Recurrence of a primary xanthoma of the humerus in a 9-year-old normolipidemic child

Xanthomas are rare bone tumors that occur more often in patients with hyperlipidemia or metabolic disorders. We report the case of a 9-year-old child presenting with right shoulder pain after a trauma. A benign osteolytic lesion was found in the proximal humeral metaphysis. Curettage of the lesion was performed. Histologic findings confirmed intraosseous xanthoma. Although there was no evidence of dyslipidemia or metabolic disease, the patient suffered local recurrence at the 8-month follow-up, requiring a second surgery with bone grafting. This difficult and rare diagnosis must be kept in mind with osteolytic lesions.     

Pubertal growth spurt in patients with bilateral anorchia after testosterone replacement therapy

Anorchia, the absence of testes in 46,XY boys, is a very rare condition. It has been suggested that the testicular tissue disappears during pregnancy, as a result of a vascular accident associated with torsion or a genetic cause. Because pubertal growth spurt is directly influenced by androgen exposure, we decided to evaluate the pubertal height gain in nine patients with anorchia who were followed up at the pediatric endocrinology unit of Bicêtre University Hospital. We retrospectively included nine patients with bilateral anorchia whose puberty had been induced by androgen replacement therapy and for whom final height measurements were available. Data were obtained from medical records. Mean gain in pubertal height was 21.7 ± 2.3 cm, lower than the expected gain during puberty (25 cm, P < 0.005). Despite limited experience in this rare condition, androgen replacement therapy seems to allow for good pubertal growth spurt in adolescents with anorchia. However, formal protocols for androgen therapy during puberty may need to be optimized.     

An unusual domestic accident: a penetrating abdominal wound with intestinal evisceration in a child

We report a penetrating abdominal injury due to a piece of glass, a very unusual domestic accident, in a 2-year-old boy who was admitted to the emergency department with an evisceration through the umbilicus. His mother reported that she was asleep when the boy came to her room, and did not know how it had happened. We primarily suspected child abuse, but the presence of a piece of glass in the boy's heel led us to think of a domestic accident. He had poured himself a glass of water, and then fell on the glass. We could not find a similar case in the literature on domestic accidents. Accepted: 25 June 1997     

Sensitivity,specificity, and accuracy of fetal echocardiography for high-risk pregnancies in a tertiary center in Egypt

BackgroundAdvances in fetal echocardiography training among pediatric cardiologists have led to substantial improvements in prenatal detection of congenital heart diseases (CHDs). Nevertheless, diagnostic accuracy varies among centers. Moreover, this subspecialty continues to evolve in developing countries, with limited studies assessing fetal echocardiography sensitivity, specificity, and accuracy in developing countries such as Egypt.Subject and methodsHigh-risk pregnancies referred for fetal echocardiography from January 2011 to January 2019 were analyzed retrospectively. All of the cases included had one of the high-risk indications for fetal echocardiography. Maternal age and gestational age at the prenatal diagnosis were determined, and detailed fetal and neonatal echocardiograms were documented. The results of fetal and postnatal echocardiography were compared to assess the sensitivity, specificity, and accuracy of fetal echocardiography. A minor lesion was defined when no postnatal intervention was needed and a major anomaly when postnatal cardiac therapy, surgery, or intervention was required.ResultsOut of 615 pregnant patients referred, comparisons between fetal and neonatal echocardiography were possible in 458 fetuses, with 157 patients excluded from the study. The mean maternal age in the study was 26.97 ± 5.871 years and the mean gestational age at referral was 27.24 ± 5.407 weeks. The most common indication for prenatal echocardiography in this cohort was a family history of CHD (142; 31%) followed by nonimmune hydrops (97; 21.18%) and abnormal obstetrical ultrasound screening (64; 13.97%). We had three false-positive minor diagnoses and four false-negative cases with only one requiring intervention. Prenatal diagnosis in this study was accurate in 98.47% of cases. The sensitivity, specificity, and accuracy of fetal echocardiography in the current work were 97.03%, 99.07%, and 98.47, respectively.ConclusionFetal echocardiography is considered a highly sensitive specific tool for prenatal detection of congenital heart diseases in high-risk pregnancies even in developing countries. However, minor fetal cardiac disorders are challenging to diagnose and family counseling should emphasize the difficulty of excluding or confirming such lesions.     

