Israel Medical Association Journal

Background: Benign paroxysmal positional vertigo is a common and treatable vestibular disorder characterized by attacks of positional vertigo. Although elderly patients often complain about unsteadiness, the symptom of positional vertigo is seldom reported. Several studies on BPPV[1] in the elderly reveal a low success rate in the treatment of this entity.

Objectives: To assess the clinical characteristics and treatment outcome of BPPV in elderly patients and to compare them with those of the general population treated at our dizziness clinic.

Methods: We reviewed the medical records of 23 patients above age 75 who were treated at our dizziness clinic for BPPV during the years 1998–2004. Their clinical data, BPPV characteristics and treatment outcome were compared with the data of 30 consecutive BPPV patients who represented the general population.

Results: No differences in gender distribution, duration of BPPV, treatment responsiveness or recurrence rate were found between elderly patients as compared to the general population. The duration of the last attack of positional vertigo was found to be longer in the elderly, probably due to the delay in recognition of symptoms and accessibility of a dizziness clinic.

Conclusions: Our study shows that BPPV characteristics and treatment effectiveness, as measured by negative Dix-Hallpike maneuver, are not age-dependent and there is no need for a special approach or cautiousness in prognosis prediction. It is important to search actively for this condition since treatment leads to amelioration of unsteadiness and improved well-being in these patients.

[1] BPPV = benign paroxysmal positional vertigo

Type 1 diabetes mellitus is caused by an autoimmune destruction of pancreatic islet beta cells, leading to insulin deficiency. Beta-cell replacement is considered the optimal treatment for type 1 diabetes, however it is severely limited by the shortage of human organ donors. An effective cell replacement strategy depends on the development of an abundant supply of beta cells and their protection from recurring immune destruction. Stem/progenitor cells, which can be expanded in tissue culture and induced to differentiate into multiple cell types, represent an attractive source for generation of cells with beta-cell properties: insulin biosynthesis, storage, and regulated secretion in response to physiologic signals. Embryonic stem cells have been shown to spontaneously differentiate into insulin-producing cells at a low frequency, and this capacity could be further enhanced by tissue culture conditions, soluble agents, and expression of dominant transcription factor genes. Progenitor cells from fetal and adult tissues, such as liver and bone marrow, have also been shown capable of differentiation towards the beta-cell phenotype in vivo, or following expression of dominant transcription factors in vitro. These approaches offer novel ways for generation of cells for transplantation into patients with type 1 diabetes.

Background: Intestinal malrotation is usually observed in the neonatal period with signs of acute high intestinal obstruction due to midgut volvulus. However, malrotation presenting beyond the neonatal period and well into adult life is associated with a variety of atypical and frequently non-specific gastrointestinal symptoms that may often cause prolonged delay in diagnosis and appropriate treatment.

Objectives: To emphasize the difficulty in predicting the risk of midgut volvulus based on age or symptoms, and to recommend surgery in all patients found to have intestinal malrotation even if they are considered asymptomatic.

Methods: We reviewed 41 patients with malrotation treated over a period of 24 years at the Soroka University Medical Center.

Results: In our series, 27 patients (66%) had acute midgut volvulus while 14 (34%) had malrotation found during investigation of various long-term gastrointestinal non-specific symptoms. Two patients died of total parenteral nutrition-related sepsis following extensive resection of small bowel. A total of 28 patients was available for long-term follow-up and are asymptomatic.

Conclusions: We recommend elective laparotomy and Ladd procedure in all patients found to have intestinal malrotation. This will prevent the catastrophic results of midgut volvulus and a variety of gastrointestinal symptoms wrongly attributed to other conditions in the span of a lifetime.

Background: Coverage of part of a soft tissue defect in the thumb, without bone shortening and without long-lasting immobilization in an inappropriate position leading to stiffness, is difficult to achieve.

Objectives: To report our experience using Foucher’s modification of the first dorsal metacarpal artery flap for thumb reconstruction in 21 cases.

Methods: Foucher’s flap is based on the neurovascular structures of the first dorsal metacarpal artery flap and radial nerve-sensitive branches on the dorsum of the second metacarpal and proximal phalanx. The cause of injury was work-related in all 21 cases. The patients' mean age was 37 (range 17–68 years), and mean follow-up was 19 months (range 12–31). Emergency surgery was performed in 13 patients, with a time delay after injury of 4–12 hours. The minimum defect was 12–18 mm and the maximum 20–40 mm. Pedicular length was 55–95 mm. A skin bridge was left intact in 16 cases. In two cases of early postoperative venous congestion and flap loss, a cross-finger flap was performed as a salvage procedure.

