תוצאת חיפוש

Pulmonary Involvement in Osler- Weber-Rendu Syndrome 

Efrat Bron-Harlev, Benjamin Zeevi, Ben-Zion Garty

Dept. of Medicine B and Cardiac Catheterization Unit, Schneider Children's Medical Center, Petah Tikva

Hereditary hemorrhagic telangiectasia (Osler-Weber-Ren-du syndrome) is a group of autosomal dominant diseases with variable penetration, characterized by vascular malformations. Recently hereditary hemorrhagic telangiectasia has been found to be a phenotypic expression of mutations in genes located on chromosomes 9 and 12, and possibly of other genes located on other chromosomes.

We describe 2 patients with hereditary hemorrhagic telangiectasia and pulmonary involvement who presented with repeated complaints of dyspnea and cyanosis and were diagnosed as having long-standing asthma. Both were treated with therapeutic catheterization and embolization with good clinical outcomes.

Endoscopic Sinus Surgery for Extracranial Complications of Sinusitis

E. Deutsch, I. Hevron

ENT Dept., Bikur Cholim Hospital, Jerusalem

Orbital subperiosteal abscess (SPA) and Pott's puffy tumor (PPT) are the major extracranial complications of acute sinusitis. These complications are aggravated by the close anatomic relationships between the nasal sinuses and the orbits and frontal bone. Furthermore, the rich diploic venous drainage of the region enhances the spread of the infection.

Between 1992 and 1997, 16 patients (mean age 12 years, range 2-15, 10 of them males), 11 of them with SPA and 4 with PPT were operated on by the senior author by means of endoscopic sinus surgery (ESS). Indications for operation included: CT findings of abscess formation and lack of clinical improvement after 48 hours of IV antibiotic therapy. Clinical resolution of symptoms was achieved in all and there were no operative or postoperative complications.

We emphasize and explain the advantages of ESS over external approaches in surgery for extracranial complications of acute sinusitis by several facts: the technique treats the source of the disease, clinical success rate is high, morbidity is low, and facial distortion and poor cosmetic results completely avoided.

Evaluation of Asthmatic Children Presenting at Emergency Rooms

Haim Bibi, Elena Shoychet, David Shoseyov, Michael Armoni, Emil Chai, Dorit Ater

Pediatric Pulmonary Clinic and Pediatric Dept., Barzilai Medical Center, Ashkelon; Pediatric Pulmonary Clinic, Bikur Cholim Hospital, Jerusalem; Emergency Room, Barzilai Medical Center, Ashkelon; and Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer Sheba

Bronchial asthma in the pediatric age group has become prevalent recently. Many children who suffer from asthma arrive at the emergency room (ER) with exacerbations which did not respond to medical treatment at home.

Between July and December 1997, 136 children 8 months to 14 years of age (61% below 3 years), were studied in our pediatric ER. Investigation included physical examination and pulse oximetry, which were used as guidelines for scoring the children on arrival and post-treatment. Spirometry was done in those who could cooperate. For each patient a detailed questionnaire about medical and sociodemographic factors was filled.

Primary pediatricians used mainly beta-agonist and corticosteroid inhalators, while pediatric pulmonologists used mainly inhaled steroids. There was no relationship between severity of attack on arrival at the ER, mode of treatment and speed of recovery in the ER. More children treated by a general pediatrician more were admitted to hospital. Low parental education and paternal smoking were risk factors for recurrent hospital admissions.

Our results indicate that parents must be educated to stop smoking, especially those with asthmatic children, and primary pediatricians should be updated with regard to proper treatment and follow-up of asthma.

Multisystem Disease Caused by BCG Imitating Miliary Tuberculosis

I. Tsikonov, D. Yeshurun, J.E. Naschitz

Dept. of Medicine A, Bnai Zion Medical Center and B. Rappaport Faculty of Medicine, The Technion, Haifa

As the prevalence of tuberculosis is on the rise in western countries, we present a 79-year-old man who developed a pulmonary tuberculosis-like syndrome following immunotherapy with BCG for carcinoma of the urinary bladder. The symptoms subsided following 3-drug antitubercular treatment, and the addition of steroids following negative cultures for Mycobacterium tuberculosis. The course of this disease, named BCG-osis, is much more favorable than miliary tuberculosis, even with milder treatment. It is important to keep in mind this phenomenon now that there is increasing treatment of cancers with BCG.

Budd-Chiari Syndrome

S. Goland, S.D.H. Malnick, L. Shvidel, E. Mor, Z.M. Sthoeger, E. Evron

Medical Depts. C and B, and Hematology Institute, Kaplan Hospital, Rehovot; and Surgical Dept. B, Rabin Medical Center, Beilinson Campus, Petah Tikva

Budd Chiari syndrome is a rare disorder resulting from occlusion of hepatic venous drainage by hepatic vein thrombosis or by a membranous web in the inferior vena cava. In western countries the commonest causes are myeloproliferative disorders and hypercoagulable states. Presentation may be acute with rapid accumulation of ascites and hepatic failure, or subacute with symptoms developing over a few months. A chronic progressive form has also been described. On presentation there is usually abdominal pain, ascites, and hepatosplenomegaly; hepatic encephalopathy is found in about a third. Noninvasive, ultrasound-Doppler is recommended in diagnosis, and has a high correlation with hepatic venography. Liver biopsy is required for therapeutic decisions. Those with advanced hepatic failure or severe fibrosis on liver biopsy are referred for hepatic transplantation. When biopsy shows only hepatic congestion and inflammatory infiltrates, portosystemic shunting is recommended. We present a 61-year-old woman with ascites and hepatosplenomegaly that had developed over the courses of a few months. Budd-Chiari syndrome with chronic myelofibrosis and congenital protein C deficiency were diagnosed. Portosystemic shunt was performed but death from sepsis followed shortly.

Pediatric Flexible Bronchoscopy

Haim Bibi, David Shoseyov, Michael Armoni, Melly Ohali, Shlomo Pollak, Menachem Schlesinger

Pediatric Dept., Pediatric Emergency Room, Pediatric Day Care Hospital and Immunology and Clinical Allergy Unit, Barzilai Medical Center, Ashkelon; and Bikur Holim Hospital, Jerusalem (Affiliated with the Faculty of Health Sciences, Ben-Gurion University of the Negev)

Between 1993-1996, 200 pediatric flexible bronchoscopies were performed. Indications were: chronic cough (158 children), persistent pulmonary infiltrates (89), recurrent stridor (28), suspected tracheobronchial foreign body (20), suspected tuberculosis (17) and hemoptysis (3). Some children had more than 1 indication. 124 patients were boys (mean 4.18+2.86 years; range 1 month-15 years) and 76 were girls (mean 4.39+2.7 years; range 4 months-15 years). The procedure included direct vision recorded by video-camera and broncho-alveolar lavage; the lavage fluid was sent for culture, Gram and Ziehl-Nielsen stains and for cytology. There were a few minor side effects: mild stridor which resolved within a few hours (10 children) and transient fever (3). This simple, flexible instrument was effective and helpful in the diagnosis and treatment of children with respiratory symptoms in a secondary hospital facility.