Clinical recommendations for the inpatient management of lower respiratory tract infections in children and adolescents with severe neurological impairment in Germany.

Children and adolescents with severe neurological impairment (SNI) require specialized care due to their complex medical needs. In particular, these patients are often affected by severe and recurrent lower respiratory tract infections (LRTIs). These infections, including viral and bacterial etiology, pose a significant risk to these patients, often resulting in respiratory insufficiency and long-term impairments. Using expert consensus, we developed clinical recommendations on the management of LRTIs in children and adolescents with SNI. These recommendations emphasize comprehensive multidisciplinary care and antibiotic stewardship. Initial treatment should involve symptomatic care, including hydration, antipyretics, oxygen therapy, and respiratory support. In bacterial LRTIs, antibiotic therapy is initiated based on the severity of the infection, with aminopenicillin plus a beta-lactamase inhibitor recommended for community-acquired LRTIs and piperacillin-tazobactam for patients with chronic lung disease or tracheostomy. Ongoing management includes regular evaluations, adjustments to antibiotic therapy based on pathogen identification, and optimization of supportive care. Implementation of these recommendations aims to improve the diagnosis and treatment of LRTIs in children and adolescents with SNI. What is Known: • Children and adolescents with severe neurological impairment are particularly affected by severe and recurrent lower respiratory tract infections (LRTIs). • The indication and choice of antibiotic therapy for bacterial LRTI is often difficult because there are no evidence-based treatment recommendations for this heterogeneous but vulnerable patient population; the frequent overuse of broad-spectrum or reserve antibiotics in this patient population increases selection pressure for multidrug-resistant pathogens. What is New: • The proposed recommendations provide a crucial framework for focused diagnostics and treatment of LRTIs in children and adolescents with severe neurological impairment. • Along with recommendations for comprehensive and multidisciplinary therapy and antibiotic stewardship, ethical and palliative care aspects are taken into account.

Multidrug-resistant organisms in paediatric palliative care patients - Prevalence, risk factors and the impact of a liberal hygiene concept.

AIM: Multidrug-resistant organisms (MDRO) deserve special attention in health-care facilities for children with life-limiting conditions because these children have an increased risk for colonisation. To avoid nosocomial transmissions to other inpatients, single-room isolation is usually recommended. In the context of paediatric palliative care (PPC), such isolation counters the aim of participation in social activities for the patients. This study aimed to determine the prevalence of MDRO, the predictive value of risk factors and the incidence of nosocomial infections and nosocomial colonisations on a PPC inpatient unit applying a special hygiene concept that enables participation in social activities through risk-adaption and barrier nursing. METHODS: Two-year surveillance with MDRO screening of all intakes (N = 386) of a PPC unit on the day of admission and discharge. To determine the predictive value of pre-defined risk factors, logistic regression analyses were calculated. Receiver operating characteristic analyses were performed to determine the predictive power of the number of risk factors on the presence of MDRO. RESULTS: The rate of MDRO colonisation at admission was 12.7%; previous positive MDRO screening was the only significant individual risk factor. Over the 2-year period, no MDRO-related nosocomial infections occurred; nosocomial colonisation incidence density was 0.6. CONCLUSION: Results demonstrate that patients with at least one risk factor have to be cared for by barrier nursing until MDRO screening results are negative. Following these guidelines prevents nosocomial MDRO transmission.

Addressing multidrug resistant pathogens in pediatric palliative care patients-the nurses point of view: A qualitative study.

