Tel Aviv Sourasky Medical Center Research Articles

Characterization of normocalcemic primary hyperparathyroidism

The introduction of multichannel serum biochemistry testing in the early 1970s uncovered a large number of patients with primary hyperparathyroidism who were asymptomatic, turning an extremely rare and severe disease into a fairly common and benign condition (1). The diagnosis of primary hyperparathyroidism—whether symptomatic or not—still requires hypercalcemia along with an elevated intact parathyroid hormone (PTH) level (2). Recently, endocrinologists have become aware of patients with normocalcemic primary hyperparathyroidism. This entity has not been characterized systematically, and it is not yet clear whether this is a “forme fruste” of the disease or a harbinger of the hypercalcemic type (3). The purpose of the present study was to define the characteristics of patients with this diagnosis in one endocrine outpatient clinic during the last 5 years. METHODS We reviewed medical records of patients with a diagnosis of primary hyperparathyroidism (International Classification of Diseases, Ninth Revision, code 252.0) seen in the endocrine clinic at Tel Aviv Sourasky Medical Center between January 1998 and October 2003. (Institutional review board approval was not required for this study.) The diagnosis of normocalcemic primary hyperparathyroidism was confirmed if a patient had never had documented hypercalcemia, and if all apparent causes of secondary hyperparathyroidism had been ruled out. Specifically, patients with impaired renal function, and those in whom vitamin D supplementation unmasked hypercalcemia, were excluded. Patients in whom elevated PTH levels decreased following treatment (vitamin D or calcium supplementation, or thiazides) were considered to have secondary hyperparathyroidism and thus were not eligible. We identified 32 patients who met these criteria. Serum calcium levels were determined on a Hitachi 747 random-access analyzer (Roche Diagnostics Boehringer Mannheim, Mannheim, Germany) or a Bayer Advia 1650 analyzer (Leverkusen, Germany) with identical analytical performances; the normal range was 8.5 to

Hospital preparedness for possible nonconventional casualties: an Israeli experience

Since 9/11, hospitals and health authorities have been preparing medical response in case of various mass terror attacks. The experience of Tel Aviv Sourasky Medical Center in treating suicide-bombing mass casualties served, in the time leading up to the war in Iraq, as a platform for launching a preparedness program for possible attacks with biological and chemical agents of mass destruction. Adapting Quarantelli's criteria on disaster mitigation to the “microinfrastructure” of the hospital, and including human behavior experts, we attempted to foster an interactive emergency management process that would deal with contingencies stemming from the potential hazards of chemical and biological (CB) weapons. The main objective of our work was to encourage an organization-wide communication network that could effectively address the contingent hazards unique to this unprecedented situation. A stratified assessment of needs, identification of unique dangers to first responders, and assignment of team-training sessions paved the way for program development. Empowerment through leadership and resilience training was introduced to emergency team leaders of all disciplines. Focal subject matters included proactive planning, problem-solving, informal horizontal and vertical communication, and coping through stress-management techniques. The outcome of this process was manifested in an “operation and people” orientation supporting a more effective and compatible emergency management. The aim of article is to describe this process and to point toward the need for a broad-spectrum view in such circumstances. Unlike military units, the civilian hospital staff at risk, expected to deal with CB casualties, requires adequate personal consideration to enable effective functioning. Issues remain to be addressed in the future. We believe that collaboration and sharing of knowledge, information, and expertise beyond the medical realm is imperative in assisting hospitals to expedite appropriate preparedness programs.

