Sudden death in incomplete Kawasaki's disease.

A boy of 12 years died suddenly of myocardial infarction, which was due to coronary artery aneurysm typical of Kawasaki''s arteritis. Two years earlier he had an obscure illness, recognised retrospectively as incomplete Kawasaki''s disease. We recommend routine echocardiography even if only one or two features of the syndrome are present.     

A Rare Case of Left Lung Hypoplasia Associated With Congenital Pulmonary Artery Aneurysm and Ventricular Septal Defect

This report describes a 3-year-old boy who presented with hemoptysis and a history of congenital heart disease detected at the age of 3 months. Clinical and laboratory evaluation showed a large true congenital pulmonary artery aneurysm of the left main pulmonary artery occupying the entire left hemithorax and two small aneurysms in the right lung. In addition, a restricted ventricular septal defect was detected. It was evident on clinical and histologic grounds that the multiple aneurysms seen in this child stemmed from congenital weakness of the arterial wall in conjunction with increased pulmonary blood flow and elevated pulmonary artery pressure.     

Reliability of Oxygen Tension Measurements on Arterialized Capillary Blood in the Newborn

Samples of blood were obtained from newborn infants simultaneously from an umbilical artery catheter, an `arterialized'' heel, an unprepared heel, and from an `arterialized'' thumb. The correlation between the arterial oxygen tension with that of capillary blood, whether arterialized or not, was poor. Thus it was concluded that arterial blood is the only satisfactory sample for the measurement of oxygen tension in the newborn.     

Reduced shear stress and disturbed flow may lead to coronary aneurysm and thrombus formations

BACKGROUND: With Kawasaki disease it is important to clarify the mechanisms of coronary artery aneurysm and thrombus to avoid acute myocardial infarction. The authors tested the hypothesis that shear stress is reduced at coronary branching sites and in coronary artery aneurysms, and that this reduction of shear stress can promote formation of coronary artery aneurysms and thrombus. METHODS: The subjects were 111 children with Kawasaki disease with left coronary artery aneurysms, classified into three groups: giant coronary artery aneurysm (n= 28, diameter of coronary artery >8 mm), aneurysm (n= 44, diameter of coronary artery =8 mm), and normal-appearing coronary (n= 39). Averaged peak flow velocity (APV), flow patterns and shear stress were measured and calculated at normal-appearing coronary vessels, left coronary artery branching sites and intra-coronary aneurysm using flow wire, and coronary angiography. Also, presence and appearance of thrombus were detected by intravascular ultrasonography. RESULTS: The authors found that 90.3% of the coronary artery aneurysms occurred at major left coronary branching sites. APV and shear stress were significantly decreased in giant coronary artery aneurysms (APV, 7.1 +/- 2.1 cm/s; shear stress, 3.8 +/- 2.1 dyne/cm(2)) and at the left coronary artery branching site (APV, 9.1 +/- 1.2; shear stress, 1+/-+/-.2 3.0). In total, 20 of 24 thrombi were detected only in giant aneurysm, and all patients exhibited disturbed flow pattern in their giant coronary artery aneurysms. CONCLUSIONS: Reduced shear stress and disturbed flow pattern may lead to coronary artery aneurysm and thrombus formation.     