BACKGROUND: Multidrug resistant pathogens are a large-scale healthcare issue. In particular, children with life-limiting conditions have a significantly increased risk of multidrug resistant pathogen colonization. Official hygiene requirements recommend children, who are colonized with multidrug resistant pathogens, to be isolated. In the context of pediatric palliative care, such isolation adversely affects the aim of social participation. To overcome this challenge of conflicting interests on a pediatric palliative care inpatient unit, a hygiene concept for patients colonized with multidrug resistant pathogens, called PALLINI, was implemented. AIM: The aim of this study was to identify the nurses' attitudes and opinions toward PALLINI. METHODS: Nurses (N = 14) from the pediatric palliative care unit were queried in guideline-oriented interviews. Interviews were analyzed qualitatively by means of content analysis. RESULTS: The following four categories were identified: (1) safety, (2) effort, (3) quality of care, and (4) participation. All categories demonstrated ambivalence by nursing staff regarding PALLINI. Ambivalence arose from guaranteeing infection control versus noncompliance by the families, additional workload for patients with multidrug resistant pathogens versus lack of resources, impaired relationship with the parents versus enabling better care for the child, as well as enabling some limited contact versus the larger goal of genuine social participation. Despite this, nurses reported the importance of arranging everyday-life for the patients so that they experience as much social participation as possible. CONCLUSION: The implementation of a new hygiene concept is challenging. Despite positive reception of PALLINI from the nurses, ambivalence remained. Addressing these ambivalences may be critical to best implement the new hygiene concept.

[Paediatric palliative care (PPC) - a particular challenge : Tasks, aims and specifics]. / Pädiatrische Palliativversorgung ­ herausfordernd anders : Aufgaben, Ziele und Besonderheiten.

In Germany, there are about 50,000 children, adolescents and young adults with life threatening or life limiting conditions, who will likely die due to their illness before reaching the age of 40. In recent years prevalence has increased significantly.The aim of paediatric palliative care (PPC) is to optimize quality of life for the child, adolescent or young adult and its whole family. Whenever possible the patient should be treated at home.The very complex and rare diseases as well as the patients' wide range of age and developmental stage are particularly challenging within PPC. Many PPC patients have cognitive disabilities and are not able to communicate verbally. In contrast to adult palliative care, PPC is often delivered for many years.To date, families with a child suffering from a life limiting condition have access to several care systems; however most of them are still financed by donations. One of PPC's most important tasks is the implementation of individual case management to find the right mix of care provision, its flexible adaption on changing needs and the appropriate intensity of care.Specific education and training courses in the field of PPC are still rare. There is a single chair for childrens' pain therapy and paediatric palliative care at Witten/Herdecke University in Germany. In addition, quality-checked multiprofessional PPC courses for the additional "palliative health care professional" designation based on the Dattelner Curriculum are offered at several institutions.

Relevance of Potential Contributing Factors for the Development and Maintenance of Irritability of Unknown Origin in Pediatric Palliative Care.

Potential contributing factors (PCFs) for irritability of an unknown origin (IUO) in children with neurological conditions are identifiable through structured diagnostics. Uncertainty exists regarding the actual relevance of identified PCFs to IUO. Assessments from parents as well as nursing, psycho-social, and medical professionals were used to determine the contribution of different PCFs in the development and maintenance of IUO. For this, individual PCFs of N = 22 inpatient children with IUO were presented to four raters. Descriptive statistics, Kruskal-Wallis tests, and Krippendorff's alpha were used to determine which PCFs were most relevant to explain IUO and rater agreement. Psycho-social aspects (44.7%), hyperarousal (47.2%), pain (24.6%), and dystonia (18.1%) were identified as the most relevant PCFs for IUO. Descriptively, physicians' relevance rating regarding psycho-social aspects, hyperarousal, and dystonia deviated the most from the overall group rating. All professional raters considered psycho-social aspects to be more relevant than did parents. Parents rated pain as more relevant than the other raters. Kruskal-Wallis tests showed no significant differences between relevance ratings (H = 7.42, p = 0.059) or the four parties' deviations (H = 3.32, p = 0.344). A direct comparison of the six two-party constellations showed that across all factors, agreement was weak to moderate. The highest agreement was between physicians and nurses (α = 0.70), and the lowest was between nurses and psycho-social experts (α = 0.61). Understanding which psycho-social and various biological PCFs are significant for IUO can facilitate more targeted and individualized pediatric palliative care for affected patients.

Characteristics of highly impaired children with severe chronic pain: a 5-year retrospective study on 2249 pediatric pain patients.

