A case of an inflammatory myofibroblastic tumor in the lung which expressed TPM3–ALK gene fusion

We herein describe a 4-year-old boy who after being treated for pneumonia showed an abnormal shadow at the hilus of the right lung on chest X-rays with continued inflammatory findings in his laboratory data. CT and MR investigations suggested the existence of a neoplasm at that site. An open biopsy was thus performed for a definite diagnosis. The histological findings and the expression of TPM3–ALK fusion gene confirmed a diagnosis of an inflammatory myofibroblastic tumor. A right upper and middle lobectomy including the tumor was thus performed for a complete resection. In addition to the histological diagnosis, the detection of the tumor specific fusion gene provided objective evidence in making a diagnosis.     

Inflammatory myofibroblastic tumor of the bladder in children: what can be expected?

Inflammatory myofibroblastic tumor of the bladder is an uncommon condition of unknown neoplastic potential. In adults the tumor is seen in association with instrumentation of the lower genitourinary tract, while in children it appears to run an idiopathic course. Its clinical and radiological presentation in children resembles sarcoma. The case of a 10-year-old girl with inflammatory myofibroblastic tumor is presented, outlining the histological and immunhistochemical features to allow differentiation between sarcomas, the most important differential diagnosis. An outcome meta-analysis of the literature identified 35 cases of inflammatory myofibroblastic tumor in the bladder of children. Conservative surgery is the strategy of choice. There is no evidence of recurrence or metastasis at a median follow up of 1.5 years.     

Long-term follow up (37–69 years) of patients with bladder exstrophy treated with ureterosigmoidostomy: Uro-nephrological outcome

ObjectiveTo describe the urological and nephrological long-term outcome of patients born with classical bladder exstrophy treated with bilateral ureterosigmoidostomies in early childhood.Patients and methodOut of 42 patients born with bladder exstrophy in Switzerland between 1937 and 1968, 25 participated in this study; seven had died, seven were lost to follow up and three refused consent. Assessment included chart review, clinical examination, and assessment of renal function and morphology.ResultsAfter a follow-up period of 37–69 years ((mean 50 years), 13 of the 25 participants (52%) had their ureterosigmoidostomy still in place. All others had different forms of urinary diversions. Fifteen (60%) patients had normal renal function or mild chronic kidney disease as assessed by estimated glomerular filtration rate. Three patients were on renal replacement therapy. MRI (n = 16) showed 10 morphologically normal kidneys. One patient suffered from adenocarcinoma of the colon, five had benign colonic polyps, one urethral papillary carcinoma and 18 no evidence of tumor.ConclusionThe majority of our patients have normal or mildly impaired renal function and a well functioning ureterosigmoidostomy. This is remarkable, given the fact that ureterosigmoidostomies are considered to be refluxing high-pressure reservoirs at risk of renal injury and malignancy.     

Small omphalocele with umbilical evagination of the bladder: A distinct entity?

A neonatal case of umbilical evagination of the bladder combined with a small omphalocele is presented. This rare congenital malformation has previously been described in only three cases. Umbilical evagination can be understood as a disturbance of development of both the vitelline and allantoic ducts, resulting in a non-descended but otherwise normal bladder opening to the inferior margin of a small omphalocele. Diagnosis is made by clinical inspection and ultrasound. Since other malformations are not present, this entity may be regarded as a minor form of a lower midline defect with excellent prognosis.     

Guilt by association - what is the true risk of malignancy in children treated with etanercept for JIA?

Recently, the Food and Drug Administration placed a "black box" label on etanercept, and other tumor necrosis factor inhibitors used to treat childhood arthritis, warning of the risk of malignancies. The Food and Drug Administration made their decision based on a review of 48 cases of malignancies identified worldwide in children treated with tumor necrosis factor inhibitors for inflammatory bowel disease, sarcoidosis, and juvenile idiopathic arthritis. Recently, an article in Pediatric Rheumatology demonstrated that there may not be an increased risk of cancer in children with juvenile idiopathic arthritis treated specifically with the tumor necrosis factor receptor fusion protein, etanercept. There are many confounding issues regarding whether or not etanercept increases the risk of malignancy, specifically lymphomas, above the background rate of cancer in children with juvenile idiopathic arthritis who are not being treated with biologic agents. Whether or not it was appropriate for the Food and Drug Administration to lump cancer patients with underlying granulomatous diseases (inflammatory bowel disease and sarcoidosis) with children with juvenile idiopathic arthritis is explored herein. Moreover, the amalgamation of etanercept with anti-tumor necrosis factor monoclonal antibodies (adalimumab and infliximab) is another point of contention. What is clear is that there is much that is currently unknown to be able to convincingly demonstrate a substantial risk of cancer in children with juvenile idiopathic arthritis treated with etanercept. Conversely, there is ample evidence demonstrating remarkable benefit of etanercept in treating juvenile idiopathic arthritis. Physicians treating childhood arthritis should weigh these potential risks and benefits with patients and their families discussing the current limitations in available data regarding the risk of cancer in children treated with etanercept for juvenile idiopathic arthritis.     

