תוצאת חיפוש

Liver Failure with Coagulopathy in an Infant with Tyrosinemia

Moshe Nussinovitch, Gadi Campino, Rivka Shapira, Benjamin Voluvitz, Jacob Amir

Department of Pediatrics, Schneider Children's Medical Center of Israel, Petah Tikva, Sackler Faculty of Medicine, Tel-Aviv University, Tel-Aviv, Israel

Tyrosinemia is an inherited autsomal recessive condition. We present a 5 week-old boy with this disorder. He was admitted because of a fever, vomiting and lethargy. The laboratory tests confirmed a coagulopathy with prolonged prothrombin time (PT), partial thromboplastin time (PTT) and a decreased serum fibrinogen. The a-fetoprotein level was markedly elevated. To confirm the diagnosis of tyrosinemia, quantitative urinary succinylacetone was measured. Although overt liver failure with coagulopathy may be part of the representation of tyrosinemia, a significant coagulopathy in the absence of overt signs of liver disease has not been emphasized as a clue to the diagnosis of this condition.

אשר בשירי, בוריס פורמן, משה מזור

החטיבה למיילדות וגינקולוגיה, מרכז רפואי אוניברסיטאי סורוקה, באר שבע

הנקת היילוד היא בעלת יתרונות מוכחים לגבי בריאות הילד ומקובל על הכל, שחלב אם מכיל את המרכיבים התזונתיים הטובים ביותר בהשוואה לתחליפים שונים, וכמו כן, נועד לו תפקיד מכריע בהישרדות הילודים במדינות מתפתחות. לאור זאת, תפקיד מכריע בהישרדות הילודים במדינות מתפתחות. לאור זאת, נודעת חשיבות רבה לאמצעי המנע הנבחר בזמן ההנקה, כך שיהיה בעל השפעות מיקטיות על החלב ועל הילוד.

בחירת אמצעי המנע (contraceptives) לאשה מיניקה צריכה להיקבע באופן אישי ויש לקחת בחשבון מספר שיקולים לגבי הסוג הנבחר ומועד תחילת הנטילה: העדפה אישית, השפעה על הפוריות, השפעה על ההנקה, עלות, שיעור השפעות-הלוואי, מידת היעילות, משך ההשפעה וזמינות השיטה.

רונית חיימוב-קוכמן, שמחה יגל

המח' לרפואת נשים ויולדות, ביה"ח האוניברסיטאי, הסתדרות מדיצינית הדסה, הר הצופים, ירושלים

חקר גנום האדם החל לפני למעלה מ-40 שנה בתגלית, שתאים סומטיים מכילים 46 כרומוסומים. בין השנים 1956 ל-1960, הוגדר הבסיס הגנטי לתיסמונות שונות שהיו ידועות זה מכבר. כל סטייה במספר הכרומוסומים מ-46 (23 זוגות כרומוסומים) קרויה אנאפלואידיות, והיא מהווה את הליקוי הגנטי הנפוץ ביותר. אנאפלואידיות נגרמת בדרך כלל מהפרעה בצימוד הכרומוסומים במהלך החלוקה התאית במיטוזה ובמיוזה.

דן ליבוביץ, אריה לינדנר

המח' לאורולוגיה, מרכז רפואי "אסף-הרופא", הפקולטה לרפואה סאקלר, אוניברסיטת תל-אביב

סרטן הוא סיבת המוות השניה בשכיחותה לאחר מחלות הלב וכלי-הדם. בשלב התהוות הגרורות, אפשרויות הטיפול מצומצמות, ועל אף ריבוי התכשירים הכימותרפיים הפועלים במנגנונים שונים, רק ממאירויות ספורות ניתנות לריפוי בכימותרפיה: סרטן האשך, ליקמיה, לימפומה, כוריוקארצינומה הריונית ושאתות פדיאטריות, כגון מחלת Wilm.

אנגיוגנזיס הוא תהליך הכרחי להתהוות שאתות, להתפשטותן, לחודרנותן ולהתרחשות גרורות. עיכוב תהליך האנגיוגנזיס נושא פוטנציאל לעצור את מהלך המחלה ובמצבים מסויימים, אף לרפא את החולה. הטיפול נוגדי-האנגיוגנזיס מתאפיינים ברעילות נמוכה, המאפשרת טיפול בטוח גם בחולים במצב ירוד בשל מחלתם הבסיסית.

