In spite of the implementation of high technology in Neonatal Intensive Care in our country, the mortality rate of the infants with RDS is high, but is not different from that in developing countries. The improvement of perinatal care and diminution of risk factors, common use of surfactant as well as antenatal steroids could most probably result in better outcome of neonatal RDS.
BackgroundAtypical hemolytic uremic syndrome (aHUS) is a rare and heterogeneous disorder. The first line treatment of aHUS is plasma therapy, but in the past few years, the recommendations have changed greatly with the advent of eculizumab, a humanized monoclonal anti C5-antibody. Although recent recommendations suggest using it as a primary treatment for aHUS, important questions have arisen about the necessity of immediate use of eculizumab in all cases. We aimed to draw attention to a specific subgroup of aHUS patients with rapid disease progression and high mortality, in whom plasma therapy may not be feasible.MethodsWe present three pediatric patients of acute complement-mediated HUS with a fatal outcome. Classical and alternative complement pathway activity, levels of complement factors C3, C4, H, B and I, as well as of anti-factor H autoantibody and of ADAMTS13 activity were determined. The coding regions of CFH, CFI, CD46, THBD, CFB and C3 genes were sequenced and the copy number of CFI, CD46, CFH and related genes were analyzed.ResultsWe found severe activation and consumption of complement components in these patients, furthermore, in one patient we identified a previously not reported mutation in CFH (Ser722Stop), supporting the diagnosis of complement-mediated HUS. These patients were not responsive to the FFP therapy, and all cases had fatal outcome.ConclusionTaking the heterogeneity and the variable prognosis of atypical HUS into account, we suggest that the immediate use of eculizumab should be considered as first-line therapy in certain small children with complement dysregulation.
Case summaryA 6-year-old boy was admitted to the hospital with presumptive diagnosis of acute pyelonephritis. He had a 2-day history of fever and vomiting. On admission he had high fever (39.7 C) and diffuse abdominal tenderness. Renal percussion was negative. Laboratory investigations revealed high erythrocyte sedimentation rate (60 mm/h), C-reactive protein 136 mg/l and leukocytosis of 21.310 9 / l. His urine was cloudy; nitrite and esterase tests were positive and microscopic examination of the sediment revealed many white blood cells. Ultrasound examination of his kidneys, liver, gallbladder, spleen and urinary bladder did not show any abnormal changes. The boy was administered intravenous fluids and ceftriaxone 1.0 g/day (50 mg/kg/day). Within 48 h he became afebrile and his urine cleared. The bacteriology report showed that his pyelonephritis was due to Escherichia coli (10 6 /CFU), which was sensitive to ceftriaxone. The parenteral treatment with ceftriaxone continued at the same dose on outpatient basis. On the fifth post-admission day, the boy's parents reported that he complained of right lumbar pains and macroscopic hematuria. Ultrasound examination was performed the same day and demonstrated a small non-obstructive calculus (5 mm) in the right kidney (Fig. 1). Also, echogenic material with acoustic shadowing was seen in the gallbladder (Fig.2). Additional laboratory investigations were performed afterwards and revealed normal complete blood counts; urinalysis was positive for blood 3+; nitrite and esterase tests were negative and pH was 5. Serum biochemistry showed normal values for calcium (2.33 mmol/l), phosphate (1.55 mmol/l), magnesium (1.0 mmol/l), uric acid (236 mmol/l), bicarbonate (23 mmol/l), alkaline phosphatase (133 U/l), and PTH (22 pg/ml). Examination of the solute/creatinine ratios (mmol/mmol) in the random urine sample were as follows: calcium/creatinine (0.22, normal <70), uric acid/creatinine (0.33, normal <1.0),The answer to this question can be found at http://dx.
Cardiomyopathies 284supply the yolk sac. The blood drains back to the heart tube via paired vitelline veins. The second circuit is the umbilical allantoic, placental extraembryonic circuit. In this instance, the dorsal aortae supply blood to umbilical arteries that in turn bring this now unoxygenated blood back to the placenta. "lood from the placenta is carried to the heart tube via umbilical veins. . . Formation of the primitive four chambered heart"s the endocardial heart tubes fuse, several bulges and sulci appear. From the cephalic end, the bulges are the bulbus cordis truncus arteriosus and the conus arteriosus , the primitive ventricle, the primitive atrium and the sinus venosus fig . The veins connect to the heart tube via the sinus venosus, while the paired dorsal aortae arise from aortic arches that in turn arise from the aortic sac. The aortic sac is at the most cephalic end of the bulbus cordis. The sulci are present as the bulboventricular sulcus, between the bulbus cordis and the ventricle, and the atrioventricular sulcus, between the atrium and the ventricle.Then, a rapid growth of the heart tube takes place, and the heart begins to convolute. With this convolution the dorsal mesocardium begins to degenerate. During the process of convolution, the first flexure seen is between the bulbus cordis and the ventricle. The bulbo-ventricular loop that is formed shifts this region of the heart to the right and ventrally. The second flexure, the atrioventricular loop, is between the atrium and the ventricle and this region of the heart is shifted to the left and dorsally. "s growth continues the atria shift cephalically.
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