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Results for “pathological imaging data”

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PubMed2000

The impact of (18)F-fluoro-2-deoxy-D-glucose positron emission tomography (FDG-PET) lymph node staging on the radiation treatment volumes in patients with non-small cell lung cancer.

PURPOSE: (18)F-fluoro-2-deoxy-D-glucose positron emission tomography (FDG-PET) combined with computer tomography (PET-CT) is superior to CT alone in mediastinal lymph node (LN) staging in non-small cell lung cancer (NSCLC). We studied the potential impact of this non-invasive LN staging procedure on the radiation treatment plan of patients with NSCLC. PATIENTS AND METHODS: The imaging and surgical pathology data from 105 patients included in two previously published prospective LN staging protocols form the basis for the present analysis. For 73 of these patients, with positive LN's on CT and/or on PET, a theoretical study was performed in which for each patient the gross tumour volume (GTV) was defined based on CT and on PET-CT data. For each GTV, the completeness of tumour coverage was assessed, using the available surgical pathology data as gold standard. A more detailed analysis was done for the first ten consecutive patients in whom the PET-CT-GTV was smaller than the CT-GTV. Theoretical radiation treatment plans were constructed based on both CT-GTV and PET-CT-GTV. Dose-volume histograms for the planning target volume (PTV), for the total lung volume and the lung volume receiving more than 20 Gy (V(lung(20))), were calculated. RESULTS: Data from 988 assessed LN stations were available. In the subgroup of 73 patients with CT or PET positive LN's, tumour coverage improved from 75% when the CT-GTV was used to 89% with the PET-CT-GTV (P=0.005). In 45 patients (62%) the information obtained from PET would have led to a change of the treatment volumes. For the ten patients in the dosimetry study, the use of PET-CT to define the GTV, resulted in an average reduction of the PTV by 29+/-18% (+/-1 SD) (P=0.002) and of the V(lung(20)) of 27+/-18% (+/-1 SD) (P=0.001). CONCLUSION: In patients with NSCLC considered for curative radiation treatment, assessment of locoregional LN tumour extension by PET will improve tumour coverage, and in selected patients, will reduce the volume of normal tissues irradiated, and thus toxicity. This subgroup of patients could then become candidates for treatment intensification.

Carcinoma, Non-Small-Cell Lung↗

PubMedDate not supplied

Do arterial phase helical CT images improve detection or characterization of colorectal liver metastases?

Our goal was to determine if arterial phase images from dual phase helical CT improve either the detection or the characterization of hepatic metastases in patients with colorectal carcinoma. Sixty-two patients with known colorectal cancer underwent 65 dual phase helical CT examinations to evaluate for possible liver metastases. Three blinded reviewers independently evaluated the portal venous phase images alone to determine if hepatic metastases were present or absent. Arterial phase images were then analyzed to determine if they identified additional lesions or aided in characterizing small hepatic lesions. Scores of the two methods for diagnosing metastases were compared with the "gold standard" established by a consensus panel of three other radiologists who reviewed all images together with clinical, pathologic, and other imaging data. The addition of arterial phase imaging did not detect any new metastases. However, in 6 of the 64 technically adequate examinations, hepatic arterial phase images increased lesion conspicuity and significantly increased diagnostic confidence when compared with portal vein phase scans alone. In patients with colorectal cancer, the addition of arterial phase imaging does not increase sensitivity, but improves the specificity in diagnosing liver metastases in a small number of cases. Dual phase helical CT does not appear to be indicated in the evaluation of liver metastases from colorectal cancer.

Adult↗

PubMed2002

Etiology of spontaneous perirenal hemorrhage: a meta-analysis.