BACKGROUND: Prevalence of pain as a recurrent symptom in children is known to be high, but little is known about children with high impairment from chronic pain seeking specialized treatment. The purpose of this study was the precise description of children with high impairment from chronic pain referred to the German Paediatric Pain Centre over a 5-year period. METHODS: Demographic variables, pain characteristics and psychometric measures were assessed at the first evaluation. Subgroup analysis for sex, age and pain location was conducted and multivariate logistic regression applied to identify parameters associated with extremely high impairment. RESULTS: The retrospective study consisted of 2249 children assessed at the first evaluation. Tension type headache (48%), migraine (43%) and functional abdominal pain (11%) were the most common diagnoses with a high rate of co-occurrence; 18% had some form of musculoskeletal pain disease. Irrespective of pain location, chronic pain disorder with somatic and psychological factors was diagnosed frequently (43%). 55% of the children suffered from more than one distinct pain diagnosis. Clinically significant depression and general anxiety scores were expressed by 24% and 19% of the patients, respectively. Girls over the age of 13 were more likely to seek tertiary treatment compared to boys. Nearly half of children suffered from daily or constant pain with a mean pain value of 6/10. Extremely high pain-related impairment, operationalized as a comprehensive measure of pain duration, frequency, intensity, pain-related school absence and disability, was associated with older age, multiple locations of pain, increased depression and prior hospital stays. 43% of the children taking analgesics had no indication for pharmacological treatment. CONCLUSION: Children with chronic pain are a diagnostic and therapeutic challenge as they often have two or more different pain diagnoses, are prone to misuse of analgesics and are severely impaired. They are at increased risk for developmental stagnation. Adequate treatment and referral are essential to interrupt progression of the chronic pain process into adulthood.

Potential Contributing Factors for Irritability of Unknown Origin in Pediatric Palliative Care.

CONTEXT: In pediatric palliative care, irritability of unknown origin (IUO) in children with severe neurological impairment is a peculiarly complex and challenging symptom, yet its etiology remains poorly understood. OBJECTIVES: Presenting a structured IUO diagnostic and therapeutic approach developed in a specialized inpatient pediatric palliative care facility for identifying IUO's potential contributing factors (PCFs). METHODS: Prospective observational study with N = 22 children showing IUO at admission to the inpatient pediatric palliative care facility. Analysis of patient records and participation in ward routine to identify and treat PCFs. Treatment outcome was assessed using a standardized protocol for tracking IUO at 24-hour intervals. RESULTS: Altogether, 136 PCFs were identified (average 6.18 per child) with pain and psycho-social problems being the most common. Two hundred and twenty-two diagnostic measures were initiated of which 159 (71.6%) helped identify a PCF. The majority of PCFs were identified by basic diagnostics (93.7%). Inpatient length of stay and duration of IUO phases correlated significantly. Between baseline (days 3-5; timing at which inpatients experientially show regular behavior) and discharge, patients showed a significant reduction in average 24-hours IUO duration by 1 hour and 26 minutes (Wilcoxon test: Z = -3.29, P < 0.01). CONCLUSION: Results lead to a more thorough understanding of PCFs to IUO. Following a systematic approach such as the one presented, PCFs can be detected even by simple diagnostics. In addition to biological aspects, diagnostics and therapy should address psycho-social aspects of IUO.

Lower Respiratory Tract Infections in Pediatric Patients with Severe Neurological Impairments: Clinical Observations and Perspectives in a Palliative Care Unit.

