Study of cardiac abnormalities in children with Gaucher disease

Gaucher disease is the most prevalent lysosomal storage disease which results from inherited deficiency in the glucocerebrosidase enzyme. Three main clinical forms have been described, type I non-neurophatic, type II acute neuropathic and type III subacute neuropathic [1]. In this study, we present specific characteristics, as well as our experience in diagnosing the cardiac abnormalities in a group of Egyptian patients with this disease. The study included 22 patients with Gaucher disease attending Children's Hospital, Alexandria University. The recombinant enzyme imiglucerase [cerezyme] was given in a dose of 60 IU/kg/2 weeks [2]. Hemoglobin, plasma chitotriosidase and abdominal ultrasound were assessed before starting therapy and every 6 months. Molecular analysis was done to 17 patients. At presentation, the mean age was 7.94 +/- 6.26 years. 6 patients [27.2%] had type I, 16 patients had type III Gaucher disease [72.7%]. The commonest genotype was homozygous L444P which was present in 11 patients [50%] followed by homozygous D409H found in three patients [13.6%]. Gaucher's disease leads to deposition of glucocerebrosides in various organs. Recently, type IIIC Gaucher's disease, homozygous for the D409H mutation, has been identified; this is an ultra-rare cardiac variant with progressive calcification of aortic and/or mitral heart valves[3]. In this study the cardiac evaluation through clinical examination and investigations of the cases revealed that [50%] had positive cardiac findings

Effect of saffron (Crocus sativus L.) on sodium valporate induced cytogenetic and testicular alterations in albino rats / 대한해부학회지

The present study investigated the cytogenetic and testicular damage induced by the antiepileptic drug, sodium valporate (SVP) in albino rats and the effect of saffron aqueous extracts. Treating rats with SVP caused a significant increase in the chromosomal aberrations either structural or numerical and decreased the mitotic index. Besides, animals administered SVP showed DNA damage appeared in the single strand breaks (comet assay). Testis of SVP-treated rats showed many histopathological changes. A significant decrease in seminiferous tubules and their epithelial heights diameters and inhibition of spermatogenesis was recorded. In addition, the number of sperm head abnormalities was increased. Biochemical results revealed an increase in malondialdhyde (MDA) which is lipid peroxidation marker and a significant decrease in the level of serum antioxidant enzyme, catalase (CAT) and reducing antioxidant power (RAP). Animals given SVP and saffron showed an improvement in chromosomal aberrations, mitotic index, DNA damage and testicular alterations caused by SVP. Moreover, MDA decreased and CAT and RAP increased. It is concluded from the present results that the ameliorative effects of saffron extract against SVP-induced cytogenetic and testicular damage in albino rats may be due to the presence of one or more antioxidant components of saffron.

Effect of Nigella sativa oil on the microbial quality of chilled chicken fillets

The present study was conducted to evaluate the effect of addition of different concentration of Nigella sativa oil on microbial quality of chicken fillets during refrigeration for 2, 4, 7, and 10 days storage depending on bacteriological assessments. There is no effect on sensory properties [odour and appearance] of treated chicken meat fillets during storage as compared with control one. Bacteriological examinations recorded high initial bacterial counts, Most probable number of coliforms, Staphylococcus aureus and yeast and molds counts]. During refrigeration period, there was increase in bacterial counts. A series of five different oil concentrations were screened for their antibacterial and antifungal effects at 0.5, 1.0, 1.5, 2.0 and 2.5% [w /w]The results revealed that all oil percentages showed antibacterial activity against bacteria, yeast and molds on this assay. The oil at 2.0% concentration was more effective as compared to other concentrations on the bacterial count, coliforms count [MPN/gm], Staphylococcus aureus and yeast and molds counts. Therefore, Nigella sativa oil may be used as an antimicrobial agent in food products to prevent spoilage and may be used as preservative agent

Effect of pasteurization on some chemical and bacteriological parameters on camel milk