PURPOSE: We determine the most common etiology of spontaneous perirenal hemorrhage. MATERIALS AND METHODS: A MEDLINE search of the English language literature from 1985 to 1999 revealed 47 publications and 165 cases of spontaneous renal hemorrhage meeting our study entry criteria. These criteria were presentation of raw data including imaging modality, pathological confirmation (123 cases) or long-term (greater than 2 years) (42) imaging and/or clinical followup and no history of recent trauma, anticoagulant use, dialysis or renal transplant. Meta-analysis was performed using analysis of counts derived from contingency tables and pooled and stratified analysis. RESULTS: Hemorrhage was identified by ultrasound in 56 of 100 cases (56%) and by computerized tomography (CT) in all 135 cases assessed (100%). Etiology was correctly identified with an overall sensitivity and specificity of 0.11 and 0.33 for ultrasound and 0.57 and 0.82 for CT. Angiography in 81 cases revealed active bleeding in 11. The most common etiology of spontaneous renal hemorrhage was benign or malignant neoplasm (101 cases, 61%) with angiomyolipoma being predominant (48) followed closely by renal cell carcinoma (43). Vascular disease was the next most common offender (28 cases, 17%) with polyarteritis nodosa occurring most frequently (20). CONCLUSIONS: The most common cause of spontaneous perirenal hemorrhage is renal neoplasm and approximately 50% of such neoplasms are malignant. CT is the method of choice for evaluation of perirenal hemorrhage, although its sensitivity for detection of underlying etiology is only moderate.

Adolescent↗

PubMedDate not supplied

Successful outcome after surgical management in two cases of the "painful variant" of Hashimoto's thyroiditis.

OBJECTIVE: To describe two cases of the rare "painful variant" of Hashimoto's thyroiditis (HT) that were refractory to medical management and in which surgical intervention provided the definitive treatment. METHODS: We thoroughly review the clinical history as well as the laboratory, imaging, and surgical pathology data in these cases, and follow-up of the clinical response over time is provided. The relevant literature is also discussed. RESULTS: A 56-year-old woman, who had remotely undergone a left hemithyroidectomy and had been diagnosed with HT, sought further assessment because of neck pain and edema. Treatment with corticosteroids was partially successful but led to the development of Cushing's syndrome. A 32-year-old man had pain and swelling of the thyroid and was diagnosed with HT shortly thereafter. Levothyroxine treatment was unsuccessful. Both patients underwent thyroidectomy. Chronic lymphocytic thyroiditis (HT) with a variable degree of fibrosis was found on assessment of pathology specimens. The patients remained asymptomatic after the surgical procedure and did not require any further anti-inflammatory therapy. CONCLUSION: In selected cases, surgical treatment may become necessary for effective and permanent control of symptoms and local signs in painful HT. Access to experienced endocrine surgeons is important in order to avoid postoperative complications because the thyroid gland may be small or fibrosed in this rare variant of HT.

Adult↗

PubMed2003

Evaluating non-invasive medical imaging for diagnosis of carotid artery stenosis with ischemic cerebrovascular disease.

OBJECTIVES: To assess the value of non-invasive medical imaging for diagnosis of carotid artery stenosis and to study the relationship between carotid stenosis and brain infarction. METHODS: Thirty-one patients with a total of 62 carotid arteries were studied using Doppler ultrasound (DUS) and magnetic resonance angiography (MRA). Eleven of the 31 patients were studied using CT angiography (CTA). CT and MRI of the brain were also done in all patients. The imaging results in 5 patients were compared with those of digital subtraction angiography (DSA). Eight patients with severe stenosis received carotid endarterectomy. The comparisons between the imaging results and pathological data were conducted in 2 patients. RESULTS: Of the 62 carotid arteries, mild stenosis was seen in 11, moderate in 14, severe in 21, obstructed in 4 and normal in 12. In 25 patients with severe stenosis or occlusion of carotid arteries, there were a total of 35 focal or multifocal infarcts on the ipsilateral cerebral hemisphere, and 15 infarcts on the contrary side. Compared with the results of the operations, DUS correctly diagnosed 6 stenoses, while MRA identified 7 correctly and CTA 8. Agreement on location of stenosis as performed by endarterectomy, DUS, MRA and CTA occurred in all patients. Histologically, areas of calcification and fibrousness were related to high densities on CTA, strong echoes on DUS, and low signal intensities on MRA. Relatively large amounts of necrotic material and foam cells filled with lipolytic materials on the intimal surface of arteries were observed during pathologically, corresponding to low and iso-densities on CTA, low echoes on DUS, and inhomogeneous signal intensities on MRA. CONCLUSIONS: A strong link exists between carotid stenosis and brain infarction. The combined use of DUS, MRA and CTA can improve diagnostic accuracy for the assessment of carotid artery stenosis, as well as assist in ascertaining the nature of the plaque.

Adult↗

PubMedDate not supplied

Hepatocellular carcinoma presenting with obstructive jaundice: a clinicopathological study of eight cases.