Pediatric palliative care (PPC) patients with a severe neurologic impairment (SNI) suffer considerable morbidity and increased mortality from lower respiratory tract infections (LRTIs). The indication and choice of antibiotic therapy for bacterial LRTIs are often challenging given the lack of evidence-based treatment recommendations for this vulnerable patient population. We conducted an observational study before the SARS-CoV-2 pandemic in an eight-bed pediatric palliative care inpatient unit. During two years of surveillance, we diagnosed and treated 33 cases of a bacterial LRTI in patients with an SNI; 5 patients were hospitalized with an LRTI more than once. Two patients died from complications due to LRTIs during hospitalization. Three patients (15%) were colonized with multidrug-resistant organisms. An initial antibiotic treatment failed in one-third of the cases; a successful therapy of the LRTI was achieved with broad-spectrum and extended-spectrum penicillins (n = 13; in combination with ß-lactamase inhibitors for n = 5 cases), cephalosporins (n = 13: n = 4 second-generation and n = 9 third-generation cephalosporins; in combination with other substances for n = 5 cases), ciprofloxacin (n = 3), and meropenem plus vancomycin (n = 2) or meropenem (n = 1). A respiratory specimen was obtained in 66.7% of cases with P. aeruginosa, E. coli, and K. pneumoniae accounting for the majority of the detected species. In most cases, there was no definite confirmation that the LRTI was caused by the species detected. The diagnostics and treatment of bacterial LRTIs in PPC patients with an SNI are challenging. The lack of controlled studies and the heterogeneity of this population often necessitate an individual approach. This lack of controlled studies may partly be compensated by a set of diagnostic and antibiotic stewardship criteria.

Differential Diagnosis of Cyclic Vomiting and Periodic Headaches in a Child with Ventriculoperitoneal Shunt: Case Report of Chronic Shunt Overdrainage.

Fourteen months after the implantation of a ventriculoperitoneal shunt catheter, a six-year-old boy developed recurrent, severe headaches and vomiting every three weeks. The attacks were of such severity that hospitalizations for analgesic and antiemetic therapies and intravenous rehydration and electrolyte substitution were repeatedly required. The patient was asymptomatic between the attacks. After an extensive diagnostic workup-including repeated magnetic resonance imaging (MRI) and neurosurgical examinations-common differential diagnoses, including shunt overdrainage, were ruled out. The patient was transferred to a specialized pediatric pain clinic with suspected cyclic vomiting syndrome (CVS). Despite intensive and in part experimental prophylactic and abortive pharmacological treatment, there was no improvement in his symptoms. Consecutive MRI studies reinvestigating the initially excluded shunt overdrainage indicated an overdrainage syndrome. Subsequently, the symptoms disappeared after disconnecting the shunt catheter. This case report shows that even if a patient meets CVS case definitions, other differential diagnoses must be carefully reconsidered to avoid fixation error.

Multidrug-Resistant Organisms in Palliative Care: A Systematic Review.

Background: Multidrug-resistant organisms (MDROs) are an important health care issue. Patients in Western societies often present an increased morbidity of chronic conditions accompanied by poor immune status and the use of devices. In particular, patients in palliative care (PC) are at greater risk of MDRO colonization, due to accompanying special devices and being hospitalized. Objective: To gain an overview of the literature regarding MDROs in PC. Design: Systematic review Data sources: On the 19th of October 2019 the databases " PubMed" and " CINAHL" were used to identify studies reporting on MDROs in PC; the search was updated on 16th of May 2020. Results: Seventeen out of 486 articles were included. Six represent qualitative data, 10 quantitative data, and one a mixed methods approach. Prevalence data range from 4.0% to 18%. MDRO colonization has a negative impact on patients and families. It leads to uncertainties and higher workload by staff members. Strategies for the management of MDROs in the field of PC are predominantly available for methicillin-resistant Staphylococcus aureus. Not even half of institutions utilize existing protocols. Recommendations for dealing with MDROs indicate required staff and time resources as well as information, communication, and specific knowledge. Conclusion: There is a great need for studies examining the prevalence of all MDROs in the PC setting. Additionally, not only patients but also a public enlightenment on MDROs should be provided to decrease knowledge gaps and therefore reduce transmission on MDROs.

The effect of multidrug-resistant organisms on social participation and quality of life in children with life-limiting conditions.