Fifty raw camel milk samples were randomly collected from different retail shops from Cairo and Giza Governorates and submitted to bacteriological and chemical evaluation. Results revealed that the mean values of mesophilic bacteria, Psychrotrophic bacteria, Coliforms, Staphylococcus aureus and Yeast and mould count were 8.1xl0[5], 3.5xl0[4], 2.4xl0[2], 3.2xl0[3] and 4.2x10[5], respectively. Forty percentages of the examined samples were positive for coagulase positive Staphylococcus aureus. Salmonellae, E. coli and Yersinia enterocolitica could not detected in all examined camel milk samples. The mean values of pH, protein and fat percent were 6.6, 2.96% and 3.5%, respectively. Camel milk samples were pasteurized by procedures specific for bovine milk at 63°C for 30 minutes. Results indicated that pasteurization of milk samples at temperature of 63°C was effective on the microbial load of the examined milk samples. The spoilage of pasteurized milk samples began after day 6 of cold storage at 5°C. The mean pH value, protein contents and fat percent were 6.6, 2.96% and 3.5%, respectively. Results indicated the potential health hazard risk of consumption of raw camel milk under the present production procedure. The public health importance of the isolated organisms as well as control measures and treatment for improving of camel milk quality were discussed

Chemosystematic studies of some species of the genus potamogeton L. in Egypt

Morphological, anatomical and a phytochemical studies were conducted on four out of seven potamogeton L. species recorded in the flora of Egypt. The stem anatomy referred to the presence of three types of steles and three types of endodermis. The chemical investigation revealed the presence of eleven flavonoid compounds, the distribution of which was traced in the studied species to evaluate the interspecific affinity through this parameter It was found that the chemical data in this work justify the classification of ascherson and graebner [1907] as regards the assignment of the four studied species to their sections. A key based on a combination of the anatomical and chemical characters was suggested for species delimitation

Midline posterior microendoscopic discectomy. Is it the future procedure of choice?

This is prospective study of 20 cases with solitary unilateral lumbar and lumbosacral disc prolapse were managed in Al-Azhar University Hospital, Ain, Shams University Hospital and Al-Sahel Teaching Hospital from January 2005 to January 2007. All the cases were operated by posterior midline microendoscopic discectomy procedure, utilizing microendoscopic tubular retractor system [METR'x] and Destandeau method. The postoperative clinical outcome was assessed according the MacNab's criteria. We assessed the factors which affect the outcome including the age, the value of clinical evaluation, pre operative MRI, degree of disc prolapse and duration of symptoms. Complication in the form of dural tear, discitis and superficial wound infection were recorded. We compare the result of our study to those reported in literature and the result of open microdiscectomy. Endoscopic lumbar discectomy is considered an alterative option of traditional open discectomy in appropriately well trained and experienced hands for appropriately selected patients

Evaluation of cerebrospinal fluid diversion procedures in treatment of pediatric midline posterior fossa tumors

Neurosurgeons still divided in their opinions concerning the best way to manage obstructive hydrocephalus secondary to posterior fossa tumors. Some authors proposed a preoperative indwelling cerebrospinal fluid shunt as the most advantageous method for the subsequent surgical approach to the tumor. Others proposed direct approach to the posterior fossa tumor, when possible, or external ventricular drainage, when necessary. This study aimed to evaluate the role of cerebrospinal fluid diversion procedures in treatment of hydrocephalus due to midline posterior fossa tumors in children. Eighty-eight patients were included in this retrospective study. They were classified into two groups: 22 patients operated for direct tumour attack without CSF diversion and 66 patients operated for CSF diversion before tumour attack. The later group was further subdivided into three subgroups according to the method of CSF diversion; V-P shunt, ETV, and EVD. This study carried out in the Neurosurgery Department, at Suez Canal University Hospital in the period between March 1995 and February 2005. The use of CSF diversion procedure was associated with improvement of hydrocephalus in 70% of patients while direct tumour attack was associated with improvement of hydrocephalus in 31% of patients. The best results were found in ventriculoperitoneal shunt [100%] followed by endoscopic third ventriculostomy [60%] then the external ventricular drainage [31%].The use of CSF diversion is beneficial in treatment of hydrocephalus due to midline posterior fossa tumour in children