תאלידומיד פותח בשנות ה- 50 כתכשיר נוגד-בחילה ומשרה שינה, שנועד להקל על נשים הרות. תוצאות הטיפול בתאלידומיד היו הרות-אסון – תיסמונת הדיסמלה (היפופלזיה של הגפיים) בילדי האימהות שנטלו את התרופה במהלך ההריון. עם זאת, בשל ההשפעה נוגדת האנגיוגנזיס של התאלידומיד, שבה ומתעוררת התעניינות מחודשת בתרופה זו כטיפול בסרטן.

[...]

בסקירה זו, מובאים הניסיונות ליישם את הטיפול בתאלידומיד בחולי סרטן.

ערן תמיר, דרור רובינסון, גבריאל אגר, נחום הלפרין

המח' לאורתופדיה, מרכז רפואי אסף הרופא, צריפין

חומצה היאלורונית היא מולקולת ענק במשקל מולקולתי של 3.5-5 מיליון דלטון, המורכבת משרשרת פוליסאכארידית של D-glucuronate ו-N-acetylglucosamine המופיעים לסירוגין. חומצה היאלורונית מיוצרת על ידי תאים מסוג A בריקמה הסינובית של המיפרק ומהווה מרכיב חשוב התורם לתכונות המגנות של הנוזל הסינובי, הכוללות בלימת זעזועים ושימון המישטחים הסחוסיים. בנוסף לכך המישטח הפנימי של הממברנה הסינובית מצופה בשיכבת חומצה היאלורונית, המונעת נדידת תאים ומולקולות גדולות לתוך המיפרק.

שרון ארליך(1), דלית מודן(1) וגדעון פרת(2)

(1) היח' לאנדוקרינולוגיה, (2) המח' לטיפול נמרץ ילדים, מרכז רפואי שיבא, תל-השומר, רמת-גן

לפטין הוא חלבון בעל 167 חומצות-אמינו ומשקלו המולקולתי הוא כ-16kDa. שמו נגזר מהמלה היוונית leptos, שמשמעותה "רזה". ללפטין מספר תפקידים, שהעיקרי ביניהם הוא ויסות מאזן האנרגיה על ידי דיכוי תיאבון והגברת הוצאת אנרגיה. כמו-כן, הוא מהווה סמן למאכת הרבייה וכהורמון המשפיע על מיגוון תהליכי חילוף-חומרים, כגון הפרשת אינסולין וליפוליזה. קיימות עדויות לכך, שללפטין יש גם תפקיד מרכזי בתגובה למצבי דחק והוא מצוי ביחסי גומלין הדוקים עם ציר ההיפותאלאמוס-היפופיזה-טוחה (adrenal). המטרה בסקירה זו לסכם בקצרה את הידע הקשור בלפטין, בהסתמך על הספרות העכשווית.

Surgery of the Carotid Body Tumors

Galit Sivak, Menashe Haddad, Ram Avrahami, Avigdor Zelikovski

Department of Vascular Surgery, Rabin Medical Center - Beilinson Campus, Petah-Tikva and the Sackler School of Medicine, Tel-Aviv University, Israel.

Carotid body tumor is a rare tumor of obscure origin, usually benign, and commonly present as a non-painful cervical mass. Resection is the treatment of choice. When the tumor is very adherent to the carotid artery, it may be necessary to also resect the artery and to reconstruct the arterial continuity using a saphenous or synthetic interposition graft. The most common complication of surgery is damage to the cranial nerves in the vicinity of the artery. We present 8 patients with carotid body tumor who underwent surgery in our department in 1996-1999. Two had a large tumor penetrating the intima which mandated resection of the involved carotid artery and a saphenous interposition graft reconstruction. Our experience with resection of carotid body tumors is detailed, with emphasis on precautions taken to prevent damage to the adjacent cranial nerves.

Following 90 Patients with Antiphospholipid Syndrome: Manifestations of the Disease, Follow-Up of Antibodies Titers and Correlations with Clinical Manifestations in the Israeli Population - Patients in the Sheba Medical Center, Tel Hashomer

Ibrahim Marai, Yair Levi, Gezal Godard, Yehuda Shoenfeld

Department of Medicine B and Research Unit of Autoimmune Diseases, Chaim Sheba Medical Center (affiliated to Tel-Aviv University), Israel.

The antiphospholipid syndrome (APS) is defined by the presence of antiphospholipid antibodies (aPLs) with venous or arterial thrombosis, recurrent pregnancy loss or thrombocytopenia. The syndrome can be either primary or secondary to an underlying condition, most commonly systemic lupus erythematosus (SLE).