Gemcitabine in the treatment of recurrent epitherlial ovarian cancer

5141 Background: Five years survival of patients with advanced epithelial ovarian cancer (EOC) is 25–40% and once the disease recurs, treatment is mainly palliative. We would like to give that treatment with the longest time to progression (TTP) together with the best quality of life. Our objectives in this study were to evaluate the safety and to determine the TTP of monotherapy Gemcitabine in heavily pretreated patients with recurrent or persistent EOC. Methods: nineteen heavily pretreated patients with recurrent or persistent EOC were treated with single agent Gemcitabine during 1995–2003 in the Oncology Division in Tel Aviv Sourasky Medical Center. Fifteen of them were retrospectively evaluated (4 had died and received the drug for =< 1.5 months - less than two cycles). All patients were previously treated with cisplatin combinations and had received a median of 3 prior chemotherapy regimens (range 2–6). Seven (47%) patients were considered platinum sensitive and 8(53%) platinum resistant. Median age was 64(43–78) years. Protocol schedule was Gemcitabine 1000mg/m2 over one hour administration days 1, 8 and 15 every 28 days. Results: Fifteen patients are evaluable for response by CA-125 reduction or stability and clinical improvement (pain, ascites etc). Patients were not on preplanned protocols and for that, follow-up CT's were not done routinely. Clinical benefit (PR or SD by CA-125 and symptom relief) was noted in 12 out of 15(80%) patients. Median TTP was 4 months (range 2–14). Median OS was 6 months (range 3–28) and 5 patients are still alive. Toxicities: Grade III-IV toxicity was found in 4(27%) patients with anemia, 4(27%) with thrombocytopenia, 1(6%) with fatigue and 2(13%) with leg edema. Conclusions: Gemcitabine is an active drug in heavily pretreated patients with EOC and has a very favorable toxicity profile. It's use as a monotherapy and in combination with platinum salts drugs should be studied. No significant financial relationships to disclose.

Phase II study of maintenance carboplatin in patients with advanced epithelial ovarian cancer after complete response to platinum based combination

5122 Background: Five years survival of patients with advanced epithelial ovarian cancer (EOC) is 25–40% and once the disease recurs, treatment is mainly palliative. We evaluated whether continued treatment with carboplatin for an extended one-year period in women with complete response after platinum based combination, could prolong time to progression (TTP) and overall survival (OS). Methods: 14 patients were assigned to the protocol between 2/1996 to 6/2002 at the Oncology Division in Tel Aviv Sourasky Medical Center. Protocol schedule was: administration of Carbopltin at an AUC=6, three treatments every 2 months followed by two treatments once every three months (for a total of one year). All patients were previously treated with platinum based combinations and had achieved complete response (CR) by CT-scan and serum CA-125. Results: As of December 2003 with a median follow up of 42 months (range 18–93 months), the median TTP is 28 months (range 8–93+ months), and a median OS of 42 months (range 18–93+ months). Eight patients are still in complete remission and two more patients are still alive with disease. Conclusions: One year treatment (5 cycles) of single agent carboplatin that was administered to women with advanced EOC who achieved CR on platinum based chemotherapy as a first line, significantly prolonged TTP and OS compared to historical control. No significant financial relationships to disclose.

Increased red blood cell adhesiveness/aggregation owing to fibrinogen elevation in hypercholesterolaemic patients and the rationale of fibrinogen‐lowering by LDL apheresis: authors’ reply

European Journal of Clinical InvestigationVolume 34, Issue 5 p. 380-381 Increased red blood cell adhesiveness/aggregation owing to fibrinogen elevation in hypercholesterolaemic patients and the rationale of fibrinogen-lowering by LDL apheresis: authors’ reply V. Schechner, V. Schechner Tel Aviv University, Tel Aviv, IsraelSearch for more papers by this authorI. Shapira, I. Shapira Tel Aviv University, Tel Aviv, IsraelSearch for more papers by this authorS. Berliner, Corresponding Author S. Berliner Tel Aviv University, Tel Aviv, IsraelS. Berliner, Tel Aviv Sourasky Medical Center, 6 Weizman Str, Tel Aviv 64239, Israel. Tel.: +972–3−6974255; fax: +972–3−6973635; e-mail: shapiraiz@tasmc.health.gov.ilSearch for more papers by this author V. Schechner, V. Schechner Tel Aviv University, Tel Aviv, IsraelSearch for more papers by this authorI. Shapira, I. Shapira Tel Aviv University, Tel Aviv, IsraelSearch for more papers by this authorS. Berliner, Corresponding Author S. Berliner Tel Aviv University, Tel Aviv, IsraelS. Berliner, Tel Aviv Sourasky Medical Center, 6 Weizman Str, Tel Aviv 64239, Israel. Tel.: +972–3−6974255; fax: +972–3−6973635; e-mail: shapiraiz@tasmc.health.gov.ilSearch for more papers by this author First published: 17 May 2004 https://doi.org/10.1111/j.1365-2362.2004.01342.xRead the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat No abstract is available for this article. Volume34, Issue5May 2004Pages 380-381 RelatedInformation