BACKGROUND: Children with life-limiting conditions have a high risk of colonisation with a multidrug-resistant organism (MDRO). To avoid the spread of hospital-aquired infections to other patients, children with a MDRO are moved to an isolated room or ward. However, such isolation prevents social participation, which may reduce the child's quality of life (QoL). To overcome this challenge of conflicting interests on a paediatric palliative care inpatient unit, a hygiene concept for patients colonised with MDRO, called PALLINI, was implemented. PALLINI advises that, instead of isolating the affected children, strict barrier nursing should be used. AIM: To identify the impact of a complex hygiene concept on children's and parents' QoL and social participation. METHODS: Cross-sectional mixed-methods research approach, comprising semi-structured interviews with parents and staff members, and a QoL-questionnaire focusing on the child which was completed by parents. FINDINGS: In paediatric patients with life-limiting conditions who have MDRO colonisation, using a complex hygiene protocol resulted in both benefits and barriers to social participation. However, the child's QoL did not appear to be affected. CONCLUSION: All staff members and families have to be familiar with the hygiene concept and the concept has to be self-explanatory and easy to apply.

Opioid-Induced Respiratory Depression in Pediatric Palliative Care Patients with Severe Neurological Impairment-A Scoping Literature Review and Case Reports.

Pediatric Palliative Care (PPC) addresses children, adolescents, and young adults with a broad spectrum of underlying diseases. A substantial proportion of these patients have irreversible conditions accompanied by Severe Neurological Impairment (SNI). For the treatment of pain and dyspnea, strong opioids are widely used in PPC. Nonetheless, there is considerable uncertainty regarding the opioid-related side effects in pediatric patients with SNI, particularly concerning Opioid-Induced Respiratory Depression (OIRD). Research on pain and OIRD in pediatric patients with SNI is limited. Using scoping review methodology, we performed a systematic literature search for OIRD in pediatric patients with SNI. Out of n = 521 identified articles, n = 6 studies were included in the review. Most studies examined the effects of short-term intravenous opioid therapy. The incidence of OIRD varied between 0.13% and 4.6%; besides SNI, comorbidities, and polypharmacy were the most relevant risk factors. Additionally, three clinical cases of OIRD in PPC patients receiving oral or transdermal opioids are presented and discussed. The case reports indicate that the risk factors identified in the scoping review also apply to adolescents and young adults with SNI receiving low-dose oral or transdermal opioid therapy. However, the risk of OIRD should never be a barrier to adequate symptom relief. We recommend careful consideration and systematic observation of opioid therapy in this population of patients.

Multidrug-resistant bacteria in a paediatric palliative care inpatient unit: results of a one year surveillance.

Aim: Nosocomial infections (NIs) and multidrug resistant (MDR) pathogens are an important paediatric healthcare issue. In vulnerable patients such as children with life-limiting conditions, MDR infections can be life-threatening. Additionally, these children have a significantly increased risk for colonisation with MDR pathogens. Therefore, it is vital to prevent new colonisations with MDR pathogens in this vulnerable patient group. However, little is known about colonisation with MDR pathogens and NIs in inpatient units for paediatric palliative care (PPC). The aim of this study was to investigate the prevalence of colonisation with MDR pathogens and the incidence of NIs in a PPC unit. Methods: Evaluation of surveillance data of a PPC unit. All patients admitted to a PPC unit from 1st April 2012 to 31st March 2013 were screened for MDR pathogens upon admission. Patients who exhibited clinical signs of an infection during their inpatient stay were screened again. Results: During the study period, 198 cases were admitted to the unit. Those cases represent 118 patients. 18% of the patients were colonised with MDR pathogens. The most common MDR pathogens were E. coli (8.1%) and Pseudomonas ssp. (8.1%). In addition, 58% of patients with tracheostomy had MDR pathogens in their tracheal secretions. The incidence density of NIs was 0.99 per 1000 inpatient treatment days with no NI caused by MDR pathogens. Conclusion: Due to a high prevalence, it is reasonable to screen PPC patients for MDR pathogen colonisation before or during admission. Special attention must be given to patients with tracheostomy. Our results provide preliminary evidence that participation in social activities in a PPC unit for patients colonised with MDR pathogens is safe if hygiene concepts are applied.

