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Biomedical subjects

H Omran

Publications and source records attributed to H Omran.

At least 19 recordsLinked to original sources
PubMed2003

[Biventricular thrombi dissolution and antibody development with lepirudin therapy].

HISTORY AND ADMISSION FINDINGS: A 50-year-old patient presented with clinical symptoms of heart failure with orthopnoe and edema (NYHA IV). INVESTIGATIONS: Echocardiography revealed a dilated left ventricle with severely reduced left ventricular function and biventricular floating thrombi, due to dilatative cardiomyopathy. TREATMENT AND COURSE: With a heart failure medication clinical symptoms reduced and body weight decreased > 10 kg in 3 weeks. Due to the high-risk constellation, anticoagulation was performed with lepirudin and the biventricular thrombi were dissolved within 17 days. At this point in time, the patient suffered from petechial bleedings, hemoptysis and gross hematuria. Despite breaking anticoagulation and substitution of PPSB with not measurable fibrinogen, subarachnoid hemorrhage occurred leading to exitus letalis. CONCLUSION: Lepirudin is a highly effective anticoagulant, that can induce severe hemorrhagic side effects in individual cases. The present case report demonstrates an immunological reaction as a rare cause with activation of prothrombin and formation of fibrin.

Antibody Formation↗
PubMed2003

Classical infantile spinal muscular atrophy with SMN deficiency causes sensory neuronopathy.

OBJECTIVE: Classic infantile spinal muscular atrophy (SMA) is believed to be a purely motor disorder, affecting neurons of the spinal anterior horn and nuclei of the lower cranial nerves. Other organ malformations or peripheral nerve involvement have been regarded as exclusion criteria for infantile SMA. Whether SMN protein deficiency can also lead to loss of sensory neurons has not been systematically addressed. METHODS: The authors evaluated the sural nerve biopsies of 19 patients with infantile SMA of varying severity. The diagnosis of SMA was confirmed by the presence of a homozygous deletion of the SMN1 gene in all patients. RESULTS: In seven unrelated infants with SMA type I, axonal degeneration of the sural nerve was noted. Five patients showed abnormal sensory conduction, thus prompting sural nerve biopsy. Sural nerves showed different degrees of axonal loss: fiber density ranged from 3.482 to 22.076/mm2 and was markedly reduced in four patients. There was no evidence of primary demyelination: the ratio of total myelinated fiber thickness to axon diameter (g-ratio) was normal in the patients examined. In seven patients with SMA II and five patients with SMA III, no sural nerve alterations were seen, and conduction velocity was normal. In addition to SMN1 gene deletions, homozygous NAIP gene deletions were detected in six out of seven infants with peripheral neuropathy, whereas there was no evidence of a large deletion including the multicopy markers C212 and Ag1-CA in two out of three families tested. CONCLUSIONS: In this series of patients with SMA I through III who underwent sural nerve biopsy, there was significant sensory nerve pathology in severely affected patients with SMA type I, whereas there were no sensory nerve alterations clinically or morphologically in patients with milder SMA type II or III.

Axons↗
PubMed2003

[Syncope with cardiac arrest in a 30-year-old woman].

HISTORY AND ADMISSION FINDINGS: A 30-year-old woman presented with a cardiac arrest, which spontaneously converted into a regular sinus rhythm. The patient had no prior history of cardiovascular diseases. On admission, the cardiovascular clinical examination of this young female was regular. INVESTIGATIONS: On admission, discrete inferior repolarisation changes were documented which were slightly progressive during the next day. Electrophysiologic examination of the heart was regular, angiography showed a coronary fistula and an atypical descense of the circumflex artery from the right coronary artery. DIAGNOSIS: Right coronary artery fistula to the right ventricle. Atypical descense of the circumflex artery from the right coronary artery. TREATMENT AND COURSE: During hospitalisation no arrhythmias or pauses were revealed. The patient underwent cardiac pacemaker implantation. We plan a transcatheter closure of the fistula. CONCLUSIONS: Coronary fistulas are a rare cause of cardiac arrhythmias. Angiography performed at an early stage is therefore crucial in the initial assessment of a young adult presenting with cardiac arrest and subsequent changes in the ECG, as a small proportion may have coronary vascular abnormalities. The occlusion of the fistula by transcatheter or cardiac surgery is appropriate, especially as the risks of the interventions are limited.

