Echocardiographic evaluation of the aortic root and mitral valve in children and adolescents with isolated pectus excavatum: Comparison with Marfan patients

Summary Pectus excavatum, mitral valve prolapse (MVP), and dilated aortic root occur frequently in patients with Marfan's syndrome (MS). Patients with isolated pectus excavatum (IPE) have a high prevalence of MVP, but it is not known whether aortic root dilatation is a risk in those patients. To test the hypothesis that IPE and MS represent a spectrum of connective tissue dystrophy with MV and aortic root involvement, two-dimensional (2D) echocardiography was used to measure the aortic root diameter and assess for MVP in IPE (n=31), MS (n=14), and normal (n=16) gender- and age-matched patients. Aortic root was measured in parasternal long- and short-axis views, just above the aortic sinuses, at end systole, in six cardiac cycles, and averaged. Parasternal long-axis view was used to assess for MVP.Aortic root diameter in IPE patients was not different from that in normal subjects, 24±4 mm vs 22±4 mm (p=NS), respectively, both were significantly smaller than that in MS patients (30±5 mm;p<0.05).MVP was present in 17 of 31 (55%) IPE patients vs 12 of 14 (86%) MS patients (p=NS) and in only 1 of 16 (6%) normal subjects (p<0.05) vs both IPE and MS. We conclude that young patients with IPE represent an isolated form of connective tissue abnormality because of the presence of pectus excavatum and MVP, but this is different from the systemic involvement of MS because of the lack of other systemic findings, including aortic root dilatation and changes in body habitus.     

Lyell's syndrome: treatment with Centoxin and selenium

Lyell's syndrome (LS) (toxic epidermal necrolysis) is also known as scalded skin syndrome. When caused by staphylococci, it has a favorable prognosis. However, as a drug reaction, primarily to sulfonamides, anticonvulsives, and antirheumatic agents, it often takes a fatal course. On the basis of a case report, the authors present a new therapeutic concept aimed at influencing the pathobiochemical changes underlying LS. After taking a single dose of a sulfonamide preparation for a mild urinary tract infection, a 14-year-old girl developed LS with skin changes covering almost the whole body surface. Within 12 h the child showed signs of sepsis with imminent organ failure, leuco- and thrombocytopenia, and symptoms of beginning enteroparalysis. Wholeblood chemiluminescence analyses revealed massive formation of reactive oxygen species. Parallel to this, a drastic increase in PMN-elastase and strong activation of the kallikrein-kinin system were found. Blood concentrations of glutathione peroxidase and reduced glutathione were extremely low. The therapeutic regimen was intended to prevent both endotoxinemia and overproduction of toxic oxygen metabolites: the girl received 100 g monoclonal antibody against endotoxin (Centoxin) in combination with sodium selenite (Selenase). By day 2 her circulatory condition had stabilized and the blood concentration of oxygen radicals decreased. Neither liver nor kidney function showed pathological changes. Although on day 2 maximum activation of the kallikreinkinin system was observed, the expected drop in blood pressure did not occur. The blood concentration of elastate fell markedly, indicating that the release of elastase from neutrophil granulocytes, stimulated by endotoxin and complement, could be prevented. Both blood and plasma activities of glutathione peroxidase increased sharply as a consequence of the selenium therapy. On the 8th day of therapy all parameters had returned to normal (elastase, kallikrein-kinin, leucocytes, selenium). The whole-blood chemiliminescence response showed normal values and the skin changes had almost completely disappeared.     

不同机械通气方式联合肺表面活性物质对新生儿急性肺损伤/急性呼吸窘迫综合征疗效比较

目的 比较高频振荡通气+肺表面活性物质 （HFOV+PS）、常频机械通气+肺表面活性物质 （CMV+PS）、常频机械通气 （CMV）3种治疗方式对新生儿急性肺损伤/急性呼吸窘迫综合征 （ALI/ARDS）的临床疗效。方法 纳入ALI/ARDS新生儿136例 （ALI73例,ARDS63例）,其中HFOV+PS组45例,CMV+PS组53例,CMV组38例,前两组应用肺表面活性物质气管内滴入 （70~100mg/kg）。分别检测机械通气0h、12h、24h、48h、72h的PaO2、PaCO2、PaO2/FiO2、氧合指数 （OI）、呼吸指数 （RI）。结果 机械通气12h、24h、48h时HFOV+PS组的PaO2高于CMV+PS组和CMV组,PaCO2低于CMV+PS组和CMV组 （P < 0.05）;在机械通气12h、24h、48h、72h时HFOV+PS组PaO2/FiO2高于CMV+PS组和CMV组,OI、RI低于CMV+PS组和CMV组 （P < 0.05）;HFOV+PS组机械通气时间、用氧时间均低于CMV+PS组和CMV组 （P < 0.05）;3组气漏、颅内出血发生率及治愈率比较差异无统计学意义。结论 与单纯CMV以及CMV联合PS治疗相比,HFOV联合PS更可改善ALI/ARDS新生儿的肺功能,缩短通气时间及用氧时间,且不增加并发症的发生。     

Tetralogy of Fallot with Absent Pulmonary Valve and Bronchial Compression: Treatment with Endobronchial Stents

Absent pulmonary valve syndrome (APVS) is a rare congenital cardiac lesion. The lesion includes ventricular septal defect, overriding aorta, and absence of the pulmonary valve, with resultant pulmonary incompetence. It has been suggested that the pulmonary incompetence induces intrauterine dilatation of the pulmonary artery, which leads to tracheobronchial compression. One of the presenting features in infants with APVS is severe airway obstruction, which may be difficult to manage. We report an infant who benefited from bilateral endobronchial endoscopic stent placement.     

Clinical features and treatment of children with hemolytic uremic syndrome caused by enterohemorrhagic Escherichia coli O157:H7 infection: Experience of an outbreak in Sakai City, 1996

Presented is a study of 15 patients (seven males and eight females ranging between 5 and 10 years of age) with hemolytic uremic syndrome (HUS) associated with hemorrhagic colitis that was caused by enterohemorrhagic Escherichia coli (EHEC) O157:H7, encountered during the outbreak in Sakai City in July, 1996. The complete form of HUS, which includes the three characteristics hemolytic anemia, thrombocytopenia and acute renal dysfunction, was noted in eight patients, while an incomplete form of HUS, which did not include all three characteristics, was noted in seven patients. Regarding treatment, intravenous gamma-globulin was administered in nine patients and dialysis was performed in five patients (two males and three females) with the complete form of HUS. In three of these five patients, plasma exchange was also performed. Weaning from dialysis was accomplished by the 15th day of disease in all patients. Some patients developed pancreatitis, central nervous system symptoms, fundal hemorrhage and elevation of transaminase, although these abnormalities subsided uneventfully. Renal biopsy, which was performed in two patients who recovered from acute renal failure but still had mild proteinuria and a decrease in creatinine clearance, showed moderate changes in the glomeruli and tubulointerstitium. One year after onset of disease, hematological and urological findings were within normal limits in all patients except one with the complete form of HUS, who still had slightly decreased creatinine clearance.