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Biomedical subjects

J T Anim

Publications and source records attributed to J T Anim.

At least 19 recordsLinked to original sources
PubMed2007

Serum prostate-specific antigen levels in Middle Eastern men with subclinical prostatitis.

OBJECTIVE: To investigate the influence of subclinical or histologically diagnosed prostatitis on serum prostate-specific antigen (PSA) in patients investigated for prostatic disease in Kuwait. MATERIALS AND METHODS: Serum PSA was assayed in patients investigated for prostatic disease in Mubarak Al-Kabeer Hospital, Kuwait, between December 2002 and December 2004. These included patients undergoing transrectal ultrasound with needle biopsy of the prostate gland and those who were treated with transurethral resection of the prostate or retropubic prostatectomy. The tissue was evaluated for prostatitis as well as the underlying disease, and the type and severity of prostatitis were compared with levels of serum PSA. RESULTS: Of the 331 tissue specimens, 18 (5.4%) did not show prostatitis, while 233 (70.4%) with benign prostate and 80 (24.2%) with malignant prostate disease showed prostatitis. Of 270 men with known serum PSA levels, 198 and 72 had benign and malignant prostate disease, respectively. Of the 198, 77 (41%) with benign prostate disease and prostatitis and of the 72, 52 (76%) with malignant prostate disease and prostatitis had serum PSA levels >10 ng/ml. CONCLUSION: The data showed that although raised serum PSA is more commonly associated with prostate cancer, subclinical prostatitis is a significant source of high serum PSA in over 40% of men in Kuwait. That local factors may obscure the usefulness of serum PSA as a screening tool suggests the need for a locally applicable paradigm to identify prostate cancer.

Adult↗
PubMed2006

Relationship between serum prostate specific antigen and the pattern of inflammation in both benign and malignant prostatic disease in Middle Eastern men.

To determine the effect of prostatitis on serum prostate specific antigen in the diagnosis of prostate cancer in Middle Eastern men, H&E-stained sections of all consecutive prostate specimens were reviewed for diagnosis (malignant or benign) and pattern of inflammation. Inflammation was categorized into acute, active chronic and chronic inactive and graded semi-quantitatively according to previously published criteria. Results were correlated with serum PSA obtained from patients' records. Of 513 prostate specimens reviewed; 435 (84.8%) were benign and 78 (15.2%) were malignant. Chronic inactive prostatitis was present in 259 (204 benign, 55 malignant) and active chronic prostatitis in 221 (204 benign, 17 malignant). Acute prostatitis alone was not observed and prostatitis was absent in 33 (27 benign, 6 malignant). There was no significant difference in the prevalence of inactive chronic prostatitis between benign and malignant specimens (p < 0.071), but active chronic prostatitis was more prevalent in benign specimens (p < 0.001). Increasing serum PSA was observed for increasing grades of both inactive and active chronic prostatitis in both benign and malignant disease. Prostate cancer showed higher serum PSA levels than benign, at different cut-off points (4 ng/ml = p < 0.0001; 8 ng/ml = p < 0.0001; 12 ng/ml = p < 0.0001). However, significant numbers of patients with benign prostate biopsies presented with PSA above 12 ng/ml (82/260 = 32%). We conclude that active chronic prostatitis is common in Middle Eastern men with benign prostatic disease and a significant number of these present with very high PSA levels, some over 300 ng/ml.

Acute Disease↗
PubMed2006

Angiotensin-(1-7) prevents development of severe hypertension and end-organ damage in spontaneously hypertensive rats treated with L-NAME.