Clinical and radiological outcomes of posterior lumbar interbody fusion with carbon fiber cage in comparison to posterior lumbar interbody fusion with iliac bone graft

This prospective, controlled, randomized clinical study critically compared the clinical and radiological outcomes in patients surgically treated by PLIF with carbon fiber cage versus those treated by PLIF with iliac bone graft. In the period between May 2004 and April 2006, sixty patients underwent posterior interbody fusion of the lumbar spine with transpedicular screw fixation for the treatment of their degenerative segmental instability. In half of then PLIF was done using carbon fiber cage and in the other half PLIF was done using iliac bone graft. Participants were evaluated pre-operatively and post-operatively at 3, 6, 9, and 12-month intervals. Pain was scored by a VAS for both lower limb and back pain both preoperatively and postoperatively. The clinical outcomes were compared using the Prolo economic and functional rating scale and the fusion status was compared using the radiological criteria of fusion proposed by Brantigan. By 12 months follow up period, 65% of the study group expressed clinical success. Prolo scale showed clinical outcomes of patients who were treated with PLIF with carbon cage are better than those treated with PLIF with iliac crest graft but this was not statistically significant. The radiographic evaluation for bone union showed that 43% of the iliac bone group had no fusion, but 80% of cases of the cage group revealed sound fusion. Complications were matched in the 2 groups except for collapse and iliac crest harvest complications that prevail in the iliac crest group. PLIF with Carbon cage gives better fusion on radiology than PLIF with iliac bone graft, but no statistical difference in the clinical outcome. Carbon cage use precludes complications associated with iliac bone harvesting

Raman and infrared spectra of ternary P[2]O[5] -PbO-Bi[2]O[3] glasses

Vibrational infrared and Raman spectroscopy were employed to investigate the structural arrangement of some selected glasses from the ternary system P[2]O[5]-PbO-Bi[2]O[3]. The obtained spectra were analyzed in order to elucidate the role of each of the component oxides and justifying their specific roles in the glassy network. The densities of the prepared glass samples were measured and their molar volumes are calculated and the data are analyzed in relation to the compactness of the structure

Validity of clinical versus genetic study for identification of the fragile X syndrome

Fragile X syndrome is the most common cause of idiopathic X linked mental retardation among boys. It presents several diagnostic problems due to variable clinical features. Inconsistencies also exist in both cytogenetic and molecular markers. This study was conducted on 100 males referred for mental retardation of unknown cause at the Alexandria University Children's Hospital with an age three years and older. The study population was screened for clinical detection of Fragile X syndrome using Ten-item checklist. Patients with positive checklist were subjected to cytogenetic analysis to detect the fragile site on X chromosome and molecular analysis for detection of the altered DNA sequence using polymerize chain reaction [PCR].The patients' ages ranged 3-14 years with a mean of 5.99 +/- 2.68 years. Their IQs ranged 33-85 with a mean of 60.22 +/- 14.05. Out of the 100 males, 22 cases scored positive on the checklist. The most frequent clinical characteristic was hyperactivity [50%] and the least frequent was macro-orchids [2%]. Cytogenic analysis was positive for the fragile site at Xq 27.3 for four cases [18.2%] and negative for 18 cases [81.8%].PCR was positive [more than 200 CGG repeats] for 7 cases [31.8%] and negative for 15 cases [68.2%]. Also, there was a highly significant correlation between the score of the checklist and the positivist of both tests [P=0.01 for each test]. In conclusion, pre-test selection based on clinical criteria is useful to identify high-risk groups and minimizes the cost-effectiveness of both cytogenesis and molecular laboratory testing of fragile X syndrome. Combination of behavioral and physical characteristics is valuable especially in pre-pubertal cases

Iridoids with antimicrobial activity from plumeria alba L.

