אלכס לבנטל (1), אריק קרסנטי (1,2)

היחידה לאפידמיולוגיה סביבתית (2), שירותי בריאות הציבור (1), משרד הבריאות

ההתפרצות של קדחת מערב הנילוס (קמ"נ) (West-Nile Fever) בסוף קיץ-סתיו 2000 גרמה לבהלה ואף לחרדה בקרב הציבור, אך יחד עם זאת גם עניין רב וסקרנות מדעית במערכות הבריאות, איכות הסביבה והרפואה הווטרינרית, ומקום מרכזי בכל אמצעי התקשורת בישראל ובעולם. נראה היה שמדובר ב"מחלה אקזוטית" חדשה, כפי שמעיד עליה שמה – צירוף של מחלת "הקדחת" יחד עם "מערב הנילוס".

בחוברת הנוכחית של "הרפואה", מופיע מאמרם של נאסר וחב', מבית החולים לגליל המערבי בנהריה, שבו מדווח על ההתמודדות של הרופאים במחלקה לרפואה פנימית עם האיבחון והטיפול בחולה קמ"נ הראשון בגליל המערבי, שלקה בתסמינים נירולוגיים קשים ונזקק לאישפוז ולטיפול נמרץ.

לכאורה התוודע ציבור הרופאים בישראל לקמ"נ רק לאחרונה, ובעיקר לתסמיני המחלה הקשים, המצריכים אשפוז במחלקות לאשפוז נמרץ ואף עלולה לגרום לפטירת החולים. אולם רק מעטים יודעים, כי הפרסומים הראשונים בספרות העולמית על ההיבטים הקליניים של מחלה זו הגיעו בשעתו ממדינת ישראל, בשנות קיומה הראשונות.

גטאס נאסר, יאן כגנוב, דוד רמון

המח' לרפואה פנימית ב', בית חולים לגליל המערבי, נהריה

קדחת מערב הנילוס היא מחלה נגיפית הנגרמת על ידי נגיף מערב הנילוס, השייך למשפחת ה- Flaviviridae. הנגיף נישא על ידי ציפורים, בעיקר יונים ועורבים ומועבר בעקיצת יתוש, וגורם למלה שמהלכה קצר בדרך-כלל. מאפייני המחלה הם חום, כאב ראש, דלקת לחמית, כאבי-גב, שרירים ומיפרקים, בחילה, הקאה, שילשול, תיפרחת ושריעות קשריות-לימפה. בחלק מהחולים, קשישים בעיקר, תיתכן התהוות דלקת-מוח (encephalitis) עם שיעור תמותה גבוה, המחלה יכולה לחקות שפעת, חצבת אדמת, טיפוס, שחפת ודלקת-עוצבה חיידקית (bacterial meningitis) ומכאן הקושי בקביעת האבחנה. לאחרונה גרם הנגיף בישראל למקרים רבים של מחלת-חום ודלקת-מוח, ודווח על חולים שנפטרו מהמחלה.

מובאת בזאת פרשת החולה הראשון בגליל המערבי שאובחן כלוקה בקדחת מערב הנילוס. מקרה זה הגביר את עירנות הרופאים והתושבים באזור, תרם לאיבחון חולים רבים הלוקים במחלה והוביל לנקיטת אמצעי-ההגנה הנדרשים.

Male Genital Self Mutilation - A Cause of Hematuria

P. Schwartzman, Z. Viner

Department of Familyu Medicine, Community Health Section, Ben-Gurion University, The Negev, Beer-Sheva, Israel

Pathological self-mutilation is defined as a deliberate destruction of body tissue without conscious intent. It is sometimes associated with several pathologies such as mental disorders and mental retardation.

We report a non-psychotic male, with borderline mental retardation who practiced sporadic moderate genital self-mutilations as a masturbation technique and presented as a case of recurrent gross hematuria.

The Effect on Family Members of Treating Home-Hospitalized Patients

Netta Bentur, Avivit Moualem

JCD-Brookdale Institute, Jerusalem

Background: In recent years, chronic patients being treated at home are being joined by increasing numbers of acute and sub-acute patients. Although there has been a considerable expansion of formal services for home hospitalization, nevertheless a significant part of the burden of caring for these patients falls on their families. Many studies have examined the effect on the family of treating chronic patients at home, but very little is known about the effects on the family caregiver of treating an acute or sub-acute patient at home.

