Apolipoprotein E in childhood idiopathic nephrotic syndrome

Idiopathic nephrotic syndrome [INS] is one of the common renal disorders in childhood. Dyslipidemia is not only an important manifestation of INS, but is also involved in cardiovascular diseases and in progressive glomerular damage leading to renal failure. Apolipoprotein E [apo E] has been identified as an important candidate gene for lipid metabolism abnormalities. In the kidney, apo E gene mutation may play a role in aggravating the glomerular basement membrane lesion and promoting proteinuria. Our objective is to investigate the association of apo E serum level and genetic polymorphism [E[2], E[3], and E[4]] with responsiveness to steroid treatment as well as renal pathology in children with INS. Design: Case-control study. One study center at Center of Pediatric Nephrology and Transplantation [CPNT], Cairo University Children's Hospital. Forty seven pediatric patients with INS and 11 age and sex-matched controls were enrolled in the study, Twenty two of the patients had steroid-sensitive nephrotic syndrome [SSNS] and 25 had steroid-resistant nephrotic syndrome [SRNS]. Genomic DNA was extracted from children with INS and controls, and ape E genotype was determined by Real time-PCR analysis. The serum ape E, total cholesterol [TC], albumin, creatinine, and urine protein to creatinine ratio [UP/UCr] were also measured in both groups. Serum ape E was significantly higher in SRNS than SSNS [p=0.01] and controls [p=0.03]. Serum total cholesterol [TC] was significantly higher in SRNS than controls [p=0.028]. Significant positive correlation was found between serum apo E and UP/UCr [r=0.327, p=0.03]. Apo E[4] allele was only found in NS [8.5%] and absent in the control group. Apo E genotype E4E4 was found in one patient only in SRNS group with pathology of Focal segmental glomeruloscierosis [FSGS] while it was absent in SSNS and control groups, but this was not statistically significant. Heterozygous E[3]E[4] was only present in MS groups and not in control group [12% In SRNS and 13.64% in SSNS]. E[2]E[2] genotype was also found only in SRNS [4%] with the pathology of FSGS, which was not significant but the allelic frequency of E[2] was significantly higher in SRNS than SSNS [P=0.036]. E[3]E[3] was the most common genotype in the 3 groups and the allelic E[3] presentation was statistically higher in SSNS than in SRNS [P=0.04] The high frequency of E[4] only in nephrotic children and not in controls suggests that E[4] may share, as a genetic marker, in predisposition to childhood INS. The higher frequency of the E[2] allele in SRNS patients suggests that the E[2] allele gives a possible genetic predilection to steroid resistance in our population while the significantly higher E[3] in SSNS group may convey a readiness to steroid responsiveness related to E3, but further studies are needed to clarify this subject

C-reactive protein as a marker for early onset neonatal sepsis: the good, the better and the best

C-reactive protein [CRP] analysis is a simple, established and widely available test to identify an evidence of plasma acute phase response to infection and tissue injury. Standard CRP assays such as: latex agglutination and quantitative assays lack sensitivity at the early onset of neonatal sepsis. They can detect CRP concentrations. The development of the new high sensitivity immunoassay technique for CRP [HSCRP] has enabled its detection at lower concentrations. In HSCRP immunoassay, anti CRP antibodies react with antigen in the serum to form antigen antibody complex measured turbidimetrically. Evaluation of the diagnostic value of highly sensitivity CRP immunoassay in comparison to standard CRP latex agglutination and quantitative assays in early onset neonatal sepsis. A prospective controlled study including 51 neonates with culture proven bacterial sepsis. Ten healthy neonates served as control group. All neonates were subjected to full clinical examination, and laboratory investigations including: CBC with differential leukocytic count, blood culture and sensitivity. CRP analysis was performed using 3 techniques for each patient: CRP latex agglutination, CRP quantitative assay where values ? 6mg/L were considered abnormal, and HSCRP immunoassay where values ? 2mg/L were considered abnormal. CRP latex agglutination was positive in 8/51 [15.68%] and had sensitivity of 15.68%, specificity 100%, positive predictive value 100%, negative predictive value 18.86% and diagnostic accuracy 29.5%. CRP quantitative assay was positive in 12/51 [23.52%]. At cut of level 6mg/L: sensitivity was 23.5%, specificity 100%, positive predictive value 100%, negative predictive value 20.4% and diagnostic accuracy 36.06%. HSCRP immunoassay was positive in 34/51 [66.66%]. At cut off level 2mg/L: sensitivity was 66.6%, specificity 100%, positive predicative value 100%, negative predictive value 37.03% and diagnostic accuracy 72.13%. CRP analysis using high sensitivity immunoassay is likely to improve the diagnostic accuracy of CRP in detection of early onset neonatal sepsis. It allows detection of CRP at low concentrations. Only 40micro l of serum is needed, and results are available within 15 minutes