Paediatric cancer pain management using the WHO analgesic ladder--results of a prospective analysis from 2265 treatment days during a quality improvement study.

OBJECTIVE: To collect data on pain management in paediatric oncology with respect to the WHO ladder approach. SETTING, DESIGN, PATIENTS AND METHODS: Eight German tertiary care paediatric oncology centres prospectively documented all their in-patient pain treatment courses from June 1999 to December 2000. Pain was scored using a 1-6 faces scale. RESULTS: Two hundred and twenty four patients (median age, 9 years; range 0.2-32.1) were enrolled. Three hundred and thirty three pain episodes comprising a total of 2265 treatment days were documented. Pain was mostly therapy associated. The most frequently administered non-opioid analgesics were dipyrone and paracetamol. On WHO step 2, tramadol was almost the only opioid used. During tramadol monotherapy average daily pain scores were lower than with a combination of tramadol and non-opioid analgesics. On WHO step 3, morphine was at least part of the analgesic regimen on most treatment days. Strong opioids were combined with a non-opioid analgesic on 41% of the treatment days. The mean intravenous morphine equivalence dose was 0.034 mg/kg/h. During opioid and non-opioid combination therapy, adverse effects were more frequent, and average pain scored higher than on opioid monotherapy. CONCLUSIONS: WHO-guidelines were closely followed in Germany and seem to provide effective analgesia for children with cancer pain. In our patient group there is no evidence that a combination of an opioid with a non-opioid is more effective than opioid therapy alone in in-patient paediatric oncology pain treatment.

Restricted use of glycopeptides in paediatric cancer patients with fever and neutropenia.

Until now, studies confirming the safety of glycopeptide restriction in the empirical treatment of prolonged fever and neutropenia included only nine children. In an open-label observational study, the use of teicoplanin in paediatric oncology patients was investigated. A period of unrestricted use (2001-2003) was compared with a second period (2004) following implementation of a restrictive treatment guideline. Empirical first-line treatment consisted of piperacillin/tazobactam; in 2004, fosfomycin was added after 72 h as the second-line combination instead of teicoplanin. In total, 213 episodes (n=163 in 2001-2003; n=50 in 2004) managed with teicoplanin or fosfomycin (only 2004) were eligible. Empirical treatment of fever of unknown origin with teicoplanin was reduced by 97%. In 2004, the mean length of stay was 0.4 days shorter, no infection-related death occurred and no vancomycin-resistant enterococci were detected. Restriction of empirical glycopeptides is safe in paediatric cancer patients after first-line treatment with piperacillin/tazobactam. Fosfomycin appears to offer a feasible and cost-saving alternative in second-line combination therapy.

Pain in pediatric oncology--children's and parents' perspectives.

There is a lack of valid epidemiological data on malignancy-associated pain in modern pediatric oncology. Pediatric oncology patients (self-assessment) and their parents from 28 hospitals were questioned using age-adapted, structured interviews and validated pain assessment tools. Pain intensity was measured by the NRS and Bieri faces scale. We conducted 363 interviews with patients and their parents, and 46 with the parents alone (if patients <2.5 years). Pain was reported at the time of the interview or within the last 24 h, 7 d, or 4 weeks in 15%, 28%, 50% and 58% of cases, respectively. The proportion of patients suffering severe to maximal pain (NRS>3; Bieri>2) increased significantly (p=0.001, chi2 test). The median pain intensity for the most severe pain episode within the last 4 weeks was 6.7 (NRS 0-10). Adverse effects of anti-tumor therapy were the most frequent cause of pain. Multivariate analyses depicted general physical condition either "severely reduced" (ASA status 3) (OR 4.0, 95% CI 1.1-14.7, p=0.037) or "moderately reduced" (ASA status 2) (OR 1.8, 95% CI 1.1-2.9, p=0.018), "in-patient status" (OR 1.8, 95% CI 1.2-2.9, p=0.010), and "co-morbidity present" (OR 3.5, 95% CI 1.1-10.7, p=0.030) as risk factors for severe to maximal pain. General anesthesia was the only factor significantly (OR 0.14, 95% CI 0.05-0.39, p<0.01) associated with a reduction in the proportion of patients suffering severe to maximal pain during bone marrow aspiration. Our data emphasize both the importance of in-house acute pain control and the need for general anesthesia during painful procedures in pediatric oncology.