Adult↗
PubMed2003

Atrial fibrillation: relation between clinical risk factors and transoesophageal echocardiographic risk factors for thromboembolism.

OBJECTIVE: To correlate clinical risk factors for thromboembolism with transoesophageal echocardiography (TOE) markers of a thrombogenic milieu. DESIGN: Clinical risk factors for thromboembolism and TOE markers of a thrombogenic milieu were assessed in consecutive patients with non-rheumatic atrial fibrillation. The following TOE parameters were assessed: presence of spontaneous echo contrast, thrombi, and left atrial appendage blood flow velocities. A history of hypertension, diabetes mellitus, or thromboembolic events, patient age > 65 years, and chronic heart failure were considered to be clinical risk factors for thromboembolism. SETTING: Tertiary cardiac care centre. PATIENTS: 301 consecutive patients with non-rheumatic atrial fibrillation scheduled for TOE. RESULTS: 255 patients presented with clinical risk factors. 158 patients had reduced left atrial blood flow velocities, dense spontaneous echo contrast, or both. Logistic regression analysis showed that a reduced left ventricular ejection fraction and age > 65 years were the only independent predictors of a thrombogenic milieu (both p < 0.0001). The probability of having a thrombogenic milieu increased with the number of clinical risk factors present (p < 0.0001). 17.4% of the patients without clinical risk factors had a thrombogenic milieu whereas 41.2% of the patients presenting one or more clinical risk factors had none. CONCLUSION: There is a close relation between clinical risk factors and TOE markers of a thrombogenic milieu. In addition, TOE examination allows for the identification of patients with a thrombogenic milieu without clinical risk factors.

Aged↗
PubMed2002

[Cardiac resynchronization therapy by biventricular pacing. How many patients with left ventricular dysfunction are eligible?].

BACKGROUND AND OBJECTIVE: Cardiac resynchronization therapy by multisite biventricular pacing presents an additive therapeutic option in the treatment of severe congestive heart failure. The objective of the study was to evaluate how many patients with left ventricular dysfunction may potentially benefit from this therapy. METHODS: A total of 975 patients were screened for the prevalence of left ventricular dysfunction. Patients with a left ventricular ejection fraction (LVEF) <45 % were included into the investigation. Potential benefit of biventricular pacing was presumed in the presence of: LVEF < 35 %, severe heart failure (NHYA class III or IV), intrinsic left bundle branch block pattern with QRS interval > 150 ms and the absence of atrial fibrillation in the last 3 months before study inclusion. RESULTS: In 203 patients (168 male, 35 female, mean age: 64 +/- 11) an LVEF <45 % was found. A total of 12 of these patients (6 %) or 12 of 113 patients (11 %) with an LVEF <35 % were identified as appropiate candidates for biventricular resynchronization therapy. CONCLUSIONS: Cardiac biventricular pacing currently serves as a therapeutic option for a relatively small subgroup of patients with left ventricular dysfunction. Focusing on estimations that the incidence of heart failure in Germany amounts to more than 100.000 cases per year our results suggest that after all more than 6.000 patients per year may potentially benefit from electric resynchronization therapy. This number may increase substantially if prospective studies can prove that patients with heart failure and atrial fibrillation or left ventricular conduction delay due to univentricular pacing also benefit from cardiac resynchronization therapy.

Aged↗
PubMed2002

Local intra-arterial thrombolysis in the carotid territory: does recanalization depend on the thromboembolus type?