This Month in ANESTHESIOLOGY

This Month in ANESTHESIOLOGY

Higher rates of neonatal respiratory morbidity after elective cesarean section

ELECTIVE CESAREAN SECTION LIMOR HELPMAN, YAEL WILNAI, JESSICA ASCHER-LANDSBERG, MICHAEL KUPFERMINC, MICHAEL SHENHAV, SHAUL DOLLBERG, ARIEL MANY, Tel Aviv Sourasky Medical Center Tel Aviv University, Obstetrics and Gynecology, Tel Aviv, Israel OBJECTIVE: Cesarean section (CS) rate has increased over the last few decades. A large proportion of it is due to elective CS. Respiratory complications (RDS, transient tachypnea of the newborn, etc) are the foremost causes of neonatal morbidity and are increased after elective CS. The reason for this higher incidence is unclear. The most widely accepted explanation is iatrogenic prematurity due to unconfirmed dating. Recently, some reports have suggested that the labor process itself affects neonatal respiratory function, thus reducing the rate of respiratory morbidity. The objective of this study was to examine the rate of respiratory morbidity in neonates delivered by elective CS at term, with a definite confirmation of gestational age (GA) by 1st-trimester ultrasound. STUDY DESIGN: Consecutive women carrying a singleton pregnancy and undergoing elective CS at term (>38 1/7 weeks), confirmed by 1st-trimester ultrasound, were included in the study group. Multiple gestation, CS in labor, and CS performed after rupture of membranes were excluded. The control group included women with a singleton pregnancy at term (>38 1/7 weeks) who delivered spontaneously. This group of women was randomly selected during the study period. RESULTS: 277 women delivered by elective CS were included in the study group. The control group consisted of 311 women. 5 newborns in the study group and none in the control group were admitted to the NICU due to respiratory disorders (P < 0.02). Excluding diabetic women did not change the results. On multivariate analysis, no other factors were found to independently influence the risk of respiratory complications. CONCLUSION: In our study, the rate of respiratory morbidity was found to be significantly higher in neonates delivered by elective CS in comparison to those delivered vaginally. The fact that GA was confirmed by 1st-trimester ultrasound makes iatrogenic prematurity an unlikely sole cause for this excess morbidity.

Role of adjuvant cryosurgery in intralesional treatment of sacral tumors.

Cryosurgery is an adjuvant surgical technique for the treatment of benign aggressive, low-grade malignant and metastatic tumors of long bones. It has been used rarely to treat sacral tumors, mainly because of potential damage to nerves, blood vessels, and intrapelvic organs. The authors described their experience with this procedure and provided medium and long-term follow-up results. Fifteen procedures of cryosurgery of the sacrum were performed in 14 patients to improve the therapeutic outcome of a variety of tumors. The patient group included 7 males and 7 females with a mean age of 42 +/- 24 years. Three patients were younger than 20 years of age. The procedures were performed at the Tel Aviv Sourasky Medical Center between January 1991 and January 1999. There were seven benign aggressive lesions (four giant cell tumors and three aneurysmal bone cysts), one benign schwannoma, one low-grade chondrosarcoma, five metastatic carcinomas, and one high-grade Ewing sarcoma, all localized at level S(2) or higher. Eight of the bone tumors also involved significant anterior or posterior soft tissue. All patients had severe preoperative pain radiating to the buttocks, perineum, and lower limbs and 9 (64%) patients had bladder and/or rectal dysfunction. Invasive diagnostic procedures and radiation (if warranted) preceded surgery. Sacral posterior fenestration and burr drilling were followed by two-cycle cryosurgery using the open pour technique or the argon-helium-based heat-freeze system. All interventions were performed under combined general and regional anesthesia and concluded uneventfully with moderate blood loss. Thirteen patients were discharged home after 8 +/- 5 days (one patient remained hospitalized for 30 days). Only two patients experienced local disease recurrence during a 3-11-year follow-up period: one was retreated successfully by cryosurgery and the other underwent sacrectomy and radiotherapy elsewhere, with a subsequent loss of visceral functions. No patient suffered chronic pain, deep wound infections, or significant neurologic deficits and all were satisfied with the esthetic outcome. Cryosurgery is a conservative, feasible, and safe adjuvant technique in the treatment of sacral tumors. It is associated with minimal permanent neurologic and vascular injury compared with sacrectomy.