The purpose of the study was to review the manifestations of the APS in Israeli patients and to investigate the difference between patients with primary or secondary syndrome. Analysis of the correlation between the manifestations of the disease and the antibody titers [LA (lupus anticoagulant), IgG aCL (anticardiolipin), IgM aCL] was also performed.

In the study there were 90 patients with APS in the Sheba Medical Center. The clinical findings for all patients were recorded according to established protocol.

Results: Arterial thrombotic events were present in 51.1% of the patients; cereberal ischemic attacks were the most frequent arterial events. Venous thrombotic events were present in 45.6% patients; deep venous thrombosis was the most frequent venous event. Obstetric complications were found in 37.3% of patients; the most frequent complication was abortions. The clinical findings of patients with primary or secondary syndrome were similar. Patients with APS associated with lupus had a high prevalence of hemolytic anemia (28.6% v 3.3%; P=0.001), and ANA (antinuclear antibody) (75% v 12.9%; P=0.0001). There was no correlation between the antibody titers (LA, IgG aCL, IgM aCL) and clinical manifestations. The patients received anticoagulant treatment and antiaggregant drugs to prevent recurrence of symptoms. Clinical manifestations in Israeli patients did not differ from those in non-Israeli patients.

Conclusions: Venous or arterial thrombosis and obstetric complications are the most frequent clinical findings in APS. There was no different in clinical manifestations between primary and secondary syndrome. The findings of autoimmune hemolytic anemia and ANA were more frequent in patients with secondary APS. There was no correlation between antiphospholipid antibody titers and manifestations of disease. Overall, the patients require therapy with anticoagulant treatment and antiaggregant drugs. The clinical manifestations were similar in Israeli and non-Israeli patients.

Clinical Mentorship as a Bridge to Life in the Treatment of Eating Disorders

Moria Golan^1,2, Tali Gogol-Ostrowsky¹

¹Shachaf-Eeting Disorders Intensive Treatment Center, Kibbutz Nasn. ²School of Nutritional Sciences, The Hebrew University of Jerusalem, Rehovot

The aim of this article is to describe a community-based multi-modality intensive treatment program, which demonstrates a significant reduction in symptoms of eating disorders while providing an opportunity to deal with the functional and social skills. Treatment is delivered through a multidisciplinary team. The uniqueness of the team is in the inclusion of clinical mentors. These are social workers, art therapists and graduate level psychology students who are trained to connect with clients in an informal, intensive manner (10-40 hours a week). The mentors focus on legitimizing healthy attitudes to food, eating and life, while highlighting the pain and loss associated with the disorder. The mentors serve as meal companion and calming figure, representing the healthy self-caring voice. The relationship established during the many hours spent together becomes a powerful experience, completely different from the isolation of the disorder. The treatment intervenes in most areas of life, focusing on an active search for possibilities, in contrast to the emptiness associated with the disorder.

This article presents the results and principles of a 2.5-year practice. Seventeen patients, ill for 6 years and more, completed this program more than a year ago. An assessment of their BMI and their general outcome using Eckert scales was performed. One year after completing the program, 76% of the patients were defined as recovered and 12% were almost recovered with only a few remaining symptoms. All of these patients function satisfactorily in the community, both in social and occupational aspects. Six percent were partially recovered and 6% suffered from regression during the first year of follow-up.

This treatment provides the intensity required in hospitalizations, while enabling patients to stay in the community and maintain those activities that survive the disorder. This program has proven to work well with chronic patients. Further data, not analyzed as yet, also indicates the efficacy of this treatment in acute cases.

The Lack of Awareness of the Israeli Population Regarding Gastrointestinal Complications from Non-Steroidal Anti-Inflammatory Drugs

Gisels Zandman-Goddard, Pnina Langevitz

Medicine Dept. B and F. The SlE Clinic and The Rheumatology Unit, Sheba Medical Center, Tel Hashomer, Israel

Non-steroidal anti-inflammatory drugs (NSAID) are potent analgesic agents. They are indicated for rheumatoid arthritis, osteoarthritis, severe acute and prolonged chronic pain. A wide range of analgesic agents are available. The objective of this study was to assess the patient's knowledge of his illness, his knowledge familiarity with NSAID medications and their side effects and thereupon, to evaluate the awareness, utilization, satisfaction and expectations associated with current available treatment.

