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Biomedical subjects

H M Al Attia

Publications and source records attributed to H M Al Attia.

15 recordsLinked to original sources

Antitopoisomerase I antibody in patients with systemic lupus erythematosus/sicca syndrome without a concomitant scleroderma: two case reports.

We describe two female patients with classic systemic lupus erythematosus (SLE) and secondary sicca syndrome associated with topoisomerase I (topo-I, Scl-70) antibody, a specific marker for scleroderma (SSc), which is rarely found in other collagen diseases. During the course of the disease, the sera of these two patients were repeatedly found to be positive for topo-I antibody following a positive screening by ANA-EIA. Neither patient had clinical evidence of scleroderma. One patient remains well nearly 4 years from the first positive serological test. The progression to sicca syndrome in that patient occurred 2 years after having tested positive for antitopo-I antibody. Her frozen serum also tested positive for anti-Scl-70 by the Western blot technique. The other patient, however, died after developing renal and cardiopulmonary complications of lupus, including Libman Sachs endocarditis and pulmonary hypertension. Contrary to the previous patient, the onset of sicca syndrome in this case had preceded the expression of positive antitopo-I antibody. The present cases and other similar previously reported ones are therefore unique in the sense of being a serological challenge to the high specificity of antitopo-I to scleroderma. In addition, they may also represent a new subset of SLE with or without sicca syndrome, which is characterised by the absence of features of scleroderma despite the presence of antitopo-I antibody.

Antibodies, Antinuclear↗

Salivary gland scintigraphy in Sjögren's syndrome: are quantitative indices the answer?

We evaluated the role of quantitative indices derived from dynamic 99mTc-pertechnetate salivary scintigraphy in the differentiation between a group of patients with Sjögren's syndrome (SS), a group of patients without xerostomia, but with underlying autoimmune disorders, and a group of controls. Seventeen patients with SS (group A), 18 patients with autoimmune disorders (group B) and 15 controls (group C) underwent dynamic salivary gland scintigraphy. Functional indices for the parotid and submandibular glands were calculated and comparisons were made between the groups. There were no significant differences between the three groups in terms of the maximum accumulation (MA), maximum secretion (MS) and pre-stimulatory oral index (PRI). The uptake ratios (URs) for both the right and left parotid glands and the left submandibular gland in group A were significantly lower than those in group C, but no different from those in group B. The URs for the parotid glands in group B were significantly less than those in group C. The percentage uptake by the right parotid gland at 4 min (U4) was significantly lower in group A than in groups B or C, and lower than the percentage uptake by the left parotid gland at 4 min in group A. The rest of the U4 values and all of the uptakes at 14 min (U14) were not significantly different between the three groups. The time taken for the right parotid gland to reach peak activity (Tmax) was significantly less in group A than in the other groups, but other glands showed no significant differences. It can be concluded that MA, MS and PRI cannot be used to differentiate between the three groups. The URs in groups A and B were no different, but were significantly lower than those in group C. However, the extensive overlap between xerostomic patients and normal controls for all the quantitative indices calculated imposes a severe limitation on their discriminatory power.

Adolescent↗

Progression of primary APS (Hughes syndrome) into serological SLE: case report.

An Arab woman presented with a history of multiple foetal losses and spontaneous venous thromboembolism, which recurred on several occasions. The presence of antiphospholipid antibodies in the absence of other clinical and serological features of systemic lupus erythematosus (SLE), including negative antinuclear antibodies (ANA), confirmed the diagnosis of primary antiphospholipid syndrome (PAPS). More than 15 years after the beginning of clinical events and 10 years after diagnosis, she progressed into the immunological domain of SLE without concurrent clinical features. The patient exhibited weakly positive ANA of a speckled pattern, strongly positive anti (ds) DNA antibodies and false positive VDRL. Lymphopenia has not been observed at any stage of the follow-up. Although the evolution of PAPS into SLE has been infrequently reported, this seems to be another case suggesting that PAPS in some patients may be an early manifestation of lupus.

Adult↗

The role of scintigraphy with technetium-99m nanocolloid in patients with peripheral joint pain.

We studied 51 patients with peripheral joint pain to assess the ability of technetium-99m-nanocolloid (99mTc NC) scintigraphy to distinguish patients with active joint disease from those without, irrespective of their underlying aetiology. Patients with peripheral joint pain due to various causes were clinically categorised as either having objective signs of active joint disease or not. Their clinical diagnoses were not made known to the nuclear physician until after scanning. Each patient was given an intravenous injection of 555 MBq of 99mTc NC 1 h prior to taking spot views of the peripheral joints. Seventeen patients with low backache but without peripheral joint pain were imaged in an identical manner to exclude peripheral joint involvement. The latter formed a control group. 99mTc NC scintigraphy demonstrated a sensitivity of 89% for identifying at least one affected joint per patient, a specificity of 87.5%, a negative predictive value (npv) of 87.5%, a positive predictive value (ppv) of 89%, and overall accuracy of 88%. There were three false negative and three false positive cases. The scan was negative in all control subjects. 99mTc NC scintigraphy proved to be sensitive in patients with active joint disease and demonstrated a good correlation with clinical assessment. The value of the high specificity, npv and negative controls together made the test highly discriminatory in excluding patients without active peripheral joint disease.