Mid-trimester severe intrauterine growth restriction is associated with a high prevalence of thrombophilia

Objective To investigate the association between severe mid-trimester IUGR, whose causes are unknown in most cases, and maternal thrombophilias. Design Case–control study. Setting Lis Maternity Hospital, Tel Aviv Sourasky Medical Center, The Sackler Faculty of Medicine, Tel Aviv University. Population Twenty-six women with severe mid-trimester (22–26 weeks of gestation) IUGR (birthweight <3rd centile) and 52 matched multiparous women with normal pregnancies (controls). Methods After excluding pregnancies with vascular maternal disease, chromosomal and structural aberrations and cytomegalovirus infection, 26 women out of 35 with severe mid-trimester IUGR remained and composed the study group. Each was matched for age, ethnicity and smoking status with two healthy women who had normal pregnancies. All the women were tested for genetic and acquired thrombophilias at least eight weeks after delivery. Main outcome measures Prevalence of maternal thrombophilias. Results The frequency of thrombophilias was 69% in the study group compared with 14% in the control group [odds ratio (OR) 4.5; 95% confidence interval (CI) 2.3–9, P < 0.001]. The frequencies of factor V Leiden mutation, prothrombin gene mutation and protein S deficiency were significantly increased in the study group compared with the control group. The frequency of multiple thrombophilias was 33% in the study group versus none among the controls. Of the 26 pregnancies with severe mid-trimester IUGR, 13 ended in intrauterine fetal death before 25 weeks of gestation: 10 of these women had thrombophilia. Conclusion Women with mid-trimester severe IUGR have an increased prevalence of inherited and acquired thrombophilias.

Mid-trimester severe intrauterine growth restriction is associated with a high prevalence of thrombophilia.

To investigate the association between severe mid-trimester IUGR, whose causes are unknown in most cases, and maternal thrombophilias. Case-control study. Lis Maternity Hospital, Tel Aviv Sourasky Medical Center, The Sackler Faculty of Medicine, Tel Aviv University. Twenty-six women with severe mid-trimester (22-26 weeks of gestation) IUGR (birthweight <3rd centile) and 52 matched multiparous women with normal pregnancies (controls). After excluding pregnancies with vascular maternal disease, chromosomal and structural aberrations and cytomegalovirus infection, 26 women out of 35 with severe mid-trimester IUGR remained and composed the study group. Each was matched for age, ethnicity and smoking status with two healthy women who had normal pregnancies. All the women were tested for genetic and acquired thrombophilias at least eight weeks after delivery. Prevalence of maternal thrombophilias. The frequency of thrombophilias was 69% in the study group compared with 14% in the control group [odds ratio (OR) 4.5; 95% confidence interval (CI) 2.3-9, P < 0.001]. The frequencies of factor V Leiden mutation, prothrombin gene mutation and protein S deficiency were significantly increased in the study group compared with the control group. The frequency of multiple thrombophilias was 33% in the study group versus none among the controls. Of the 26 pregnancies with severe mid-trimester IUGR, 13 ended in intrauterine fetal death before 25 weeks of gestation: 10 of these women had thrombophilia. Women with mid-trimester severe IUGR have an increased prevalence of inherited and acquired thrombophilias.