Little is known about whether recanalization of carotid territory occlusions by local intra-arterial thrombolysis (LIT) depends on the type of the occluding thromboembolus. We retrospectively analysed the records of 62 patients with thromboembolic occlusions of the intracranial internal carotid artery (ICA) bifurcation or the middle cerebral artery who were undergoing LIT with urokinase within 6 h of symptom onset. We determined the influence of thromboembolus type (according to the TOAST criteria), thromboembolus location, leptomeningeal collaterals, time interval from onset of symptoms to onset of thrombolysis, and patient's age on recanalization. The thromboembolus type was atherosclerotic in six patients, cardioembolic in 29, of other determined etiology in four, and of undetermined etiology in 23 patients. Thirty-three (53%) thromboembolic occlusions were recanalized. The thromboembolus location but not the TOAST stroke type nor other parameters affected recanalization. In the TOAST group of patients with cardioembolic occlusions recanalization occurred significantly less frequently when transoesophageal echocardiography showed cardiac thrombus. The present study underlines the thromboembolus location as being the most important parameter affecting recanalization. The fact that thromboembolic occlusions originating from cardiac thrombi had a lower likelihood of being resolved by thrombolysis indicates the thromboembolus type as another parameter affecting recanalization.

Carotid Artery Thrombosis↗
PubMed2002

[Submillimeter 3D coronary MR angiography with real-time navigator correction in 107 patients with suspected coronary artery disease].

PURPOSE: To evaluate the clinical value of high-resolution coronary MR angiography (coronary MRA) in a large group of patients with suspected coronary artery disease. METHODS AND MATERIAL: 107 patients with suspected coronary artery disease underwent free-breathing coronary MRA (Intera, 1.5 T, Philips Medical Systems). To compensate for artefacts due to respiratory motion, a right hemidiaphragmatic navigator with real time-time slice correction was used. An ECG-gated, fat-suppressed, 3D segmented-k-space gradient echo sequence (in plane resolution 0.70 x 0.79 mm(2)) was used. Cardiac catheterization with selective coronary angiography was performed in all patients. Visualization of the coronary arteries (CA) was qualitatively assessed using a four-point grading scale. RESULTS: Image quality of grade 1 was achieved in 24 %, grade 2 in 48 %, grade 3 in 24 % and grade in in 4 % of patients. Based on an evaluation of the coronary MRAs of all patients (n = 107) sensitivity and specificity for the detection of stenoses > 60 % in the proximal and middle main coronary arteries were 74 % and 63 %, respectively. In coronary MRAs with good quality [grade 1 and 2, n = 77/107 (72 %)] sensitivity and specificity for the detection of coronary stenoses were 88 % and 91 %, respectively. CONCLUSION: Submillimeter 3D coronary MRA with real-time navigator correction allows high quality imaging of the proximal and middle main coronary arteries with good sensitivity and specificity for detection of stenoses > 50 % in selected patients. However, in about 28 % of patients image quality is severely impaired.

Adult↗
PubMed2002

[Stress cine MRI for detection of coronary artery disease].

Stress testing is the cornerstone in the diagnosis of patients with suspected coronary artery disease (CAD). Stress echocardiography has become a well-established modality for the detection of ischemia-induced wall motion abnormalities. However, display and reliable interpretation of stress echocardiography studies are user-dependent, the test reproducibility is low, and 10 to 15 % of patients yield suboptimal or non-diagnostic images. Due to its high spatial and contrast resolution, MRI is known to permit an accurate determination of left ventricular function and wall thickness at rest. Early stress MRI studies provided promising results with respect to the detection of CAD. However, the clinical impact was limited due to long imaging time and problematic patient monitoring in the MRI environment. Recent technical improvements - namely ultrafast MR image acquisition - led to a significant reduction of imaging time and improved patient safety. Stress can be induced by physical exercise or pharmacologically by administration of a beta1-agonist (dobutamine) or vasodilatator (dipyridamole and adenosine). The best developed and most promising stress MRI technique is a high-dose dobutamine/atropine stress protocol (10, 20, 30, 40 microgram/kg/min; optionally 0.25-mg fractions of atropine up to maximal dose 1 mg). Severe complications (myocardial infarction, ventricular fibrillation and sustained tachycardia, cardiogenic shock) may be expected in 0.25 % of patients. Currently, data of three high-dose dobutamine stress MRI studies are available, revealing a good sensitivity (83 - 87 %) and specificity (83 - 86 %) in the assessment of CAD. The direct comparison between echocardiography and MRI for the detection of stress-induced wall motion abnormalities yielded better results for dobutamine-MRI in terms of sensitivity (86.2 % vs. 74.3 %; p < 0.05) and specificity (85.7 % vs. 69.8 % p < 0.05) as compared to dobutamine stress echocardiography. The superior results of MRI can mainly be explained by the better image quality with sharp delineation of the endocardial and epicardial borders. Currently, stress MRI is already a realistic clinical alternative for the non-invasive assessment of CAD in patients with impaired image quality in echocardiography.

