Whole-body magnetic resonance imaging for detection of skeletal metastases in children and young people with primary solid tumors - systematic review.

BACKGROUND: Many solid neoplasms have a propensity for osteomedullary metastases of which detection is important for staging and subsequent treatment. Whole-body magnetic resonance imaging (WB-MRI) has been shown to accurately detect osteomedullary metastases in adults, but these findings cannot be unconditionally extrapolated to staging of children with malignant solid tumors. OBJECTIVE: To conduct a literature review on the sensitivity of WB-MRI for detecting skeletal metastases in children with solid tumors. MATERIALS AND METHODS: Searches in MEDLINE and EMBASE databases up to 15 May 2017 were performed to identify studies on the diagnostic value of WB-MRI. Inclusion criteria were children and adolescents (age <21 years) with a primary solid tumor who were evaluated for skeletal metastases by WB-MRI and compared to any type of reference standard. The number of included patients had to be at least five and data on true positives, true negatives, false-positives and false-negatives had to be extractable. RESULTS: Five studies including 132 patients (96 patients with solid tumors) were eligible. Patient groups and used reference tests were heterogeneous, producing unclear or high risk of bias. Sensitivity of WB-MRI ranged between 82% and 100%. The positive predictive value of WB-MRI was variable among the studies and influenced by the used reference standard. CONCLUSION: Although WB-MRI may seem a promising radiation-free technique for the detection of skeletal metastases in children with solid tumors, published studies are small and too heterogeneous to provide conclusive evidence that WB-MRI can be an alternative to currently used imaging techniques.

Community-acquired pneumonia and positive urinary antigen tests: Factors associated with targeted antibiotic therapy.

BACKGROUND: The use of rapid microbiological tests is supported by antimicrobial stewardship policies. Targeted antibiotic therapy (TAT) for community-acquired pneumonia (CAP) with positive urinary antigen test (UAT) has been associated with a favorable impact on outcome. We aimed to determine the factors associated with TAT prescription. PATIENTS AND METHODS: We conducted a retrospective multicenter study including all patients presenting with CAP and positive UAT for Streptococcus pneumoniae or Legionella pneumophila from January 2010 to December 2013. Patients presenting with aspiration pneumonia, coinfection, and neutropenia were excluded. CAP severity was assessed using the Pneumonia Severity Index (PSI). TAT was defined as the administration of amoxicillin for pneumococcal infection and either macrolides or fluoroquinolones (inactive against S. pneumoniae) for Legionella infection. RESULTS: A total of 861 patients were included, including 687 pneumococcal infections and 174 legionellosis from eight facilities and 37 medical departments. TAT was prescribed to 273 patients (32%). Four factors were found independently associated with a lower rate of TAT: a PSI score≥4 (OR 0.37), Hospital A (OR 0.41), hospitalization in the intensive care unit (OR 0.44), and cardiac comorbidities (OR 0.60). Four other factors were associated with a high rate of TAT: positive blood culture for S. pneumoniae (OR 2.32), Hospitals B (OR 2.34), E (OR 2.68), and H (OR 9.32). CONCLUSION: TAT in CAP with positive UAT was related to the hospitals as well as to patient characteristics.

Gastric adenocarcinoma in a 13-year-old boy: a diagnosis not often seen in this age group.

Gastric adenocarcinoma is not uncommon in the adult population, but in the pediatric population it is an extremely rare entity. A 13-year-old boy was referred to a pediatric oncology unit for evaluation of a tumor in the upper abdomen. Further investigation revealed an advanced stage gastric carcinoma with metastases suggestive for a hereditary cause. Awareness for uncommon diagnoses is a key issue in regard of accurate treatment and overall prognosis.

[Pyomyositis of the M. quadriceps femoris in an 8-month-old boy following pneumococcal meningitis]. / Een 8 maanden oude jongen met een pyomyositis van de m. quadriceps femoris volgend op een pneumokokkenmeningitis.

An 8-month-old boy developed pyomyositis of the M. quadriceps femoris following meningitis caused by Streptococcus pneumoniae. The child had no underlying immune defect. Conservative treatment with antibiotics led to complete clinical and radiological resolution of the infection. In Europe, pyomyositis is rarely seen in children. Pyomyositis should be considered in the differential diagnosis in children with fever and complaints of joint pain or myalgia. Ultrasound and MRI are helpful techniques for establishing the diagnosis and for differentiating pyomyositis from other pathological conditions. Treatment consists of adequate antibiotics and in some cases drainage.

[Bilious vomiting due to malrotation, also in older children]. / Gallig braken door malrotatie, ook bij oudere kinderen.

Three children presented with bilious vomiting due to malrotation at the age of 3, 5 and 12 years, respectively. They were treated surgically and recovered fully. In the period 1989-2002, 12 patients were operated for malrotation after the first year of life in the Emma Children's Hospital AMC, Amsterdam, the Netherlands. The mean age at operation was 5 years (range: 1-15). The most important symptoms were (bilious) vomiting and abdominal pain. Four patients had a history of bilious vomiting in the neonatal period. Nine patients had been previously admitted to a hospital with abdominal complaints. The most sensitive imaging technique was an upper gastrointestinal contrast study. Malrotation with intermittent volvulus can cause recurrent abdominal complaints and vomiting in children. Bilious vomiting is pathologic at any age and should lead to further investigations, preferably an upper gastrointestinal contrast study to exclude malrotation or other obstructions.