Dedication: gearing up for war

The terrorist attack of September 11, 2001, that targeted the World Trade Center in New York City and the Pentagon in Arlington, Virginia, awakened the entire world to the absolute horror of megaterrorism. These were the most barbarous acts committed against the United States of America since December 7, 1941, with the attack on Pearl Harbor, “a day that will live in infamy.” The assault against America and the free world was so massive, so unimaginable, and so unprecedented that even on the publication of this special issue of Clinics in Dermatology we find the events difficult to fathom. Several thousands of innocent victims of terrorism perished in the crash of planes, in the destruction of buildings, and in the aftermath of the devastation. Such horrid terrorist attacks were immediately recognized by the vast majority of the world community as vicious. These were evil-doing acts against civilization, freedom, and most of all, humanity. These actions contravene all sensible religious and human values. In the weeks after the September tragedy, we have seen that persons in leading positions with significant responsibilities, regardless of race, color, or religion, must not only denounce and condemn every type of terrorism but also fight this evil and eliminate its roots and motives from the face of the earth. We now know that we must acknowledge the possibility of more catastrophic terrorist acts, including the use of weapons of mass destruction, be they chemical, biologic, or nuclear, and that we must be prepared. We must deny potential terrorists access to facilities and to technology that they might use in their evil doings. They should be held accountable for their crimes. What happened on September 11, 2001, concerns the entire world as one community, Scientists and physicians are committed to helping mankind; they need the moral voice of every nation and have the commitment to speak out on this important issue. They have the obligation to be at the forefront of the dedicated efforts to protect the health and life of everyone. Dermatologists, in particular, should have the full knowledge of megaterrorist weapons and learn how to handle their hazardous effects. We must stay alert and have the expertise to deal with any crisis. The cardinal challenge facing civilized nations is the preservation of the integrity of their values and the ethical standards of respect for human dignity. Allowing disrespect for human rights and the violation of international law will play into the hands of the terrorists. We, as the guest editors, and our contributors dedicate this special issue of Clinics in Dermatology to the memory of the innocent victims of 9/11. We must ensure that the continuation of our message of hope and our responsibility toward humanity and future generations will continue. We are committed to promoting knowledge, to developing scientific research and continuing medical education, and to solving community health problems. All of this will contribute to building stable and solid societies so that the September tragedies must never occur again. From the Amman Clinic, Amman, Jordan, and the Department of Dermatology, Tulane University, School of Medicine, New Orleans, Louisiana; the Department of Dermatology, Tel-Aviv Sourasky Medical Center and the Sackler Faculty of Medicine, Tel-Aviv, Israel; and the Department of Dermatology and Cutaneous Biology, Jefferson Medical College of Thomas Jefferson University, Philadelphia, Pennsylvania. Address correspondence to Oumeish Youssef Oumeish, MD, FRCP (Glasgow), PO Box 65, Prince Rashid Suburb, Amman 11831, Jordan. E-mail: oumeishdermatol@hotmail.com

