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Minimal change disease accounts for up to 25% of the cases of nephrotic syndrome in adult population. The allergic mechanism has been associated with minimal change disease and allergens have been implied, namely insect stings. We present a case report of a woman with new onset of nephrotic syndrome after a non-hymenoptera insect sting, with biopsy-proven minimal change disease, that was accompanied by a pulmonary thromboembolism process. Complete remission with glucocorticoid therapy was observed, with sustained response for 6 months after discontinuation. A new exposure to insect sting in the same geographical region and season triggered a nephrotic syndrome relapse. Subsequent avoidance of the place resulted in a sustained remission for more than 4 years.

The regulation of hematopoietic stem cell (HSC) emergence during development provides important information about the basic mechanisms of blood stem cell generation, expansion, and migration. We set out to investigate the role that cytokine signaling pathways play in these early processes and show here that the 2 cytokines interleukin 3 and thrombopoietin have the ability to expand hematopoietic stem and progenitor numbers by regulating their survival and proliferation. For this, they differentially use the Janus kinase (Jak2) and phosphatidylinositol 3-kinase (Pi3k) signaling pathways, with Jak2 mainly relaying the proproliferation signaling, whereas Pi3k mediates the survival signal. Furthermore, using Jak2-deficient embryos, we demonstrate that Jak2 is crucially required for the function of the first HSCs, whereas progenitors are less dependent on Jak2. The JAK2V617F mutation, which renders JAK2 constitutively active and has been linked to myeloproliferative neoplasms, was recently shown to compromise adult HSC function, negatively affecting their repopulation and self-renewal ability, partly through the accumulation of JAK2V617F-induced DNA damage. We report here that nascent HSCs are resistant to the JAK2V617F mutation and show no decrease in repopulation or self-renewal and no increase in DNA damage, even in the presence of 2 mutant copies. More importantly, this unique property of embryonic HSCs is stably maintained through ≥1 round of successive transplantations. In summary, our dissection of cytokine signaling in embryonic HSCs has uncovered unique properties of these cells that are of clinical importance., (© 2016 by The American Society of Hematology.)

Objectives: To study the effect of two intravenous maintenance fluids on plasma sodium (Na), and acid-base balance in pediatric intensive care patients during the first 24h of hospitalization., Methods: A prospective randomized controlled study was performed, which allocated 233 patients to groups: (A) NaCl 0.9% or (B) NaCl 0.45%. Patients were aged 1 day to 18 years, had normal electrolyte concentrations, and suffered an acute insult (medical/surgical)., Main Outcome Measured: change in plasma sodium. Parametric tests: t-tests, ANOVA, X(2) statistical significance level was set at α=0.05., Results: Group A (n=130): serum Na increased by 2.91 (±3.9)mmol/L at 24h (p<0.01); 2% patients had Na higher than 150 mmol/L. Mean urinary Na: 106.6 (±56.8)mmol/L. No change in pH at 0 and 24h. Group B (n=103): serum Na did not display statistically significant changes. Fifteen percent of the patients had Na<135 mmol/L at 24h. The two fluids had different effects on respiratory and post-operative situations., Conclusions: The use of saline 0.9% was associated with a lower incidence of electrolyte disturbances., (Copyright © 2015 Sociedade Brasileira de Pediatria. Published by Elsevier Editora Ltda. All rights reserved.)

Background: Children and adolescents with sickle cell disease (SCD) have a higher incidence of sleep pathology and obstructive sleep apnea syndrome (OSAS). The nocturnal hypoxemia is a risk to vaso-occlusive crisis among other SCD morbidities. Our aim was to compare polysomnography (PSG) results in a sample of children with SCD with a sample of children with suspected OSAS without SCD., Design and Methods: A retrospective study compared clinical and PSG parameters. A descriptive analysis and t-test were done considering P < 0.05 as significant., Results: PSG was done in 65 children with SCD and 65 control-children. Control sample was selected to be equal to SCD sample considering gender (53.8% were male), age (mean age was 9.4 years (SD ± 4.6) and AHI (mean 3.57 events/hr). Mean efficiency, latency and percentage of sleep phases in both groups showed no statistically significant differences. Mean SpO2 and minimum SpO2 were lower in SCD group and it was statistically significant (P < 0.01). Enuresis was more frequent in the SCD children group (35.4% vs. 6.2%, P < 0.01)., Conclusion: Comparing children with and without SCD, sleep architecture was similar in both groups and minimum SpO2 was significantly lower in SCD children although both groups had a similar AHI. This is an important issue in these children, so it is essential to have a sleep evaluation in order to prevent complications and co-morbidities., (© 2014 Wiley Periodicals, Inc.)

A tuberculoma is a rare form of presentation of tuberculosis (TB) in children. We describe the case of a 13-year-old girl, with 3&emsp14;weeks of progressive tiredness and asthenia and a 48 h fever and cough. Physical examination revealed diminished pulmonary sounds in the left lower hemithorax. A chest radiograph showed an oval hypotransparency image in this location. The TB skin test was anergic and sputum was negative for acid-fast bacilli (AFB). The thoracic CT revealed a cystic mass in the left lower half hemithorax, compressing the adjacent pulmonary lobe, with double non-calcificated membrane. The exeresis of the pulmonary mass was performed and the anatomopathological study revealed a tuberculoma with AFB. TB treatment was established with a favourable clinical and radiological evolution. TB is a prevalent disease around the world. In this case, due to the mass dimensions and adjacent-organ compression, surgery was essential for a favourable clinical evolution.