The protective arm of the renin–angiotensin system may counteract the intense inflammatory process in fetuses with posterior urethral valves

ObjectivePosterior urethral valve is the most common lower urinary tract obstruction in male children. A high percentage of patients with posterior urethral valve evolve to end-stage renal disease. Previous studies showed that cytokines, chemokines, and components of the renin–angiotensin system contribute to the renal damage in obstructive uropathies. The authors recently found that urine samples from fetuses with posterior urethral valve have increased levels of inflammatory molecules. The aim of this study was to measure renin–angiotensin system molecules and to investigate their correlation with previously detected inflammatory markers in the same urine samples of fetuses with posterior urethral valve.MethodsUrine samples from 24 fetuses with posterior urethral valve were collected and compared to those from 22 healthy male newborns at the same gestational age (controls). Renin–angiotensin system components levels were measured by enzyme-linked immunosorbent assay.ResultsFetuses with posterior urethral valve presented increased urinary levels of angiotensin (Ang) I, Ang-(1-7) and angiotensin-converting enzyme 2 in comparison with controls. ACE levels were significantly reduced and Ang II levels were similar in fetuses with posterior urethral valve in comparison with controls.ConclusionsIncreased urinary levels of angiotensin-converting enzyme 2 and of Ang-(1-7) in fetuses with posterior urethral valve could represent a regulatory response to the intense inflammatory process triggered by posterior urethral valve.     

Outcome of infants with neuroblastoma detected by mass screening and surgically treated in Shiga Prefecture,Japan: what is the role of surgery?

To investigate retrospectively the clinical and biological features that influence the outcome of infants with neuroblastoma (NB) detected by mass screening (NBMS), and to construct surgical strategies to deal with NBMS, 20 infants diagnosed as having either NB or ganglioneuroblastoma (GNB) between 1986 and 1998 were enrolled in a study. They comprised 15 boys and 5 girls ranging in age from 7 to 14 months. The following factors were analyzed by multivariate analysis: age, stage according to the Japanese staging system at the time of diagnosis, site of the primary tumor, histologic findings, preoperative urinary vanillylmandelic acid (VMA) and homovanillic acid (HVA) levels, VMA/HVA ratio, lactate dehydrogenase, neuron-specific enolase, Shimada's histologic classification, amplification of the N- myc oncogene by Southern blot analysis, nuclear content, and chromosomal abnormality. The 2-year survival was 95.0% (19/20). The site of the primary tumor was the adrenal gland in 12 cases, retroperitoneum in 6, and retrothoracic space in 2. Four infants had stage I, 6 stage II, 3 stage III, 3 stage IVB, and 4 stage IV disease. Complete resection was achieved in all cases except for 1 retroperitoneal GNB. Histologic examination showed that 8 patients had NB. Of the 12 GNBs, 8 were poorly-differentiated and 1 was well-differentiated. Only 1 of the 14 examined tumors showed amplification of N- myc (20 copies). The infant with N- myc-positive NB (stage II) died 23 months after surgery in spite of aggressive postoperative chemotherapy. Multivariate analysis revealed the plasma NSE level to be a significant predictor of survival (p < 0.0143). This suggests that N- myc amplification and plasma NSE level could be closely related to the survival of infants with NBMS. The N- myc-positive NB case implies that even in locoregional NB detected by NBMS, surgical excision should play a central role in the diagnosis of its oncogenic characteristics and indicate any subsequent therapy.     

Long-term follow-up of male patients after reconstruction of the bladder–exstrophy–epispadias complex: Psychosocial status,continence, renal and genital function

