עמוד בית
יום א, 17.01.21

אוקטובר 2000


1 באוקטובר
בן-עמי סלע, יהודית בלונדר, אילנה פלד וג'ראר שוורץ

Treatment of Phenyl-Ketonuria due to Dihydropteridinereductase Deficiency 


B. Sela, J. Blonder, I. Peled, G. Schwartz

 

Institute for Pathological Chemistry and Child Development, Sheba Medical Center, Tel Hashomer; Clinical Biochemistry Dept., Sackler Faculty of Medicine, Tel Aviv University

 

Most cases of hyper-phenylalaninemia are due to deficiency of phenyl-alanine hydroxylase that converts phenyl-alanine to tyrosine. This enzymic reaction is facilitated by the co-factor tetrahydrobiopterin (BH4). A defect in the latter substrate leads to increased phenyl-alanine in 1-2 cases per million live births. Such cases are characterized by a degenerative brain process, and pronounced neurologic symptoms that cannot be prevented by a low phenyl-alanine diet alone.

In 3 male newborns a deficiency of dihydropteridine reductase (DHPR) activity was diagnosed, the last in a sequence of 3 enzymes involved in the formation of BH4. Successful outcome of treatment as well as the results of failure to diagnose and treat affected newborns are described, with emphasis on the logistic problems involved in mass screening.

גל גולדשטיין, עופר שפילברג, פיה רענני, אנג'לה שטרית ויצחק בן בסט

Acute Lymphoblastic Leukemia in Adults Treated with German Multicenter Study Group Protocols

 

G. Goldstein, O. Shpilberg, P. Raanani, A. Chetrit, I. Ben-Bassat

 

Institutes of Hematology and of Clinical Epidemiology, Sheba Medical Center and Sackler Faculty of Medicine, Tel Aviv University

 

Acute lymphoblastic leukemia (ALL) is a malignant disease whose incidence is relatively low among adults, unlike in children. Adults with ALL have a lower rate of long-term disease-free survival. During the last 20 years, a German multicenter group has shown that their protocol have achieved good results in adult ALL.

We reviewed the medical records of 35 ALL patients, aged 19-63 years, whome we treated with these protocol (1988-1997). The remission rate was 94%. At a median follow-up of 46 months the 2-year overall survival was 54% and the disease-free survival was 94%. Although 2 patients died of bone marrow transplant complications, no death was directly associated with drug toxicity. The main grade 3 or 4 side effects (WHO classification) were neutropenia (91%), thrombocytopenia (71%) and anemia (71%).

With there protocols we achieved high overall and disease-free survival rates, especially in comparison with other reports. Despite the high rate of severe treatment toxicity, there were no fatalities directly related to treatment. These results emphasize the need to concentrate treatment of adult ALL patients in large medical centers with expertise in the use of the complicated treatment protocols required.

מיכה פלד, דרור איזנבוד, דנה גולדשטיין, עדי רחמיאל ודב לאופר

Secondary Bone Grafting in Cleft Lip and Palate

 

M. Peled, D. Aisenbud, D. Goldstein, A. Rachmiel, D. Laufer

 

Maxillofacial and Oral Surgery Dept. and Cleft-Palate Clinic, Rambam Medical Center; and Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa

 

Results of reconstruction of residual alveolar bone defects in 52 patients operated between 1990-1998 were evaluated clinically and radiographically in a retrospective study.

Ages ranged between 9-37; 30 were males. The donor site of bone grafts in all was particulate cancellous marrow from the anterior iliac crest. 32 had unilateral clefts and 20 bilateral. Total cleft sites treated was 72.

Best results were achieved when bone grafting was carried out prior to the eruption of the canine tooth. The cleft space was closed and oro-nasal fistulas were eliminated in 42 (80%). Success rates in unilateral and bilateral cases were significantly different.