During the year 2000, a randomized telephone screening questionnaire, was completed over a 2 month period for a sample group of the general Israeli population over 50 years of age.

Of the 3179 persons contacted, 2028 responded and 292 persons were treated with NSAIDs and further investigated. Fifty percent suffered from chronic musculoskeletal pain, 22% had osteoarthritis, 10% had rheumatoid arthritis, and 18% had undefined chronic pain. The average age of the respondent was 65 years old. The average duration of treatment was 11 years. The most common drugs used by the 292 respondents that were taking NSAIDs were diclofenac (Voltaren) - 51% and naproxen (Naxyn) - 21%. Only 4% were treated with COX-2 specific inhibitors. Twenty percent of the respondents utilized more than one NSAID simultaneously. Forty seven percent of the patients had peptic ulcer disease but only 39% of them were simultaneously treated with an NSAID and a gastroprotective agent. Sixty eight percent of patients expected amelioration of symptoms while taking NSAIDs. Seventy seven percent of respondents were dissatisfied with treatment. The patients expressed that they did not have a reliable easily accessible source of information on their disorder or on available treatment options.

Many respondents were not aware of their medical diagnosis. In general, patients were not aware of all the available NSAIDs or the side effects of gastrointestinal complications. Enhancement of information available to the patient is warranted to improve the provision of efficient therapy, reduce side effects and increase patient satisfaction from therapy.

Stealing Madness - Kleptomania

Dana M. Polak, Pinhas N. Dannon

Psychiatry Department C, Sheba Medical Center Tel Hashomer, Israel

Kleptomania is one of the common less diagnosed impulse control disorders, which could be treated by the combination of psychological and pharmacological therapy. The most effective treatment regimens include cognitive behavioral therapy and interpersonal psychotherapy in the psychological field, and antidepressants and mood stabilizers as pharmacological treatment.

Most of the patients with kleptomania are initially treated only for a comorbid psychiatric disorder, since kleptomaniac symptoms had not been raised in the anamnesis.

The aim of our article is to inform the physicians about the possible diagnosis and treatment options for this disorder in order to prevent co-morbidity with other psychiatric disorders. Some specific cases are presented in order to explain symptomatology.

Eosinophilic Ascites - Presenting Symptom of the Hypereosinophilic Syndrome

Sofia Rimbrot1, Michael Bennett², Marina Komorovski², Yishai Levy³

¹Department of Medicine A, ²Department of Haematology, Haemek Medical Center, Afula and ³Technion Faculty of Medicine, Haifa, Israel

We report a case of a 22 year old man with a history of bronchial asthma, suffering from diarrhea, eosinophilic ascites and prominent blood and bone marrow eosinophilia. The patient responded to corticosteroid treatment.

Later, hydroxyurea was added to this treatment because of recurrence of eosinophilia, ascites and hepatosplenomegaly.

Eosinophilic ascites is rarely the outstanding symptom of the hypereosinophilic syndrome.

Following the exclusion of other reasons for eosinophilia, concomitant unexplained hepatosplenomegaly suggested the diagnosis of a hyperereosinophilic syndrome.

Sentinel Lymph Node Biopsy in Breast Cancer: A Validation Study and Preliminary Results

Aviram Nissan, Ram M. Spira, Roland Chisin, Oded Zamir, Diana Prus, Martin Klein, Mahmoud Badriyyah, Herbert R. Freund

Departments of Surgery. Biophysics and Nuclear Medicine, and Pathology. Hadassah University Hospital Mount Scopus and Hebrew University - Hadassah Medical School, Jerusalem

Introduction: Sentinel lymph node biopsy (SLNB) has been recently proven to be an accurate staging method for breast cancer, replacing axillary lymph node dissection (ALND) in selected cases. We present our initial experience and the process of introduction and implementation of SLNB in a University Hospital setting.

Material and methods: 46 SLNB were performed in 42 consecutive female patients with invasive breast cancer. Treatment included 0.4mCi-2mCi of Tc-99m rhenium colloid injected either 2 hours before surgery (0.4 mCi) or the night before surgery (2 mCi). Four milliliters of Patent Blue V were injected peritumoral 10 minutes prior to skin incision in all patients. Following SLNB all women underwent subsequent ALND. Sentinel nodes were processed both with multiple (10-15) H&E sections and immunohistochemistry with cytokeratin antibodies stain.

