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Biomedical subjects

K Eid

Publications and source records attributed to K Eid.

15 recordsLinked to original sources
PubMed2006

Enzyme replacement therapy and bony changes in Egyptian paediatric Gaucher disease patients.

BACKGROUND: In Gaucher disease, the infiltration of the bone marrow by glucocerebroside-laden macrophages (Gaucher cells) triggers a diverse pattern of skeletal disease that results in crippling complications. Reliable ascertainment of the severity and pattern of skeletal disease is essential to determine disease status and the response to enzyme replacement therapy (ERT). Although there is ample documentation of reversal of haematological and visceral disease by ERT, there is a paucity of data on skeletal response to ERT in children. AIM: To delineate the pattern of bone disease in children with Gaucher disease in Egypt and to evaluate its response to ERT. METHOD: Twenty-two children with Gaucher disease were treated with ERT. Phenotyping by clinical, laboratory and radiological criteria was performed at baseline and following 11.2 +/- 4 months of ERT. Genotyping for glucocerebrosidase (GBA) mutations was performed by gene sequencing, and genotype-phenotype correlations were performed.Results. Two-thirds of the patients were from consanguineous pedigrees and 14/22 patients were homozygous or compound heterozygous for L444P and D409H mutations. Bone involvement was detected by plain radiology in 11 children (50%) and in 16 (73%) by magnetic resonance imaging (MRI). There was no correlation of severity of bone involvement and GBA genotype. ERT ameliorated bone disease: 10 of the 11 children with abnormal radiographic findings at baseline showed improvement in skeletal lesions; while 9/16 showed improvement of marrow disease by MRI. Radiographic sensitivity and specificity were 62% and 82% compared to MRI for detection of bone involvement in this patient population. At baseline, bone pain was present in 5 patients and ERT resulted in complete symptomatic remission in all of them. ERT was associated with significant improvement in growth parameters and amelioration of haematological and visceral involvement. CONCLUSION: Symptomatic and radiological skeletal disease is common in children with Gaucher disease in Egypt. MRI is the most accurate technique for detecting early skeletal involvement. There was no correlation between severity of skeletal involvement and GBA genotype. ERT was effective in ameliorating radiological manifestations of skeletal disease and achieving complete remission of bone pain.

Adolescent↗
PubMed2006

Systemic effects of severe trauma on the function and apoptosis of human skeletal cells.

Systemic factors are believed to be pivotal for the development of heterotopic ossification in severely-injured patients. In this study, cell cultures of putative target cells (human fibroblastic cells, osteoblastic cells (MG-63), and bone-marrow stromal cells (hBM)) were incubated with serum from ten consecutive polytraumatised patients taken from post-traumatic day 1 to day 21 and with serum from 12 healthy control subjects. The serum from the polytraumatised patients significantly stimulated the proliferation of fibroblasts, MG-63 and of hBM cells. The activity of alkaline phosphatase in MG-63 and hBM cells was significantly decreased when exposed to the serum of the severely-injured patient. After three weeks in 3D cell cultures, matrix production and osteogenic gene expression of hBM cells were equal in the patient and control groups. However, the serum from the polytraumatised patients significantly decreased apoptosis of hBM cells compared with the control serum (4.3% vs 19.1%, p = 0.031). Increased proliferation of osteoblastic cells and reduced apoptosis of osteoprogenitors may be responsible for increased osteogenesis in severely-injured patients.

Adult↗
PubMed2005

[Influence of sacral fracture on the long-term outcome of pelvic ring injuries].

Initial treatment of pelvic ring fractures with involvement of the iliosacral complex is directed at bleeding control and fixation of the pelvic ring. However, long-term outcome is determined by persisting neurological deficits, malunion of the posterior pelvic ring with low back pain, and urological lesions. Between 1991 and 2000, 173 patients with sacral fractures were treated at our institution. Sacral fractures as part of type B2 ("lateral compression") or type C ("vertical shear") pelvic ring fractures were treated conservatively, if dislocation was less than 1 cm. Fractures with a dislocation of more than 1 cm were treated operatively (n=33, 19%). A total of 112 patients were examined after an average of 4.9 years. Of the 39 patients with primary neurological deficits (35%) only 4 showed complete neurological recovery. Chronic low back pain was rarely observed (n=8, 7%) and only in type C injuries. The low incidence of chronic low back pain justifies conservative treatment of minimally (<1 cm) displaced sacral fractures. Long-term outcome is largely determined by neurological deficits, which persist in 30% of all patients with sacral fractures.

Adolescent↗
PubMed2003

[Embolization of the splenic artery after splenic gunshot wound].