Three major iridoids; viz. plumieride, protoplumericin A and plumieride acid were isolated from the bark and leaves of Plumeria alba L. Protoplumericin A and plumieride acid are reported for the first time from the titled plant. These compounds were identified through different chemical and spectroscopic methods. They displayed distinct activity against some pathogenic bacteria and fungi

Histopathological changes in liver and kidneys of male albino rat due to tribenuron- methyl

Histopathological changes were detected in liver and kidneys of male rats Rattus norvegicus var. albus following treatment with tribenuron- methyl. Rats were given orally tribenuron-methyl technical or formulation once per 48 hours as 5, 25, 50 and 100 mg/kg b.w over 10 days. Acute hepatitis was the common feature among all tested doses represented by focal necrosis and proliferation of Kupffer cells with infiltration of mononuclear cells in liver. Acute nephritis showed as degeneration in kidney tubules without seeing any phagocytes, also acute interstitial nephritis at 25 and 100 mg/kg b.w of the formulation and technical form, respectively. The changes seem to be dose related

Continent cutaneous urinary diversion versus ileal conduit post-radical cystectomy: a comparative study

Objectives: to evaluate the efficacy and outcome of continent cutanuous urinary diversion using the extramural serous lined tunnel [CCD] and compare it with the standard ileal conduit procedure [ICD] after radical cystectomy due to bladder cancer

Patients and Methods: Between 1998 and 2002, 68 patients were underwent radical cystectomy and were not candidates for orthotopic diversion. Of these patients, 24 underwent CCD as described by Abo-Enein and Ghoneim in 1995 and 44 patients underwent ICD. Outcome analysis included operative time, hospital stay, early postoperative complications, quality of life and cosmetic appearance

Results: Overall, mean age at surgery and follow up was 55, 3 years, respectively. Mean operative time was 8.3 and 6.8 hours for CCD and ICD, respectively. Sixty cm of ileum was used to fashion the CCD pouch compared to 1520 cm used in ICD. Mean capacity of the pouch was 450 in CCD. Of CCD patients, 23 were continent and only one patient had cutaneous incontinence, one had stoma1 stenosis and one had pouchoureteral reflux associated with continence. Serum creatinine, Na, K acid acid-base profile were within normal. Most of the patients had better quality of life than ICD group. Eady postoperative complications were comparable. No intraoperative or postoperative mortality was encountered

Conclusion: Continent Cutaneous diversion is technkally feasible, aapplnce free, applicable for urinary diversion with low complication rate and good quality of life with cosmetically accepted stoma for this selected group of patients. It is considered an alfemative diversion when orthotopic type is not feasible

Plasma and red blood cells membrane lipid concentration of sickle cell disease patients

The present study aimed to determine the concentration of plasma and red blood cells [RBCs] membrane lipids in Saudi sickle cell disease [SCD] patients. This study was carried out at the Hematology Clinic, King Abdul-Aziz University Hospital, Jeddah, Kingdom of Saudi Arabia from October 1998 to October 1999. Lipid concentrations were determined in plasma and RBC membrane of 81 SCD patients and 66 normal healthy matched individuals [control]. Different lipid parameters were measured according to standardized enzymatic assay methods. The plasma concentrations of total cholesterol and low density lipoprotein cholesterol of SCD patients were significantly decreased [p<0.001], whereas the plasma concentrations of high density lipoprotein phospholipids were significantly increased [p<0.001]. The plasma concentrations of apo A and apo B were significantly decreased [p<0.001] in SCD patients. However, the concentration of total cholesterol of RBC membrane was significantly increased [p<0.001] in SCD patients, while the phospholipid content was significantly decreased [p<0.001]. The significant increase of RBC membrane cholesterol concentration in SCD patients possibly is responsible to the change in RBC membrane fluidity that may play a direct role in the sickling phenomenon of RBCs in SCD