BACKGROUND/AIMS: The prognosis of icteric type hepatocellular carcinoma is extremely poor, not only because of obstructive jaundice, but also because of difficulties for early diagnosis. The aim of this study is to evaluate characteristics of icteric hepatocellular carcinoma for early diagnosis. METHODOLOGY: Eight patients with icteric hepatocellular carcinoma among 326 patients with hepatocellular carcinoma in our hospitals were retrospectively examined by laboratory data, image studies and pathology studies. RESULTS: Most cases were already advanced, with a portal tumor thrombus at the time of diagnosis. Imaging studies fail to reveal tumors because this type of hepatocellular carcinoma has an irregular faint margin and has lost the characteristic pattern of hepatocellular carcinoma, such as capsular formation or early enhancement. Pathology observations demonstrated poorly or moderately differentiated hepatocellular carcinoma in all our cases. CONCLUSIONS: This type of hepatocellular carcinoma should be considered in cirrhotic patients with obstructive jaundice or in patients with high tumor marker levels even if image studies fail to reveal tumors. For better prognosis, combination therapies such as biliary drainage, support for portal flow as well as treatment for the hepatocellular carcinoma, are necessary.

Aged↗

PubMedDate not supplied

MR imaging of acute spinal cord trauma.

The thoracic spinal cords of five mongrel dogs were imaged with a 1.5 T MR scanner before and after trauma induced by a well-established method of spinal cord impaction that produces central cord hemorrhagic necrosis. The anesthetized dogs were studied acutely with a 5-in. circular surface coil, 12-cm field of view, sagittal and axial partial-saturation (TR = 600, TE = 25 msec) and spin-echo (TR = 2000, TE = 25-100 msec) techniques. One normal dog was used as a control. The cords were surgically removed and histologically examined. Direct correlation of the pathologic findings and imaging data showed that at the level of trauma there was obliteration of epidural fat and CSF spaces secondary to central cord hemorrhage and edema. The traumatized cords expanded to fill the bony canal, and there was loss of visualization of the internal anatomy of the cord (gray- and white-matter structures). We conclude that MR can accurately identify cord hemorrhage and edema within a few hours of spinal trauma.

Animals↗

PubMed1998

Parathyroid carcinoma.

BACKGROUND: Parathyroid carcinoma is rare and represents 0.1% to 5% of the cases of hyperparathyroidism. New accounts are important to the understanding of these tumors. Experience in 9 cases is reported. METHODS: From 1970 to 1995, 10 patients with parathyroid carcinoma demonstrated by clinical course or pathologic features of malignancy were treated. The patient's clinical data, laboratory and imaging examinations, surgical findings, pathology, recurrences, and survival were analyzed. RESULTS: One male patient was excluded because of insufficient data. Average age was 51 years, with female:male ratio of 2:1. Average calcium level was 14,3 mg/dL. Palpable mass was found in 55%, bone disease in 89%, and renal disease in 78%. Four patients were reoperations. Five were operated on for hyperparathyroidism (1/tertiary). Capsular invasion was the most incident pathologic feature. Local recurrence occurred in 55%; neck node and bone metastasis in 11%, and lung in 33%. Two patients are alive and 5 died of disease. CONCLUSIONS: Parathyroid carcinoma has clinical and laboratory features that can help diagnosis at the first surgery. It seems to have variable malignancy.

Adenoma↗

PubMed1997

Optical imaging of anatomical maps derived from magnetic resonance images using time-independent optical sources.

We present a model suitable for computing images of absorption cross sections of thick tissue structures illuminated at near infrared (NIR) wavelengths from tomographic projection data. Image reconstruction is accomplished by solving a system of linear equations derived from transport theory. Reconstruction results using different algebraic solvers are shown for anatomical maps of the breast, derived from magnetic resonance imaging data, containing two simulated pathologies, in which case qualitatively good reconstructions were obtained. Evaluation of magnetic resonance (MR) data to optimize NIR optical tomographic imaging methods and to assess the feasibility of a combined MR-optical measurement scheme is discussed.

Adipose Tissue↗

PubMed2003

Volume registration using needle paths and point landmarks for evaluation of interventional MRI treatments.