Early detection of melanoma: the best strategy for a favorable prognosis

Of all the skin tumors, cutaneous melanoma represents the greatest challenge for secondary prevention and early detection aimed at finding early curable disease. Melanoma is a highly malignant tumor with an alarming increase in incidence over the last few decades, among the highest in cancer, that is directly related to a rise in the mortality rate. The current lifetime risk is 1 in 71 Americans.1 These statistics compel physicians to make the utmost effort to detect melanomas at an early, treatable stage when simple excision can provide a cure. Most melanomas develop in the skin and are clinically classified into four major subtypes, with recent identification of additional variants (Table 1). Melanoma can arise in any melanocyte-containing tissue, including the uveal tract, central nervous system, and mucosal surfaces, including the oral cavity, vulva, vagina, nasopharynx, paranasal sinuses, tracheobronchial tree, anal canal, and urinary tract. Frequently neglected are subungual melanomas, which are often mistaken for benign or traumatic conditions of the nailbed; they occur at a higher frequency in African Americans, who have 12% of all subungual melanomas in contrast to 1% of cutaneous melanomas.2 Malignant melanoma is very rare before the age of 20, accounting for 2% of cases,1 of which 0.3%–0.4% are prepubertal melanomas.1 Childhood melanoma is divided into five categories based on the mode of occurrence3: development from healthy skin, accounting for most cases; transformation from a giant congenital nevus, accounting for a third of the cases4,5; transplacental transmission from mother to fetus; transformation of a pre-existing nevus; and associated conditions like xeroderma pigmentosum, albinism, or dysplastic nevus syndrome. Melanoma is the second most common cancer in men aged 30–49 years and the fourth most common cancer in men aged 50–59.6 It is the most common cancer in women aged 25–29 and second only to breast cancer in women aged 30–35 years.7 The overall incidence is highest in New Zealand and Australia, exceeding 55 per 100,000.8 These rates have risen sharply with a more modest increase in the mortality rate, mainly due to a substantial increase in melanoma surveillance that has led to identification of early, curable melanomas and reduction in the emergence of advanced disease and melanoma-related mortality.9 Melanoma incidence and mortality rates are influenced by gender and geography. In Europe, melanoma occurs at a higher frequency among women than men, whereas in Australia and America the incidence is slightly higher in men.10 Mortality rate is higher in southern than in northern European countries and rose 3% per year for men and 1% for women for each year from 1976 to 19876 (Table 2). Large studies have shown a female survival advantage over men of 22% and a disease-free survival advantage at 5 years of 17% for woman over men.11 This survival advantage was most commonly seen in early stages of the disease and was often lost in advanced disease. The most pronounced discrepancy occurred in the progression of the disease to stage III, while there was no evident survival advantage at further progression to stage IV. This indicates that female sex steroids could slow the tumor progression at early stages of the disease.12 With regard to hormone status, the female advantage was equally strong in preand post-menopausal periods in some studies13 but restricted to premenopausal women in other studies.14 Despite widespread research, the basis for the female survival advantage is still not established. It has been suggested that estrogenic compounds reduce the invasion of human melanoma cells through fibronectin, as opposed to androgenic compounds, which seem to have no anti-invasive capacities.12,15 How estrogenic compounds affect melanoma cell invasion through fibronectin remains to be determined. Another known effect of estradiol, progesterone, and dihydrotestosterone is their ability to inhibit interleuFrom the Department of Dermatology, Tel Aviv Sourasky Medical Center and Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel. Address correspondence to Sarah Brenner, MD, Department of Dermatology, Tel Aviv Sourasky Medical Center, 6 Weizman St., Tel Aviv 64239, Israel. E-mail address: derma@tasmc.health.gov.il

Gait festination in Parkinson's disease

Background: Festinating gait (FSG) was first associated with parkinsonism by Sir James Parkinson, in his original essay on “The Shaking Palsy”. Its frequency and relation to other parkinsonian features have never been assessed. Objective: To study the relationships between gait festination and other parkinsonian clinical features among patients with Parkinson's disease (PD). Method: During an open lecture to patients with PD who are followed at the Movement Disorders Unit (MDU) of Tel-Aviv Sourasky Medical Center one of us explained verbally and imitated festinating gait on stage. All attending patients with the help of their care-givers or family members, were asked to answer two written questions regarding their own experience with FSG as well as the degree of disability it causes. Clinical information about each patient was taken from his/her chart at the MDU and missing data was completed during the next office visit or from the family physician. Statistical analysis was performed using t-tests for comparison between groups, Chochran–Armitage test for trends and logistic regression to assess the contribution of age of onset, disease duration and disease severity to the development of FSG. Results: Eighty-one PD patients (58 males, mean age 67.5±10.7 years) answered the FSG questionnaire. Our study population's mean disease duration was 8.5±6.4 years, mean Hoehn and Yahr (H&Y) clinical stage of 2.6±0.8 and mean levodopa dose of 608±375 mg/day (15 patients were not on levodopa). Twenty-six patients (32.1%) experienced FSG during the previous month and 56% of them reported that FSG was a significant and disabling symptom. FSG was strongly associated with higher stage of H&Y ( p<0.001) with a significant trend as the disease progresses ( p=0.001) but not with total score in the motor part of the Unified Parkinson's Disease Rating Scale (UPDRS). Longer disease duration was the only clinical factor, which was found to be associated with FSG in the multivariate model. Thirty seven percent (37%) of the patients with FSG reported frequent falls with association between occasional or frequent falls, as reported on the activity of daily living (ADL) part of the UPDRS, and the presence of FSG ( p<0.08). There was no association between significant postural reflex abnormalities as rated on the objective part of the UPDRS and the presence of FSG. There was a significant association between the presence of freezing of gait (FOG) as reported in the ADL part of the UPDRS and the presence of FSG ( p<0.001) as well as a significant trend towards more frequent FSG in patients with more severe FOG ( p<0.001). Conclusion: FSG was clearly associated with longer duration of PD symptoms but not with disease severity as reflected in the motor part of the UPDRS. The relationships between FSG and postural reflexes abnormalities is unclear but it is frequently associated with falls and freezing of gait.