[Diagnostic image (132). A 3-year old boy with growth retardation]. / Diagnose in beeld (132). Een 3-jarige jongen met groeiachterstand. Osteopetrose.

A 3-year-old boy was referred for treatment of chronic myelomonocytic leukemia. Radiological examination revealed a generalized increase in bone density, harlequin face, Erlenmeyer flask deformity of the metaphysial regions and pathological fractures, findings which are characteristic of osteopetrosis.

Effect of exercise on portal vein blood flow in man.

PURPOSE: Doppler pulsed flow and electronic scanning allow for instantaneous measurement of portal vein flow. This method was used to monitor alterations in splanchnic blood flow during exercise. METHODS: Measurements of portal vein blood flow were performed in eight healthy males at rest and at 10-min intervals during cycle ergometry at 70% of maximal aerobic capacity. Subjects stopped cycling briefly (approximately 30 s) and stopped breathing while measurements were made. Flow was calculated from values obtained for velocity of red blood cell passage and cross-sectional area of the vessel. RESULTS: Cross-sectional area decreased during exercise, resulting in a significant decrease in flow over time (P = 0.0001 by ANOVA). The flow within the portal vein had decreased by 80% after 60 min of exercise (absolute flow, 0.63 +/- 0.13 L x min(-1) at rest and 0.13 +/- 0.04 L x min(-1) at 60 min). CONCLUSION: Electronic Doppler flow measurement of portal vein flow is a viable, noninvasive technique that can be used to measure splanchnic blood flow. Values obtained as a result of intensive exercise are in line with earlier results obtained with other techniques.

Coexistence of an adrenocortical carcinoma with an abdominal ganglioneuroma in a child.

We report a 3-year, 5-month-old boy with an adrenocortical carcinoma. These tumours are rare and highly malignant in childhood. In most cases they are functional, secreting adrenocortical hormones. In this case there was a misleading coexistence with a second abdominal neoplasm, which was a ganglioneuroma; this is a rare benign tumour arising from the sympathetic nervous system. The imaging investigations and their findings are discussed and correlated with pathology.

Syntenin, a PDZ protein that binds syndecan cytoplasmic domains.

The syndecans are transmembrane proteoglycans that place structurally heterogeneous heparan sulfate chains at the cell surface and a highly conserved polypeptide in the cytoplasm. Their versatile heparan sulfate moieties support various processes of molecular recognition, signaling, and trafficking. Here we report the identification of a protein that binds to the cytoplasmic domains of the syndecans in yeast two-hybrid screens, surface plasmon resonance experiments, and ligand-overlay assays. This protein, syntenin, contains a tandem repeat of PDZ domains that reacts with the FYA C-terminal amino acid sequence of the syndecans. Recombinant enhanced green fluorescent protein (eGFP)-syntenin fusion proteins decorate the plasmamembrane and intracellular vesicles, where they colocalize and cosegregate with syndecans. Cells that overexpress eGFP-syntenin show numerous cell surface extensions, suggesting effects of syntenin on cytoskeleton-membrane organization. We propose that syntenin may function as an adaptor that couples syndecans to cytoskeletal proteins or cytosolic downstream signal-effectors.

Pulmonary and mediastinal lesions in children with Langerhans cell histiocytosis.

Langerhans cell histiocytosis (LCH) is an uncommon group of disorders affecting mainly children and young adults. In children, pulmonary involvement occurs mostly in the disseminated forms; isolated pulmonary lesions are unusual. A retrospective study was undertaken on a group of 42 children diagnosed with LCH over a 19-year period. Eight children (19 %) had radiological evidence of pulmonary involvement. The lung lesions were either present at the time of diagnosis or, when appearing during the course of the disease, always coinciding with exacerbation or recurrence of the disease in other sites. Lung involvement did not appear to be an unfavourable prognostic factor. However, the toxic effects of treatment on the lungs might lead to important pulmonary sequelae.

Fibrotic eye muscles, Axenfeld anomaly, flat face, and mild developmental retardation: a new example of the Chitty syndrome.

We have studied a girl with fibrotic extrinsic eye muscles, Axenfeld anomaly, unusual facial appearance, mild hydrocephaly, and neurodevelopmental delay. Her condition is similar to the one described recently in members of a single family by Chitty et al. [1991, Am J Med Genet 40:417-420]. We suggest that she represents a second example of what may be called the Chitty syndrome.

Clinical tolerance and diagnostic efficacy of iobitridol in contrast-enhanced CT in children.