We created a method for three-dimensional (3-D) registration of medical images (e.g., magnetic resonance imaging (MRI) or computed tomography) to images of physical tissue sections or to other medical images and evaluated its accuracy. Our method proved valuable for evaluation of animal model experiments on interventional-MRI guided thermal ablation and on a new localized drug delivery system. The method computes an optimum set of rigid body registration parameters by minimization of the Euclidean distances between automatically chosen correspondence points, along manually selected fiducial needle paths, and optional point landmarks, using the iterative closest point algorithm. For numerically simulated experiments, using two needle paths over a range of needle orientations, mean voxel displacement errors depended mostly on needle localization error when the angle between needles was at least 20 degrees. For parameters typical of our in vivo experiments, the mean voxel displacement error was < 0.35 mm. In addition, we determined that the distance objective function was a useful diagnostic for predicting registration quality. To evaluate the registration quality of physical specimens, we computed the misregistration for a needle not considered during the optimization procedure. We registered an ex vivo sheep brain MR volume with another MR volume and tissue section photographs, using various combinations of needle and point landmarks. Mean registration error was always < or = 0.54 mm for MR-to-MR registrations and < or = 0.52 mm for MR to tissue section registrations. We also applied the method to correlate MR volumes of radio-frequency induced thermal ablation lesions with actual tissue destruction. In this case, in vivo rabbit thigh volumes were registered to photographs of ex vivo tissue sections using two needle paths. Mean registration errors were between 0.7 and 1.36 mm over all rabbits, the largest error less than two MR voxel widths. We conclude that our method provides sufficient spatial correspondence to facilitate comparison of 3-D image data with data from gross pathology tissue sections and histology.

Anatomy, Cross-Sectional↗

PubMed1996

[Computer-assisted surgery of the paranasal sinuses with an opto-electronic stereotaxic system].

BACKGROUND: A basic problem common to all systems for computer-assisted surgery (CAS) is the exact registration and referencing, that is, the transfer of preoperative image data to the intraoperative pathology. A system for computer-assisted ENT surgery should provide high precision as well as noninvasive registration and referencing. We present a system designed for such use in paranasal sinus surgery that is based on optical digitizing with several custom-made self-localizing surgical instruments. METHODS AND RESULTS: The system tracks the correct alignment of the head during CT data acquisition and continuously throughout surgery. A transformation matrix and a plane equation of the CT scan are used to match points of the object space to the image space detecting fiducial markers on the CT scans by an automatic algorithm. For position measurements a special reference frame and surgical instruments were developed and equipped with infrared light-emitting diodes that could be detected in real time. Measurements for repositioning of the reference system in a model and during real operations demonstrated a mean error ranging from 0.69 to 1.01 mm and from 0.76 to 1.67 mm respectively. Clinical experience with the application of the system for 15 patients who underwent surgery for different paranasal sinus pathologies is reported. CONCLUSIONS: Results suggest that the non-invasive reference system and the locatable surgical tools may be effective, accurate and useful for computer-assisted identification of intranasal structures. Such systems may contribute to further improvement of minimal invasive intranasal sinus surgery.

Equipment Design↗

PubMed2002

Prognostic value of [18F]FDG-PET imaging in small cell lung cancer.

Positron emission tomography (PET) utilizing fluorine-18 fluorodeoxyglucose (FDG) has been used in the evaluation of non-small cell lung cancer (NSCLC). Recently its use in the staging of small cell lung cancer (SCLC) has been reported. However, the prognostic value of FDG-PET imaging in SCLC has not been studied. We performed a retrospective analysis to assess this, with the following hypotheses: (1) PET-positive patients would have a less favorable prognosis than PET-negative patients and (2) a high standardized uptake value (SUV) would be associated with a poor prognosis. Retrospective review of a mixed population of treated and untreated patients imaged between 1995 and 2000 was performed. Results of 62 scans in 46 patients were analyzed. There were 8 untreated and 38 treated patients. Findings were correlated with pathology, computed tomography/magnetic resonance imaging and clinical data. The sensitivity of PET scanning was 100% with pathological correlation. The prognostic value of a positive PET study was determined. Overall survival in PET-positive cases was significantly worse than that in PET-negative cases ( P=0.0108). Correlation of SUV(max) with survival showed a significant negative correlation ( P=0.0021). In the eight untreated patients, scans were strongly positive and in all cases the scan results concurred with the final clinical stage assigned on the basis of conventional methods. We conclude that FDG-PET imaging provides prognostic information in treated patients. A positive study and a high SUV(max) are significantly associated with poor survival. Additionally, FDG-PET may be helpful in staging and follow-up.

