Hollow visceral myopathy in a 5-year old boy: a case report.
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Biomedical subjects
Publications and source records attributed to Z Zaidi.
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OBJECTIVE: To correlate the density of the yeast Malassezia with the clinical seventy of seborrhoeic dermatitis. METHOD: Fifty patients and twenty control subjects were selected for the study. The patients were evaluated both clinically for the severity of seborrhoeic dermatitis and microscopically for the presence of the yeast Malassezia. RESULTS: Out of 50 patients Malassezia was present in 41 patients (82%). On microscopic evaluation it was found that patients with mild seborrhoeic dermatiis had a density of 2+ (more than 5 but less than 10 yeast cells per high power field (hpf). Patients with moderate seborrhoeic dermatitis had a density of 3+ (more than 10 but less than 20 yeast cells per hpf) and patients with severe seborrhoeic dermatitis had a density of 4+ (more than 20 yeast cells per hpf). Of the 20 normal subjects only 8 (40%) had Malassezia and they had a density of 1+ (5 or fewer yeast cells per hpf). The results show a strong correlation of the yeast Malassezia to the severity of seborrhoeic dermatitis (p value < 0.05). CONCLUSION: Malassezia increases in proportion with the severity of seborrhoeic dermatitis; an antifungal agent should therefore be used in the treatment of seborrhoeic dermatitis.
Multiple studies have shown that intracellular signal transduction by the protein kinase C (PKC) family participates in the initiation of megakaryocyte differentiation. In this study, multiple approaches addressed the functional contributions by specific PKC isozymes to megakaryocytic lineage commitment of two independent cell lines, K562 and human erythroleukemia (HEL). Pharmacologic profiles of induction and inhibition of megakaryocytic differentiation in both cell lines suggested a role for the calcium-independent novel PKCs, in particular PKC-epsilon. In transfection studies, the isolated variable domain of PKC-epsilon selectively blocked exogenous activation of the megakaryocyte-specific alpha IIb promoter. Constitutively active mutants of PKC-epsilon, but not of other PKC isozymes, cooperated with the transcription factor GATA-1 in the activation of the alpha IIb promoter. The functional cooperation between GATA-1 and PKC-epsilon displayed dependence on cellular milieu, as well as on the promoter context of GATA binding sites. In aggregate, the data suggest that PKC-epsilon specifically participates in megakaryocytic lineage commitment through functional cooperation with GATA-1 in the activation of megakaryocytic promoters.
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Congenital generalized lipodystrophy (CGL, Berardinelli-Seip Syndrome, OMIM # 269700) is a rare autosomal recessive disorder characterized by near complete absence of adipose tissue from birth. Affected individuals have marked insulin resistance, hypertriglyceridemia and acanthosis nigricans, and develop diabetes mellitus during teenage years. The genetic defect for CGL is unknown. A semi-automated genome-wide scan with a set of highly polymorphic short tandem repeats (STR) was carried out in 17 well-characterized pedigrees and identified a locus for CGL to chromosome 9q34. The maximum two-point lod score obtained was 3.6 at D9S1818 (theta(max) = 0.05). There was evidence for genetic heterogeneity (alpha = 0.73) and 2 of the pedigrees were unlinked. Multipoint linkage analysis excluding the 2 unlinked families yielded a peak lod score of 5.4 between loci D9S1818 and D9S1826. The CGL1 critical region harbors a plausible candidate gene encoding the retinoid X receptor alpha (RXRA) that plays a central role in adipocyte differentiation. Identification of the CGL gene(s) will contribute to our understanding of the adipocyte differentiation and elucidation of the mechanisms of insulin resistance in disorders of adipose tissue.
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OBJECTIVES: To assess the use of a multipurpose stent (the 'Blue stent', Angiomed Urosoft Pyeloplasty Stent, Bard, UK) in children undergoing pyeloplasty and ureteric reimplantation. PATIENTS AND METHODS: Between August 1994 and August 1996, the Blue stent was used in 50 renal units in 46 children aged 2 months to 12 years and 6 months. Twenty-five children underwent pyeloplasty, 11 had ureteric reimplantation for vesico-ureteric reflux (VUR), eight had ureteric reimplantation with remodelling for obstructed megaureters and in two patients it was used during the removal of stones. The mean follow-up was 18 months (range 6-30 months). RESULTS: After pyeloplasty, 22 patients (88%) had improved renal function and drainage with a decrease in hydronephrosis; two patients had a decrease in hydronephrosis only, one had an anastomotic leak and needed a repeat pyeloplasty and four developed a urinary tract infection (UTI). After ureteric reimplantation, VUR was not detected in any patient. Two patients had no change in drainage after remodelling and reimplantation of a megaureter, one was later diagnosed as having a neuropathic bladder and one child developed a UTI after ureteric reimplantation. The hospital stay was 3 days after pyeloplasty and 5 days after reimplantation. CONCLUSION: The design of the multipurpose Blue stent provides versatility; it can be used as a stent, and both an internal and external drain. Its use does not prolong the hospital stay. Insertion causes minimal trauma to the renal parenchyma, and removal is easy, pain-free and requires no anaesthesia. The complication rates in the present series compare favourably with other reported series.