广州市儿童医院2001至2004年川崎病患儿冠状动脉损害的特点及随访

目的 探讨广州市儿童医院2001至2004年川崎病（KD）患儿冠状动脉损害的发生及恢复期变化特点。方法 采用彩色多普勒超声心动图检查广州市儿童医院收治的KD患儿，对伴冠状动脉损害者进行随访，KD 患儿恢复期接受三磷腺苷负荷超声心动图检查。按急性期冠状动脉扩张的程度，将KD患儿分为冠状动脉扩张组、中小型冠状动脉瘤组和巨大型冠状动脉瘤组，分别比较其发病及变化特点。结果 共纳入KD患儿356例，冠状动脉损害发生率为41.3％（147/356）；巨大型和中小型冠状动脉瘤组确诊前发热时间明显长于冠状动脉扩张组［(18.5±12.0) d、(12.9±8.2) d vs (7.4±3.6) d， P均<0.01］］。有119例（81.0％，119/147）冠状动脉损害患儿定期门诊随访。随访2年，超声心动图显示冠状动脉扩张组72例患儿冠状动脉全部恢复正常；中小型冠状动脉瘤组77.8%（28/36）患儿冠状动脉恢复正常；巨大型冠状动脉瘤组11例患儿无一例恢复正常。40例冠状动脉有损害的KD患儿恢复期三磷腺苷负荷超声心动图检查结果显示中小型和巨大型冠状动脉瘤组室壁运动异常、冠状动脉血流储备下降的发生率显著高于冠状动脉扩张组（P< 0.05）。结论 KD患儿急性期冠状动脉扩张损害为一过性；大部分中小型冠状动脉瘤改变可恢复；巨大型冠状动脉瘤造成的损害可长期存在。超声心动图和三磷腺苷负荷超声心动图对追踪观察KD冠状动脉损害的变化及心肌供血有重要帮助。     

Common iliac artery aneurysm due to fibromuscular dysplasia in infants.

A seven-month old female with a huge aneurysm of the right common iliac artery and multiple stenoses of her bilateral renal arteries is described. At surgery, a Gore-Tex graft was interposed between the proximal common iliac artery and the external iliac artery. A histological examination of the aneurysmal wall was compatible with medial fibromuscular dysplasia (FMD). After surgery, her blood pressure was controlled in the normal range on medical treatment. This case would be the first case of a huge common iliac artery aneurysm due to FMD in infants.     

氯吡格雷联合阿司匹林对川崎病并发中小型冠状动脉瘤患儿抗血栓治疗的疗效及安全性研究

目的 探讨川崎病（KD）合并冠状动脉瘤（CAA）患儿使用氯吡格雷联合阿司匹林抗血栓治疗的疗效及安全性。方法 选取2013年1月至2018年6月经超声心动图诊断为合并多发中小型CAA的KD患儿77例为研究对象,随机分为观察组（n=38）和对照组（n=39）,分别接受氯吡格雷+阿司匹林和低分子肝素+阿司匹林治疗。所有患儿定期随访,以病程前3个月为观察期,总结两组患儿的冠状动脉变化及其他并发症发生情况。结果 随访至3个月时,观察组6例冠状动脉恢复正常,11例冠状动脉较前回缩,19例冠状动脉稳定,2例发展为巨大冠状动脉瘤;对照组7例冠状动脉恢复正常,12例冠状动脉较前回缩,19例冠状动脉稳定,1例发展为巨大冠状动脉瘤;两组冠状动脉变化情况比较差异无统计学意义（P > 0.05）。观察组合并鼻衄2例,皮肤瘀斑6例;对照组合并鼻衄1例,出现注射部位瘀点瘀斑7例;两组均未出现其他严重出血事件。结论 氯吡格雷联合小剂量阿司匹林对KD合并CAA患儿的抗血栓治疗安全有效。     

川崎病治疗前发热时间与丙种球蛋白耐药的相关性临床研究北大核心CSCD

目的 探讨川崎病(Kawasaki disease,KD)患儿丙种球蛋白(intravenous immunoglobulin,IVIG)治疗前发热时间与IVIG耐药的关系。方法 回顾性收集2018年1月至2020年12月收治KD患儿317例的病例资料,根据IVIG治疗前发热时间分为短热程组(发热时间≤4 d,n=92)和长热程组(发热时间>4 d,n=225),根据是否发生IVIG耐药将每组再分为耐药组和非耐药组。分析比较不同热程耐药组及非耐药组的基线资料及实验室结果,并采用多因素logistic回归分析IVIG耐药的影响因素。结果 短热程组中IVIG耐药19例(20.7%),并发冠状动脉瘤5例(5.4%);长热程组中IVIG耐药22例(9.8%),并发冠状动脉瘤19例(8.4%);短热程组IVIG耐药率明显高于长热程组(P<0.05),而冠状动脉瘤发生率在两组间差异无统计学意义(P>0.05)。短热程组中,耐药患儿治疗前血钠水平低于非耐药患儿,而降钙素原、C反应蛋白及N末端B型利钠肽原水平则明显高于非耐药患儿(P<0.05)。长热程组中,耐药患儿治疗前血钠及肌酸激酶水平低于非耐药患儿(P<0.05)。多因素logistic回归分析显示,血钠水平降低与长热程组KD患儿IVIG耐药有关(P<0.05)。结论 KD患儿IVIG耐药因IVIG治疗前发热时间不同而异。在治疗前发热时间>4 d的KD患儿中,血钠降低与IVIG耐药具有相关性。     