Computed tomography (CT) of the abdomen is a reliable method for evaluation of spleen injuries and has the potential to exclude further abdominal injuries. Blunt and penetrating injuries of the spleen have to be managed immediately due to a high mortality rate. Two therapeutical options, conservative or operative, are currently available. In general, a hemodynamic stable patient, no further injuries of the abdominal organs or the skull as well as no history of abdominal surgery of the abdomen are prerequisites for a non-surgical therapy. Catheter-based angiography gives the possibility to diagnose and to treat injuries of blood vessels of the spleen, which were seen on the CT scans. Gunshots are relatively rare in Europe, but the mortality of such traumas is high. The present case demonstrates a patient with a penetrating gunshot trauma of the left hemiabdomen with a bleeding injury of the spleen. Due to the stable hemodynamic conditions, absence of further injuries of the abdomen or the skull and, because of previous pancreas surgery a non-surgical therapy was chosen, consisting of proximal embolisation of the splenic artery.

Adult↗
PubMed2001

Effects of hyaluronan on engineered articular cartilage extracellular matrix gene expression in 3-dimensional collagen scaffolds.

Hyaluronan (HA) is a component of cartilage matrix with known effects on chondrocytes. We tested the effects of adding HA to 3-dimensional (3-D) collagen. sponges on chondrocyte function in vitro. Bovine articular chondrocytes isolated by collagenase digestion were injected into either collagen or HA/collagen scaffolds comprising different amounts of HA (2, 5, 10, and 14% w/w). Expression of aggrecan and type II collagen genes was measured by gene-specific quantitative competitive reverse transcriptase-polymerase chain reactions, and the extracellular matrix was estimated by histomorphometrical analyses. After 7-day culture, the chondrocytes in 2% (w/w) HA sponges expressed fourfold more mRNA transcripts for type II collagen (p = 0.002) and twofold more mRNA transcripts for aggrecan (p = 0.022) than in control collagen sponges. Furthermore, there was 45% more extracellular matrix in 2% (w/w) HA sponges and 43% less matrix in the 10% (w/w) HA sponges compared with plain collagen sponges (p > 0.05). In sum, a small amount of HA in 3-D collagen scaffolds enhanced chondrogenesis, but a greater amount was inhibitory. This 3-D system represents a novel tool to identify mechanisms by which extracellular matrix molecules influence chondrocyte function. Further, these results show the potential for modifying scaffolds to improve production of engineered cartilage for in vivo applications.

Aggrecans↗
PubMed2001

Effect of RGD coating on osteocompatibility of PLGA-polymer disks in a rat tibial wound.

Osteocompatibility of porous polylactic-glycolic acid (PLGA) disks coated with synthetic peptides was assessed in 5-mm diameter unicortical tibial osseous wounds in rats. The coatings consisted of various ratios of peptides including the tripeptide arginine-glycine-aspartic acid (RGD) and the inactive arginine-glycine-glutamic acid (RGE). When left empty, the tibial wounds healed spontaneously with proliferation of intramedullary woven bone within 1 week. The reactive bone was resorbed, and by 3 weeks, the cortical wound was healed with lamellar bone, and the medullary space was repopulated with marrow. When PLGA disks were implanted there was a delay in repair with reduced bone fill and no bone bridging at 3 weeks. When disks were coated with increasing amounts of RGD peptide, there was a biphasic effect on osteocompatibility and on osseous ingrowth. Evaluation at 10 days showed a dose-dependent increase, with 1.5-fold greater osteocompatibility (p < 0.05) and 1.6-fold more osseous ingrowth into the polymer (p < 0.01) than uncoated disks. With more RGD and with undiluted RGE, osteocompatibility and osseous ingrowth were the same as with uncoated disks. At 3 weeks, there were no significant differences among all the groups. These data indicate that RGD coating enhanced early stages of osteocompatibility and ingrowth.

Animals↗
PubMed2001

Tissue reactions to particles of bone-substitute materials in intraosseous and heterotopic sites in rats: discrimination of osteoinduction, osteocompatibility, and inflammation.

Two rat models were used to characterize tissue-specific reactions to particles of bone-substitute materials: one for osteocompatibility in a healing tibial wound and the other in a heterotopic, subcutaneous site. Small, unicortical tibial wounds in rats healed spontaneously, beginning with the rapid proliferation of intramedullary woven bone. That temporary bone was resorbed by osteoclasts and finally, the cortical wound was healed with lamellar bone and the medullary space was repopulated with marrow. When various particulate materials were implanted into fresh wounds, three types of reactions were observed. (1) Demineralized bone powder (DBP) and non-resorbable calcium phosphate (nrCP) were incorporated into the reactive medullary and cortical bone. (2) Polymethylmetlhacrylate (PMMA) particles were surrounded with a fibrous layer, but did not impair bone healing. (3) Polyethylene (PE) shards and resorbable calcium phosphates (rCPs) were inflammatory and inhibited osseous repair. Subcutaneous sites showed osteoinductive, fibrotic, or inflammatory responses to these materials. Only DBP induced endochondral osteogenesis subcutaneously. The nrCP evoked a fibrous reaction. In contrast, rCPs, PMMA, and PE shards generated inflammatory reactions with each particle being surrounded by fibrous tissue and large multinucleated giant cells. In conclusion, only DBP showed osteoinductive as well as osteocompatible properties. The nrCP was osteocompatible. The rCPs stimulated various degrees of inflammatory responses. PMMA was osteocompatible and did not interfere with the bone healing process. PE was not osteocompatible and generated foreign body reactions in both sites. Use of the two sites distinguishes osteoinductive, osteocompatible, and inflammatory properties of particles of bone-substitute materials.