Results: Blue dye, isotope or the combination of both identified 43/46 (93%) of the sentinel lymph nodes. ALND was performed only unilaterally in 4 patients with bilateral breast cancer bringing the total evaluable SLNB to 39. In the 39 patients in whom the sentinel node was successfully identified and underwent ALND, the SLNB was true positive (TP) in 17/39 (44%) true negative (TN) in 20/39 (51%) and false negative in 2/39 [(5%), both T2 lesions] with overall accuracy of 95%. In the last 10 cases all sentinel nodes were successfully identified with 70% TP and 30% TN.

Conclusions: Experience with at least 30-40 consecutive cases for safe implementation of SLNB in clinical practice. Specific training and dedication is required for the entire team involved, including surgeons, nuclear medicine physicians and technicians and pathologists.  

Laparoscopy as Part of the Management of Gynecologic Neoplasms - Report of Our Clinical Experience

Yehuda Ben David, Moshe Bustan, Eliezer Shalev

The Department of Obstetrics and Gynecology, HaEmek Medical Center, Afula

Introduction: Surgery is the treatment of choice in most early stages of cervical cancer and advanced stages of ovarian cancer. Failing to preoperatively diagnose para-aortic and parametrial metastases in cervical cancer or a non-resectable, ovarian cancer, may results in a superfluous laparotomy.

Aim: To evaluate the advantage of using laparoscopy in cervical and ovarian cancer.

Patients and Methods: Study population includes patients with ovarian or cervical cancer referred between 1997-1999. A CT scan and a trans-vaginal sonography were used to detect involvement of pelvic and para-aortic lymph nodes, parametrium and other metastases. In patients with cervical cancer, para-aortic lymph node dissection was laparoscopically performed. When para-aortic nodes were negative and parametrium was clear, radical hysterectomy and pelvic lymph nodes dissection was conducted through laparotomy. When para-aortic lymph nodes or parametrium were positive, patients were referred for radiation therapy. In ovarian cancer patients, the upper abdomen and the pelvis were examined laparoscopically to evaluate the possibility of optimal debulking surgery. Staging was done for patients who were not candidates for optimal debulking surgery and second debulking surgery was considered.

Results: Nine patients with stage lb-lla cervical cancer were included. Preoperatively, 2 of them were suspected for lymph node involvement. Following laparoscopy one was confirmed to have para-aortic lymph node involvement and the other did not. In the remaining 8 patients, one was found to have parametrial involvement and laparotomy was avoided, while the rest were treated surgically.

Eighteen ovarian cancer patients were included in this study. Laparoscopy revealed an extensive disease in 7 patients and therefore staging laparoscopy was completed. In the remaining 11 patients, laparotomy was performed, operable disease was found and complete debulking surgery was conducted. In only one patient of the 11 complete debulking surgeries was not possible.

Conclusions: In accordance with the experience and skills of the surgical team, we propose utilizing laparoscopy in cases where laparotomy may be avoided.

Animal Models of Psychiatric Diseases: Possibilities, Limitations, Examplesand Demonstration of Use

Haim Einat, RH Belmaker

Beer Sheva Mental Health Center, Faculty of Health Sciences, Ben Gurion University of the Negev, Beer Sheva, Israel

Animal models of psychopathology serve as a central tool for psychopharmacologists in their attempts to develop new, more efficient medications for psychiatric disorders, and in the efforts to explore the mechanisms of conventional and novel drugs. The development of efficient models for psychiatric diseases is complicated since the mechanisms of the disorders are not clear, major parts of the diagnosis depend on verbal communication with the patient and many of the symptoms are expressed mainly through the subjective experiences of the afflicted individual.

Inspite of these difficulties, effective models were developed for most psychiatric diseases. The development of such models is based on their validation in three different dimensions: face validity - behavioral similarities between the model and the disorder; construct validity - similarities between the mechanisms related to the model and a mechanistic theory of the disease; predictive validity - that the model response to the conventional medications that are effective in the disease and will not respond to drugs that are not effective in the disease.

The present paper presents three known models of depression; each induced in a different way and therefore represents a group of models:

*     Reserpine-induced hypoactivity - represents the group of pharmacologically induced models.

*     Forced swim test - represents the group of behaviorally induced models.

*     Flinders Sensitive Line - represents the group of genetically induced models.

The use of these models in an attempt to examine the range of action of a new potential antidepressant and its mechanisms of action is demonstrated with a recent set of experiments with inositol.