Giant cerebral arterial aneurysm in an infant: Report of a case and review of 42 previous cases in infants with cerebral arterial aneurysm

A giant cerebral aneurysm with subarachnoidal hemorrhage in a 4-month-old female infant is reported. The clinical course was complicated by an electrolyte imbalance and unexpected intoxication by phenobarbital. She died as a result of the third episode of rerupture of the aneurysm on the 16th day after onset. Histological findings showed that the aneurysm was of the saccular type, 35 × 20 × 22 mm in size, arising from the basilar artery. The aneurysmal wall consisted microscopically of fibrous tissue with partial absence or fragmentation of the internal elastic lamina and muscular lamina. Forty-three cases of cerebral aneurysms during the first year of life including the present case were summarized and it was found that they could be characterized as follows: (i) a high incidence of onset in early infancy; (ii) larger than 10 mm; (iii) a large number arose from the posterior circulation of the circle of Willis; and (iv) a poor prognosis of non-operated groups, especially in the posterior circulation.     

川崎病患儿冠状动脉血流储备测定与影像形态学诊断的对照研究

目的评价持续冠状动脉病变对川崎病（KD）患儿冠状动脉血液动力学的影响, 探讨冠状动脉血流储备（CFR）检测在KD患儿冠状动脉病变远期随访中的应用价值。方法选择因胸痛、心悸以及各类偶发早搏的患儿为对照组，KD并发持续左冠状动脉病变患儿为KD组，均予以ATP 160 μg· kg-1·min-1持续静脉注射，共6 min。运用经胸多普勒超声心动图（TTDE）检测冠状动脉左前降支(LAD) 在ATP注射前后的舒张期峰值流速(PDV)及平均流速(MDV)，并通过计算比值得出相应CFR（PDV）和CFR（MDV）值。结果 对照组纳入25例，CFR（PDV）和CFR（MDV）值均与年龄呈显著正相关(r=0.596, 0.591, P=0.002, 0.002)；<2岁、～8岁和>8岁组的CFR (PDV) 95%参考值范围分别为≥1.22、≥1.94和≥2.22。KD组纳入8例，静息状态下PDV和MDV值分别为（50.0±15.07） cm· s-1和(35.16±11.52) cm· s-1，显著高于对照组(P=0.004, 0.015)；而CFR（PDV）和CFR（MDV）值明显降低，分别为（1.67±0.38） cm· s-1和（1.69±0.47） cm· s-1(P=0.002,0.018)。与对照组比较，KD组7/8例(87.5%)患儿CFR（PDV）值显著降低。结论持续冠状动脉病变可导致KD患儿CFR值显著降低，TTDE检测患儿CFR具无创、安全和便捷的优点，对于KD冠状动脉病变的长期随访与临床诊断治疗策略的选择具有指导意义。     

Lumbar and iliac artery aneurysms in Menkes’ disease: endovascular cover stent treatment of the lumbar artery aneurysm

We report lumbar and iliac artery aneurysms in a 3-month-old boy with Menkes disease. The iliac artery aneurysm thrombosed spontaneously, documented by follow-up colour Doppler sonography. The lumbar artery aneurysm was successfully treated using a cover stent. There was no filling of the lumbar artery aneurysm and no stenosis of the cover stent during the 9-month follow-up.     