Identification of a novel mutation in the coding region of the grey-lethal gene OSTM1 in human malignant infantile osteopetrosis.

Autosomal recessive malignant infantile osteopetrosis (ARO) is characterized by severe osteosclerosis, pathologic fractures, hepatosplenomegaly, and pancytopenia. The pathophysiological basis is inadequate bone resorption due to osteoclast dysfunction. In the majority of cases, mutations in either of two human genes cause this fatal disorder: TCIRG1, encoding a subunit of the osteoclast H(+)-ATPase, and the voltage-gated chloride channel gene CLCN7. We excluded both genes in a small inbred family with malignant infantile osteopetrosis and undertook linkage analysis of several candidate loci that are involved in murine osteopetrosis. A region spanning more than 20 cM between the markers D6S1717 and D6S1608 on chromosome 6q21 was found to be homozygous in the affected child. This locus is syntenic to the genomic region harboring the gene for the osteopetrotic mutant mouse grey-lethal (gl). Recently, mutations in a novel gene of unknown function were described in the grey-lethal mouse and in one human patient. Mutation screening of the grey-lethal gene (OSTM1), revealed a homozygous 2-bp deletion in exon 2 (c.415_416delAG) in the affected child. No mutations could be found in six independent ARO patients who had tested negative for mutations in TCIRG1 and CLCN7. In summary, we describe the identification of a novel mutation in the coding sequence of the human grey-lethal gene, which is the second OSTM1 mutation found in human ARO, confirming the involvement of this gene in the pathogenesis of this severe bone disease.

Sex differences in pain intensity in adolescents suffering from cancer: differences in pain memories?

UNLABELLED: Although sex differences have been investigated in chronic pain populations, little is known about sex differences in the pain experience of paediatric oncology patients and also whether their parents rate the pain experience differently for boys and girls. The aim of the present study was to determine if (1) boys and girls with cancer differ in current perception and past recollection of cancer-related pain and (2) if adolescents' and parents' pain ratings differ in relation to the sex of the adolescent. One hundred twelve adolescents with malignant diagnoses (12 to 18 years) and their parents participated in the study. Girls reported higher pain intensity within the last 7 days and 4 weeks despite similar diagnosis, physical status, duration of diagnoses, and main pain causes. When asked for pain intensity that dated back in time, parent and adolescent ratings diverged, with a trend for parents to reporting higher pain intensity in boys and lower pain intensity in girls, particularly for pain in the preceding 7 days. The present study provides preliminary evidence for sex differences in the recalled pain experience of adolescents with malignant diagnoses. Although boys and girls experience present pain similarly and hence should be treated similarly, girls recall higher pain intensity than boys. Future studies should address whether negative memories in girls play a significant role and may have an impact on girls' well-being and pain-related distress. Additionally, psychosocial factors such as gender role expectations may need to be investigated. Parental variables and their impact on parents' pain ratings, especially for ratings of precedent pain, warrants further investigation. PERSPECTIVE: Girls with malignant diagnoses differ from boys in their recalled pain intensity ratings, with girls reporting higher pain intensity. Additional pain management strategies referring to the memory of pain may need to be implemented.

Stop the pain! A nation-wide quality improvement programme in paediatric oncology pain control.