维生素D和钙剂治疗营养性佝偻病疗效的Meta分析

目的：采用Meta分析对联合补充钙剂、维生素D与单用维生素D或钙剂治疗佝偻病疗效进行定量评价。方法检索Cochrance Library、Pubmed、Web of science、Scirus、中国期刊全文数据库、中国生物医学文献数据库和万方数据库,检索时间均从建库至2013年3月。获得维生素D和钙剂治疗营养性佝偻病相关的RCT文献。选取血清25-( OH)维生素-D3、磷、ALP作为主要测量指标,选取钙、甲状旁腺素、白蛋白、影像评分作为次要测量指标。采用Review Manage 5．0．2对纳入的RCT文献进行Meta分析。结果共检索到437篇文献,对符合纳入标准的3篇文献(n=218)进入Meta分析。 Meta分析结果显示：(1)血清25-(OH)维生素 D3上升程度：联合治疗组与维生素 D 组 MD =-7．88(95%CI：-12．24~-3．52);联合治疗组与钙剂组MD=-18．32(95%CI：-22．61~-14．04);(2)血清磷上升程度：联合治疗组与维生素D组MD=-0．64(95%CI：-0．86~-0．42);联合治疗组与钙剂组MD=-0．16(95%CI：-0．84~0．51);(3)血清 ALP 下降程度：联合治疗组与维生素 D 组 MD =109．99(95%CI：20．40~199．58);联合治疗组与钙剂组MD=59．89(95%CI：10．09~109．59)。(4)血清钙上升程度：联合治疗组与维生素D组MD=-0．71(95%CI：-0．91~-0．52);影像学评分下降程度：联合治疗组与维生素D组MD=0．68(95%CI：0．42~0．95)。以上指标除联合治疗组与钙剂组对血清磷的上升程度差异无统计学意义外,其余差异均具有统计学意义。结论维生素D联合钙剂长期联合治疗佝偻病的疗效要优于单用维生素D或钙剂。     

Report of severe accidental baclofen intoxication in a healthy 4-year-old boy and review of the literature

The number of reports on baclofen intoxication has increased in recent years. Here we report the case of a 4-year-old boy in deep coma who was referred to the pediatric intensive care unit. The patient was intubated and mechanically ventilated. A computerized tomography scan without contrast showed a collapsed appearance of the ventricular system suggesting diffuse cerebral edema. A multichannel electroencephalogram registered 6 h after admission showed a very slow and ample continuous pattern, without structure, nonreactive to stimulations, expressing diffuse and severe nonspecific cerebral pain. A targeted analysis to determine the baclofen plasma levels was performed. Test results of plasma samples were highly positive for baclofen (2009 ng/mL). Following 36 h of mechanical ventilation, the patient rapidly regained consciousness and recovered normal neurological behavior. The present case demonstrates the importance of considering baclofen overdosage in cases of deep coma with areflexia, and emphasizes the importance of warning parents about the potential toxicity of baclofen when prescribing the drug to a family member. A review of the literature on pediatric baclofen overdose is included.     

Scar wars: implications of fetal wound healing for the pediatric burn patient

Scar formation and fibrosis often cause devastating disabilities in children suffering severe burn injury. In contrast to the child, the fetus has the ability to heal skin injury without scar formation, and instead with regeneration of epithelial and mesenchymal tissues and restoration of normal skin architecture. In this paper we review those unique features of the fetus and fetal wound healing that may contribute to the scarless repair process. It is hoped that an understanding of these remarkable reparative capabilities may lead to the development of new wound healing therapies that reduce or prevent scar formation and fibrosis in the management of children with burns.     

Neonatal end-of-life decision-making almost 20 years after the EURONIC study: A French survey