Introduction: In Cavernous transformation of portal vein (CTPV), therapeutic options are limited; however the restoration of circulation by mesenteric-portal bypass (MPB) is an option in selected cases. CASE REPORT 1: 13-year-old female with polymalformative syndrome. Admission at 4 months of age to Intensive Care Unit due to severe pneumonia with hemodynamic instability. Follow up due to thrombocytopenia and splenomegalia she was diagnosed CTPV at7-years old. At 13y-old she was submitted to MPB. CASE REPORT 2: 9-years-old male; severe neonatal Rh isoimmunization treated with exsanguinations. Followed-up since 6-months ofage due to thrombocytopenia and splenomegalia, and at 3 years of age he was diagnosed CTPV. Due to disease progression he was proposed as candidate to MBP which was contraindicated due to vascular thrombosis of the Rex recess., Comments: MBP presents as one of the few therapeutic options to CTPV in children; due to its specificity and rigid requirements it is vital the close follow up and early diagnosis.

High-output cardiac failure is rare in newborns. Emergent diagnosis and management of this pathology is crucial. We report the case of a child, currently 12-months old; obstetric background is non-contributory. Clinic observation on D1 was normal except for the presence of a systolic cardiac murmur; cardiological evaluation revealed mild ventricular dysfunction of the right ventricle. On the third day of life, she developed cardiac failure with gallop rhythm, hepatomegaly and a murmur in the anterior fontanel; an echocardiogram confirmed clinic aggravation with biventricular dysfunction and right cavities and superior vena cava dilatation. The cranial MRI confirmed the presence of a pial arteriovenous malformation (AVM) involving the anterior and middle cerebral arteries with an associated fronto-parietal ischaemic lesion. The infant underwent embolisations of AVM with successful flow reduction and cardiac failure improvement. The multidisciplinary follow-up showed no cardiac dysfunction or permanent lesions but confirmed a severe psycho-motor delay and left hemiparesia.

Local anesthetics (LA) are frequently used in medical and surgical and dental procedures. Adverse reactions to LA are rare, and hypersensitivity reactions are very rare. Nevertheless, they are the third more frequent cause of referral to Allergy Clinics due to drug allergy, after betalactamic antibiotics and non-steroidal anti-inflammatory agents. In this paper we review hypersensitivity reactions to LA and propose a diagnostic approach to them.

The first adult-repopulating hematopoietic stem cells (HSCs) are detected starting at day 10.5 of gestation in the aorta-gonads-mesonephros (AGM) region of the mouse embryo. Despite the importance of the AGM in initiating HSC production, very little is currently known about the regulators that control HSC emergence in this region. We have therefore further defined the location of HSCs in the AGM and incorporated this information into a spatial and temporal comparative gene expression analysis of the AGM. The comparisons included gene expression profiling (1) in the newly identified HSC-containing region compared with the region devoid of HSCs, (2) before and after HSC emergence in the AGM microenvironment, and (3) on populations enriched for HSCs and their putative precursors. Two genes found to be up-regulated at the time and place where HSCs are first detected, the cyclin-dependent kinase inhibitor p57Kip2/Cdkn1c and the insulin-like growth factor 2, were chosen for further analysis. We demonstrate here that they play a novel role in AGM hematopoiesis. Interestingly, many genes involved in the development of the tissues surrounding the dorsal aorta are also up-regulated during HSC emergence, suggesting that the regulation of HSC generation occurs in coordination with the development of other organs.

This study compared four treatments for bacterial endometritis in mares experimentally infected with Streptococcus zooepidemicus. Twenty-five mares were used, 20 resistant and five susceptible to endometritis. Mares would be in estrus when infected. Twenty-four hours after inoculation, clinical, bacteriological and cytological examinations were performed and repeated until the first occurrence: negative cytology and no Streptococcus growth or the seventh day post-infection. All mares showed clinical signs of endometritis and were assigned to one of the following treatments: (1) intrauterine infusion of fresh leukocytes; (2) intrauterine infusion of frozen-thawed leukocytes; (3) intrauterine infusion of lysed leukocytes; (4) intrauterine infusion of recombinant human interleukin-8 (rhIL-8); (5) control. Mares were submitted to all treatments, with at least a 14-day interval between treatments in a Latin square design. Treatment did not affect (P=0.121) time needed for resistant mares to eliminate bacteria. Time needed for elimination of bacteria was similar in susceptible mares treated with fresh and frozen leukocytes (P=0.333). Susceptible mares treated with frozen leukocytes also did not differ from those treated with lysed leukocytes (P=0.227) for time to eliminate bacteria, but were significantly different (P>0.02) from those treated with rhIL-8 and control. In resistant mares, physical clearance ability was probably the responsible for bacterial elimination. Intrauterine infusions in susceptible mares with viable or lysed leukocytes associated or not to opsonizing factors, reduced the time to elimination of bacteria. Infusions with bactericidal effect (functional neutrophils and granules) was likely effective and responsible for the more rapid elimination of bacteria in susceptible mares.