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PURPOSE: In 1986 we reported the placement of a silicone sheath sandwiched between layers of omentum around a newly reconstructed bladder neck. We now present long-term followup of 94 cases of silicone sheath bladder neck reconstruction. MATERIALS AND METHODS: A total of 94 silicone sheaths was placed in 84 patients between August 1983 and October 1992. We retrospectively reviewed our results and divided the reconstructions into 3 groups according to modifications in surgical technique. We report the results of each modification and current recommendations for use. RESULTS: Each sequential modification of silicone sheath bladder neck reconstruction significantly reduced the risk of erosion from 100%, 32% and 7%, respectively (p < 0.05). Erosion was independent of patient age, sex, pathological condition or whether bladder neck reconstruction was a repeat procedure. Patients who had silicone sheath erosion did not have different continence or loss of urethral continuity than those with no erosion. In 13 patients (16%) artificial sphincter placement was clearly facilitated by the silicone sheath. CONCLUSIONS: Modifications in the surgical technique of silicone sheath placement around a reconstructed bladder neck have resulted in a decrease in the erosion rate to 7%. The primary benefit of silicone sheath placement is ease of subsequent sphincter placement. Currently we reserve silicone sheath placement for a small subset of patients who are most likely to benefit from subsequent artificial sphincter placement.
An open label trial of Lamisil (Terbinafine) 500 mg daily for 16 weeks was conducted on 20 patients with Onychomycosis due to candida. Of these, 15 patients could complete the trial. Total number of target nails infected was 21. The follow-up period was from 16 to 52 weeks. At 16 weeks, 71% cases showed clinical improvement and 67% had mycological cure. Thirty-three percent had complete cure at 52 weeks. The results show that Terbinafine is effective against candida but requires a longer duration of treatment. The drug was well tolerated.
The complete sequence of human gamma s-crystallin has been determined and confirmed using a combination of MS methods, peptide sequencing and cDNA sequencing. Regions 21-35 and 102-107, which were previously assumed to be the same as the bovine sequence, differ from the bovine sequence at residues 22, 28, 31 and 104. An additional six residues were also found to be different from the original sequence determined for Pakastani lenses. Whether these differences represent errors in the original sequence or two different sequences among human lens crystallins is not yet known.
Hundred people were examined for piezogenic papules (cutaneous hernia). Of these 80 had pedal papules, 86 wrist papules and 74 both pedal and wrist papules. Most of these papules were bilateral and multiple. All cases examined were painless. Biopsy showed fragmentation of dermal elastic tissue and herniation of subcutaneous fat into the dermis. Histology was similar for both pedal and wrist papules.
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A rapid and sensitive high-performance liquid chromatographic (HPLC) procedure is described for the analysis of the antitussive dextromethorphan hydrobromide in several cough-cold syrup preparations and compared with a gas chromatographic (GC) procedure. In the HPLC procedure, the active ingredient is analyzed as the hydrobromide salt by dilution in the mobile phase and separation on a reverse-phase cyano column. In the GC method, the active ingredient is analyzed as the free base, in which an aqueous solution of the antitussive is made alkaline and extracted with dichloromethane before injection onto the GC column. Excellent resolution of the antitussive agent was obtained by both systems; however, the HPLC assay is preferred for routine analysis (RSD 1%), as compared with the GC assay (RSD 4%).
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The maternal mortality in the Maternity and Children Hospital, Riyadh, during the years 1978-1980 was 52 per 100 000 births, when the total births were 55 428. This is higher than the rate reported from the hospitals in developed countries but lower than rates reported by the university hospitals of developing countries such as India, Thailand and Nigeria. Haemorrhage, associated disease, pulmonary embolism and infection, in that order, were the main causes of maternal deaths. The main avoidable factor was failure by the patient to seek the medical care. Much could be done in reducing deaths due to haemorrhage by improving blood transfusion facilities in the peripheral hospitals. Adequate health education, especially of rural women and their midwives, is a crucial factor in improving the maternal death rate for the country as a whole.
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