Is it safe to avoid bone marrow examination in suspected itp?

Two children with suspected ITP are described. One child was treated outside with corticosteroids and was diagnosed acute lymphoblastic leukemia. Another child was fresh and diagnosed as acute myeloid leukemia on bone marrow aspirate examination. Both the children had no physical or peripheral smear finding suggestive of leukemia. We suggest that a bone marrow examination is required in developing countries for evaluation of thrombocytopenia before labeling it an immune thrombocytopenic purpura.     

A case with extraosseous Ewing's sarcoma: a late effect related to bone marrow transplantation for thalassemia or a component of a familial cancer syndrome?

Allogeneic bone marrow transplantation has proved to be a radical form of cure in patients with beta-thalassemia major who have a human leukocyte antigen identical donor. Although malignant neoplasms are serious late complications of bone marrow transplantation, very few reports describing the development of malignant tumors after allografting for thalassemia appeared in the literature. A case is presented here of extraosseous Ewing's sarcoma that developed 8 years after allogeneic bone marrow transplantation performed for beta-thalassemia major. The phenotypic features of the patient's family fullfill the criteria for Li-Fraumeni syndrome. The patient was treated with chemotherapy and radiotherapy and died with recurrent disease. To the authors' knowledge, this is the first case of extraosseous Ewing's sarcoma after bone marrow transplantation for thalassemia. The possible contribution of transplantation procedure and the genetic factors as well as the primary genetic hemoglobinopathy to the development of this malignant tumor are discussed.     

Lymphatic preservation in varicocele in the adolescent: Does prior embolization limit spermatic dissection?

ObjectiveOne of the complications after complete section of the spermatic pedicle in the treatment of adolescent idiopathic varicocele is the formation of a scrotal lymphocele. This can be avoided by preserving lymphatic vessels using dye, but there is a risk that dissection may be complicated in patients who have previously undergone embolization. The aim of this study was to determine whether prior embolization limits spermatic dissection.Material and MethodWe used lymphography with dye (Patent Blue) prior to surgery in order to mark and preserve the lymph vessels during spermatic section. This was done by laparoscopy with a single umbilical port.ResultsWe treated six patients aged 12.5–15 years (mean 13.12 years), two of whom had grade 2 varicoceles and four grade 3. Prior percutaneous embolization with metallic coils had been undertaken in all cases but had not been curative. Post-surgery controls were undertaken for a mean duration of 5 months without any lymphoceles appearing. No testicles were lost, nor did any other complication arise.ConclusionThe presence of embolization material in the spermatic veins and perivascular fibrosis does not complicate surgery, enabling the single port laparoscopic technique to be undertaken.     

Tolerance: is it achievable in pediatric solid organ transplantation?

Significant advances have been made in the understanding of allograft rejection. There is growing awareness that allograft acceptance, or tolerance, is also an active process rather than a passive absence of rejection. Mechanistic awareness of this process has spawned many preclinical strategies for the prevention of allograft rejection without the need for chronic immunosuppression. These therapies are currently entering clinical trials. This article reviews the prevailing therapies that hold promise for future clinical application. In particular, their application in children is discussed, as are biologic aspects of childhood immunity that may play a role in the success or failure of these strategies.     

Predictive genetic testing in young people: When is it appropriate?

Predictive genetic testing is currently offered to adults for a range of conditions, even when there is no possibility of prophylaxis or treatment. However, similar testing in children and adolescents is advised against in international guidelines. Despite this, several authors have argued against the existing guidelines. Given the lack of empirical evidence, it is important that clinicians are aware not only of the guidelines, but also of the arguments in favour of such testing.     

The Goldilocks principle. Oxygen in the delivery room: When is it too little,too much,and just right?