Nearly 20 years ago the EURONIC study reported that French neonatologists sometimes deemed it legitimate to terminate the lives of newborn infants when the prognosis appeared extremely poor. Parents were not always informed of these decisions. Major change has occurred since then and is described herein.Material and methodsA survey was conducted in the Île-de-France region, from 1 January to 31 January 2016. Professionals from 15 neonatal intensive care units (NICUs) were invited to complete a questionnaire.ResultsA total of 702 questionnaires were collected and 670 responses were analyzed. Knowledge of the law differed according to professional status, with 71% of MDs (medical staff, MS), compared with 28% of nonmedical staff (NMS) declaring that they had good knowledge of the law. Most MDs and NMS believed that withholding or withdrawing life-sustaining treatments (WWLST) could be decided and implemented after a delay. Half of them thought that WWLST would always result in death. Although required by law, a consulting MD attended the collegial meeting required before deciding on WWLST in only half of the cases. Parents were almost always informed of the decision thereafter by the physician in charge of their infant. The most frequent disagreement with parents was observed when WWLST was the option selected. In this case, most professionals suggested postponing WWLST, continuing intensive care and dialogue with parents, aiming at a final shared decision. Major differences were observed between NICUs with regard to the withdrawal of artificial nutrition and hydration. Finally, 14% of MDs declared that infant active terminations of life still occurred in their NICU. Major differences concern WWLST and active termination of life, whose meaning has been partly modified since 2001.ConclusionSeveral major changes were observed in this survey: (1) treatment withdrawal decisions are made today in agreement with the law; (2) parents’ information and involvement in the decision process have profoundly changed; (3) active termination of life (euthanasia) very rarely occurs; only at the end of a process in accordance with ethical principles and within the law is this decision made.     

Delineating mechanisms of change in child and adolescent therapy: methodological issues and research recommendations

BACKGROUND: Mechanisms of therapeutic change are rarely studied in child and adolescent therapy. Our central thesis is that the study of mechanisms of treatment is an excellent investment for improving clinical practice and patient care. Indeed, extending treatment trials to clinical settings, without complementary research that studies why and how treatment works, could have great limitations. METHOD: In this article, we discuss the importance of studying mechanisms, the logical and methodological requirements, and why almost no studies to date provide evidence for why or how treatment works. Standard statistical practices (tests of mediation) and designs (randomized controlled clinical trials) contribute greatly to outcome research but have little to say about mechanisms given the way they are commonly used. CONCLUSIONS: The article ends with recommendations to guide research on mechanisms of therapeutic change.     

Effect of calcium therapy in the sick premature infant with early neonatal hypocalcemia

Twenty-seven sick premature infants with serum calcium concentrations less than 6.0 mg/dl during the first day of age were enrolled in a prospective controlled study involving two treatment regimens--calcium given as a bolus or a drip--or no treatment. Mean total calcium concentration was 5.5 +/- 0.8 mg/dl, and ionized calcium was 3.1 +/- .3 mg/dl, with no significant difference between treatment groups. By 24 hours, in all groups total calcium had increased to greater than 6.0 mg/dl (bolus 6.5 +/- 1.1, drip 7.0 +/- 0.4, control 6.6 +/- 0.4) and ionized calcium to greater than 3.5 mg/dl (bolus 3.9 +/- 0.3, drip 3.6 +/- 0.6, control 3.6 +/- 0.3). Ionized and total calcium concentrations were significantly correlated (r = 0.562; P less than 0.001), but total calcium did not predict ionized calcium in any group. These data support the concept that, even in sick infants, early neonatal hypocalcemia is a physiologic phenomenon that may not require treatment.     

Social representations of mandatory vaccination in patients and general practitioners in Moselle,France

Vaccine controversies affect the image of vaccination. At the end of 2014, there was a shortage of tetra- and pentavalent vaccines, although immunization obligation was being discussed. Our objective in this context was to study the social representations of mandatory vaccination in general practitioners and patients. Thirty general practitioners and 30 patients were recruited for this study based on hierarchal evocation. Each provided seven associations, which were revealed spontaneously in response to the evocation of the inductive concept: “mandatory vaccination”. An order of importance and a connotation were attributed to them. These associations were classified into subthemes and themes. For each, the frequency of occurrence, the median rank of importance, and the polarity index were calculated and then compared. The image of vaccination was positive (polarity index from doctors and patients: +0.22 and +0.24, respectively). The doctors mentioned first the organization of the vaccine follow-up (21.9%). For patients, the benefits of vaccination predominated (22.38%), although these benefits were considered more important by the doctors. Doctors raised vaccine controversies more frequently than patients and were more concerned with the practical aspects of vaccination (injection, pain). The current controversies have not eroded the representations of mandatory vaccination. However, doctors are critical towards the media and vaccination policy. Patients, on the other hand, are afraid of side effects and do not have complete confidence in vaccination. Immunization obligation does not appear to be challenged by either of the two populations. To sustain immunization, our main asset remains communication and information to patients.