Young Women with PD

ABSTRACT Parkinson's Disease (PD) prior to the age of 40 affects between 5-10% of the PD population. The psychosocial changes that patients with early PD encounter, may be more devastating and disabling than the actual motor disability. The paper describes a unique experience in groupwork with young female PD patients treated in the Movement Disorders Unit of the Tel Aviv Sourasky Medical Center. The paper focuses on the special issues which characterized this group's experience: stigma, body and sexual image, and personality traits.

METASTATIC RENAL CELL CARCINOMA OF BONE: INDICATIONS AND TECHNIQUE OF SURGICAL INTERVENTION

We describe the efficacy of surgical excision of metastatic renal cell carcinoma of bone for achieving local tumor control, pain control and functional outcome with emphasis on the indications and techniques of surgical intervention as well as oncological outcome. Between 1980 and 1997 we performed surgery on 45 patients (56 lesions) with metastatic renal cell carcinoma of bone. Indications for surgery were solitary bone metastasis, intractable pain, or impending or present pathological fracture. Surgery involved wide excision in 29 cases, marginal excision with adjunctive liquid nitrogen in 25 and amputation in 2. None of the patients had significant bleeding intraoperatively. Mean hospital stay was 9.8 days, during which there was no flap necrosis, deep wound infection, nerve palsy or thromboembolic complication. Postoperatively pain was significantly relieved in 91% of patients, while 89% achieved a good to excellent functional outcome, and 94% with metastatic lesions of the pelvic girdle and lower extremities were ambulatory. Local recurrence developed in only 4 of the 56 lesions (7.1%), including 3 after marginal resection. Survival was more than 2 years in 22 patients (49%) and more than 3 in 17 (38%). Surgical excision is safe and reliable for restoring mechanical bone stability, relieving pain and providing good function in most patients with metastatic renal cell carcinoma who meet the criteria for surgical intervention. Relatively prolonged survival in these cases justifies considering surgical intervention when feasible.

The risk of fragile X premutation expansion is lower in carriers detected by general prenatal screening than in carriers from known fragile X families.

The Fragile X syndrome is the most common cause of inherited mental retardation. For a female premutation carrier, the risk of having a child with a full mutation is positively correlated with the size of the premutation. The current study was performed to evaluate the risk of premutation expansion in the offspring of average-risk carriers detected by general prenatal screening. Over a 4-year period, 9,660 women underwent DNA screening for FMR1 mutation/premutation at the Tel Aviv Sourasky Medical Center. A premutation was defined as a CGG repeat number >50 in the 5' untranslated region (UTR) of exon 1 in the FMR1 gene. The study included only individuals with no family history of X-linked mental retardation or known FMR1 mutations. A premutation was found in 85 women (1 in 114), 68 of whom consented to have prenatal diagnoses in 74 pregnancies. The abnormal allele was transmitted to the offspring in 44 pregnancies. Of these, no change in allele size was noted in 35 pregnancies (79.6%), and expansion within premutation range was evident in 4 pregnancies (9%). In 5 pregnancies (11.4%), expansion to the full mutation was noted. This occurred only in carriers having more than 90 repeats. We conclude that the likelihood of Fragile X premutation expansion to full mutation is significantly lower in individuals ascertained by general prenatal carrier testing than in those from known Fragile X families.

Hyperhomocystinemia in patients with nonarteritic anterior ischemic optic neuropathy, central retinal artery occlusion, and central retinal vein occlusion

Objective This study aimed to determine the prevalence of hyperhomocystinemia among patients with nonarteritic anterior ischemic optic neuropathy (NAION), central retinal artery occlusion (CRAO), or central retinal vein occlusion (CRVO). Design Retrospective, case-control study. Participants The study cohort consisted of 74 consecutive patients with NAION, CRAO, or CRVO who were examined at the Retina or Neuro-ophthalmological Unit of the Tel-Aviv Sourasky Medical Center from 1998 through 1999. The control group consisted of 81 consecutive patients of similar gender and age with no history of these pathologic conditions. Main outcome measures Plasma homocystine levels of all study participants were obtained. Results Eighteen of 40 patients (45%) with NAION and eight of 13 patients (61.5%) with CRAO had hyperhomocystinemia compared with three of 21 (14.3%) in the CRVO group ( P < 0.001) and eight (9.8%) in the control group ( P < 0.0001). Hypertension and ischemic heart disease were significantly more prevalent in the NAION patients with elevated plasma homocystine. Conclusions Our findings suggest that hyperhomocystinemia is a risk factor for NAION and CRAO.

