The spectrum of infantile myofibromatosis includes both non-penetrance and adult recurrence

Infantile myofibromatosis is characterized by benign myofibroblastic tumors within skin, muscle, bone or viscera which have a characteristic staining pattern on immunohistochemistry. The condition typically presents in infancy and the tumors often disappear by the third year of life. Mutations in the PDGFRB gene and NOTCH3 genes have been identified in familial forms of the condition. We present two families with molecularly confirmed germline mutations in the PDGFRB gene, one demonstrating a phenotype ranging from complete non-penetrance to neonatal lethality; and the other illustrating adult recurrence of the tumors.     

奥美拉唑治疗小儿消化道出血临床疗效分析

目的：探讨奥美拉唑治疗小儿消化道出血的临床疗效。方法随机选取我院2010年7月～2013年11月期间收治的消化道出血的小儿患者68例，将其随机分为实验组和对照组，其中对照组患儿采用常规方法治疗，实验组患儿采用奥美拉唑进行治疗，对两组患儿的治疗结果进行分析比较。结果实验组的临床治疗总有效率为94.1%，对照组患儿的临床治疗总有效率为58.8%，实验组患儿的临床疗效明显优于对照组患儿，差异具有统计学意义（P＜0.05）。结论采用奥美拉唑对小儿消化道出血进行治疗，能够有效改善患儿的出血状况，安全可靠，较传统方法患儿的不良反应发生率低，建议在临床上进一步推广。     

The incidence of infantile hypertrophic pyloric stenosis and its association with folic acid supplementation during pregnancy: A nested case–control study

Background and Rationale

Several studies have suggested that the incidence of infantile hypertrophic pyloric stenosis (IHPS) has decreased in recent decades. This decrement is controversial and not fully explained. Concurrently, there has been a major increase in folic acid consumption by pregnant women to prevent neural tube defects. We aimed to describe IHPS incidence in Israel in recent years and to assess its potential association with folic acid consumption.

三指捏脊法联合王不留行籽耳穴贴压治疗小儿厌食

小儿厌食病因很多,喂养不当、饮食无度等导致脾胃不和,受纳运化失职,最终诱发厌食。诊断应与现代医学结合,排除消化系统严重疾病。采用三指捏脊法联合王不留行籽耳穴贴压治疗,疗效满意,大大减少患儿服药打针之痛苦,易取得患儿配合,避免药物毒副作用。     

对比热毒宁与痰热清治疗小儿呼吸道感染的效果差异

目的 比较热毒宁与痰热清在治疗小儿呼吸道感染疾病上的疗效差异,为总结该疾病有效治疗方法提供参考.方法 收集80例小儿呼吸道感染患儿作为研究对象,随机分为研究组和对照组,各40例.研究组给予热毒宁治疗,对照组选用痰热清治疗方案,比较两组治疗效果及不良反应.结果研究组起效时间、症状恢复时间分别为(37.22±2.21)h、(76.42±10.25)h,均少于对照组的(45.13±1.54)h、(103.61±13.15)h,对比差异有统计学意义(P＜0.05).研究组总有效率为97.5％,与对照组的95.5％对比差异无统计学意义(P＞0.05).结论 热毒宁和痰热清均可作为治疗小儿呼吸道感染有效的药物选择,不过相比痰热清,热毒宁见效早,症状消退快,值得临床作为首选药物应用.     

Vascular Anomalies of the Head and Neck: A Pediatric Overview

Vascular anomalies, further classified into vascular tumors and malformations, often involve the head and neck region of children. These entities may raise diagnostic dilemmas, as they often demonstrate heterogenous and overlapping histologic features. The aim of this paper is to provide an overview of the common vascular anomalies in the head and neck region of children. Specific entities discussed include infantile hemangioma, congenital hemangioma, tufted angioma, kaposiform hemangioendothelioma, and various vascular malformations. Clinicopathologic features and associated molecular associations are reviewed.     

Infantile cortical hyperostosis of the mandible

Infantile cortical hypersotosis is a rare disease that affects children during the first six months of life, and is characterized by new periosteal bone formation. We report a case that affected the left mandible in a 3-month-old boy treated with indometacin.     

Cardiovascular drugs in the treatment of infantile hemangioma

Since the introduction of propranolol in the treatment of complicated infantile hemangiomas(IH) in 2008, other different beta-blockers, including timolol, acetabutolol, nadolol and atenolol, have been successfully used for the same purpose. Various hypotheses including vasoconstriction, inhibition of angiogenesis and the induction of apoptosis in proliferating endothelial cells have been advanced as the potential beta-blockerinduced effect on the accelerated IH involution, although the exact mechanism of action of beta-blockers remains unknown. This has generated an extraordinary interest in IH research and has led to the discovery of the role of the renin-angiotensin system(RAS) in the biology of IH, providing a plausible explanation for the beta-blocker induced effect on IH involution and the development of new potential indications for RAS drugs such as angiotensin-converting enzyme inhibitors and angiotensin-receptor blockers in the treatment of IH. This review is focused on the current use of cardiovascular drugs in the treatment of IH.     

Combined distal tibial rotational osteotomy and proximal growth plate modulation for treatment of infantile Blount’s disease

Infantile Blount’s disease is a condition that causes genu varum and internal tibial torsion. Treatment options include observation, orthotics, corrective osteotomy, elevation of the medial tibial plateau, resection of a physeal bar, lateral hemi-epiphysiodesis, and guided growth of the proximal tibial physis. Each of these treatment options has its disadvantages. Treating the coronal deformity alone (genu varum) will result in persistence of the internal tibial torsion (the axial deformity). In this report, we describe the combination of lateral growth modulation and distal tibial external rotation osteotomy to correct all the elements of the disease. This has not been described before for treatment of Blount’s disease. Both coronal and axial deformities were corrected in this patient. We propose this combination (rather than the lateral growth modulation alone) as the method of treatment for early stages of Blount’s disease as it corrects both elements of the disease and in the same time avoids the complications of proximal tibial osteotomy.     

Serial elongation-derotation-flexion casting for children with early-onset scoliosis

Various early-onset spinal deformities, particularly infantile and juvenile scoliosis (JS), still pose challenges to pediatric orthopedic surgeons. The ideal treatment of these deformities has yet to emerge, as both clinicians and surgeons still face multiple challenges including preservation of thoracic motion, spine and cage, and protection of cardiac and lung growth and function. Elongation-derotation-flexion (EDF) casting is a technique that uses a custom-made thoracolumbar cast based on a three-dimensional correction concept. EDF can control progression of the deformity and - in some cases-coax the initially-curved spine to grow straighter by acting simultaneously in the frontal, sagittal and coronal planes. Here we provide a comprehensive review of how infantile and JS can affect normal spine and thorax and how serial EDF casting can be used to manage these spinal deformities. A fresh review of the literature helps fully understand the principles of the serial EDF casting technique and the effectiveness of conservative treatment in patients with early-onset spinal deformities, particularly infantile and juvenile scolisois.