CHEST PAIN DUE TO A GIANT THYMOMA IN AN ADOLESCENT BOY.

Chest pain in children is a common referral for emergency examination, although it is believed that the underlying condition is not a serious event. The authors present the case of a 17 years old male with complaints of chest pain with a very recent onset, constant, experienced after physical effort. All medical history and physical examination findings were normal, the poster-anterior chest X-ray revealed a heterogenous opacity in the middle third of the right hemithorax confirmed on computerized tomography as a large solid anterior mediastinal mass with a diameter of 7.5/10.3 cm. By thorax surgery the mass was identified in the anterior and superior mediastinum and total resection was achieved for the tumor mass and thymus. Pathology examination showed poor small and medium epithelial cells proliferation and the tumor was classified as thymoma type 1B. Mediastinum is a rare location of space occupying processes in children. Also, there is a high rate of asymptomatic lesions in this area, considering that half of them are incidentally discovered for various chest X ray examinations. Many causes of chest pain in children are benign; nonetheless, some serious events do exist sometimes and pediatricians must pay attention to identify and manage those cases.

Another way of thinking about ADHD: the predictive role of early attachment deprivation in adolescents' level of symptoms.

PURPOSE: Attention deficit and hyperactivity disorder (ADHD) is one of the most frequent disorders in childhood and adolescence. Both neurocognitive and environmental factors have been related to ADHD. The current study contributes to the documentation of the predictive relation between early attachment deprivation and ADHD. METHOD: Data were collected from 641 adopted adolescents (53.2% girls) aged 11-16 years in five countries, using the DSM oriented scale for ADHD of the Child Behavior Checklist (CBCL) (Achenbach and Rescorla, Manual for the ASEBA school-age forms and profiles. University of Vermont, Research Center for Children, Youth and Families, Burlington, 2001). The influence of attachment deprivation on ADHD symptoms was initially tested taking into consideration several key variables that have been reported as influencing ADHD at the adoptee level (age, gender, length of time in the adoptive family, parents' educational level and marital status), and at the level of the country of origin and country of adoption (poverty, quality of health services and values). The analyses were computed using the multilevel modeling technique. RESULTS: The results showed that an increase in the level of ADHD symptoms was predicted by the duration of exposure to early attachment deprivation, estimated from the age of adoption, after controlling for the influence of adoptee and country variables. The effect of the age of adoption was also demonstrated to be specific to the level of ADHD symptoms in comparison to both the externalizing and internalizing behavior scales of the CBCL. CONCLUSION: Deprivation of stable and sensitive care in infancy may have long-lasting consequences for children's development.

What is the significance of a microscopically positive resection margin in the curative-intent treatment of rectal adenocarcinoma? A retrospective study.

PURPOSE: The aim of this study was to analyze the characteristics of patients with rectal cancer operated with a microscopic positive margin (R1) and thus avoid these situations or adapt treatment in these particular cases. METHODS: We reviewed all the pathology data of resected specimens from patients with rectal or recto-sigmoid cancer operated with curative intent at the Institute of Oncology "Prof. Dr. Ion Chiricuta" between 2000-2011 (763 patients in 12 years) and the pathology files of patients from other institutions referred for adjuvant treatment to our hospital (318 patients). We included patients with anterior resection, Hartmann's procedure and abdomino-perineal resection, but we excluded patients with local excision and patients with R2/R1 at first, but R0 after re-resection (56 patients). We have identified 31 patients with R1, but had to exclude one case from analysis because this patient was lost to follow-up. RESULTS: With surgery alone the local relapse (LR) was unavoidable. In the neoadjuvant chemoradiation (CRT) group 85.7% of the patients did not develop LR despite of R1. In the adjuvant CRT cohort 50% of the patients were LR-free at 2 years after conventional radiotherapy (p<0.01). CONCLUSION: Based on these results it is concluded that a clear resection margin is extremely important for the local control of rectal cancer, because it cannot be always compensated by adjuvant CRT. In R1 cases neoadjuvant CRT seems to offer better prognosis than adjuvant CRT. To avoid R1 and its consequences a good quality control of total mesorectal excision (TME) is needed and CRT should be done before and not after surgery. R1 after primary surgery needs to be compensated by re-resection if possible, otherwise probably high dose radiotherapy with chemotherapy is needed.