We examined the influence of chronic treatment with ANG-(1-7) on development of hypertension and end-organ damage in spontaneously hypertensive rats (SHR) chronically treated with the nitric oxide synthesis inhibitor L-NAME (SHR-L-NAME). L-NAME administered orally (80 mg/l) for 4 wk significantly elevated mean arterial pressure (MAP) compared with SHR controls drinking regular water (269 +/- 10 vs. 196 +/- 6 mmHg). ANG-(1-7) (24 microg x kg(-1) x h(-1)) or captopril (300 mg/l) significantly attenuated the elevation in MAP due to L-NAME (213 +/- 7 and 228 +/- 8 mmHg, respectively), and ANG-(1-7) + captopril completely reversed the L-NAME-dependent increase in MAP (193 +/- 5 mmHg). L-NAME-induced increases in urinary protein were significantly lower in ANG-(1-7)-treated animals (226 +/- 6 vs. 145 +/- 12 mg/day). Captopril was more effective (96 +/- 12 mg/day), and there was no additional effect of captopril + ANG-(1-7) (87 +/- 5 mg/day). The abnormal vascular responsiveness to endothelin-1, carbachol, and sodium nitroprusside in perfused mesenteric vascular bed of SHR-L-NAME was improved by ANG-(1-7) or captopril, with no additive effect of ANG-(1-7) + captopril. In isolated perfused hearts, recovery of left ventricular function from 40 min of global ischemia was significantly better in ANG-(1-7)- or captopril-treated SHR-L-NAME, with additive effects of combined treatment. The beneficial effects of ANG-(1-7) on MAP and cardiac function were inhibited when indomethacin was administered with ANG-(1-7), but indomethacin did not reverse the protective effects on proteinuria or vascular reactivity. The protective effects of the ANG-(1-7) analog AVE-0991 were qualitatively comparable to those of ANG-(1-7) but were not improved over those of captopril alone. Thus, during reduced nitric oxide availability, ANG-(1-7) attenuates development of severe hypertension and end-organ damage; prostaglandins participate in the MAP-lowering and cardioprotective effects of ANG-(1-7); and additive effects of captopril + ANG-(1-7) on MAP, but not proteinuria or endothelial function, suggest common, as well as different, mechanisms of action for the two treatments. Together, the results provide further evidence of a role for ANG-(1-7) in protective effects of angiotensin-converting enzyme inhibition and suggest dissociation of factors influencing MAP and those influencing end-organ damage.

Angiotensin I↗
PubMed2005

Utility of volume adjusted prostate specific antigen density in the diagnosis of prostate cancer in Arab men.

BACKGROUND: This study was undertaken to assess the utility of prostate specific antigen (PSA) and PSA density (PSAD) in discriminating between benign and malignant prostate disease in the Kuwaiti Arab population. METHODS: A total of 100 consecutive patients suspected of having prostate cancer because of serum PSA > 4 ng/ml, or detection of a prostatic nodule on rectal examination were further investigated by determination of PSAD, TRUS of prostate, sexant prostatic biopsy and histological analysis to establish the correct diagnosis. Other diagnostic measures included the determination of the area under the receiver operating characteristic (ROC) curve, sensitivity and specificity. RESULTS: Of the 100 prostate biopsies that were performed, 33 cases were confirmed to be prostate cancer and 67 were described as benign lesions comprising benign prostatic hyperplasia (BPH) with or without prostatitis. The age range for patients with prostate cancer was 42-90 years, and 52-90 years for those without prostate cancer. The mean prostate volume was 58.82 cc (range 9-177 cc) and 62.60 cc (range 15-140 cc), the mean PSA value was 36.65 ng/ml (range 5.8-200 ng/ml) and 16.49 ng/ml (range 1.4-46.0 ng/ml), while the mean PSAD was 0.92 (range 0.046-5.714) and 0.452 (range 0.034-2.294) for patients with prostate cancer and patients without prostate cancer respectively. Patients with PSA less than 4 ng/ml (3 cases) all had benign prostate lesions, and 7 cases with PSA more than 50 ng/ml all had prostate cancer and were excluded because values above 50 ng/ml have close to 100% specificity for prostate cancer. Further analysis was done on the remaining 90 cases which were patients with a PSA between 4 and 50 ng/ml. The discriminating power of serum PSA for detecting prostate cancer as estimated by the area under ROC was 0.686 while that for PSAD was 0.732. The maximum likelihood for a positive PSA was at a PSAD cut-off point of 0.32. For the PSA cut-off point of l0 ng/ml, the sensitivity was 80%, and specificity was 42.2%. For the PSAD cut-off point of 0.32, the sensitivity was 58% and the specificity 76.6%. CONCLUSIONS: Determination of PSAD is not a useful adjunct to serum PSA values in the range of 10-50 ng/ ml in our population. PSAD value less than 0.32 with PSA less than l0 ng/ml strongly suggests benign disease.