Ocular manifestations of Graves' disease

The present study concluded that 60% of hyperthyroid patients examined showed ocular manifestations. The commonest signs of ocular manifestations of hyperthyroidism are: Poor convergence [90%], extra-ocular muscle palsies [84%], upper lid retraction [50%] and exophthalmos [50%]. While the rarest ocular manifestations are: Increased pigmentation of lids, jerky pupillary consensual light contraction [7%]. T3 estimation in the sera of the patients is more important than T4 estimation. As 77% of hyperthyroid patients with ocular manifestations showed increase in T3 above normal level. Ultrasonographic study is helpful to demonstrate extra-ocular muscles enlargement in patients with exophthalmos

Operative and immediate post-operative complications of cataract extraction

Study of two hundred and forty four cases of cataract surgery. The rate of operative and immediate post operative complications were 66 cases [27.04%]. The study revealed that 182 cases [75.20%] of operations have been done for senile cataract with 44 complicated cases [24.17%] while developmental cataract operations were preformed in J4 cases [5.78%] with 10 complicated cases [71.42%]. For complicated cataract 46 cases [19.00%] operations were performed and the rate of complications were highest in cataract operations complicating iridocyclitis 4 from 12 cases [33.33%], then in cataract complicating myopia 4 cases frome 20 cases [20%] and lastly cataract operations comlicating diabetes 2 cases from 14 cases [14.28%]. As regard methods of extractions of cataractous lens, linear extractions technique were used in 10 cases [4.13%] and the complicated cases were 8 cases [80.00%], scoop method were used in 6 cases [2.47%] with 4 complicated cases [66.66%], extraction with non toothed capsule forceps were used in 22 cases [9.09%] with 8 cases [36.36%] complicated operations, cryoextraction were used in 200 cases [82.64%] and 44 cases [22%] complicated cases, and lastly aspiration technique were used in 4 cases [1.65%] with no complications. The most common types of complications were vitreous loss 42 cases [17.21%] striate keratitis 30 cases [12.29%], rupture of lens capsule 12 cases [4.91%], after cataract 8 cases [3.27%], Shallow anterior chamber 8 cases [3.27%], and lastly 2 cases of retro-bulber haemorrhage [0.81%]. No case of endophthalmits, expulsive haemorrhage, post operative iris prolapse were happened during the study

Strabismic amblyopia: practical considerations in diagnosis and management

The authors conducted a research project of screening children under the age of 10 for visual defect. They found that amblyopia constituted 6.0% of the total ophthalmic outpatient children, but 44.0% of the strabismic cases. Early treatment of amblyopia is essential but before commencing any treatment of this visual defect, appropriate correction of ametropia or anisometropia must be dealt with. The authors recommend a combination of part-time occlusion of the fixing eye and attentive visual exercises on television games using the amblyopic eye for 20 minutes twice daily until maximum vision is attained on two consecutive visits. The authors therapeutic regimen achieved 86% success rate