An unusual case of double anterior descending artery originating from the left and right coronary arteries

Double left anterior descending coronary artery originating from the left and right coronaries is an extremely rare coronary artery anomaly. In the present report, an unusual case with double left anterior descending coronary artery arising from the left and right coronary arteries is described. To our knowledge, only a few such cases have been published in the literature so far.     

Individual variation in organ histogenesis as a causative factor in the developmental origins of health and disease: Unnoticed congenital anomalies?

Morphological studies of congenital anomalies have mainly focused on abnormal shape (i.e. malformation) and thus on disturbed organogenesis. However, in regard to postnatal functions of organs that develop through branching mechanisms, organ size is another important morphological feature. These organs consist of a large number of structural and functional units, such as nephrons in the kidney, and the total number of these units, that is approximately proportional to the organ size, has been shown to vary widely among individuals. Organ‐specific cells are differentiated and organized to form structural units and realize organ‐specific functions during the histogenetic period (i.e. from mid‐gestation to the early postnatal period). The total number of units is attained at the end of histogenesis and determines the total functional capacity, including the functional reserve of the organ, and thus may be related to predispositions to postnatal organ‐based diseases, because the functional reserve decreases during the course of life and eventually become short of the minimum requirement of each organ. Therefore, it may be hypothesized that a smaller number of units of organs at the end of histogenesis is one of the predisposing factors for postnatal diseases (i.e. a form of unnoticed but late‐manifested congenital anomalies), in this era of extended longevity. However, the mechanisms that control the total number of units in each organ during histogenesis and the possible relationship among the numbers of units in different organs remain unknown. Here, we review our trials based on the above hypothesis in order to (1) mathematically analyze the morphometric data of the different organs in fetuses to elucidate relationship among developing organs, (2) analyze the developing neuro‐immuno‐endocrine network as a series of mechanisms to systemically correlate the histogenesis of multiple organs, and (3) examine the maternal environment, including dietary fat, as a factor to influence histogenesis and thus the predisposition to type 1 diabetes.     

Cardiac ischemia in pediatric patients

Cardiac ischemia in children is usually not an isolated disease in an otherwise normally formed coronary artery but is part of more complex congenital or acquired diseases. Although cardiac ischemia is not a frequent occurrence, it must be recognized as a serious, life-threatening event. This article lists and characterizes major causes of cardiac ischemia in children, describes signs and symptoms of each, and provides therapeutic considerations.     

Mesonephric remnant with tubular function in a 15-year-old female

Congenital abnormalities of the genital or urinary tract are not uncommon and often occur together. This article discusses a unique case in which a functioning mesonephric remnant was found.     

Urinary control after the definitive reconstruction of cloacal anomaly

PURPOSE: Urinary control after definitive repair of a cloacal anomaly is difficult to achieve. The present report aims to describe the clinical course of urinary control, and the need for the management of bladder dysfunction after reconstruction. METHODS: The present consecutive series consisted of 11 girls who underwent definitive repair of cloacal anomalies over a period of 11 years. Eight patients were associated with hydrocolpos. Radiological examination included a plain X-ray radiograph of the lumbosacral spine and a voiding cystourethrography with or without a urodynamic study. RESULTS: Reconstruction of the cloaca was performed on patients aged between 1 and 3 years using a posterior sagittal approach. Vaginal reconstruction was carried out 13 times in 11 patients using tubularized vaginal flap, distal rectal segment, perineal skin flap, or total urogenital sinus mobilization. Cystostomy or vesicostomy was carried out in four newborns/infants. Another seven patients could void spontaneously but incompletely with residual urine. Occult spinal dysraphism was found in five patients and hemisacrum in two patients. After definitive reconstruction, most patients acquired an adequate to normal bladder volume for 1-year-olds. Normal detrusor-sphincter function was seen in three patients. Detrusor areflexia was seen in two patients who underwent in utero vesico-amniotic shunt. Detrusor underactivity was observed in six patients. Bladder compliance was good in all patients except for one. No patients in the present series showed persistent urinary incontinence from the bladder neck or urethral dysfunction. CONCLUSION: It is postulated that wetting after definitive repair may be the result of overflow incontinence and poor bladder contractility rather than sphincter injury. The main clinical characteristic of bladder dysfunction was a failure to empty. We could not define the exact etiology, but iatrogenic injury from extensive dissection can lead to the higher risks of peripheral nerve damage. Accomplishment of definitive repair involves not only anatomical reconstruction, but also postoperative urinary control, including the initiation of clean intermittent catheterizations under repeated urodynamic evaluations.     

164例法洛四联症的外科治疗

目的总结164例法洛四联症(tetralogyofFallot,TOF)患者手术治疗的经验,探讨手术时机、危险因素和围术期处理等。方法对164例TOF患者行根治手术,其中单纯右心室流出道漏斗部狭窄37例,漏斗部及肺动脉瓣狭窄14例,主肺动脉及左右肺动脉狭窄113例。合并肺动脉闭锁5例。分别给予自体心包片或自体心包卷成心外管道加宽右心室流出道、同种带瓣血管行右心室-肺动脉连接等处理;合并的心脏畸形作相应的矫正。结果全组手术死亡6例,手术死亡率为3.66%(6/164)。死亡原因严重低心排血量综合征2例,冠状动脉移植后不能脱离体外循环机1例,发生室性心律失常1例,术后发生急性呼吸窘迫综合征2例。术后发生心肺并发症55例,均经治疗后恢复。结论行TOF根治术患者年龄应减小,低心排血量综合征已不是导致术后并发症和死亡的主要原因,术中、术后处理的重点应为预防肺动脉残余梗阻和肺部并发症。     

Klinefelter综合征患者卵细胞胞质内单精子注射治疗和子代患染色体病的风险

Klinefelter综合征患者以47,XXY核型,睾丸精曲小管发育不良,无精子症和不育为特征。但极少数患者仍有 局部生精灶和少量精子发生。近年来由于辅助生育技术的发展,Klinefelter综合征患者经睾丸活检取精子和卵细胞 胞质内单精子注射(ICSI)治疗后已有30多个健康胎儿出生,但也有1例报告妊娠胚胎的核型为47,XXY而选择流 产。本文对近年来Klinefelter综合征患者的ICSI治疗的现状及子代患染色体病风险进行综述。     

二维及彩色多普勒超声心动图对Ebstein畸形的诊断

应用二维彩色多普勒超声心动图对１２例Ｅｂｓｔｅｉｎ畸形患者作出诊断，均经手术证实，本组患者的三尖瓣隔叶与后叶均有不同程度下移，达瓣环水平以下≥２ｃｍ。提出二维右室流出道切面对三尖瓣后叶的观测具有重要价值，与心尖四腔心切面相结合可以全面地评价三尖瓣每个瓣叶与瓣环的结构形态。加之，彩色多普勒提供血流动力学的依据，可对该病作出诊断，且准确，安全，可靠。