Results: Subjective satisfaction score was 8.37/10 (range 4–10); cold intolerance was experienced in 60% and dysesthesia in 33%. All except one patient are able to use their thumb in daily activity. Loss of mobility in the proximal interphalangeal joint of the index finger was less than 20 degrees. Semmes-Weinstein sensitivity evaluation score was 3.61–4.31 on the flap and 0–6.65 on the donor site. Two-point discrimination was 10.8 mm (range 8–20). Grip strength was reduced by 15% compared to the unaffected hand (hand dominance was not taken into consideration). Rehabilitation was not consistent as almost all the patients were living in another location.

Conclusions: First DMCA[1] pedicle flap is a successful thumb reconstruction method, especially in patients not disturbed by its cosmetic appearance.

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[1] DMCA = dorsal metacarpal artery

Background: The Bloom syndrome gene, BLM, was mapped to 15q26.1 and its product was found to encode a RecQ DNA helicase. The Fanconi anemia complementation group C gene was mapped to chromosome 9q22.3, but its product function is not sufficiently clear. Both are recessive disorders associated with an elevated predisposition to cancer due to genomic instability. A single predominant mutation of each disorder was reported in Ashkenazi Jews: 2281delATCTGAinsTAGATTC for Bloom syndrome (BLM-ASH) and IVS4+4A®T for Fanconi anemia complementation group C.

Objectives: To provide additional verification of the mutation rate of BLM and FACC[1] in unselected Ashkenazi and non-Ashkenazi populations analyzed at the Sheba Medical Center, and to trace the origin of each mutation.

Methods: We used polymerase chain reaction to identify mutations of the relevant genomic fragments, restriction analysis and gel electrophoresis. We then applied the Pronto^TM kit to verify the results in 244 samples and there was an excellent match.

Results: A heterozygote frequency of 1:111 for BLM-ASH and 1:92 for FACC was detected in more than 4,000 participants, none of whom reported a family history of the disorders. The Pronto^TM kit confirmed all heterozygotes. Neither of the mutations was detected in 950 anonymous non-Ashkenazi Jews. The distribution pattern of parental origin differed significantly between the two carrier groups, as well as between each one and the general population.

Conclusions: These findings as well as the absence of the mutations in non-Ashkenazi Jews suggest that: a) the mutations originated in the Israelite population that was exiled from Palestine by the Roman Empire in 70 AD and settled in Europe (Ashkenazi), in contrast to those who remained; and b) the difference in origin distribution of the BS[2] and FACC mutations can be explained by either a secondary migration of a subgroup with a subsequent genetic drift, or a separate geographic region of introduction for each mutation.

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[1] FACC = Fanconi anemia complementation group C

Background: About one-third of patients with severe ulcerative colitis do not respond to conventional therapy and require urgent colectomy. It was recently shown that cyclosporin is effective in some of these patients.

Objectives: To review the current experience of six hospitals in central Israel that used cyc-losporin in patients with severe ulcerative colitis.

Methods: The files of all 32 patients treated with cyclosporin for corticosteroid-resistant ulcerative colitis were reviewed. Activity of disease was measured by a clinical activity, index colonoscopy and laboratory tests.

Results: The average duration of treatment with intravenous cyclosporin was 12.7 days (range 9–28) after which the disease activity index dropped from an average of 14.22 to 4.74. The mean time for response was 7.5 days (4–14). Twelve patients (40%) required surgery within 6 months and another 6 patients (18.8%) were operated on after more than 6 months. Twelve patients (37%) maintained remission for at least 6 months and did not require surgery. In one patient treatment was stopped because of non-compliance and one was lost to follow-up. There were numerous side effects, but in only one case with neurotoxicity was treatment withdrawn.

Conclusions: Cyclosporin is a relatively safe and effective treatment for severe ulcerative colitis. It induced long-term remission in 37% of the patients, and in those who required surgery the treatment resulted in an improved clinical condition before the operation.

Objective: To examine to what extent and for how long nedocromil sodium prevents exercise-induced asthma when given immediately before exertion compared to chronic administration.

Patients and Methods: Eighteen asthmatic patients were given 4 mg NS at 30 min or 3.5 hours before exertion. We compared the resultant effect with that of the same protocol measured after 2 and 4 weeks of continuous treatment with the drug.

Results: Nedocromil sodium decreased exercise-induced asthma similarly at both points when given acutely. Chronic treatment of up to 4 weeks did not improve this protective effect at either interval following the inhalation.

Conclusion: Nedocromil sodium most likely reaches its maximal effect on exercise-induced asthma upon the first administration, although treatment for longer than 4 weeks might be required to prove a chronic effect of the drug.