Israel Medical Association Journal

Objective: To investigate the effect of the Dead Sea environment (climatotherapy) on the signs, symptoms and clinical course of chronic uveitis.

Methods: Fifty-five patients with chronic uveitis were examined at the beginning and end of a 3–4 week stay at the Dead Sea region and on repeat visits to the region. Study data included demographic information, medical history, etiology, diagnosis, medication, and a complete ophthalmic examination.

Results: Statistically significant improvements were seen between the two examinations within each visit in four parameters (negative values indicate improvement): a) visual acuity for near and far: Jaeger (‑1.18 ± 0.28, P < 0.0001) and best corrected visual acuity (‑0.08 ± 0.02, P < 0.0001); b) anterior chamber flare (-0.18 ± 0.06, P < 0.01); c) anterior chamber cells (-0.16 ± 0.05), P < 0.001); and d) vitreous cells (-0.15 ± 0.09, P < 0.05). There was a significant mean improvement during visits to the Dead Sea area and a slight dissipation of the effect during the intervals between visits. Sixty-four percent of the patients reported that they required less medication and had fewer and milder attacks of uveitis following the visits.

Conclusions: The results of this study provide evidence of short- and possibly long-term improvement in the signs and symptoms of uveitis following exposure to the Dead Sea environment.

Background: In patients with Graves’ ophthalmopathy, orbital decompression surgery is indicated for compressive optic neuropathy, severe corneal exposure, or for cosmetic deformity due to proptosis. Traditionally this has been performed through a transantral approach, but the associated complication rate is high. More recently, endoscopic orbital decompression has been performed successfully with significantly fewer postoperative complications.

Objective: To report our experience of endoscopic orbital decompression in patients with severe Graves’ ophthalmopathy.

Methods: Three patients (five eyes) underwent endoscopic orbital decompression for Graves’ ophthalmopathy at Soroka Medical Center between the years 2000 and 2002. The indications for surgery were compressive optic neuropathy in three eyes, severe corneal exposure in one eye, and severe proptosis not cosmetically acceptable for the patient in one case. An intranasal endoscopic approach with the removal of the medial orbital wall and medial part of the floor was performed.

Results: In all five eyes an average reduction of 5 mm in proptosis was achieved. Soon after surgery, visual acuity improved in the three cases with compressive optic neuropathy, and exposure keratopathy and cosmetic appearance improved. The diplopia remained unchanged. No complications were observed postoperatively.

Conclusions: Endoscopic orbital decompression with removal of the medial orbital wall and medial part of the floor in the five reported eyes was an effective and safe procedure for treatment of severe Graves’ ophthalmopathy. A close collaboration between ophthalmologists and otorhinolaryngologists skilled in endoscopic sinus surgery is crucial for the correct management of these patients.

Background: The appearance of pericarditis following insertion of a permanent pacemaker is not widely acknowledged in the literature.

Objectives: To describe our experience with pericarditis following 395 permanent pacemaker implantations over 2 years.

Methods: We retrospectively reviewed the medical records of 395 consecutive patients in whom new pacing systems or pacemaker leads had been implanted over a 2 year period. We searched the records for pericarditis that developed within 1 month after pacemaker implantation according to the ICD-9 code. The incidence, clinical picture, response to treatment and relationship to lead design and location were studied.

Results: Eight cases (2%) of pericarditis following implantation were detected. Clinical manifestations in all patients were similar to those of post-pericardiotomy syndrome and included chest pain (n=7), friction rub (n=1), fever (n=2), fatigue (n=2), pleural effusion (n=2), new atrial fibrillation (n=2), elevated erythrocyte sedimentation rate (n=4) and echcardiographic evidence of pericardial effusion (n=8). All affected patients had undergone active fixation (screw-in) lead implantation in the atrial position. The incidence of pericarditis with screw-in atrial leads was 3% compared to 0% in other cases (P < 0.05).

Conclusions: Pericarditis is not uncommon following pacemaker implantation with active fixation atrial leads. Special attention should be paid to identifying pericardial complications following pacemaker implantation, especially when anticoagulant therapy is resumed or initiated. The use of passive fixation leads is likely to reduce the incidence of pericarditis but this issue should be further investigated.

Background: Pulmonary disease is the most frequent cause of morbidity and mortality in cystc fibrosis patients. New techniques such as non-invasive positive pressure ventilation have resulted in prolongation of life expectancy in CF[1] patients with end-stage lung disease.