Adenosine↗
PubMed2002

Candidate gene analysis of KIAA0678 encoding a DnaJ-like protein for adolescent nephronophthisis and Senior-Løken syndrome type 3.

Nephronophthisis (NPH), an autosomal recessive cystic kidney disease, causes progressive renal failure. The gene for adolescent nephronophthisis (NPHP3) has been mapped to chromosome 3q21-->q22. Senior-Løken syndrome (SLS) describes the association of NPH and Leber congenital amaurosis. Recently a locus for Senior-Løken syndrome (SLSN3) has been localized on chromosome 3q21-->q22 containing the whole critical NPHP3 region. Within the critical NPHP3/SLSN3 region we identified the gene KIAA0678 encoding a DnaJ-like protein. KIAA0678 was considered a good functional candidate gene for NPH3 and SLS3, because molecular cha- perones are involved in the etiology of renal and retinal diseases. Analysis of the genomic structure of KIAA0678 identified 25 exons. For mutational analysis all exons and intron-exon boundaries were amplified and directly sequenced. Affected individuals of two NPH3 families and one SLS family with haplotypes indicative for homozygosity by descent for the NPHP3/SLSN3 locus were studied. No mutation in KIAA0678 was detected. We conclude, KIAA0678 most likely is not responsible for NPH and SLS in the patients studied.

Adolescent↗
PubMed2002

Identification of the human ortholog of the t-complex-encoded protein TCTE3 and evaluation as a candidate gene for primary ciliary dyskinesia.

Primary ciliary dyskinesia (PCD) is a heterogeneous autosomal recessive disease that is caused by impaired ciliary and flagellar functions. About 50% of PCD patients show situs inversus, denoted as Kartagener syndrome. In most cases, axonemal defects in cilia and sperm tails can be demonstrated by electron microscopy, i.e. PCD patients often lack inner and/or outer dynein arms in their sperm tails and cilia, supporting the hypothesis that mutations in dynein genes may cause PCD. In order to identify novel PCD genes we have isolated the human ortholog of the murine TCTE3 gene. The human TCTE3 gene encodes a dynein light chain and shares high similarity to dynein light chains of other species. The TCTE3 gene is expressed in tissues containing cilia or flagella, it is composed of four exons and located on chromosome 6q25-->q27. To elucidate the role of TCTE3 as a candidate gene for PCD a mutational analysis of thirty-six PCD patients was performed. We detected five polymorphisms in the coding sequence and in the 5' UTR of the TCTE3 gene. In one patient a heterozygous nucleotide exchange was identified resulting in an arginine to isoleucine substitution at the amino acid level. However, this exchange was also detected in one control DNA. Our results indicate that mutations in the TCTE3 gene are not a main cause of primary ciliary dyskinesia.

Amino Acid Sequence↗
PubMed2001

The impact of emission power on the destruction of echo contrast agents and on the origin of tissue harmonic signals using power pulse-inversion imaging.