Pelvic fracture urethral injuries in children and adults

We attempted to identify the particular features of pelvic fracture urethral injuries in children compared to adults. A total of 43 boys, 2 to 15 years old, with pelvic fracture, were studied prospectively. This is a subset of a series of 203 consecutive male patients, 2-80 years old, with fracture of the bony pelvis. All patients underwent radiographic examination of the pelvis while retrograde urethrography was performed in 194 patients. The most common types, of pelvic fracture in children were Malgaigne's [32.6%] and straddle [23.2%] fractures. Urethral injury was found in 13 children [30%] in the form of stretching of an intact posterior urethra in 1 case [8%], partial rupture in 3 [23%], and complete rupture in 9 [69%]. In 3 of the partial rupture cases, injury involved the prostatic urethra or bladder neck. Whereas the more serious fractures, were more common in children [56 versus 24%], the less serious fractures were more common in older patient [76 versus 44%] [p < 0.05]. In children, associated urethral injury is more frequent [30 versus 24%], more likely to be a complete rupture [69 vs 42%], and with a higher incidence of prostatic displacement [22.2 versus 18.7%] than in adults. Injury of the prostatic urethra or bladder neck is peculiar to the children group

Rapid carrier screening using short tandem repeats in the phenylalanine hydroxylase gene

Phenylketonuria [PKU] is an autosomal recessive genetic disorder caused by defects in the phenylalanine hydroxylase [PAH] system. Our work aimed to screen the PAH locus for the presence of potentially useful short tandem repeats [STR] as markers for carrier detection in PKU families in Egypt, and to determine the level of PAH heterozygosity within the Egyptian population. The system contains at least eight independent alleles in the Egyptian population, transmitted in a Mendelian fashion. Variations in the number of STR in the 16 families studied gave rise to polymorphisms that proved to be suitable markers for PKU carrier detection and prenatal diagnosis. The most frequent allelic fragment size in PKU patients was 246 bp [35.7%], which together with a fragment of 254 bp accounted for 60.7% of the mutant chromosomes

Blastocystis hominis among infants and preschool children in an urban and a rural area in Alexandria

Blastocystis hominis is now gaining acceptance as an agent of human intestinal disease. This case-control study of the prevalence of B. hominis infection in children less than 6 years old was conducted in an urban area and a rural area in Alexandria. A total of 600 stool samples were examined by wet mount preparation, Merthiolate iodine Formaldehyde concentration technique and permanent staining by using modified Ziehl Neelsen and trichrome stains. The difference between the prevalence of B. hominis in infants and preschool children from an urban area [20.33%] was not significantly different from that in a rural area [24%]. The most common clinical manifestations of B. hominis infection were diarrhea, abdominal pain, fever and vomiting. B. hominis was found in 85 [28.33%] of 300 stool specimens of cases with diarrhea and in 48 [16%] of 300 control specimens. Seventy cases [82.35%] of diarrhea had heavy infection [>5 organisms/20 HPF]. The presence of diarrhea with B. hominis infection was more common in boys aged from one to six years, in severely malnourished cases and in areas with no sewage system and in absence of in-house piped water supply. Contaminated water was suspected to be the major source of infection, since several cases were associated with Giardia infection. These findings support the concept of B. hominis pathogenicity in children with diarrhea

Use of colonic pouches in treatment of mid and low rectal cancer: better patient selection result in better functional outcome

This study was conducted to evaluate the selective use of colonic pouches based upon the diameter of the proximal colon. Twenty-nine rectal cancer patients underwent ultra-low anterior resection or colon anastomosis were included in this study, 17 had a stapled straight anastomosis, while 12 had a stapled colonic pouch-anal anastomosis. Colonic pouches resulted in a superior functional result in the first six months postoperatively compared with straight anastomoses. Pouch patients had less frequency, urgency and need for constipating drugs. None of the pouch patients had evacuation difficulties. Adaptation of straight anastomosis patients resulted in comparable bowel function after six months in most of the patients

Effect of length of oesophageal stump on postoperative gastro-oesophageal function after resection of lower oesophageal carcinoma