בלה בר-כהן, פרידה דקייזר ונורית וגנר

Reactions of Patients to Complementary Medicine

 

Bella Bar-Cohen, Freda DeKeyser, Nurit Wagner

 

Division of Nursing and School of Nursing, Hadassah Medical Center, Jerusalem

 

350 patients attending 11 large out-patient clinics completed questionnaires evaluating attitudes to, and experience with complementary medicine. 129 (36%) respondents reported using complementary medicine. 14% of them used complementary medicine for the current medical problem for which they were attending the clinic.

Pain was the most common medical problem for which complementary medicine was used, followed by respiratory problems and cancer. Common therapeutic modalities used were acupuncture, homeopathy, nutrition and herbal medicine.

Women, the secular as opposed to the religious, and those with higher education were more apt to use complementary medicine. No differences were found in age, national origin, length of living in Israel, and diet (vegetarian, natural foods or regular diet) between those who used complementary medicine and those who did not. No relationship was found between the use of complementary medicine and perceived poor health status, locus of control, or satisfaction with the doctor-patient relationship.

י' דומניץ, ר' אבישר וח' סביר

Corneal Infection in Wearers of Contact Lenses

 

Y. Domniz, R. Avisar, H. Savir

 

Assaf Harofeh Medical Center, Zerifin; and Hasharon Hospital, Petah Tikva

 

This is a 5-year retrospective survey of corneal infection in wearers of optical contact lenses (OCL). 23 of the 61 patients (38%; Hasharon Hospital) with positive cultures wore OCL. Visual acuity improved in 15 (65%), no change was noted in 4 (17.5%) and there was deterioration in 4 (17.5%), as compare with status on admission.

 

Pseudomonas aeruginosa was the most common cause of infections among OCL wearers. The improvement in visual acuity expected due to wearing OCL was affected by infections. After Staphylococcus albus infections had the highest rate (100%) of improvement in visual acuity and after Ps. aeruginosa the lowest rate (57.2%) of improvement, as well as the highest rate of deterioration (42.8%) found following recovery.

 

OCL wearers are at higher risk for damage to visual acuity following corneal infection, and highly virulent infections in OCL wearers are responsible for a high risk of damage to visual acuity.

אורי רובינשטיין, מאיר ויסברוד ובן-ציון גרטי

Life-Threatening Echovirus 11 Infection During First Month of Life

 

U. Rubinstein, M. Weisbrod, B. Garty

 

Neonatal Dept., Laniado Hospital, Kiriat Zans, Natanya; and Pediatrics B, Dept., Schneider Children's Medical Center, Petah Tikva

 

Infection with Echovirus 11 is mostly asymptomatic, but it may cause a wide variety of clinical diseases, from gastroenteritis to serious diseases such as meningitis and myocarditis. In small infants, especially during the first days of life, echovirus infection may appear as a sepsis-like illness, and cause disseminated intravascular coagulopathy and shock.

We present 2 infants with severe echovirus 11 infections. A 3.5-month old died within 24 hours of shock and probably myocarditis. The other, 6-days old, presented with meningitis, hepatitis and disseminated intravascular coagulopathy. It recovered after treatment with intravenous immunoglobulin.

Echovirus 11 may cause life-threatening infections in small infants. Pediatricians should be alert to the special characteristics of this disease.

אדוארד רוזנבלט, נאוה זיגלמן-דניאלי, ג'מאל זידאן, ניסים חיים ואברהם קוטן

Preservation of the Larynx in Advanced Cancer

 

Edward Rosenblatt, Nava Siegelmann-Danieli, Jemal Zidan, Nisim Haim, Abraham Kuten

 

Oncology Dept., Rambam Medical Center and Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa

 

The effectiveness of sequential chemo-radiotherapy in preserving the larynx in advanced laryngeal carcinoma was assessed. 4 Unselected patients (19 men and 2 women, mean age 60 years) with advanced squamous cell carcinoma of the larynx (T3-4/N0-3) received induction chemotherapy consisting of 2-3 cycles of cisplatin (100 mg/m2) and 5-flourouracil (1000 mg/m2/day) as a continuous infusion on days 1-5 followed by definitive radiotherapy: 50 Gy to the whole neck, 70 Gy to the larynx and clinically involved nodes, using a combination of 6 MV photons and 9-12 MeV electrons.