The purpose of this study was to determine the impact of emission power on ultrasound (US)-induced destruction of echocontrast microbubbles during real-time power pulse inversion imaging (PPI) in myocardial contrast echocardiography (MCE) and to evaluate the magnitude of noncontrast PPI signals arising from myocardial tissue at variable emission power to define the cut-off emission power for optimal MCE using low power technologies. In vitro studies were performed in a flow phantom using Optison, Definity and AFO 150. PPI signal intensity during real-time imaging at 27 Hz was compared with intermittent imaging at 0.1 Hz to evaluate bubble destruction at variable emission power (MI: 0.09 to 1.3). In healthy volunteers, PPI signal intensities during constant infusion of Optison(R) was studied in real-time PPI 22 HZ and during intermittent imaging triggered end-systolic frames every, every 3rd and every 5th cardiac cycle. In addition, the impact of emission power on nonlinear PPI signals from myocardial structures was studied. In vitro, there was a 40% decrease of real-time PPI signal intensity for Optison and AFO 150 at lowest emission power (0.09), whereas no signal loss was observed for Definity. Increase of emission power resulted in a faster decay for Optison(R) and AFO 150 as compared to Definity. In vivo, real-time PPI during continuous infusion of Optison(R) resulted in a 40% decrease of myocardial signal intensity as compared to intermittent imaging every 5th cardiac cycle, even at lowest possible emission power (mechanical index = 0.09). There was a strong positive relationship between MI and noncontrast myocardial PPI signals in all myocardial segments. PPI signal intensity was found to be lower than 1 dB only for extremely low emission power (MI < 0.2). Destruction of microbubbles during real-time imaging by use of PPI at low emission power varies considerably for different echo contrast agents. However, bubble destruction and the onset of tissue harmonic signals focus the use of real-time perfusion imaging to very low emission power.

Adult↗
PubMed2001

Prevalence of left atrial chamber and appendage thrombi in patients with atrial flutter and its clinical significance.

OBJECTIVES: The study was done to assess the prevalence of left atrial (LA) chamber and appendage thrombi in patients with atrial flutter (AFl) scheduled for electrophysiologic study (EPS), to evaluate the prevalence of thromboembolic complications after transesophageal echocardiographic (TEE)-guided restoration of sinus rhythm and to evaluate clinical risk factors for a thrombogenic milieu. BACKGROUND: Recent studies showed controversial results on the prevalence of atrial thrombi and the risk of thromboembolism after restoring sinus rhythm in patients with AFl. METHODS: Between 1995 and 1999, patients with AFl who were scheduled for EPS were included in the study. After transesophageal assessment of the left atrial appendage and exclusion of thrombi, an effective anticoagulation was initiated and patients underwent EPS within 24 h. RESULTS: We performed 202 EPSs (radiofrequency catheter ablation, n = 122; overdrive stimulation, n = 64; electrical cardioversion, n = 16) in 139 consecutive patients with AFl. Fifteen patients with a thrombogenic milieu were identified. All of them had paroxysmal atrial fibrillation (AF). Transesophageal echocardiography revealed LA thrombi in two cases (1%). After EPS no thromboembolic complications were observed. Diabetes mellitus, arterial hypertension and a decreased left ventricular ejection fraction were found to be independent risk factors associated with a thrombogenic milieu. CONCLUSIONS: The findings of a low prevalence of LA appendage thrombi (1%) in patients with AFl and a close correlation between a history of previous embolism and paroxysmal AF support the current guidelines that patients with pure AFl do not require anticoagulation therapy, whereas patients with AFl and paroxysmal AF should receive anticoagulation therapy. In addition, the presence of clinical risk factors should alert the physician to an increased likelihood for a thrombogenic milieu.

Aged↗
PubMed2001

Mutation of BSND causes Bartter syndrome with sensorineural deafness and kidney failure.

