Biomedical subjects
Publications and source records attributed to M el-Khateeb.
A novel form of autosomal recessive distal hereditary motor neuronopathy (distal HMN) is reported. The presence of pyramidal signs within the early stages of the disease with persistence of knee hyperreflexia form distinctive clinical features. We have mapped the HMN-J gene to chromosome 9p21.1-p12, within an estimated interval of 1.2-Mb.
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The reactions of cis-[PtCl(NH3)2(H2O)]+ with L-methionine have been studied by 1D 195Pt and 15N NMR, and by 2D[1H, 15N] NMR. When the platinum complex is in excess, the initial product, cis-[PtCl(NH3)2(Hmet-S)]+ undergoes slow ring closure to [Pt(NH3)2(Hmet-N,S)]2+. Slow ammine loss then occurs to give the isomer of [PtCl(NH3)(Hmet-N,S)]+ with chloride trans to sulfur. When methionine is in excess, a reaction sequence is proposed in which trans-[PtCl(NH3)(Hmet-S)2]+ isomerises to the cis-isomer, with subsequent ring closure reactions leading to cis-[Pt(Hmet-N,S)2]2+. Near pH 7, methionine is unreactive toward cis-[PtCl(OH)(NH3)2]. By contrast, L-cysteine reacts readily with cis-[PtCl(OH)(NH3)2] at pH 7, but there were many reaction products, including bridged species. Cis-[PtCl(OH)(NH3)2] reacts with reduced thiols in ultrafiltered plasma but these are oxidized if the plasma is not fresh or appropriately stored. With very low concentrations of the platinum complexes (35.5 microM), HPLC experiments (UV detection at 305 nm) indicate that the thiolate (probably cysteine) reactions become simpler as bridging becomes less important.
In a 2-year hospital-based study (1992 and 1993), there were 131 multiple sclerosis patients attending 2 large referral hospitals in Jordan. Based on MS/ALS case ratios an overall rate of 32.1 (95% CI 19.7-55.2) was estimated. There were 126 Arabs of whom 84 were Palestinians and 36 indigenous Jordanians. Comparison of these subgroups, which had a similar age distribution revealed that the disease was twice as frequent in Palestinians (estimated 42.0/100,000 (CI 2.8-90.8)) among Jordanians (estimated 20.0/100,000 (CI 9.5-47.2)). Clinical presentation, pattern of disease, disability and HLA association were similar to that in the disease reported in Caucasians in the West. All investigations including neurophysiology and imaging were also very similar to Western reports.
During the year 1987, 106 cases of human brucellosis were studied prospectively at the Jordan University Hospital. The disease was more often diagnosed among adults (73.6%) than children (26.4%). Serious clinical complications were observed in 5.7% patients. An initial Brucella antibody titre greater than or equal to 160 proved to be reliable in confirming suspected cases of acute and subacute brucellosis. Culture of blood was found to be more sensitive (44.4%) and significant (P less than 0.02) than bone marrow culture (27.7%) for detecting Brucella melitensis. All patients treated with rifampicin plus tetracycline or co-trimoxazole were considered to be clinically cured by disappearance of all major clinical features of brucellosis. By contrast, 2/10 patients treated with rifampicin alone, as well as 1/56 patients treated with tetracycline and streptomycin, clinically relapsed. It is evident from this study that the treatment with rifampicin alone is not as effective in brucellosis as it is when given with another appropriate drug.
Two patients with a long-standing history of familial Mediterranean fever (FMF) presented with gross hematuria, oliguria, and acute renal failure; both required dialysis support. Kidney biopsies from both patients revealed crescentic rapid progressive glomerulonephritis (RPGN) without amyloidosis. One patient recovered renal function with methylprednisolone pulse therapy and cyclophosamide. The second patient did not improve and required regular hemodialysis. He is asymptomatic on colchicine therapy. To our knowledge, these are the first cases documenting the presence of RPGN in patients with FMF.
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