Adolescent↗

99Tc(m) nanocolloid scintigraphy: a reliable way to detect active joint disease in patients with peripheral joint pain.

We evaluated 99Tc(m) nanocolloid (NC) scintigraphy as a method for identifying patients with active joint disease in a group with peripheral joint pain of varying aetiology. Fifty-nine patients with peripheral joint pain were divided into two groups: those with clinical signs of active joint inflammation and those without objective signs. Thirty-four patients clinically diagnosed as having active joint disease had a total of 117 joints (95 large and 22 small) involved. 99Tc(m) NC identified 96 (79 large and 17 small) of these joints. Twenty-five patients were negative clinically. Twenty-two of these were scan negative. The other three had a total of 11 scan positive joints. The correlation coefficient between clinical and scan findings was 0.79. There was no significant difference shown between clinical evaluation and scintigraphy (z=-1.64, P = 0.1004). 99Tc(m) NC proved to be an effective method for identifying patients with active peripheral joint disease from among a group with arthralgia.

Adolescent↗

Cephalad renal ectopia, duplication of pelvicalyceal system and patent ductus arteriosus in an adult female.

An unusual cause for a shadow in the plain CX-ray of a female with uncomplicated patent ductus arteriosus is presented herein. The chest CT scan and IVU revealed the presence of a high (cephalad) right kidney in an eventrated hemidiaphragm. A bifid ureter and duplication of the pelvis were found in the contralateral kidney. This constellation of anomalies is exceedingly rare.

Adult↗

Buschke-Ollendorff syndrome in a grande multipara: a case report and short review of the literature.

A 36-year-old short-statured grande multipara (gravida 10, para 8) with diabetes mellitus and hyperlipidaemia was incidentally found to have Buschke-Ollendorff syndrome (osteopoikilosis and dermatofibrosis lenticularis disseminata). The pelvis and hips, followed by the knees, were the sites mainly affected by the osteopoikilosis. The lumbosacral spine was also affected. She had a single connective tissue naevus on the right thigh. Apart from two abortions, the repeated pregnancies were uneventful and all her deliveries were normal at full-term or nearly full-term. With regard to the relationship between Buschke-Ollendorff syndrome and multiparity, it was apparent that multiparity did not affect the involved weight bearing bony structures, nor did the disseminated osteopoikilosis interfere with the endurance of multiple pregnancies. Buschke-Ollendorff syndrome is known generally to have a benign course, a feature that is illustrated in this case, even when associated with the stresses of multiparity. The report also provides a short and updated review of various clinical aspects of the syndrome and its associations, some of which are of a serious nature.

Adult↗

Serological markers in Arabs with lupus nephritis.

The aim of this study was to characterize the autoantibody profile of Arabs with lupus nephritis (LN). Therefore, the records of 42 Arabs with classical systemic lupus erythematosus (SLE) were reviewed. Twenty one (50%) with LN and 21 without. Most patients with LN have developed their nephropathy within the first five years of the disease. Only C3 hypocomplementemia achieved statistically significant correlation in nephritic patients and anti ribonucleoprotein (RNP) antibodies in non-nephritic patients. The difference in anti double stranded DNA (dsDNA) antibodies was insignificant (P=0.107), as both groups had markedly high prevalence of these antibodies. In addition, there was no significant negative association between detectable rheumatoid factor (RI) and patients with LN. We also examined the spectrum of specific entities of antibodies that had previously been reported in association with LN in other series (anti Smith (Sm), RNP and SS-A without anti SS-B, anti SS-A and SS-B, anti RNP and Smith, anti RNP and dsDNA and anti RNP and SS-A antibodies). None has shown any correlation with renal disease, P=1.00, P=0.591, P=0.613, P=0.195 and P=0.148, respectively. In conclusion, the study demonstrates that Arab patients with LN do not exhibit a distinctive serological profile, nonetheless it provides useful information to studies of SLE in different ethnic or racial groups.

Adolescent↗

Hirsutism in the United Arab Emirates: a hospital study.

In a study of 102 women (Arabs and Asians) with hirsutism, polycystic ovary syndrome was diagnosed in 93 patients (91%) including 26 cases with hirsutism and regular menstrual periods. In more than half of the cases the diagnosis was made on the basis of transabdominal ultrasonography, which was the single test with the highest yield. Luteinising hormone hyperresponsiveness to gonadotropin-releasing hormone was positive in only seven of 50 patients tested. Late onset congenital adrenal hyperplasia due to 21-hydroxylase deficiency was identified in two cases, thus forming a prevalence of 8% in those who underwent adrenocorticotropin stimulation. In one case, polycystic ovary syndrome was an associated condition. In this study of a population of women with hirsutism and regular menses, the majority had polycystic ovaries. It is suggested that the term 'idiopathic or racial' hirsutism should not be applied unless a thorough evaluation has failed to reveal a diagnosable underlying disorder.

Adolescent↗