Antenatal Bartter syndrome (aBS) comprises a heterogeneous group of autosomal recessive salt-losing nephropathies. Identification of three genes that code for renal transporters and channels as responsible for aBS has resulted in new insights into renal salt handling, diuretic action and blood-pressure regulation. A gene locus of a fourth variant of aBS called BSND, which in contrast to the other forms is associated with sensorineural deafness (SND) and renal failure, has been mapped to chromosome 1p. We report here the identification by positional cloning, in a region not covered by the human genome sequencing projects, of a new gene, BSND, as the cause of BSND. We examined ten families with BSND and detected seven different mutations in BSND that probably result in loss of function. In accordance with the phenotype, BSND is expressed in the thin limb and the thick ascending limb of the loop of Henle in the kidney and in the dark cells of the inner ear. The gene encodes a hitherto unknown protein with two putative transmembrane alpha-helices and thus might function as a regulator for ion-transport proteins involved in aBS, or else as a new transporter or channel itself.

Animals↗
PubMed2001

Establishing an algorithm for molecular genetic diagnostics in 127 families with juvenile nephronophthisis.

BACKGROUND: Juvenile nephronophthisis (NPH1), an autosomal recessive cystic disease of the kidney, represents the most common genetic cause of end-stage renal disease in the first two decades of life. On the basis of identification of the gene (NPHP1) defective in NPH1 and the presence of homozygous deletions of NPHP1 in the majority of NPH1 patients, molecular genetic diagnosis for NPH1 is now possible. Molecular genetic testing offers the only method for definite diagnosis of NPH1 and avoids invasive diagnostic measures like renal biopsy. METHODS: We examined 127 families (204 patients) with the presumed diagnosis of NPH using molecular genetic diagnostic techniques. In 68 families, renal biopsy was performed and was consistent with NPH, and in 61 families, there was more than one affected child ("multiplex families"). RESULTS: In 74 families (115 patients), there was proof of the diagnosis of NPH1 by detection of a homozygous deletion of the NPHP1 gene, and in 5 families a heterozygous deletion in combination with a point mutation in NPHP1 was demonstrated. Furthermore, for 16 families, NPH1 was excluded with high likelihood by linkage analysis, and for 20 families by detection of heterozygosity for two newly identified polymorphic markers within the deletion region. In 5 of the remaining 12 families, which were noninformative for these markers, fluorescence in situ hybridization did not detect any further heterozygous deletions. CONCLUSIONS: The diagnosis of NPH1 was proven by molecular genetic techniques in 62% of families with one or more children with the presumed diagnosis of NPH. We present evidence that there is a fourth locus for NPH, since only 6 of the 26 multiplex families in whom the diagnosis of NPH1 was excluded were compatible with linkage to other loci for NPH. On the basis of the presented data, we propose an algorithm for molecular genetic diagnostics in NPH.

Adaptor Proteins, Signal Transducing↗
PubMed2001

Blood flow assessment by ultrasound-induced destruction of echocontrast agents using harmonic power Doppler imaging: which parameters determine contrast replenishment curves?

OBJECTIVE: To evaluate the feasibility of flow determinations by contrast replenishment using harmonic power Doppler imaging (H-PDI). BACKGROUND: The application of indicator dilution principles on contrast echocardiography is limited by numerous methodical problems. Recently, a new method was introduced that relies on ultrasound-mediated microbubble destruction and evaluation of the contrast replenishment. METHODS: Definity, a perfluorocarbon-derived contrast agent under development, was continuously infused into a steady flow phantom and H-PDI registrations were performed within a silicone tube (d = 8 mm). Replenishment interval between destruction and imaging frame was varied from 0.04-2 seconds. Nonlinear curve fitting was performed using an exponential mathematical model. RESULTS: Strong linear correlation between contrast dose and maximum signal intensity as well as between flow and the slope variable beta of the replenishment curve was found for all settings (r > 0.96). Maximum signal intensity and contrast replenishment rate were found to be a function of emission power and were significantly influenced by depth and focus position. CONCLUSION: The feasibility of flow assessment using replenishment curves obtained by H-PDI was demonstrated. However, in experimental conditions, flow analysis was severely influenced by ultrasound system settings and imaging conditions such as emission power, sound field geometry, and investigation depth. For a clinical use of this promising approach, algorithms that take specific system settings and imaging conditions into account have to be found. Imaging modalities that enable a most homogeneous scan field are best suited for the assessment of contrast replenishment.

Contrast Media↗