INTRODUCTION: Little is known about the impact of translation of pain management clinical practice guidelines on pain control in paediatrics. In an effort to overcome this, a longitudinal, nation-wide, multi-centre paediatric quality improvement (QI) study was initiated by the German Society of Pediatric Haematology and Oncology (GPOH) entitled Schmerz-Therapie in der Onkologischen Paediatrie (STOP). OBJECTIVE: The project's primary major aims were to improve paediatric oncology pain control in Germany, and to evaluate the project's impact on the pain management quality. To achieve these aims, STOP encompassed six sequential phases to evaluate present practice, develop recommendations for practical pain control, actively engage participants in improvement strategies, and assess change. The purpose of this paper is to briefly describe STOP in its entirety, report on comparisons between active quality management (QM) departments that actively participated in the project and non-active QM departments regarding differences in pain control, patients' and parents' perspectives on pain control and health professionals' knowledge, and to discuss the impact of STOP as a whole. METHODS: Four hypotheses were examined: (1) changes in health care professionals' knowledge on pain in paediatric oncology and pain management after a three-year period (2) impact of active participation in the STOP-project; (3) differences in patients' and parents' perspective in active QM versus non-active QM departments; (4) impact of the STOP-project on the health care professionals' knowledge in active QM versus non-active QM departments. Data included surveys, interviews, and standardised pre-/post-intervention documentation of pain control. All German paediatric oncology departments were invited to participate. The prime means of intervention was education (printed material, passive participation; additional lectures and feed-back, active participation). Quality indicators were defined and compared with regards to the four hypotheses. RESULTS: Sixty-eight departments participated passively. Eight departments participated actively, enrolling 224 patients (median age, 9 years) and documenting a total of 2265 treatment days. In the areas addressed, all health professionals demonstrated increases in knowledge on pain and pain control after a three-year period. STOP objectively improved pain control in the actively participating departments. Painful modes of drug administration were used less frequently; the usage of mixed opioid agonists-antagonists was reduced; the physicians' knowledge of the treatment of neuropathic pain increased; pain ratings significantly decreased, and less episodes of strong pain were observed. There was a significant increase in the proportion of health-care professionals who post-interventionally judged that pain therapy had been initiated earlier and at exactly the right time. Neither patients nor parents felt, however, that there was any quality improvement. According to participants' self-assessment, STOP improved practical pain management in actively participating departments, while in passively participating departments the change to the better was negligible. CONCLUSION: STOP predominantly aimed at and succeeded in the improvement of structure, process and outcome quality. With regard to patients' and parents' opinions, the interview tools might have been unsuited to measure the quality of pain control, or STOP was insufficient to improve pain control to a magnitude significant to the patient.

111In-pentetreotide scintigraphy in medulloblastoma: a comparison with magnetic resonance imaging.

Medulloblastoma (MB) is a primitive neuroectodermal tumour constituting a grade IV brain malignancy. Early and correct detection of recurrence or metastasis is desirable for follow-up of patients in this entity. Frequent expression of somatostatin receptors by MB lesions facilitates functional tumour imaging by somatostatin receptor scintigraphy (SRS). To investigate the value of SRS in the follow-up of MB, the results of ten consecutive patients (seven children and three adults) undergoing additional imaging with 111In-pentetreotide were reviewed. Four, 24 and 48 h p.i. planar and whole body images as well as a SPECT study at 4 h p.i. were acquired after intravenous injection of 109 +/- 35 MBq 111In-pentetreotide (Octreoscan). SRS yielded 11 positive and ten negative imaging results, compared to 17 positive and four negative in magnetic resonance imaging (MRI). The lesion-by-lesion analysis with a total of 44 lesions revealed a sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) of 42%, 83%, 94%, 18% for SRS and 89.5%, 50%, 92%, 43% for MRI. Based on a per-patient analysis, considering the patient as to be either tumour-free or tumour-positive by one imaging modality, the following values for sensitivity, specificity, PPV and NPV were obtained: 61%, 100%, 100%, 30% for SRS and 94%, 67%, 94%, 67% for MRI. MRI remains the first step imaging technique in medulloblastoma patients before and after surgery and during the follow-up providing the highest sensitivity. However, to improve specificity and contribute to correct diagnosis in MB 111In-pentetreotide scintigraphy should be considered as a confirmatory second step imaging tool, especially in case of equivocal MRI results. Moreover, a positive SRS scan might serve as a reference before and after somatostatin receptor targeted radiotherapy.