Adult↗
PubMed2005

Pleomorphic adenoma of salivary gland: to what extent does fine needle aspiration cytology reflect histopathological features?

OBJECTIVE: The histological diversity encountered in pleomorphic adenoma may cause diagnostic difficulty in fine needle aspiration (FNA) cytology due to limited and selective sampling. The present study based on 25 histologically confirmed pleomorphic adenoma cases attempts to find out to what extent FNA cytology reflects the histopathological features. METHODS: May-Grunwald-Giemsa and Papanicolaou stained smears, and haematoxylin and eosin stained paraffin sections of 25 pleomorphic adenomas of parotid and submandibular glands were reviewed. The cellularity, which was assessed in a sliding scale of 1+ to 4+, and proportions of epithelial to mesenchymal components in FNA smears and histology was determined and compared. The frequency of morphological features such as squamous metaplastic cells, cells with oncocytic change, acinus formation, mucus globules, papilla formation, giant cells, myxoid and chondroid matrix as well as specific nuclear features was compared between the two diagnostic methods, and the statistical significance was determined using Fisher's exact test of probability. RESULTS: There was complete concordance between cytology and histology with respect to overall cellularity in 14 (56.0%) cases and in the proportions of epithelial to mesenchymal components in 13 (52.0%). Epithelial cells and myxoid matrix were present in all cases. There was no significant difference between smear and tissue section with respect to frequency of squamous metaplasia, oncocytic change, acinus formation, papilla formation, mucus globules, giant cells, nuclear pleomorphism, nuclear chromatin pattern, and mitotic figures. Morphological parameters that were significantly higher in FNAC compared with histology included intranuclear cytoplasmic inclusions (36.0% versus 8.0%, p = 0.0374), nuclear grooves (84.0% versus 48.0%, p = 0.0090), and reniform nuclei (20.0% versus 0.0%, p = 0.0502). Chondroid matrix was the only parameter which was significantly more common in histology than in cytology (44.0% versus 4.0%, p = 0.019). CONCLUSION: FNA cytology demonstrates well most of the histological features of pleomorphic adenoma of salivary gland and may be considered a useful tool in initial assessment of the tumour.

Adenoma, Pleomorphic↗
PubMed2005

Morphological responses of the rabbit testis to ischemic/reperfusion injury due to torsion.

OBJECTIVE: To investigate the morphological effects of free radical injury on spermatogenic cells in both testes of the rabbit experimental model of testicular torsion. MATERIALS AND METHODS: The left testes of 8 peripubertal NZW rabbits (3-6 months) were subjected to 0, 15, 30, 60, 90, 120, and 180 min of ischemia by applying a clamp to the spermatic cord, followed by reperfusion. Another set of 8 rabbits was subjected to 60 min of ischemia and administered antioxidants (acetylsalicylic acid, ascorbic acid, allopurinol, quercetin, superoxide dismutase) before reperfusion. Both testes of 4 animals per group were harvested at 24 h and the remaining 4 at 3 months. Johnsen scores for spermatogenic activity and other changes were assessed histologically and these were compared with testicular malondialdehyde (MDA), a measure of free radical damage, assayed on testicular homogenates using the thiobarbiturate method. RESULTS: In the 24-hour reperfusion group, apoptotic bodies and giant cells were more prominent in the seminiferous tubules of the left testes compared to the right, and were maximal after 90 min. In the 3-month reperfusion group, giant cells were absent, and apoptotic bodies were reduced in both testes. Testicular MDA showed an increase only in the left testes in the 24-hour reperfusion group, while the 3-month group showed increased MDA levels in both testes, but more on the left. The Johnsen score fell only to 8.0 in the left testes in the 24-hour reperfusion group, but dropped to 2.3 in the 3-month reperfusion group. Only in the 3-month reperfusion group, did antioxidant-treated animals show a fall in Johnsen scores in the left testes, regardless of the type of antioxidant. CONCLUSION: These findings confirm a role for reactive oxygen species (ROS) in damage to spermatogenic cells in both the ipsilateral and contralateral testes following torsion, with longer term effects in the torted testis. Currently available antioxidants do not provide any significant long-term protection against morphological damage to the testis by ROS generated in testicular torsion.