ObjectiveThere is a paucity of knowledge about long-term outcome issues in the bladder–exstrophy–epispadias complex (BEEC). Adult male BEEC patients were investigated in respect of bladder and renal function, fertility, genital function and psychosocial facts.Patients and methodsIn a cross-sectional study, 17 adult male BEEC patients (mean age 23.4 years) from a single centre were evaluated with a questionnaire, renal and bladder ultrasound, blood tests, hormonal profile and semen analysis.ResultsPhenotypically one patient had complete epispadias and 16 had classical bladder exstrophy. Five patients underwent a one-stage functional reconstruction as a primary and 12 as a redo procedure. After a mean follow-up of 19.4 years, 15 bladders were preserved with 12 voiding per urethram and 3 performing intermittent catheterization; 2 were secondarily diverted. Significant residual urine was present in 10; kidneys were normal in 14 patients. Sixteen patients proved ejaculations, 3 had normospermia, 7 oligoasthenospermia and 6 azospermia. In patients with only one single bladder neck procedure normospermia was statistically significant.ConclusionAfter functional BEEC reconstruction, long-term bladder function is preserved with mostly normal renal function. The number of bladder neck attempts has a significant influence on andrologic outcome. Detailed analysis may detect multifactorial pathogenesis from the impaired sperm quality in the BEEC.     

Which method is the most reliable in determination of bladder capacity in children with idiopathic overactive bladder? A comparison of maximum voided volume,uroflowmetry and maximum cystometric capacity

ObjectiveTo compare uroflowmetry, maximum cystometric capacity and maximum voided volume in terms of bladder capacity (BC) estimation in children with idiopathic overactive bladder.Materials and methodsA total of 84 children with idiopathic overactive bladder were enrolled. Bladder diaries were kept by parents to measure maximum voided volume. The sum of voided volume and post-void residual urine represented uroflowmetric BC. Maximum cystometric capacity was recorded for each patient. Patients with and without urge incontinence and both sexes were compared, and correlations were tested between estimated capacities, patient age and continence status.ResultsMean age was 10.88 (range 6–16) years. There were 52 females and 32 males. A total of 48 patients had urge incontinence (group 1), and the remaining 36 (group 2) did not. Mean bladder capacities were 165.44 ml for uroflowmetry, 204.37 ml for cystometry and 260.07 ml for bladder diary. Maximum cystometric capacity and capacity in terms of maximum voided volume by diary were significantly higher in patients without urge incontinence. There were significant correlations between age and all other variables, and between the presence of urge incontinence and all variables except maximum cystometric capacity. There were significant differences between bladder capacities estimated by the three methods; however, on age-adjusted re-analysis this significance was lost.ConclusionThere is no significant difference between BCs estimated by uroflowmetry, maximum cystometric capacity and maximum voided volume in children with idiopathic overactive bladder. Bladder diary is a reliable and non-invasive way of estimating BC in this patient population.     

Absence of breast-feeding is associated with the risk of type 1 diabetes: a case–control study in a population with rapidly increasing incidence

There are indications that the effect of environmental factors on the risk of type 1 diabetes mellitus (T1DM) is increasing over time. This can be documented by the rapid increase of T1DM incidence in genetically stable populations. Our aim was to study an association of T1DM with the variable factors of the perinatal period and of early infancy, using data from children born over a period of changing exposure to some of the studied factors. A case–control dataset was analysed, consisting of 868 diabetic children and 1,466 anonymous controls, mostly schoolmates of the children with T1DM. The data were collected using structured questionnaires completed by parents. After performing univariate analyses, the associations were analysed using multiple logistic regression adjusted for potential confounders, including the year of birth. The risk of T1DM decreased with increasing duration of breast-feeding, while no breast-feeding was associated with an increased T1DM risk, OR=1.93 [95% CI: 1.33–2.80], breast-feeding for more than 12 months was protective, OR=0.42 [95% CI: 0.22–0.81], both being relative to the reference category of breast-feeding for 1–3 months. A short duration of day-care attendance (none or less than 1 year) was weakly associated with the risk of T1DM, OR=1.65 [95% CI: 1.05–2.62]. No association was detected between T1DM and signs of prenatal infections, perinatal stress factors, birth size and weight, indicators of crowding or the presence of a domestic pet in the household. Short breast-feeding period and short attendance to day care is associated with the risk of T1DM in Czech children.     

Transcatheter Occlusion of a Modified Blalock–Taussig Shunt Using the Amplatzer Vascular Plug with the Catheter–Snare Technique

A 16-month-old boy with previous repair of a critical pulmonary stenosis had persistence of a right modified Blalock–Taussig shunt. Transcatheter occlusion of the modified Blalock–Taussig shunt was achieved using the Amplatzer vascular plug with the catheter–snare technique.     