Absorbable Implants↗
PubMed1989

Coronary vasomotor tone during static and dynamic exercise.

Coronary vasomotion is an important determinant of myocardial perfusion in patients with angina pectoris, and it influences not only normal but also stenotic coronary arteries. The ability of a stenotic coronary artery to change its size is dependent on the presence of a normal musculo-elastic wall segment within the stenosis (i.e., eccentric stenosis). Coronary vasoconstriction of normal and stenotic coronary arteries has been reported by Brown and coworkers (Circulation 1984; 70: 18-24) during isometric exercise. The effect of dynamic exercise on coronary vasomotion was evaluated in one group of 13 patients with ischaemia-like symptoms and normal coronary arteries (group 1) and in a second group of 12 patients with coronary artery disease with exercise-induced angina pectoris (group 2). Luminal area of a normal and a stenotic vessel segment was determined by biplane quantitative coronary arteriography at rest, during supine bicycle exercise and 5 min after administration of 1.6 mg sublingual nitroglycerin. Coronary sinus blood flow was measured in group 1 at rest and after 0.5 mg kg-1 intravenous dipyridamole using coronary sinus thermodilution. Coronary flow reserve was calculated from coronary sinus flow after dipyridamole divided by coronary sinus flow at rest. In group 1, coronary vasodilation of the large (i.e., proximal) and the small (i.e., distal) coronary arteries was observed during exercise in seven patients (subgroup A). However, in the remaining six patients (subgroup B) coronary vasoconstriction of the small arteries (-24%, P less than 0.001) was found during exercise, whereas the large vessels showed coronary vasodilation (+26%, P less than 0.001).(ABSTRACT TRUNCATED AT 250 WORDS)

Angina Pectoris↗
PubMed1984

Plasma cholesteryl sulfate in Friedreich's ataxia.

Alteration of membrane fluidity and anomalies of membrane structural proteins have been suspected in Friedreich's ataxia. Plasma lecithin:cholesterol acyltransferase (LCAT) activity is also lowered in this disease, presumably because of a substrate effect. The membrane-stabilizing effect of cholesteryl sulfate (CS) and its inhibitory effect on LCAT activity prompted us to measure this substance in the plasma of Friedreich's ataxia patients as well as in normal subjects and in patients with Charlevoix-Saguenay disease. Plasma cholesteryl sulfate concentrations were significantly higher in Friedreich's ataxia, with levels above the upper limit of normal in nearly half of the cases. This increase was unrelated to age, sex or plasma cholesterol levels, but closely associated with the severity of the disease and thus considered to be secondary. A similar phenomenon (except the association with severity) was observed in Charlevoix-Saguenay ataxia. Levels also tended to be higher in first-degree relatives of Friedreich cases. The significance of these findings is discussed in the light of recent knowledge and experimental data obtained in this laboratory on rats made deficient in essential fatty acids. The highest concentrations of CS observed in Friedreich's ataxia (1097 micrograms/dL, 6 times the normal mean) was only 25% as high as the concentrations reported to inhibit LCAT activity.

Adolescent↗
PubMed1982

Plasma methyl sterol sulfates in familial hypercholesterolemia after partial ileal bypass.

We demonstrate, in this study, the presence of several unidentified components in the sterol sulfate fraction of familial hypercholesterolemia patients treated with partial ileal bypass surgery. The sterols obtained after solvolysis and derivatization of this fraction had a retention time, on gas-liquid chromatography, intermediate between cholesterol and beta-sitosterol. They were not present in the sterol sulfate fraction obtained from normal subjects, hypercholesterolemic patients, or ileal bypass subjects before surgery or after reanastomosis. The substances isolated from the sterol sulfate fraction were identified by combined gas-liquid chromatography and mass spectrometry to be 24,25-dihydrolanosterol, 4,4-dimethyl-5alpha-cholest-8-en-3beta-ol, 4,4-dimethyl-5alpha-cholest-9(11)-en-3beta-ol, 4alpha-methyl-5alpha-cholest-7-en-3beta-ol, and 4alpha-methyl-5alpha-cholest-8-en-3beta-ol. Their free forms are known to be biosynthetic intermediates in the transformation of lanosterol into cholesterol.