Neonatal Burns - A Romanian Experience From A Tertiary Care Burn Unit.

Burns in neonates is a unique pathology that poses management challenges, as, besides treating the burn injury, the medical team needs to be familiar with the unique physiology of the newborn. This study aimed to review our center's experience with the management of neonatal burns. A 7-year retrospective study of neonatal burns managed at a tertiary national referral unit in Bucharest, Romania, was performed. Patients were divided into two groups, preterm and term, based on gestational age. Collected data included demographics, treatment, complications and follow-up. Our center managed 13 neonates with burns (5 term and 8 preterm), with a mean age at injury of 8.6 days. All neonatal burns were thermal injuries. For preterm babies, all burns were iatrogenic, consisting of victims of a fire in a Maternity Hospital, while for the term group only one was iatrogenic. Burns were more extensive in the premature group (65% vs. 20% mean TBSA, p=0.0001). All premature patients had associated inhalation injuries. Surgical treatment was indicated for two term patients. Survival rate was 77% and was negatively influenced by inhalation injury, burn shock and TBSA >50%. In conclusion, the majority of hospitalized neonatal burns in Romania were the result of a fire disaster secondary to an infrastructure deficiency. Their management is complex, posing specific challenges, and needs to be led by a multidisciplinary team that can ensure an individualized continuum of acute burn care and recovery.

Les brûlures du nouveau- né (NN) posent une problème physiopathologique spécifique, tenant non seulement aux conséquences de la brûlure mais aussi à la physiologie particulière du NN. Cet article décrit notre expérience. Il s'agit d'une étude rétrospective reprenant les 13 NN pris en charge dans un CTB de référence de Bucarest, pour une brûlure survenue en moyenne à 8,6J. Les patients ont été répartis entres prématurés (P,8) et à terme (T,5). Toutes les brûlures étaient thermiques, toutes iatrogènes dans le groupe P, consécutives à un incendie à la maternité quand une seule était iatrogène dans le groupe T. Elles étaient plus étendues dans le groupe P (65% SCT) que dans le groupe T (20%, p= 0,0001). Tous les P avaient des lésions d'inhalation. Une greffe a été nécessaire pour 2 T. La mortalité était de 23%, influencée par la surface brûlée, l'inhalation et une surface atteinte >50 %. En conclusion, la plupart des brûlures de NN roumains ayant nécessité une hospitalisation étaient dues à l'incendie d'une maternité à l'architecture déficiente. Leur prise en charge est complexe et spécifique, ce qui nécessite une équipe entraînée, à même de proposer des soins adaptés, de la phase aiguë à la rééducation.

Etiology Of Neonatal Burns: A Systematic Review.

Burns in neonates are a rare pathology with a significant impact on the patient, the family and the healthcare system. At present, scarce information exists on the etiology of this type of injury. A comprehensive worldwide literature review of all publications, including our personal case series, was conducted in order to analyse the etiology of neonatal burns and summarize the available evidence. A total of 41 publications were identified in the last 4 decades, comprising of 105 cases from 20 countries, thus confirming that neonatal burns are, indeed, a rare pathology. All types of burns were recorded in neonates, the most frequent being contact burns, followed by flames and scalds. Almost 60% of burns occurred in a hospital setting due to human factors, malfunctioning devices, or hospital infrastructure deficiency. Compared to domestic burns, iatrogenic injuries occurred at an earlier age (5.4±9.1 days vs. 15.7±9.8 days, p<0.0001) and in neonates with significantly smaller weights (2102.7±1350g vs. 3197±300.8g, p<0.0001). The number of cases was almost equally distributed between developed and developing countries, but developed countries were characterised by a significantly higher preponderance of iatrogenic burns, while in developing countries domestic burns were more frequent (p<0.0001). We believe that this systematic review outlines the potential causes of neonatal burns and provides essential information for formulating prevention strategies.