Oesophageal carcinoma is not uncommon. One common goal of curative and palliative resection of oesophageal carcinoma is to achieve good functional outcome. During the last decade, Ivor Lewis operation with intrathoracic oesophagogastric anastomosis has been rising as the operation of choice for lower oesophageal carcinoma. It is wdl known that no oesophageal substitute is able to function as proper as the original organ, the peristalsis of which is essential to propel food into the gastric reservoir. Many authors think that the greater the length of the remaining oesophageal stump the better the postoperative functional outcome. The aim of this work was to evaluate the functional outcome after Ivor Lewis oesophagectomy and gastric pull-up for oesophageal cancer and to correlate the results with the length of the preserved oesophageal stump. Twenty patients [15 males, mean age 60 ys] were studied at the sixth postoperative month by questionnaire, stationary manometry and upper GI endoscopy. The commonest postoperative unpleasant complaints were early postprandial sensation of fullness [70%], diarrhea [40%] and gastrooesophageal reflux disease [GORD] [40%]. GORD was more profound in the left lateral position. According to the actual site of the oesophago-gastric anastomosis away from the central incisors as seen during endoscopy, patients were classified into 2 groups: High anastomosis group [A] with anastomosis lying less than 25cm from the central incisors [i.e. the anatomical length of the oesophageal stump < 10 cm] and low anastomosis group [B] with anastomosis lying at or below 25cm from the central incisors [ie. the anatomical length of the oesophageal stump >/= 10 cm]. It has been found that in low anastomosis group, there was a significant difference between the anatomical length of oesophageal stump and the functional length [as measured by manometry]. Patients in low anastomosis group had significantly better postoperative oesophageal body functions as revealed by better mean peristaltic pressure amplitudes [37 mmHg in group B versus 20.1 mmHg in group A, P < 0.05], better duration of contractions [2.11 sec. in group B versus 1.62 sec. in group A, P < 0.05] and better propagation velocity of the muscular contractions [2.7 mm/sec, in group B versus 2.01 mm/sec, in group A, P < 0.05]. This was reflected clinically by significant increase in body weight and significantly higher proportion of patients regaining their normal preoperative body weight in the low anastomosis group. It has been concluded that, provided oncological rules are not breached, Ivor Lewis operation with low intrathoracic anastomosis gives better functional outcome than high anastomosis in patients with lower oesophageal carcinoma

Internal colonic fistulae: experience with en-bloc extended low anterior resections

Thus study was undertaken to assess the results of using extended en bloc low anterior resection in patients with internal colonic fistulae regardless of the aetiology of the fistula. Eighteen patients [10 women, 66%; 8 men, 44%] who ranged in age from 23 to 86 years [median 67.5 years] were seen between 1996 to early 2000. The types of fistulas included 8 colovesical [44%], 5 colovaginal [28%] and 3 coloenteric [17%]. Two patients had complex fistulae involving more than 2 organs [11%]. The cause of the fistula was diverticulitis in 10 patients [56%] cancer in 5 patients [28%], Crohn's disease in 2 patients [11%] and localized bowel ischaemia in 1 patient [6%]. Fifteen patients [83%] had definitive en bloc resections, while 3 patients had diverting transverse loop colostomy only because of advanced malignancy. Stapled low anterior resection en bloc- with a second organ and omental interposition was performed in 11 patients [9 patients had omental interposition and 3 had covering stomas]. En bloc colonic and small bowel resections with hand-sewn anastomoses was done in 3 patients, One patient had a Hartmann's procedure. The hospital stay ranged from 6 to 63 days [median 12 days]. There were no operative deaths and no clinical anastomotic leaks. The postoperative course was uncomplicated in 72%, while 5 patients [28%] experienced complications. En bloc radical resections with primary anastomosis can be accomplished safely in patients with internal colonic fistulae. Extended resections allow safe anastomosis to be carried out in pliable non-inflammed tissues, guards against future recurrence of diverticulitis without any increase in morbidity. They also ensure adequate resections in cancer patients and when the diagnosis is in doubt