Israel Medical Association Journal

 Background:  Pulmonary alveolar proteinosis is a rare disease in which a surfactant-like phospholipid-rich protein accumulates in the lungs. The disease is amenable to effective therapy by total lung lavage.       

Objectives: To investigate the prevalence, ethnic distribution and course of PAP  in Israel.

Methods: A countrywide survey was conducted during which pulmonologists were questioned about patients with PAP. The patients were examined and their charts, radiological images, pathological slides and physiological data were reviewed.

Results: The survey yielded 15 patients (8 females) during the period 1976–98 (14 in the last decade), giving a prevalence of 3.7x10⁶ and an incidence of 0.36x10⁶/year.

Mean age of the patients was 33±13 years (range 0.5–46 years). Seven patients were North African (two were siblings), four were from Iraq and two were Arabs; there was only one Ashkenazi Jew (a child). Symptoms at the onset were dyspnea and chest pain. Spontaneous remission occurred in at least 3 patients, and 10 patients required 1–4 bronchoalveolar lavage treatments. The subjective and physiological response was favorable, but there was less consistent radiological improvement.

Conclusion: The prevalence of PAP in Israel is approximately 3.7x10⁶. Most cases occurred in Jews who had immigrated from North Africa or Iraq, and two were siblings. The prevalence among the Arab population appears to be similar. This clustering suggests the existence of a genetic predisposition. The course of the disease appears to be similar to that reported elsewhere.

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PAP = pulmonary alveolar proteinosis

Objectives: To present a case series of foyr patients with phenytion-induced severe CDRs, including toxic epidermal necrolysis (2 patients), exanthematous eruption (1 patient) and hypersensitivity syndrome (1 patient). In all patients the reactions appeared following the combined intake of phenytion, corticosteroids and H2 blockers.

Conclusions: Our case series may imply the role of drug interactions between phenytion, corticosteroids and H2 blockers in the induction of severe CDRs.

Objectives: To help clinicians and laboratory scientists recognize and utilize vasculitis-related ANCAs as an aid in diagnostic workup and patient follow-up, and be aware that ANCAs with different characteristics are commonly found in other chronic inflammatory conditions that persistently engage neutrophils in the inflammatory process.

Methods: Indirect immunofluorescence and enzyme immunoassay methods were used to detect ANCAs with both known and unknown neutrophil autoantigenic targets.

Results: Primary necrotizing small vessel vasculitides such as Wegener’s granulomatosis, Churg-Strauss syndrome, microscopic polyangiitis, and renal-limited rapidly progressive necrotizing glomerulonephritis target either the serine protease proteinase 3 or myeloperoxidase  in azurophilic granules. In ulcerative colitis and rheumatoid arthritis, we found multiple ANCA targets contained in azurophilic and specific granules, the cytosol and the nucleus, whereas PR3² and MPO³ were not, or only weakly, recognized.

Conclusions: ANCAs typically found in active SVV⁴ are demonstrable both by indirect immunofluorescence and antigen-specific enzyme immunoassay, and strong reactivity to either PR3 or MPO is characteristic. Strong ANCA with MPO reactivity is also found in some patients with drug-induced syndromes (lupus, vasculitis). Intermediate to strong perinuclear ANCAs are found in a substantial proportion of patients with UC⁵ (40–60%) and RA⁶ (30–70%), but in these conditions the ANCAs have many antigen targets that are only weakly recognized.

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¹ ANCA = anti-neutrophil cytoplasm antibody

² PR3 = protease proteinase 3

³ MPO = myeloperoxidase

⁴ SVV = small vessel vasculitides

⁵ UC = ulcerative colitis

⁶ RA = rheumatoid arthritis

Objectives: To report the experience at the first Israeli Linear Accelerator Radiosurgery Unit in the management of 44 patients with acoustic neurinomas.

Methods: We analyzed the clinical records and imaging studies of all patients undergoing radiosurgery for acoustic neurinomas between 1993 and 1997, and quanitified the changes in tumor volume, hearing status, and facial and trigeminal nerve function. The contribution of radiation dose and original tumor volume upon those variables was also studied.

Results: At a mean follow-up of 32 months (range 12–60), 98% of the tumors were controlled (75% had shrunk; 23% had stable volume). The actuarial hearing preservation rate was 71%. New transient facial neuropathy developed in 24% of the patients, persisting in mild degrees in 8%. Neuropathy correlated primarily with tumor volume. Tumors with volumes 4 ml were at high risk when marginal radiation doses were 1,400 cGy. Dose reduction to a maximum of 1,400 cGy produced no neuropathies in the last 20 patients, still preserving tumor control rates.

Conclusions: Radiosurgery is an effective and cost-efficient therapeutic modality for newly diagnosed acoustic neurinomas in the elderly or medically infirm population, and for all residual or recurrent tumors after conventional surgery.

Methods: The demographic data and clinical manifestations of 144 TA¹ patients in this large multicenter study were recorded and compared with data obtained in a previous study.

Results: The patient population was older, with 24% ≥80 years compared to 6% in the previous study.  There was an increase in the number of nonspecific presenting symptoms, and less patients presented with the “classical” manifestations of headache (81% vs. 71%), fever (83% vs. 40%), jaw claudication (21% vs. 13%), and visual symptoms (47% vs. 24%). The median time from presentation to diagnosis was significantly reduced, from 5 to 1.5 months.

Conclusions: There were substantial changes in the clinical presentation of TA patients in Israel during 1980–95 compared to patients diagnosed prior to 1978. It is suggested that these changes may be attributed not only to the influence of aging of the population, but are due largely to increasing physician awareness to the spectrum of manifestations of TA, which leads to earlier diagnosis.

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¹TA = temporal arteritis