Intrauterine insemination before transfer of frozen-thawed embryos may improve the pregnancy rate for couples with unexplained infertility: preliminary results of a randomized prospective study

Objective: To evaluate whether a combination of IUI and frozen-thawed embryo transfer (FT-ET) with ovulation induction would improve the PR in couples with unexplained infertility. Design: Prospective, randomized study. Setting: In Vitro Fertilization Unit, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel. Patient(s): Sixty-two patients with unexplained infertility were assigned into two groups. The study group was composed of 32 women (38 cycles) who received ovulation induction followed by IUI and FT-ET. The control group was composed of 30 women (33 cycles) who received ovulation induction followed by FT-ET. Intervention(s): Clomiphene citrate (CC) and hCG, IUI, and FT-ET. Main Outcome Measure(s): Pregnancy rate (PR) per cycle, PR per ET. Result(s): In the study group, the PR per cycle and per ET were 36.8% (14 of 38) and 40.6% (13 of 32), respectively. In the control group, the PR per cycle and per ET were 12.1% (4 of 33) and 14.3% (4 of 28), respectively. Statistically significant differences were found between the two groups in the PR per cycle ( P=.02) and PR per ET ( P=.03). No statistically significant difference was found between the groups for the stage in which the embryos were cryopreserved, the survival cleavage rates after thawing, grading of thawed embryos, and number of embryos transferred. Conclusion(s): In couples with unexplained infertility, the PR may be improved by combining IUI and FT-ET with ovulation induction. Performing IUI before thawing may prevent treatment cancellation in cycles with no surviving embryos.

Second-trimester multifetal pregnancy reduction facilitates prenatal diagnosis before the procedure

Objective: To evaluate the pregnancy outcome of selective second-trimester multifetal pregnancy reduction (MFPR) compared to first-trimester MFPR. Design: Cohort analysis. Setting: In Vitro Fertilization Unit, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel. Patient(s): The study groups comprised 38 and 70 patients who underwent selective second-trimester MFPR (group 1) and first-trimester MFPR (group 2) at mean gestational ages of 19.7 ± 3.3 weeks and 11.7 ± 0.7 weeks, respectively. Intervention(s): Ultrasonographically guided intracardiac injection of potassium chloride (KCl) solution. Main Outcome Measure(s): Pregnancy outcome and obstetric complications. Result(s): No statistically significant difference was found between group 1 and group 2 regarding mean gestational age at delivery (35.4 ± 3.4 weeks and 35.9 ± 3.1 weeks, respectively); mean birth weight (2,318.9 ± 565.7 g and 2,138.1 ± 529.4 g); and the incidence of obstetric complications. These complications included pregnancy loss (5.2% and 15.7%), pregnancy-induced hypertension (0 and 10%), discordancy (12% and 18.4%), intrauterine growth restriction (0 and 40%), and gestational diabetes (0% and 6%). However, the rate of all pregnancy complications was lower among second-trimester MFPR patients. Conclusion(s): Selective second-trimester MFPR is associated with favorable perinatal outcome and may facilitate detection of structural and chromosomal anomalies before the procedure and selective reduction of the affected fetus.

Stages of Breast Cancer

The Breast Health Center at the Tel-Aviv Sourasky Medical Center is the first center in Israel to provide medical and psychoso-cial services under one roof. The authors describe a model developed for the special needs of Israeli patients with breast cancer. The model includes three phases of intervention: diagnostic, surgical, and recovery. The underlying assumption is that each phase is characterized by specific stressors, each requiring distinctive adjustment and coping mechanisms. The phasic approach enables the therapist to meet the various needs of the patients.