Les brûlures néonatales, rares, ont un impact important sur l'enfant, ses parents et le système de santé. Nous ne disposons de que de peu de données étiologiques à leur sujet. Nous avons de ce fait réalisé une revue bibliographique et y avons adjoint nos données personnelles. Sur les quarante dernières années, nous avons trouvé 41 publications sur les 40 dernières années, compilant 105 cas dans 20 pays, confirmant la rareté des brûlures néonatales. On trouve tous les groupes de cause, par ordre de fréquence contact, flammes et ébouillantement. Presque 60% d'entre elles surviennent en milieu hospitalier, dues à une erreur humaine, un dispositif fonctionnant mal ou un défaut structurel. Les brûlures nosocomiales surviennent plus tôt (5,4 +/-9,1 j VS 15,7 +/-9,8; p<0,0001) que les brûlures au domicile, chez des enfants de plus petit poids (2 102,7 +/-1 350 g VS 3 197 +/-300,8 g; p< 0,0001). Le nombre de cas était également répartis selon les revenus des pays, les brûlures iatrogènes étant plus fréquentes dans les pays à IDH élevé (<0,0001). Les causes que nous avons retrouvées devraient permettre le développement de stratégies de prévention.

[New generation chemotherapy in the treatment of operated gastric cancer--an alternative to traditional chemotherapy]. / Chimioterapia de generatie noua in cancerul gastric operat--o alternativa la chimioterapia clasica.

Gastric cancer remains one of the most difficult tumour type despite his relative reduction of his global incidence, it remains on of the most deadly cancer. The latest advances in therapy of the gastric cancer ameliorate the results in terms of survival. In our prospective, non-randomized study, we enrolled 40 patients with gastric adenocarcinoma stage IB to IV (M0) who were surgically treated, treated with chemoradioterapy, but with chemotherapy modified by ECX (epirubicine, cisplatine, xeloda) which is now considered standard in metastatic setting. We report the toxicities and possible predictive factors to toxicity. The ECX regimen seems to be a reliable alternative to the traditional regimen with convenient toxicity and therapeutic index.

How often we diagnose allergy to ranitidine?

H2 receptors' antagonists (H2RA) are widely used drugs and they are generally well-tolerated. Ranitidine hypersensitivity reactions (HR) are rarely reported. The article emphasizes the importance of recognizing ranitidine as a cause of anaphylaxis and the advantages and limits of allergological evaluation to establish a positive diagnose. We reviewed a series of published cases of ranitidine-induced hypersensitivity reactions, starting from a clinical case presentation. Moreover, we analyzed the ranitidine related adverse events in the Eudravigilance European database of adverse reactions. Most of the allergic reactions induced by ranitidine are type I HR with immediate onset after exposure, with variable clinical presentation. But in a few cases, there were also described delayed reactions, some after occupational exposure. The article underlines the importance of allergy evaluation to avoid future contact with the drug to reduce the risk of more severe reactions. The suspected reactions should be reported, allowing pharmacovigilance systems to analyse them and to establish further recommendations for clinicians.

The PAF complex regulation of Prmt5 facilitates the progression and maintenance of MLL fusion leukemia.

Acute myeloid leukemia (AML) is a disease associated with epigenetic dysregulation. 11q23 translocations involving the H3K4 methyltransferase MLL1 (KMT2A) generate oncogenic fusion proteins with deregulated transcriptional potential. The polymerase-associated factor complex (PAFc) is an epigenetic co-activator complex that makes direct contact with MLL fusion proteins and is involved in AML, however, its functions are not well understood. Here, we explored the transcriptional targets regulated by the PAFc that facilitate leukemia by performing RNA-sequencing after conditional loss of the PAFc subunit Cdc73. We found Cdc73 promotes expression of an early hematopoietic progenitor gene program that prevents differentiation. Among the target genes, we confirmed the protein arginine methyltransferase Prmt5 is a direct target that is positively regulated by a transcriptional unit that includes the PAFc, MLL1, HOXA9 and STAT5 in leukemic cells. We observed reduced PRMT5-mediated H4R3me2s following excision of Cdc73 placing this histone modification downstream of the PAFc and revealing a novel mechanism between the PAFc and Prmt5. Knockdown or pharmacologic inhibition of Prmt5 causes a G1 arrest and reduced proliferation resulting in extended leukemic disease latency in vivo. Overall, we demonstrate the PAFc regulates Prmt5 to facilitate leukemic progression and is a potential therapeutic target for AMLs.

Mutated Ptpn11 alters leukemic stem cell frequency and reduces the sensitivity of acute myeloid leukemia cells to Mcl1 inhibition.