Animals↗
PubMed2003

Effect of castration on acetyl salicylic acid metabolism in rabbits.

OBJECTIVES: The biotransformation of acetyl salicylic acid (ASA) differs within species, and gender differences have been documented and attributed to the effect of sex hormones. Castration remains a standard therapy for men with advanced prostate cancer. We studied the effect of castration on the metabolism of ASA in rabbits to find out whether the metabolism of ASA is adversely affected after castration. METHODS: ASA in doses of 12.5, 25, and 50 mg/kg body weight was given intravenously to male and female prepubertal and adult rabbits, castrated adult male rabbits, and castrated male rabbits given testosterone (3 animals per group). Blood samples were collected at 0, 10, 30, 60, 120, and 180 minutes. The high-performance liquid chromatography method was used for the quantitation of salicylic acid (SA) in serum. The percentage of SA not metabolized was determined by comparing the serum level at 10 and 180 minutes for each group. RESULTS: At a dose of 50 mg/kg in the adult rabbits, the mean +/- SD of SA in serum at 10 and 180 minutes was 146.54 +/- 29.54 microg/mL and 19.12 +/- 5.93 microg/mL for males, 158.25 +/- 6.70 microg/mL and 33.24 +/- 2.78 microg/mL for females, 229.72 +/- 47.85 microg/mL and 44.33 +/- 5.64 microg/mL for castrated male rabbits, and 170.88 +/- 12.03 microg/mL and 68.1 +/- 37.54 microg/mL for castrated male rabbits given testosterone, respectively. Also, at 180 minutes, the percentage of SA not metabolized in adult male rabbits was 12.82% +/- 1.65% compared with 21.04% +/- 2.14% (P <0.01) in adult females, 19.53% +/- 1.73% (P <0.01) in castrated adult male rabbits, and 38.95% +/- 19.48% (P <0.001) in castrated male rabbits given testosterone. At all doses of ASA, the serum SA concentration in male and female prepubertal rabbits was not significantly different for each time point. CONCLUSIONS: These results indicate that male rabbits are able to metabolize ASA faster than are females. After castration, this ability is significantly decreased. If these experimental results are confirmed in humans, men who are undergoing hormonal manipulation for advanced prostate cancer and who require high-dose ASA, such as in the treatment of stroke or rheumatoid arthritis or as an antioxidant, may need lower doses to reduce the possible toxic effects of ASA.

Androgen Antagonists↗
PubMed2003

High serum prostate-specific antigen levels in the absence of prostate cancer in Middle-Eastern men: the clinician's dilemma.

OBJECTIVE: To investigate the common causes of total serum prostate-specific antigen (PSA) values of> 10 ng/mL in an Arab population, as in the USA and Europe the risk of prostate cancer is considered high in men with such PSA levels. PATIENTS AND METHODS: Serum total PSA was measured in men presenting to our hospital as part of the investigation for prostate cancer screening and/or in elderly men with prostatism. Men with a serum PSA level of> 10 ng/mL were further investigated by transrectal ultrasonography (TRUS) of the prostate and biopsy of suspicious lesions for histological diagnosis. In addition, the percentage of free PSA, PSA velocity and PSA density were determined. All the patients included in this study were men of Arab origin residing in Kuwait. RESULTS: In all, 1700 men (mean age 55.6 years, range 35-94) were assessed; of these, 161 had a serum PSA of> 10 ng/mL, attributable to benign prostatic hyperplasia (BPH) in 110 (68%), BPH with histological features of prostatitis in 33 (21%) and prostate cancer in 18 (11%). TRUS of the prostate in 143 of the 161 men with either BPH or BPH with prostatitis showed varying grades of intraprostatic calcifications in 22 (15%). Both PSA density and percentage free PSA did not contribute to determining the causes of total PSA levels of> 10 ng/mL. There was a progressive decline in PSA in all patients with BPH and prostatitis, except one who at re-biopsy had prostate cancer (T1N0M0, G1). CONCLUSION: Total PSA values of> 10 ng/mL in Arab men may be a result of BPH, BPH with prostatitis or prostate cancer, in that order. A gradual decline in total PSA (decreased PSA velocity) with time to < 4 ng/mL often confirms the diagnosis of BPH with prostatitis. The percentage of free PSA and PSA density may not be helpful in diagnosing prostate cancer with certainty in these patients. Compared with Caucasians in the USA and Europe, BPH and BPH with prostatitis appear to be more frequent causes of serum PSA levels of> 10 ng/mL in Arab men.