Gas Chromatography-Mass Spectrometry↗
PubMed1981

Cholesteryl sulfate: measurement with beta-sitosteryl sulfate as an internal standard.

A new method is described for the measurement of cholesteryl sulfate (CS) in plasma. Taking advantage of the use of beta-sitosteryl sulfate (SS) as an internal standard it is simpler and more rapid than the methods currently in use. It does not require the use of radioactive isotopes. A potential contamination of the CS band by free cholesterol is excluded by developing the thin-layer chromatography plate on which the sterols are first separated with a second solvent system which pushes free cholesterol to the solvent front without appreciably moving the CS band. The method is sensitive and reproducible and may be advantageous for the screening of large populations. The values obtained for plasma CS concentrations in normal subjects compare well with those measured by more elaborate techniques.

Cholesterol↗
PubMed1979

Skin abnormalities of the back in diastematomyelia.

The presence of congenital skin abnormalities on the back may be associated with a serious underlying spinal anomaly, diastematomyelia. The significance of the progressive development of neurological deficits, due to the presence of a bony or cartilaginous spur in the spinal cord, is stressed. An awareness of this condition should lead to an early diagnosis with a neurological evaluation and, if necessary, prophylactic surgery before irreversible nerve damage develops.

Adolescent↗
PubMed1976

Mandibular fractures: the problem patient.

Of 110 adult patients with 163 mandibular fractures treated at University of Pennsylvania Graduate Hospital from 1964 to 1974, 17% had a history of previous mandibular fractures. All patients' medical records and X-rays were reviewed. Because of the characteristics of this patient population, it seems well suited for discussion as a problem group. Mandibular fractures in this urban area are most commonly caused by blows of a fist and the patient is often intoxicated, which impedes diagnosis, delays treatment, and probably results in further complications from injuries. Patients frequently do not comply with treatment recommendations, do discontinue immobilization, and fail to return for followup, even for removal of intermaxillary fixation. Almost one third had insufficient teeth for fixation; many had poor oral hygiene, and teeth in the fracture line, which led to complications. Osteomyelitis resulting in nonunion and requiring bone grafting occurred in seven patients. Recommended are: simplest fixation methods, minimal amounts of surgery, and as few extractions as possible. Experience from this patient group may be of help whenever a problem patient is seen with a mandibular fracture.

Adolescent↗
PubMedDate not supplied

Control of severe hemorrhage using C-clamp and pelvic packing in multiply injured patients with pelvic ring disruption.

OBJECTIVES: Evaluation of diagnostic and therapeutic workup in multiply injured patients with pelvic ring disruption and hemorrhagic shock. DESIGN: Prospective study. PATIENTS: Twenty consecutive multiply injured patients (ISS: 41.2 +/- 15.3 points) with pelvic ring disruption and hemorrhagic shock. INTERVENTION: A C-clamp was used for primary stabilization of the pelvic ring instability. In patients with persistent or massive hemorrhage, laparotomy and pelvic packing were performed. Consecutive measurements of blood lactate levels during the early period after injury. MAIN OUTCOME MEASUREMENTS: Lactate, mortality. RESULTS: A C-clamp was applied in all patients within 57.4 +/- 30.6 minutes of arrival. Fourteen patients underwent laparotomy with pelvic packing for control of hemorrhage, three patients additional resuscitation thoracotomy (aortic clamping: n = 2). Four patients died of exsanguinating hemorrhage during the first 5.4 +/- 3.3 hours from arrival, one patient because of septic multi-organ failure twenty-three days after injury (total mortality: 5/20; 25 percent). Lactate levels at admission were elevated in all patients (5.1 +/- 2.6 mmol/l). Increased blood lactate levels (4.8 +/- 1.7 mmol/l) (+71 percent; p < 0.05) were observed in survivors undergoing laparotomy compared with survivors without laparotomy (2.8 +/- 1.1 mmol/l). In contrast, hemoglobin (7.0 +/- 2.6 g/dl versus 7.9 +/- 2.2 g/dl) and hematocrit (21.4 +/- 6.4 percent versus 23.2 +/- 6.8 percent) were similar in both groups. In patients who died during the first hours after admission, lactate levels were elevated (8.6 +/- 2.5 mmol/l) compared with survivors (4.2 +/- 1.8 mmol/l) and increased further. CONCLUSIONS: Sequential measurements of blood lactate levels during the early period after injury may provide a more rapid and reliable estimation of true severity of hemorrhage than routinely used parameters. Pelvic packing in addition to pelvic ring fixation with a C-clamp allows for effective control of severe hemorrhage in multiply injured patients with pelvic ring disruption.

Adolescent↗