19 of The 21 patients responded to combined therapy but there was no response to induction therapy in 2 (10%) and 2 did not complete therapy due to severe toxicity. At a mean follow-up of 40 months, 7 had undergone total laryngectomy (33%), for an overall 5-year laryngeal preservation rate of 66%. Reasons for total laryngectomy in 2 patients were no response and in 5 tumor recurrence.

Mean survival was 39 months (range 11-46 months); at last follow-up 17 of 21 were alive and disease-free, 11 of whom had a functional larynx (65% of survivors). 2 died due to disease progression and 1 due to a cardiovascular event. Sequential chemo-radiation allows laryngeal preservation in about 2/3 of surviving patients without compromising survival.

י' קלוגר, ב' שגיא, ד' סופר, נ' חי וד' אורון

Combined Approach in Management of Penetrating Injury of Vertebral Artery

 

Y. Kluger, B. Sagie, D. Soffer, N. Hai, D. Oron

 

Rabin Trauma Center and Division of Invasive Radiology, Tel Aviv Medical Center

 

Penetrating injuries of the vertebral artery are not common. Although surgical control of this type of injury is well- documented, the combined approach (surgery and packing for temporary control of bleeding, followed by angiography and embolization for permanent control) is now recognized and practiced.

We describe a 40-year-old man who was stabbed in the neck. He was rushed to the operating theater in hemorrhagic shock. A vertebral artery injury was identified and packed. Angiography and embolization permanently controlled the bleeding.

The combined approach is safe and we recommend it in those in whom bleeding from the vertebral artery is initially well controlled with packing.

חיים גולן, מרינה לנדאו, אילן גולדברג ושרה ברנר

Dermatitis from Contact with Agave Americana

 

Haim Golan, Marina Landau, Ilan Goldberg, Sara Brenner

 

Dermatology Dept., Tel Aviv-Sourasky Medical Center

 

Various plants induce dermatitis in man. There have been only a few published cases of contact dermatitis caused by Agave americana (AA).

We report intentional exposure to AA in a soldier seeking sick leave, and review our previously reported cases. Treatment with oral antihistamines and topical saline compresses resulted in subsidence of the systemic symptoms within 24h and regression of cutaneous manifestations in 7-10 days.

Physicians should be alert to the possibility of self-inflicted contact dermatitis induced by exposure to plants, especially to A. americana. Systemic signs may accompany the cutaneous lesions.

רונן מרום, דן מירון, הרצל גבריאל ויוסף הורביץ

Thrombocytopenic Purpura as Sole Manifestation of Brucellosis in a Child

 

Ronen Marom, Dan Miron, Herzel Gabriel, Yosef Horowitz

 

Pediatric Dept. A, Pediatric Infectious Disease Service, and Pediatric Hemato-Oncology Unit, HaEmek Medical Center, Afula and Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa

 

Thrombocytopenic purpura associated with brucellosis has rarely been described in children. The thrombocytopenic purpura is usually part of the array of manifestations of brucellosis, such as fever, malaise, arthralgia, arthritis, hepatosplenomegaly and lymphadenopathy.

We describe a 4-year-old girl in whom severe thrombocytopenic purpura was the only manifestation of brucellosis which resolved after appropriate antibiotic therapy. We conclude that brucellosis should be included in the differential diagnosis of thrombocytopenic purpura in areas endemic for brucellosis, and when there is a history of exposure to infected food products.

הבהרה משפטית: כל נושא המופיע באתר זה נועד להשכלה בלבד ואין לראות בו ייעוץ רפואי או משפטי. אין הר"י אחראית לתוכן המתפרסם באתר זה ולכל נזק שעלול להיגרם. כל הזכויות על המידע באתר שייכות להסתדרות הרפואית בישראל. מדיניות פרטיות
כתובתנו: ז'בוטינסקי 35 רמת גן, בניין התאומים 2 קומות 10-11, ת.ד. 3566, מיקוד 5213604. טלפון: 03-6100444, פקס: 03-5753303