Adult↗
PubMed2002

Cyclooxygenase-2 inhibition and experimental colitis: beneficial effects of phosphorothioated antisense oligonucleotide and meloxicam.

BACKGROUND: The effects of cyclooxygenase-2 (cox-2) inhibition by a cox-2 selective antisense phosphorothioated oligonucleotide (AS) and meloxicam were examined in experimental colitis. METHODS: Colitis was induced by trinitrobenzenesulphonic acid (TNBS) and acetic acid (Hac) separately in male Sprague-Dawley rats. Both groups of animals were treated daily intraperitoneally with AS and a mismatched control oligo (CO) (3 mg/kg), and orally with meloxicam (7.5 mg/kg) 1 h before induction of colitis. The animals were killed on day 4 (Hac) and on day 5 (TNBS). Tissue samples from colon, ileum, liver, kidney and spleen were collected for mRNA, myeloperoxidase activity (MPO), prostaglandin E2 (PGE2) estimation and for histology, and blood samples for PGE2, thromboxane B2 (TxB2) and TNF-alpha. RESULTS: Both TNBS and Hac increased colonic MPO activity, PGE2 concentrations and infiltration of colonic wall by inflammatory cells. Serum levels of TNF-alpha were increased in both models, whereas PGE2 was increased only in TNBS colitis. Only meloxicam suppressed the level of PGE2 significantly below the basal level. The animals in both models also showed splenomegaly. The colitis-induced changes were significantly suppressed by the treatment of the test compounds but not by the CO. Cox-2 mRNA but not cox-1 was decreased by the AS, but not by meloxicam or in CO-treated colitic animals. CONCLUSION: The findings demonstrate comparable beneficial effects of the cox-2 selective antisense oligonucleotide and meloxicam, which seem to be mediated by a combined inhibition of both PGE2 and TNF-alpha in the present models of colitis.

Animals↗
PubMed2001

Antiinflammatory effects of Cordia myxa fruit on experimentally induced colitis in rats.

Products of certain species of Cordia are reported to have antiinflammatory properties. In the present study we examined the effects of Cordia myxa fruit on experimentally induced colitis in rats. Colitis was induced by intrarectal administration of 4% acetic acid. Colitic, normal, and corresponding control animals were included. Body weight was recorded daily. All the animals were sacrificed 4 days after the fruit treatment. Colitis was monitored histologically and by activity of myeloperoxidase. Glutathione peroxidase, superoxide dismutase, as well as total antioxidant status and concentrations of zinc, copper, manganese, selenium, and iron were assayed in plasma, liver, and colon using standard methods. Histology of the colon of colitic rats showed acute colitis that was confirmed by a significant increase in the myeloperoxidase activity. Colitis was associated with significant decreases in the tissue activities of glutathione peroxidase and superoxide dismutase and lower concentrations of trace elements. Histologic examination and myeloperoxidase activity showed that the fruit treatment reversed the above findings in the inflamed colon, and in liver and plasma of colitic rats. The present results suggest that the observed antiinflammatory effect of the Cordia myxa may be attributed partly to its antioxidant property and to restoration of the levels of trace elements in the inflamed colon, liver, and plasma.