Objectives: To determine the role of NIPPV[2] in CF patients awaiting lung transplantation.

Methods: Between 1996 and 2001 nine CF patients (5 females) with end-stage lung disease were treated with bi-level positive airway pressure ventilation in the "spontaneous" mode.

Results: The patients' mean age at initiation of BiPAP[3] was 15 years (range 13–40 years) and the mean duration of BiPAP usage was 8 months (range 3–16 months). Four patients underwent successful lung transplantation, three patients died while awaiting transplantation, and the remaining two are still on NIPPV while waiting for transplantation. Patients' body mass index increased significantly (P < 0.05) during BiPAP therapy (from 16.1 to 17.2 kg/m²). Blood pH, p_aCO₂, and bicarbonate improved significantly (from 7.31 to 7.38, 90.8 to 67.2 mmHg, and 48.9 to 40.3 mEq/L, respectively). Pulmonary function tests were not affected by BiPAP usage. The patients experienced a significant alleviation in morning headaches and improvement in quality of sleep (P < 0.003). There were no major complications during BiPAP usage.

Conclusions: We demonstrated that long-term NIPPV can stabilize and improve physiologic parameters such as ventilation, arterial blood gases and body mass index, as well as subjective symptoms such as sleep pattern, daily activity level, and morning headaches in CF patients with end-stage lung disease. Further prospectively controlled studies are needed to evaluate the potential of BiPAP therapy and its influence on morbidity and mortality in the post-lung transplantation period.

Histiocytosis of childhood is characterized by localized or generalized proliferation of cells of the mononuclear phagocyte system and the dendritic cell system. In patients with Langerhans cell histiocytosis, the orbita is the most involved site encountered in ophthalmic practice, usually as a lytic lesion in the zygomaticofrontal suture. Patients usually present with acute or chronic periorbital swelling. Electron microscopic findings of Birbeck granules and positive staining for CD1 antigenic determinant confirm the diagnosis.

Background: Among the various new technologies in the field of parathyroid surgery are intraoperative quick parathormone measurements.

Objectives: To evaluate the contribution of QPTH[1] measurements during parathyroidectomy to the achievement of higher success rates. 

Methods: QPTH assay using Immulite Turbo Intact PTH[2] was measured in 32 patients undergoing parathyroidectomy: 30 for primary and 2 for secondary hyperparathyroidism.  QPTH levels were measured at time 0 minutes (before incision) and at 10, 20, and 30 minutes after excision of the hyperfunctioning gland.  Only a drop of 60% or more from the 0’ level was considered to be a positive result.

Results: The mean QPTH level at time 0’ for PHPT[3] patients was 38.12 ± 25.15 pmol/L (range 9.1–118 pmol/L).  At 10 minutes post-excision of the hyperfunctioning gland (or glands), QPTH dropped by a mean of 73.80% to 9.89 ± 18.78 pmol/L. 

Conclusions: Intraoperative QPTH level measurement is helpful in parathyroid surgery.  A drop of 60% or more from 0’ level indicates a successful procedure, and further exploration should be avoided.

Background: Cystinuria is an autosomal recessive disease that is manifested by the development of kidney stones. Mutations in SLC3A1 cause type I disease, while mutations in SLC7A9 are associated with non-type I disease. In Israel cystinuria is especially common among Libyan Jews who suffer from non-type I disease.

Objectives: To compare clinical manifestations of patients with mutations in SLC3A1 to those with mutations in SLC7A9, and to assess the carrier rate among unaffected Libyan Jewish controls.

Methods: Clinical manifestations were evaluated in patients with mutations in SLC3A1 and in patients with mutations in SLC7A9. Carrier rates for two SLC7A9 mutations were assessed in 287 unaffected Libyan Jewish controls.

Results: Twelve patients with mutations in SLC3A1 were compared to 15 patients with mutations in SLC7A9. No differences were detected between the patients with mutations in SLC3A1 and those with mutations in SLC7A9 in relation to the age of disease onset, the estimated number of stones, the number of invasive procedures, the number of patients receiving drug therapy, or the patients’ urinary pH. Eleven of the unaffected Libyan Jewish controls were found heterozygotes for the V170M mutation, establishing a carrier rate of 1:25. The 1584+3 del AAGT mutation was not found in any of the Libyan Jewish controls.

Conclusion: Mutations in SLC3A1 and SLC7A9 cystinuria patients result in indistinguishable disease manifestations. The high carrier rate among Libyan Jews is a result of a single missense mutation, V170M.