Adult↗

PubMed2002

Optic neuromyelitis syndrome in Brazilian patients.

OBJECTIVES: To report the clinical features and outcome of 24 Brazilian patients with optic neuromyelitis syndrome (ONM); discuss the underlying pathological events associated with the ONM syndrome; review the nosological situation of ONM in the group of inflammatory and demyelinating diseases of the central nervous system. PATIENTS AND METHODS: Patients with ONM treated at the Hospital da Lagoa, Rio de Janeiro were studied. Demographic, clinical, magnetic resonance imaging, cerebrospinal fluid, and pathological data were analysed. RESULTS: The study consisted of 20 women, four men of whom 10 were white and 14 Afro-Brazilians. Clinical course was recurrent in 22 cases and monophasic in two. Neurological manifestations at inclusion were: sensory impairment (66%), bilateral (41.6%) or unilateral blindness (20.8%), paraplegia or quadriplegia (37.5%). The EDSS was moderate/severe in 70.8%. The underlying pathological events were respectively pulmonary tuberculosis and upper respiratory infection in the two monophasic cases; in the 22 recurrent ONM patients: pulmonary tuberculosis (3), neurocysticercosis (1), polyarteritis nodosa (1), antinuclear antibody and rheumatoid factor (1), antiphospholipid antibody primary syndrome (1), diabetes mellitus (1), hypothyroidism (1), and amenorrhea-galactorrhea (4). Normal cerebrospinal fluid was found in 52% and an inflammatory profile in 48%. Only four recurrent ONM white patients had brain and spinal cord magnetic resonance imaging and cerebrospinal fluid findings compatible with the diagnosis of multiple sclerosis. Large lesions were seen in 62% of spinal magnetic resonance images. Six of 12 recurrent ONM Afro-Brazilian died. There were no statistical differences in the demographic data of the two ethnic groups. Afro-Brazilians were significantly more severely impaired and had a higher mortality rate than the white patients. CONCLUSION: These cases were classified as follows: two monophasic acute disseminated encephalomyelitis; one recurrent disseminated encephalomyelitis; three recurrent ONM associated with Hughes syndrome, autoantibodies and polyarteritis nodosa; six recurrent ONM with endocrinopathies; and finally, four multiple sclerosis cases. The remaining cases were not associated with any other condition. It would seem clear that ONM is a syndrome rather than a single disease.

Adult↗

PubMed1996

Minimally invasive neurosurgery.

BACKGROUND: The philosophy and practice of minimally invasive surgery have fundamentally altered the practice of general and gynaecological surgery, and are currently transforming the practice of neurosurgery. The goal of minimally invasive surgery is to reduce tissue disruption and thus morbidity. This is a review of the development, applications, and benefits of minimally invasive neurosurgery, and its wider surgical implications. METHODS: A synthesis of the literature is presented. RESULTS: Interactive computer imaging with frame-based and frameless stereotaxy, ultrasonographic and endoscopic techniques are increasingly being used independently and in combination in order to execute minimally invasive approaches and to navigate a safe path within the cranium or spine. Interaction using an interlinking stereotactic instrument or robot between the surgeon, a graphic interface (such as a computer workstation) and the patient, enables precise planning and execution of surgery with exact correspondence of imaging data and the living pathology. The direct influence of virtual reality, cybernetics, robotics, and telepresence will further revolutionize the practice of neurosurgery and will impact increasingly on other surgical disciplines. Safety and precision are further enhanced by intra-operative physiological monitoring techniques. Interventional neuroradiology and stereotactic radiosurgery add further dimensions to the minimally invasive approach and may not only diminish the need for open surgery, but in selected cases obviate the need altogether. CONCLUSIONS: Minimally invasive neurosurgery is a major force in contemporary neurosurgery and many of the current neurosurgical applications will have far-reaching effects on the practice of surgery in general.

Brain↗

PubMed1997

A non-invasive patient registration and reference system for interactive intraoperative localization in intranasal sinus surgery.

