A Near Miss and Salvage Management of Aortoesophageal Fistula Secondary to Cell Battery Ingestion.

We report a case of an infant surviving aortoesophageal fistula secondary to lithium cell battery ingestion. In the setting of a delayed vascular complication, computed tomography and magnetic resonance imaging are essential to establishing the correct diagnosis and surgical management. Management of children after battery ingestion must be guided by a high index of clinical suspicion.

Point-of-care prothrombin time testing in paediatric intensive care: an observational study of the ease of use of two devices.

BACKGROUND: Two major difficulties arise when taking blood samples in children: the challenge of venous access and the comparatively large amount of blood required. OBJECTIVE: To assess the value of point-of-care prothrombin time testing in paediatric intensive care patients. We evaluated two point-of-care devices, CoaguChek XS Plus and CoaLine, assessing ease of use in clinical practice and correlation with the standard prothrombin time measurement of the haematology laboratory. DESIGN: Single-centre observational study. SETTING: Between October 2007 and March 2008, patients in an interdisciplinary paediatric ICU of a tertiary centre were analysed. PATIENTS OR OTHER PARTICIPANTS: Thirty-eight patients, 22 female and 16 male (58 and 42%), aged between 0 and 13 years, participated in the study. The intention was to evaluate the ease of use of the devices in daily clinical practice, and no exclusion criteria were applied. MAIN OUTCOME MEASURES: The usefulness of the two point-of-care devices in the paediatric setting was evaluated. Measurements of point-of-care and standard laboratory prothrombin time were compared in terms of agreement and correlation. RESULTS: CoaguChek XS Plus had a failure rate of 2%, CoaLine 17% and the laboratory standard 4%. CoaguChek XS Plus received a better ease of use rating than CoaLine by the study personnel. Compared with the laboratory standard, there was considerable variability of the observed measurements with both devices. The measurements of CoaguChek XS Plus and the standard had a correlation coefficient r of being 0.79. CoaLine and the standard had a correlation coefficient r value of 0.72. CONCLUSION: CoaguChek XS Plus showed 'good' agreement, whereas CoaLine showed 'moderate' agreement compared with prothrombin time values using the standard method. The fast availability of results and the reduction of the required blood volume are advantages of point-of-care tests in the paediatric setting.

Chickenpox is not always benign: postvaricella purpura fulminans requires prompt and aggressive treatment.

We present 2 patients, who were admitted owing to rapidly progressing purpuric lesions due to postvaricella purpura fulminans, a coagulopathy leading to life- or limb-threatening thrombosis caused by a severe transient autoimmune protein S deficiency. Laboratory results were being consistent with disseminated intravascular coagulation secondary to protein S deficiency; treatment with fresh frozen plasma, intravenous immunoglobulins, and prednisone was started. In our experience, a prompt therapy may limit the course and the extent of the disease. We present a review of the topic with supporting literature for the therapeutic options. Therefore, we should be reminded that purpura fulminans is a rare but severe complication of chickenpox, which demands quick action.

Patent arterial duct, bottle-meal, and fatal myocardial ischaemia.

A patent arterial duct in pre-term neonates is frequent. Systemic complications consecutive to left-to-right shunting are well known but fatal myocardial ischaemia has not been described till now. The presented premature baby died from catecholamine refractory cardiogenic shock. Autoptic examination revealed acute ischaemic changes predominantly in the inner third of myocardium, speaking of coronary hypoperfusion due to a steal phenomenon secondary to the patent arterial duct.

Mycoplasma pneumoniae associated opsoclonus-myoclonus syndrome in three cases.

Opsoclonus-myoclonus syndrome (OMS) is a rare acquired movement disorder occurring in all age groups, predominantly in infants. Although the exact pathogenesis is still undefined, there is strong evidence for a paraneoplastic or parainfectious immune process resulting in central nervous system dysfunction. Mycoplasma pneumoniae has been implicated in a number of immune-mediated neurologic diseases [28]. However, the association of M. pneumoniae and opsoclonus-myoclonus-ataxia syndrome is not well established so far. We present three cases with opsoclonus-myoclonus-ataxia syndrome in adolescents following an infection with M. pneumoniae. Monophasic disease course and full recovery correspond to the favorable prognosis known from parainfectious cases in young adults. This should affect therapeutic consideration. OMS should be added to the spectrum of M. pneumoniae-associated neurologic complications. Nevertheless, neuroblastoma has to be ruled out in all cases of OMS.

Complex rearranged small supernumerary marker chromosomes (sSMC), three new cases; evidence for an underestimated entity?

BACKGROUND: Small supernumerary marker chromosomes (sSMC) are present ~2.6 x 106 human worldwide. sSMC are a heterogeneous group of derivative chromosomes concerning their clinical consequences as well as their chromosomal origin and shape. Besides the sSMC present in Emanuel syndrome, i.e. der(22)t(11;22)(q23;q11), only few so-called complex sSMC are reported. RESULTS: Here we report three new cases of unique complex sSMC. One was a de novo case with a dic(13 or 21;22) and two were maternally derived: a der(18)t(8;18) and a der(13 or 21)t(13 or 21;18). Thus, in summary, now 22 cases of unique complex sSMC are available in the literature. However, this special kind of sSMC might be under-diagnosed among sSMC-carriers. CONCLUSION: More comprehensive characterization of sSMC and approaches like reverse fluorescence in situ hybridization (FISH) or array based comparative genomic hybridization (array-CGH) might identify them to be more frequent than only ~0.9% among all sSMC.

High-dose therapy and autologous stem cell transplantation for children with HIV-associated non-Hodgkin lymphoma.

In contrast to adults, autologous stem cell transplantation (ASCT) as part of the salvage strategy after high-dose chemo/radiotherapy in human immunodeficiency virus (HIV) related Non-Hodgkin lymphoma (NHL) is not yet established for children. We report on a 13-year patient with congenital HIV infection and refractory Burkitt lymphoma, who was successfully treated by high-dose therapy (HDT) including rituximab followed by ASCT. After 26 months follow-up the patient remains in complete remission and his HIV parameters have normalized with continued highly active antiretroviral therapy (HAART). HIV infection may no longer exclude children from ASCT as part of salvage therapy.