Novel Influenza a (H1N1) Infection in a Pediatric Hematology Oncology Clinic During the 2009–2010 Pandemia

Pandemic influenza A infection (2009 H1N1) was associated with a worldwide outbreak of febrile respiratory infection. Although usually it results in a mild illness, certain patient groups are at increased risk for complications. The authors reviewed their experience in a pediatric hematology-oncology unit to determine the outcome of this disease in children with hematological conditions and solid tumors. During the second outbreak (1 November 2009 to 14 January 2010), a total of 187 children from pediatric clinic were tested for H1N1 influenza A by multiplex polymerase chain reaction (PCR), 63 of them were positive. Patients’ signs and symptoms were recorded prospectively. Ten (35.7%) (5 children with solid tumors, 4 with leukemia, 1 with hereditary spherocytosis) of 28 tested children with hematological conditions were diagnosed with 2009 H1N1 influenza infection. Fever (100%) and cough (90%) were the most common symptoms. Five were neutropenic (neutrophil count <1000/mm³), 4 had severe neutropenia (neutrophil count <500/mm³). Systemic antibiotics were given in 5 patients with the diagnosis of febrile neutropenia. Four were inpatients, others were hospitalized after the diagnosis. One patient required mechanical ventilation; however, he had concomitant invasive fungal infection. Eight patients were treated by oseltamivir, all tolerated the drug well. A total of 4 cases from 9 cancer patients had a delay in their planned chemotherapy for 7 to 15 days. Pandemic H1N1 influenza caused mild symptoms in children with cancer and/or hematological conditions but resulted in delay in anticancer therapy and increase in hospitalization and antibiotic usage.     

Familiarity with current practices of granting and maintaining privileges in pediatric interventional radiology—a worldwide survey of the members of the Society for Pediatric Interventional Radiology (SPIR)

Background

Physician credentialing is a complex process driven by the demand for quality improvement in health care. In the U.S., the Joint Commission Standard of 2007 has tied hospital accreditation to credentialing through mandated use of the Focused Professional Practice Evaluation (FPPE) and Ongoing Professional Practice Evaluation (OPPE).

Objective

To assess pediatric interventional radiologists’ knowledge of how institutions grant them privileges.

Materials and methods

Members of the Society for Pediatric Interventional Radiology (SPIR) were sent a web-based survey regarding credentialing.

Results

Of 122 members from 19 countries, 81 (66%) responded, and of these 81, 59 (73%) were familiar with their hospital’s privileging process. Of 49 U.S. respondents and 32 non-U.S. respondents, 37 (76%) and 17 (53%), respectively, stated that interventional radiology credentialing was different from diagnostic radiology credentialing. Of the 49 U.S. respondents, 24 (49%) reported an OPPE, and of the 32 non-U.S. respondents, 8 (25%) reported an ongoing evaluation. The U.S. OPPE is performed at shorter intervals than its international equivalent.

Conclusion

Four years after the Joint Commission defined the FPPE and OPPE, separate credentialing of pediatric interventional radiology from pediatric diagnostic radiology is more likely in the U.S. than internationally, and U.S. pediatric interventional radiologists are more likely to have a defined ongoing professional evaluation and to be evaluated every 6?months or more frequently. There are many SPIR members who do not know how they obtain privileges and/or are not knowingly subject to an OPPE. This lack of knowledge may affect future education of interventional radiologists as well as the definition of pediatric interventional radiology practices within individual institutions.     

Concurrent Validity of the Functional Independence Measure for Children (WeeFIM(™)) and the Pediatric Evaluation of Disabilities Inventory in Children with Developmental Disabilities and Acquired Brain Injuries

TheFunctional IndependenceMeasure forChildren (WeeFIM(tm)) and the Pediatric Evaluation of Disability Inventory (PEDI) are the most commonly used measures of functional performance in children. The purpose of this study was to determine the concurrent validity of the instruments when used with children with developmental disabilities and acquired brain injury. The subjects were 41 children, age 1.3 to 9.5 years, who were receiving inpatient or outpatient services at a pediatric rehabilitation unit in Brisbane, Australia. Spearman correlation coefficients between the two tests were greater than 0.88 for self care, transportation/locomotion, and communication/social function. The high correlations indicate that the two tests measure similar constructs. Choosing between the tests depends on situational requirements and depth of information required.     

The child with immune thrombocytopenic purpura: is pharmacotherapy or watchful waiting the best initial management? A panel discussion from the 2002 meeting of the American Society of Pediatric Hematology/Oncology

The initial management of immune thrombocytopenic purpura is a topic of debate among pediatric hematologists. The decision whether to start a patient on pharmacotherapy or to employ an approach of watchful waiting and patient education is problematic for this group of physicians. A wide variety of research studies and review articles have been published on either side of this debate. Here, the proceedings from a panel discussion, held at the 2002 American Society of Pediatric Hematology/Oncology meeting, are presented. The panel, composed of experts on both sides of the debate, presented the rationale, benefits, and risks of both pharmacotherapy and the watchful waiting strategy.