Serum levels of mutant p53, soluble FAS [S-FAS] in children with acute lymphoblastic leukemia

In a trial to improve the outcome of acute lymphoblastic leukemic patients, the serum concentrations of mutant p53 and soluble Fas [s- Fas] as antiapoptotic markers were assessed in the sera of 45 children with acute lymphoblastic leukemia [ALL] [on admission, after remission and in relapsed cases] as well as in 15 healthy children of matched age and sex as controls [on admission]. The study revealed a significant increase of mutant p53 and s-Fas in newly diagnosed cases than in the controls and ALL cases in remission, in non responders than in responders and in cases with organomegaly than those without. Although mutant p53 and s-Fas had the same specificity and negative predictive values, mutant p53 had significantly higher sensitivity and positive predictive value than s-Fas in ALL cases with metastases as well as in relapsed cases. On admission, there was a significant positive correlation between blast cells% and each of mutant p53, s- Fas levels as well as between the levels of the last two antiapoptotic markers

Real time three dimensional [RT3-D] and two dimensional echocardiography evaluation of atrioventricular septal defect [AVSD]

This study included 60 patients with repaired AVSD that were scanned by 2-D echocardiography to detect and evaluate the degree of residual mitral regurge [MR] and to assess the cardiac function. The preoperative data including age, weight, associated anomalies or Down's syndrome and two-dimensional echocardiography were reviewed. Using real time 3-D echocardiography, the three components of mitral valve [superior, inferior and lateral or mural leaflets] surface areas were measured in 20 out of these 60 patients. A common AVV orifice was present in 73.3% [first group] and the rest had separate AVV orifice [second group]. The prevalence of associated cardiac anomalies was significantly higher in the first group. Down's syndrome was present in 22 cases, 12 of them in the first group and 10 in the second group. It was also observed that late repair [<1 year] was a risk factor for postoperative MR. So, the first year of life is the golden time for definitive repair of AVSD. It can be concluded that late [>12 months] repair of AVSD and associated cardiac anomalies were identified to be significant risk factors for the persistence of MR postoperatively. To improve the surgical results of AVSD repair, operation should be best done before the first year of life. Real time 3-D echocardiography preoperative scanning of the surface areas of the mitral valve leaflets may give a guide to the best procedure of repair. Real time 3-D echocardiography avoids deficiencies encountered with reconstructive 3-D techniques by acquiring volumetric data at a rate sufficient to show real time movements of the heart and intracardiac structures in a very short time

N-acetyl-beta-d glucosaminadase and retinol-binding protein versus albuminuria in insulin dependent diabetic children.

Markers of tubular dysfunction [N-acetyl-beta-D-glucosaminidase and retinol binding protein], markers of glomerular dysfunction [glomerular filtration rate, urinary albumin excretion, blood urea and serum creatinine]and blood pressure were estimated in 32 insulin dependent diabetic children [IDDM] [16 males and 16 females] aged from 7 to 15 years, who had negative reagent strip test for proteinuria and in 10 age and sex matched healthy control children [6 males and 4 females]. Ultrasonography of the kidneys and fundus examination for the detection of retinopathy were done for all cases. The results indicated that tubular dysfunction, as evidenced by RBP and NAG, is more sensitive and precedes glomerular dysfunction in insulin dependent diabetics. However, RBP is a convenient, more sensitive, specific and early diagnostic tool for the detection of tubular affection than NAC. Albuminuria and increased GFR are early markers of glomerular dysfunction preceding the elevations of blood urea and serum creatinine. A reduction in hyperfiltration or GFR with increased albumin excretion is a marker of progression of renal disease. Hypertension is a feature of diabetic nephropathy after the appearance of microalbuminuria. However, the role of dietary modification programs and blood pressure control in normalization of kidney function in IDDP remains to be evaluated

Acute renal failure in jaundiced-isoimmunized full term neonate

This study included 85 full-term newborns with unconjugated hyperbilirubinemia, due to ABO and RH incompatibility and renal impairment as well as 20 healthy, age and sex matched full-term newborns as a control group. None of the cases showed any stress factors which may cause renal failure. All cases were subjected to full clinical examination, abdominal ultrasonography to exclude cases with congenital renal abnormalities, laboratory investigations of blood were done at the 5th day then repeated at 30th day of postnatal life for follow up. According to the investigations, the patients were subdivided into 2 groups: 25 patients with acute renal failure [ARF] and 60 patients without acute renal failure [non ARF]. They represent 4.2% and 10.1%, respectively. of the total admissions of the jaundiced, isoimmunized full-term newborns in the NICU in one year [596 neonates]. Follow up studies for one month revealed that renal functions were normalized in cases with non-ARF except the beta 2 microglobulin, while cases with ARF still have significantly higher differences in all the studied parameters when compared to the control group after one month except for urine pH and specific gravity. In conclusion, renal impairment should be expected in cases having total serum bilirubin >25 mg/dL. They should be closely monitored regarding their renal functions for at least one month of postnatal life. Those who develop ARF should be followed up for a longer period