A basic problem common to all systems for computer assisted surgery (CAS) is patient referencing, or the transfer of preoperative image data to the intraoperative pathology. The authors describe a highly precise CAS system with non-invasive referencing that can be used in ear, nose and throat (ENT) surgery of the paranasal sinuses. It is based on optical digitizing with several custom-made self-localizing surgical instruments. The accuracy of the system was tested in an experimental model using a plastic head. Measurements of repositioning the reference bow had a mean error of 0.81 mm +/- 0.31 mm. The system was evaluated clinically with 11 patients who received surgery for different pathologies of the paranasal sinuses. These trials met with a high rate of success and specific results are reported.

Biopsy↗

PubMedDate not supplied

High-resolution parathyroid sonography.

The purpose of this study was to assess the diagnostic utility of parathyroid ultrasonography to differentiate causes of hypercalcemia in dogs. We analyzed qualitative and quantitative ultrasound imaging findings and clinical pathology data from 33 dogs that underwent parathyroid ultrasound examination as part of the diagnostic evaluation for hypercalcemia. Diagnoses of the diseases causing hypercalcemia included parathyroid carcinoma (n = 5), parathyroid adenoma (n = 15), parathyroid adenomatous hyperplasia (n = 6), chronic renal insufficiency (n = 3), and hypercalcemia of malignancy (n = 4). All parathyroid lesions were round or oval and hypoechoic compared with surrounding thyroid parenchyma. Adenomatous and adenocarcinomatous glands were 4 mm or larger in longest linear measurement on US examination and were statistically significantly larger than hyperplastic glands. (p < 0.001) Linear measurements of parathyroid glands acquired at the time of ultrasound examination correlated well with direct size determination after surgical excision. (r2 = 0.9, p < 0.0001) Parathyroid lesions > or = 4 mm are highly suspicious for parathyroid adenoma or carcinoma, while US lesions < 4 mm most likely represent primary adenomatous hyperplasia or secondary parathyroid hyperplasia. Parathyroid size estimation from ultrasound examination is an accurate predictor of true size.

Animals↗

PubMed2002

Expression of MMP-2,TIMP-2 protein and the ratio of MMP-2/TIMP-2 in gallbladder carcinoma and their significance.

AIM: To study the correlation between expression of MMP-2, TIMP-2 protein and the ratio of MMP-2/TIMP-2 and clinical-pathological parameters of patients with gallbladder carcinoma. METHODS: Carcinomas (n=45) and polypoid lesions (n=15) of the gallbladder were studied for the expression of MMP-2 and TIMP-2 protein by immunohistochemical avidin-biotin-complex method and image analysis. Clinical-pathological data of patients with gallbladder carcinoma such as histological type, grade of differentiation, level of infiltration, liver invasion and lymph node involvement, etc, were recorded. RESULTS: There was significant difference between the average level (1.123+/-0.108 vs 1.030+/-0.054, P=0.002) of MMP-2, the ratio (1.050+/-0.013 vs 0.937+/-0.078, P=0.003) of MMP-2/TIMP-2 in gallbladder carcinomas and in polypoid lesions of the gallbladder. Significant difference was found between the expression of MMP-2 in early stage and advanced tumors, but there was no correlation between MMP-2 protein expression and histological type, differentiation degree, infiltration level, lymph node involvement or liver invasion. Although no difference was observed between TIMP-2 expression and histological type or differentiation degree, significant difference was found between TIMP-2 expression and different Nevin stage, infiltration level, local lymph node involvement or liver invasion (1.168+/-0.067 vs 1.048+/-0.075, 1.170+/-0.062 vs 1.039+/-0.069, 1.039+/-0.076 vs 1.147+/-0.083, 1.048+/-0.074 vs 1.103+/-0.095, P<0.05). MMP-2/TIMP-2 ratio did not correlate with histological type, grade of differentiation and liver invasion, but significant differences were found between MMP-2/TIMP-2 ratio and different Nevin stage, infiltration level and lymph node involvement in patients with carcinoma of gallbladder. CONCLUSION: TIMP-2 and MMP-2/TIMP-2 ratio could reflect more accurately biological characteristic of gallbladder carcinoma and MMP-2/TIMP-2 ratio might be a new significant marker in early diagnosis, in the judgment of invasion or metastasis and the estimate of prognosis in patients with gallbladder carcinomas.

Adenocarcinoma↗

PubMed1999

Pulmonary alveolar proteinosis in Israel: ethnic clustering.

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 3.7 x 10(6) and an incidence of 0.36 x 10(6)/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 3.7 x 10(6). 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.

Adolescent↗