Acetic Acid↗
PubMed2001

Influence of in-vivo endotoxin liberation on anti-anaerobic antimicrobial efficacy.

The ability of cefoxitin, clindamycin, imipenem, meropenem, metronidazole and piperacillin-tazobactam to cause gram-negative anaerobic bacteria to release endotoxin and the influence of such liberated endotoxin on antibiotic efficacy were investigated in in-vivo experiments in animal models. Experimental infections in various animal models (mice, hamster and infant rats) with cultures of wild and reference strains of Bacteroides fragilis group and Fusobacterium spp. were carried out by injecting these animals with different inocula (10(6), 10(7) and 10(8) cfu/ml) of the bacterial suspension, Appropriate doses of the test antibiotics were then injected and the plasma lipopolysaccharide (endotoxin) release measured by the Limulus Amoebocyte Lysate (LAL) Assay. Evidence of worsening of the outcome of the infections post-therapy was assessed, including histopathological changes in the internal organs. Infection with generalized septicemia was established with F. nucleatum in the mice and hamster models while with the B. fragilis group, infections only led to intra-abdominal abscess formation. Plasma endotoxin release was higher in animals infected with F. nucleatum than B. fragilis and was unrelated to the bacterial inoculum. Imipenem, meropenem and cefoxitin, in that order, induced the highest levels of endotoxin activities in the animal model, particularly following F. nucleatum infection. Histological examination of the internal organs of various animals showed variation in the pattern of histopathological changes; grades 3-4 inflammatory changes in the liver were observed in the Fusobacterium-infected animals that were treated with the carbapenems and cefoxitin. Therapy with the other antibiotics did not exacerbate anaerobic sepsis. In this study, bacteremia did not lead to massive endotoxin release and antibiotic therapy appeared not to have negatively influenced the outcome of most of the gram-negative anaerobic infections, except for infections caused by Fusobacterium spp. However, it is conceivable that if the gastrointestinal tract is the source of the endotoxin in patients with systemic inflammatory response syndrome, then the obligate anaerobes like Bacteroides and Fusobacterium species, which are members of the gut flora, may play a major role in the unfavorable outcome of antibiotic therapy in some of these infections.

Animals↗
PubMed2000

Assessment of different methods for staining Helicobacter pylori in endoscopic gastric biopsies.

The recent implication of Helicobacter pylori in the pathogenesis of gastritis-peptic ulcer syndrome and its relevance for the development of upper gastrointestinal malignancy warrant efficient methods for the detection and demonstration of the organism in biopsy specimens. We have compared 5 staining methods, namely, haematoxylin and eosin (H & E), immunohistochemistry (IHC), the silver staining HpSS, the alcian yellow-toluidine blue (Leung) method (A-Y) and Genta staining, for the demonstration of the organism in gastric biopsies taken from antrum, body and fundus of 118 patients who presented to our hospital with upper gastrointestinal symptoms. We found no significant differences in the efficacy of H & E, IHC, HpSS and A-Y in the demonstration of H. pylori in all 3 gastric sites. The least reproducible stain in our hands was the Genta stain. We conclude that H & E is adequate for the initial assessment of gastric biopsies in symptomatic upper gastrointestinal patients. This is because it is a well-tested, cheap and easy staining method, requiring a relatively short period of time to perform, with highly reproducible results. It has an added advantage of enabling simultaneous assessment of morphological changes accompanying H. pylori infection. When the density of the organism is expected to be low, we recommend addition of HpSS staining because of its high sensitivity and low cost. The disadvantages of the other staining methods (IHC, A-Y and Genta) are discussed.

Adolescent↗
PubMed1998

Characterisation of inflammatory cells in benign prostatic hyperplasia.