PURPOSE: The purpose of this double-blind, randomized parallel trial was to evaluate and compare the clinical safety and the diagnostic efficacy of the new nonionic triiodinated contrast agent iobitridol (300 mg I/ml) with those of iohexol (300 mg I/ml) in CT examinations in children. MATERIAL AND METHODS: Eighty children of either sex were included in the study. Of those, 40 patients received iobitridol, 40 iohexol. Mean volume injected i.v. was 1.8 ml/kg b.w. Adverse reactions occurring during 24 hours after the injection of the contrast agent were recorded. RESULTS: Image quality was judged good or excellent in all study examinations and all were diagnostically informative. There was no significant difference in clinical safety between the 2 groups and only adverse reactions of mild or moderate intensity were reported in both groups. CONCLUSION: Iobitridol appears to be a safe, well tolerated and effective contrast agent, when used for brain and body CT in children.

Idiopathic infantile arterial calcification: a surviving patient with renal artery stenosis.

Idiopathic infantile arterial calcification (IIAC) is a rare hereditary, fatal disease. Death occurs usually within the first 28 months of life. IIAC is characterized by calcifications along the internal elastic membrane and proliferation of the intimal layer of muscular arteries. Specific therapy consists of administration of diphosphonates, but its effectiveness has been a matter of controversy. We report a case treated with diphosphonates which has had an unusual outcome.

Magnetic resonance imaging in tracheal compression by the innominate artery.

Tracheal compression by the innominate artery is frequently suspected in infants with noisy breathing and no obvious aortic arch anomalies or pulmonary artery sling. However the diagnosis is difficult to confirm. The findings after using magnetic resonance imaging in two infants with tracheal compression syndrome are presented.

[Technetium 99m labeled dimercaptosuccinic acid (99m Tc-DMSA) scintigraphy in the diagnosis and follow-up of urinary infections in children]. / Apport de la scintigraphie à l'acide dimercaptosuccinique marqué au technétium 99m (99mTc-DMSA) dans le diagnostic et le suivi des infections urinaires de l'enfant.

BACKGROUND: The site of a urinary tract infection (UTI) is located by clinical findings plus imaging techniques. Renal imaging with technetium 99mTc-dimercaptosuccinic acid (99mTc-DMSA) has shown promise but its efficacy for detecting acute pyelonephritis is still debated as is its ability to differentiate between acute, potentially curable disease and scarring, definitive changes. PATIENTS AND METHODS: The files of all the 166 patients that underwent one or two (32 patients) 99mTc-DMSA imaging sessions for UTI in 1989 and 1990 in our department were analysed. The results of this technique were compared with the clinical and laboratory data and with those of other imaging techniques (ultrasonography and retrograde voiding cystourethrogram). The patients (92 girls and 74 boys, mean age: 5 years, range 15 days-17 years) were assigned to one of 3 categories: 1) acute pyelonephritis (27 patients), 2) low UTI (60 patients) and 3) uncertain UTI (79 patients), on the basis of the clinical and laboratory data. RESULTS: The first 99mTc-DMSA imaging was normal in 110 children; changes were unilateral in 45 and bilateral in 11 children. Images suggesting either acute or chronic changes were observed respectively in 19 and 48 children. 4 of the 7 acute changes were normalized on the second radioisotope scanning while 2 became worse; 19 of the 23 chronic changes were unchanged at the second scanning. Radioisotope scanning changes were seen in 81% of the patients classified as acute pyelonephritis and in only 27% of those with lower UTI. Reflux was detected in 56% of abnormal kidneys and in 36% of normal kidneys. The results of radioisotope scanning and ultrasound scan findings were not correlated in 24 patients (19 only abnormal radioisotope imaging, 5 only abnormal ultrasound scan). CONCLUSIONS: DMSA scans are useful for investigating and following UTI. They help to distinguish acute pyelonephritis and lower UTI. It is a reliable method of detecting structural abnormalities and identifying children at risk of progressive renal damage.

Systems and symptoms: family cohesion/adaptability and childhood behavior problems.

The relation between family systems and child symptomatology was examined among children aged 6 to 11 and adolescents aged 12 to 16 who had been referred for clinical services. On the basis of an assessment of family cohesion and adaptability, the families were divided into three groups: extreme, midrange, and balanced. Problem behaviors were assessed with a symptoms checklist, and the child's self-esteem was also measured. Families in the balanced range had children with fewer symptoms than did midrange or extreme families; this relation was less strong among families with adolescents. Self-esteem and symptoms were negatively correlated in these samples. Self-esteem did not moderate the relation between systems and symptoms but was independently related to family functioning. These results suggest the operation of bilateral processes within the family that link cohesion and adaptability, on the one hand, with low self-esteem and indications of psychopathology, on the other.

A group treatment program for adolescent sex offenders: five steps toward resolution.

This article describes a five-step group therapy program for adolescent sex offenders. The key elements of this approach are peer interaction and a system of incentives to move on in therapy, both of which are believed to be crucial in treating adolescents most effectively. While progressing through the steps, the participants acknowledge their adjudication, detail their offenses and personal sexual development, enhance their insights into their behavior and sexuality at large and plan how to avoid offending again. This paper describes the program of a rural outpatient guidance clinic in which a male-female therapist team met with several groups of boys (21 boys). The group program is part of a total treatment approach which also includes assessment, individual and family therapy.