Electrocardiographic depolarization and repolarization: long-term after Kawasaki disease

To assess myocardial electric potentials late after Kawasaki disease (KD) we measured signal-averaged electrocardiography (SAECG) and QT dispersion parameters. Thirteen patients with persistent coronary aneurysm (group I), 12 with late resolution of the aneurysm (>3 months) (group II), and 13 with early resolution (group III) were studied 7.9 ± 3.9, 6.7 ± 3.9, and 7.2 ± 3.6 years after the initial diagnosis (p = NS). In group I, myocardial infarction occurred in one patient during the acute illness, and coronary thrombosis in another; all except two patients had giant aneurysm (n = 8) and/or stenosis (n = 7). At 40-Hz high-pass filter SAECG, terminal 40-msec root mean square amplitude (RMS40) was significantly lower in group I versus II and III (64.1 ± 40.8 mV, 79.9 ± 47.2 mV, and 115 ± 65.4 mV, respectively; p <0.05). Global QT dispersion was significantly greater in group I versus III (52 ± 11 msec and 37 ± 11 msec, respectively; p <0.05), but not in comparison to group II (45 ± 13 msec). The same trend was present for rate-corrected QT dispersion, without reaching statistical significance (84.0 ± 34, 71.5 ± 31, and 61.8 ± 21 respectively). Both depolarization and repolarization parameters are altered in patients with persistent coronary artery aneurysms long-term after KD. This may represent risk factors for developing ventricular arrhythmia in a growing population.     

Splenic artery aneurysm: a diagnostic challenge in the setting of extensive portal venous collaterals

We present a 16-year-old boy with autoimmune liver disease and longstanding portal hypertension in whom a CT arteriogram demonstrated a large aneurysm arising from the distal, extra-parenchymal portion of the splenic artery. Because of its location adjacent to multiple venous collaterals, the aneurysm was indistinguishable from splenic varices on initial imaging with Doppler sonography and on portal venous-phase CT. There is an increased risk of rupture of splenic artery aneurysms in the post-liver transplant period, with high associated mortality, and therefore diagnosis of splenic artery aneurysm prior to liver transplantation is clinically important. It is quite possible that the diagnosis of splenic artery aneurysm in this case would have been missed in the absence of dedicated arterial-phase imaging. As radiologists strive to reduce radiation exposure in children, this case highlights a potential diagnostic pitfall of both Doppler sonography and venous or single-acquisition arterial/venous-phase CT angiogram in children with venous collaterals and an undiagnosed splenic artery aneurysm.     

Origin of intraventricular haemorrhage in the preterm infant.

A technique has been developed for the injection and stereomicroscopic examination of blood vessels in the preterm newborn brain. Using this technique it can be seen that in the immature brain there is a rich capillary bed in the germinal layer region supplied mainly by Heubner''s artery. Capillary channels drain directly into the terminal vein and its main branches. Study of 19 cases with spontaneous germinal layer haemorrhage (GLH) with or without intraventricular haemorrhage (IVH) failed to show rupture of the terminal vein or germinal layer infarction. In babies of up to 28 weeks'' gestation GLH developed most frequently over the body of the caudate nucleus, whereas in babies of 29 weeks'' gestation or more the haemorrhages were usually over the head of the caudate nucleus. Histological study of 10 cases of GLH failed to show rupture either of arteries or veins, though evidence of rupture at a capillary-vein junction was seen in one case and masses of fibrin adjacent to the vein wall in 2 others. Injection through the carotid artery caused prominent leaks of injection mass within the germinal layer capillary bed, often adjacent to the veins. Injection through the jugular veins in 2 cases failed to rupture the terminal vein but caused multiple vein ruptures at the junction of deep and cortical venous systems. Additional small ruptures in the germinal layer occurred in one of the cases only. It is suggested that the capillaries within the germinal layer may be ruptured by a rise in arterial pressure, particularly in conditions of hypercapnia and hypoxia.     

Aneurysm of the superficial femoral artery in an infant

An isolated arterial aneurysm in childhood is extremely rare. We report a 1-year-old girl with an aneurysm of the right superficial femoral artery, presenting as an asymptomatic mass of the thigh. The aneurysm involved the whole superficial femoral artery (9 cm in length), and surgical treatment would have required replacement of the affected artery. Conservative treatment was chosen, influenced by the patient's rapid growth at that time. Non-invasive, 3-D contrast-enhanced magnetic resonance angiography (MRA) was useful as an alternative to conventional angiography for detailed evaluation of the femoral arteries, including the aneurysm.     