Oxygen has been used to stabilize newborn infants for more than a century. Over the last two decades, a paradigm shift towards using less oxygen has occurred but without firm evidence of benefit. Using lower levels of oxygen has also added new conundrums to clinical care. Can oxygen delivery to sick newborn babies meet the Goldilocks principle, of being “just right”? This review discusses the history of oxygen use in the delivery room and the impetus to change from the long-established practice of using pure oxygen to using lower oxygen concentrations. The review also highlights knowledge gaps, particularly for oxygen exposure and monitoring, as well as the sequelae of oxygen administration, including short- and long-term outcomes.     

When is a mediastinal mass critical in a child? An analysis of 29 patients

The aims of this study were to determine the pattern of presentation of childhood mediastinal masses in our community and to identify factors associated with the development of acute airway compromise. The authors retrospectively reviewed the records of 29 consecutive patients with mediastinal masses managed at their institution between January 1995 and December 2001. Demographic data, mass characteristics, clinical presentation, and surgical procedures were recorded. Seven patients (24.1%) were asymptomatic at presentation. Eight (27.6%) were classified as having acute airway compromise at presentation. Respiratory symptoms and signs were the most common mode of presentation (58.6% and 55.2%, respectively). The most common histological diagnosis was neurogenic mass (37.9%), followed by lymphoma (24.1%). Most masses were located in the superior mediastinum (41.1%). Factors associated with the development of acute airway compromise were (1) anterior location of the mediastinal mass ( P =0.019), (2) histological diagnosis of lymphoma ( P=0.008), (3) symptoms and signs of superior vena cava syndrome ( P =0.015 and 0.003, respectively), (4) radiological evidence of vessel compression or displacement ( P =0.015), (5) pericardial effusion ( P =0.015), and (6) pleural effusion ( P =0.033). Clinical presentation of childhood mediastinal masses is often nonspecific or incidental. Yet they have the propensity of developing acute airway compromise, which is closely associated with superior vena cava obstruction. Such patients should be managed as a complex cardiorespiratory syndrome, termed critical mediastinal mass syndrome, by an experienced multidisciplinary team.     

Assessing bone health in children: who to test and what does it mean?

The foundation of bone health is established during childhood and adolescence. Unfortunately, pediatric bone health may be threatened in a variety of genetic and acquired disorders. Low bone mineral density (BMD) and/or fragility fractures may result. This paper addresses the pediatric indications for bone densitometry testing in children at risk for poor bone health and the challenges of interpreting the results. Dual energy x-ray absorptiometry (DXA) is the most commonly used of the bone densitometry methods. Most DXA software programs report BMD in terms of a T-score, which compares the results to adult norms. Z-scores, based upon age- and gender-matched reference data, should be used instead to avoid the mislabeling of a child as "osteoporotic." Delayed growth and maturation also complicate the interpretation of DXA findings in chronically ill patients. Although children with low BMD values may have an increased risk of fracture, a pediatric "fracture threshold" has not been established. Further research is needed to refine the indications for bone density testing in children and to aid in the interpreting the results.     

Parent-controlled PCA for pain management in pediatric oncology: is it safe?

Patient-controlled analgesia offers safe and effective pain control for children who can self-administer medication. Some children may not be candidates for patient-controlled analgesia (PCA) unless a proxy can administer doses. The safety of proxy-administered PCA has been studied, but the safety of parent-administered PCA in children with cancer has not been reported. In this study, we compare the rate of complications in PCA by parent proxy versus PCA by clinician (nurse) proxy and self-administered PCA. Our pediatric institution's quality improvement database was reviewed for adverse events associated with PCA from 2004 through 2010. Each PCA day was categorized according to patient or proxy authorization. Data from 6151 PCA observation days were included; 61.3% of these days were standard PCA, 23.5% were parent-proxy PCA, and 15.2% were clinician-proxy PCA days. The mean duration of PCA use was 12.1 days, and the mean patient age was 12.3 years. The mean patient age was lower in the clinician-proxy (9.4 y) and parent-proxy (5.1 y) groups, respectively. The complication rate was lowest in the parent-proxy group (0.62%). We found that proxy administration of PCA by authorized parents is as safe as clinician administered and standard PCA at our pediatric institution.