Inflammation is a common finding in benign prostatic hyperplasia (BPH) and may be classified as acute, chronic active or chronic inactive prostatitis. The aim of the present study was to localise the different types of inflammatory cells in prostatic lesions to determine the sequence of events in the cellular reaction. We have carried out immunohistological characterisation of the inflammatory cells, using CD45RO and CD3 antibodies to detect T-lymphocytes, CD20 antibodies to detect B-lymphocytes, CD68 to detect macrophages, kappa and lambda immunoglobulin light chains, and antibodies against prostate specific antigen (PSA) and prostate specific acid phosphatase (PSAP). Macrophages accumulated in the lumen and glandular epithelial layers of damaged prostatic glands and were found in the periglandular cuff of inflammatory cells in acute and chronic active prostatitis. Lymphocytes also accumulated in large numbers in the glandular epithelial layers and around the glands, indicating an association with macrophages. B-lymphocytes were scanty, if at all present, in acute and chronic active prostatitis, but were prominent within well-organised follicle centres in chronic active prostatitis. Cells positive for light chains were few and scattered in prostatic tissue. PSA and PSAP activity was lost in recently damaged prostatic glandular epithelium and reappeared only in regenerating secretory epithelium, indicating leakage as a result of damage. We suggest that the initial response to prostatic injury is cellular, and probably related to leakage into the periglandular tissues of PSA, PSAP and other antigenic molecules normally present in prostatic secretion. Macrophages respond, followed by recruitment of T-lymphocytes which participate in the inflammatory response and accumulate around the damaged glands. B-cell activity appears to be a late event.

Acid Phosphatase↗
PubMed1997

Hereditary spastic paraparesis with distal muscle wasting, microcephaly, mental retardation, arachnodactyly and tremors: new entity?

We present a consanguineous Pakistani family in which four patients (two males and two females) had a new Troyer-like phenotype. All four patients showed some marfanoid features (span more than height, arachnodactyly, high arched palate), and generalized hyper-reflexia. The two affected males and the younger female also had microcephaly and mental retardation. Features only present in the affected males included short stature, dysarthria, amyotrophy of the distal muscles, fasciculations and tremor. The distal muscle wasting in the two affected brothers reflected the presence of axonal neuropathy demonstrated both electrophysiologically and by nerve biopsy. Although neither of the sisters had any degree of distal muscle wasting, both had reduced M-wave amplitude of the tibial and peroneal nerves, bilaterally. The described phenotype does not fit any of the recognized forms of hereditary spastic paraparesis with distal muscle wasting. The specific axonal neuropathy differentiates it from familial motor neuron disease with mental retardation. The reported phenotype represents a possible new Troyer-like syndrome similar to that described by Neuhäuser (1976), but differs from it by the lack of major dysmorphic features.

Adolescent↗
PubMed1997

Endocarditis due to Aspergillus flavus.

Fungal endocarditis has emerged as an important complication of patients undergoing cardiovascular surgery. Our patient had no past history of cardiac surgery, intravenous drug abuse or immunosuppressive therapy. He had received broad-spectrum antibiotics for varying periods, which might have predisposed him to this infection. The diagnosis was based on the demonstration of hyaline, septate branched fungal elements in the infected valvular tissue and isolation of Aspergillus flavus in culture. The delay in establishing the ante-mortem diagnosis because of repeatedly negative blood cultures, presence of disseminated intravascular coagulopathy and rapidly deteriorating kidney function were the major factors contributing to his poor prognosis and death, despite surgical removal of infected valves and antifungal therapy. This is the first report of endocarditis due to A. flavus from the Middle East.

Amphotericin B↗
PubMed1997

Marinesco-Sjögren syndrome in a Bedouin family.

Marinesco-Sjögren syndrome is rarely reported in the Middle East. This is the 2nd report of Marinesco-Sjögren syndrome in an Arab family. The clinical features of 2 affected brothers are described. Electrophysiological studies of the 2 patients showed primarily myopathic changes, whereas sural nerve biopsy revealed segmental demyelination and axonal degeneration. The role of tissue biopsy and the relationship to different electrophysiological studies are discussed. Both patients were noticed to have abnormally short lateral 3 metatarsals, a feature not present in other healthy members of the family. We suggest that this feature should be considered part of the syndrome profile.

Abnormalities, Multiple↗