Tumor bleeding from a de novo aneurysm associated with optic glioma

Aneurysms in children are rare and potentially devastating lesions. The authors report the case of a 16-year-old girl with a complicated medical history related to a chiasmal glioma diagnosed at 18 months of age. She had previously received multiple modalities of radiation treatment, including external beam, proton therapy, and Gamma Knife. She presented with hemorrhage centered in the tumor and extending into the ventricular space. There was no subarachnoid blood. Magnetic resonance imaging demonstrated the hemorrhage and tumor anatomy. Magnetic resonance angiography revealed an aneurysm at the internal carotid artery bifurcation, but the lesion was more clearly delineated on CT angiography. A comparison MR imaging study obtained 6 months earlier, even in retrospect, did not show evidence of an aneurysm. This case illustrates the salient point that the clinician must search for vascular lesions in the patient with spontaneous "tumor bleeding," especially if that patient has risk factors for aneurysm formation. The authors also suggest that a CT angiogram is better at radiographically demonstrating an intratumoral aneurysm than an MR angiogram in this scenario.     

Effect of antenatal administration of thyrotrophin releasing hormone on fetal flow velocity waveforms

AIM—To determine whether antenatal administration of thyrotrophin releasing hormone (TRH), to promote lung maturation, alters blood flow through the fetal middle cerebral, umbilical artery, or ductus arteriosus and through the maternal uterine arteries.METHODS—The effect of transplacentally administered TRH on the fetal circulation was prospectively evaluated in 30 patients between 24 and 34 weeks'' gestation. TRH (400 µg) was given to the mother intravenously either as a bolus or an infusion. Fetal effects were determined by measuring the maximum velocity and pulsatility index (PI) in middle cerebral artery, ductus arteriosus, uterine artery and umbilical artery Doppler waveforms. Measurements were made immediately before, and 10 and 60 minutes after maternal TRH administration.RESULTS—Intravenous injection of TRH had no significant effect on PI in the uterine, umbilical, or middle cerebral artery and the ductus arteriosus within 60 minutes of administration in either group.CONCLUSION—The antenatal use of TRH in conjunction with steroids for fetal lung maturity does not affect utero-placental or fetal haemodynamic variables, as measured by Doppler. These findings, therefore, do not support the suggestion that antenatal intravenous administration of TRH either as bolus or infusion may have immediate adverse vascular effects in the fetus.     

Percutaneous treatment of a ruptured superior mesenteric artery aneurysm in a child

Splanchnic artery aneurysms are very rare in children. We report a 10-year-old girl with a large atraumatic ruptured superior mesenteric artery aneurysm that was considered inoperable. She was ultimately treated with two percutaneous US-guided thrombin injections, which led to complete occlusion of the aneurysm. The aetiology of the aneurysm remained unclear, but a family history was suggestive of a congenital connective tissue disease such as Ehlers-Danlos syndrome subtype IV.     

The pediatric stroke: A prospective study of cerebral angiography

Cerebral angiography was performed in 34 children having sudden hemiplegia of obscure orgin. Arterial occlusions were detected in 61.7%. Occlusions were seen more often in cases where angiography was done early in the course of illness. The involvement of internal carotid artery with middle cerebral artery and/or anterior cerebral artery was most common. Arteritis was the suggested etiology of occlusion.     

Angioplasty for recurrent renal artery stenosis in flare‐up of Hashimoto's thyroiditis

Takayasu arteritis is the most common disease seen in children presenting with renovascular hypertension (RVH) in Asia, and can manifest anatomically as renal artery stenosis (RAS). We report the case of a 16‐year‐old girl presenting with RVH due to recurrent and novel RAS in Hashimoto's thyroiditis (HT) flare‐up. After treatment with thyroxin and percutaneous transluminal renal angioplasty (PTRA), she was free of hypothyroidism and systemic hypertension. RVH due to recurrent and novel RAS in HT flare‐up has not previously been reported in the English‐language literature. PTRA is the procedure of choice, providing there is no renal artery dissection or aneurysm.