PTPN11 encodes the Shp2 non-receptor protein-tyrosine phosphatase implicated in several signaling pathways. Activating mutations in Shp2 are commonly associated with juvenile myelomonocytic leukemia but are not as well defined in other neoplasms. Here we report that Shp2 mutations occur in human acute myeloid leukemia (AML) at a rate of 6.6% (6/91) in the ECOG E1900 data set. We examined the role of mutated Shp2 in leukemias harboring MLL translocations, which co-occur in human AML. The hyperactive Shp2E76K mutant, commonly observed in leukemia patients, significantly accelerated MLL-AF9-mediated leukemogenesis in vivo. Shp2E76K increased leukemic stem cell frequency and affords MLL-AF9 leukemic cells IL3 cytokine hypersensitivity. As Shp2 is reported to regulate anti-apoptotic genes, we investigated Bcl2, Bcl-xL and Mcl1 expression in MLL-AF9 leukemic cells with and without Shp2E76K. Although the Bcl2 family of genes was upregulated in Shp2E76K cells, Mcl1 showed the highest upregulation in MLL-AF9 cells in response to Shp2E76K. Indeed, expression of Mcl1 in MLL-AF9 cells phenocopies expression of Shp2E76K, suggesting Shp2 mutations cooperate through activation of anti-apoptotic genes. Finally, we show Shp2E76K mutations reduce sensitivity of AML cells to small-molecule-mediated Mcl1 inhibition, suggesting reduced efficacy of drugs targeting MCL1 in patients with hyperactive Shp2.

[The ocular manifestations in rheumatoid polyarthritis]. / Manifestari oculare în poliartrita reumatoida.

Systemic diseases as rheumatoid arthritis and ankylosing spondylitis have unknown etiologies, unclear pathogenic mechanisms and multiorgan involvements. Ocular involvement is variable and may effect several levels: sclera, cornea, uvea and conjunctiva. Because an ocular event signify an enhancement of inflammatory-immune reactions, the patient may undergo a complete investigation and general therapy must complete local treatment. The paper showing the ocular manifestation in patients with rheumatic arthritis through a ten-years period.

[Painful ophthalmoplegia--a clinical case]. / Oftalmoplegia dureroasa--caz clinic.

We present the case of the patient S.M., female, 32 years old, who came in our eye clinic for left painful ophthalmoplegia. The onset of the clinical manifestations was a year and half ago, with the decrease of visual acuity at left eye, left partial ophthalmoplegia (partial lesion of the left oculomotor nerve) and secondary left trigeminal neuralgia. The paraclinical investigations excluded the systemic and locoregional diseases. CT exams, the left internal carotid angiography and the surgical intervention at the Neurosurgery Clinic in Timisoara revealed a left juxtasella tumor at the base skull, located extradural, which capsule invade the left nerve oculomotor. We conclusion that the painful ophthalmoplegia are complex clinical syndromes, with a different etiopathogenesis (inflammatory, tumoral, vascular malformations: aneurysm etc.) and their diagnosis and treatment need a good interdisciplinary collaboration: ophthalmologist-neurologist-neurosurgeon-endocrinologist-paraclinical exams.

[Cystic iris tumors]. / Tumori iriene chistice.

The paper approaches a rare ophthalmological pathology, cystic iris tumors. The authors show two clinical cases treated in the Clinic of Ophthalmology, Timisoara. In both cases the clinical and paraclinical diagnosis was confirmed by postoperator anatomopathological examination.

[The clinico-statistical aspects in allergic conjunctivitis]. / Aspecte clinico-statistice în conjunctivitele alergice.

Ocular allergy represents the hypersensitivity reaction of the ocular surfaces in contact with the external environment. Allergic conjunctivitis are one of the frequent disorders of the clinical practice. In this paper we present statistically the cases attended in our service for one year, a few pathogenetical factors; the positive diagnose and the therapeutic strategy.

Oscillatory pulses in FitzHugh-Nagumo type systems with cross-diffusion.

We study FitzHugh-Nagumo type reaction-diffusion systems with linear cross-diffusion terms. Based on an analytical description using piecewise linear approximations of the reaction functions, we completely describe the occurrence and properties of wavy pulses, patterns of relevance in several biological contexts, in two prototypical systems. The pulse wave profiles arising in this treatment contain oscillatory tails similar to those in travelling fronts. We find a fundamental, intrinsic feature of pulse dynamics in cross-diffusive systems--the appearance of pulses in the bistable regime when two fixed points exist.