Goals of the study: a. To determine the main areas of care involving family members within the framework of home hospitalization. b. To assess the caregivers' sense of burden. c.To identify the causes of the feelings of burden. d. To examine the caregivers' specific difficulties. e. To identify the areas in which they are particularly interested in additional guidance.

Methodology: The study population included 179 primary caregivers of patients registered in four home hospitalization units. The data were collected during face-to-face interviews with the primary caregivers, in the patients' homes.

Findings: Half (49%) of the caregivers were the patients' spouses, and the rest were other family members, 67% were women. The average age was 56, and about a third were over 65. On average, they were involved in six areas of care. Fifty three percent reported that the burden they had to contend with was too heavy, 28% said it was not so heavy, and 19% said it was not heavy at all. A multi-variate analysis revealed that the feeling that the burden was too heavy had a statistically significant correlation with caring for another family member at home, being involved in 3+ areas of treatments, being married, 9+ years of education, the patients suffering from frequent pain or unable to reach the bathroom independently. Most (75%) of the caregivers reported three or more areas of burden which include the time devoted to care giving, and the effect of the treatment on the patient's health, mental and physical condition. Although 77% of them had received instruction on how to treat the patients, 29% cited at least one area in which they would have liked to receive more instruction, noting in particular the need to learn how to deal with changes in the patient's mental status.

Conclusions: Family members caring for home-hospitalized patients require more instruction and advice from the formal agencies during the daily care of the patient, particularly in specific subjects. There is a need to develop additional instruments for advice and instruction, in a broad range of areas, in order to assist the caregivers in their complex role. There is a need to continue to examine specific areas in which the family members require additional counselling and instruction, in order to better plan these services, and help improve the quality of care for home-hospitalized patients.
 

יעקב ברקון ומאיר שליט

היח' לאלרגיה ואימונולוגיה קלינית, האגף לרפואה פנימית, ביה"ח הדסה, ירושלים

בשנים האחרונות, הצטבר מידע רב על מעורבות ליקוטריאנים בגנחת הסימפונות ובמחלות דלקתיות למיניהן. במטרה למנוע את פעולתם בתהליך האלרגי-דלקתי, פותחו תרופות נוגדות ליקוטריאנים (נלק"ט), ולאחר שהוכחה יעילותן, הן הוכנסו לשימוש נרחב בטיפול בחולי גנחת. לאחרונה התברר, שתרופות אלו פעילות גם בנזלת אלרגית וכעת נערכים מחקרים רב-מרכזיים במטרה לבדוק את יעילותן כנגד מחלה זו. כן דווח בשנתיים האחרונות על הצלחות טיפוליות של נלק"ט בחולים הלוקים בחרלת כרונית (ח"כ). המטרה בסקירה זו היא לעמוד על תפקיד הליקוטריאנים בחרלת כרונית ולסכם את הידוע לגבי יעילות תרופות נלק"ט בטיפול בלוקים במחלה זו.

Neurological Manifestations in West Nile Fever

R. Grossman, Y. Levi

Department of Medicine B and Research Unit of Autoimmune Diseases, Chaim Sheba Medical Center (Affiliated to Tel-Aviv University), Tel-Hashomer, Ramat-Gan

The West Nile fever is a viral disease transferred by a mosquito bite. It is well known in the world for the last 70 years. Recently, there was an outbreak of this disease in Israel. We will describe case reports of 2 patients who were afflicted by the disease. The first one was hospitalized with an unusual presentation including paralysis to the lower limbs, while the second one was admitted with meningitis. In both, the course was quite dramatic, and one of them expired. Reviewing the literature revealed that neurological manifestations are quite frequent among these patients (90%), while in elder people, they were fatal.

Surgery for Blepharoptosis in Muscular Dystrophy

Ori Mahler, Asher Milstein, Ayala Pollack, Daniel Hauben

Eye Dept., Kaplan Medical Center, Rehovot; and Plastic Surgery Dept., Rabin Medical Center, Petah Tikva

In some muscular dystrophies there is ocular involvement characterized by blepharoptosis and ophthalmoplegia. These conditions occur in chronic progressive external ophthalmoplegia, oculopharyngeal muscular dystrophy, mitochondrial myopathy, myotonic dystrophy, and ocular myasthenia, among others. Although they differ in their systemic clinical manifestations and in genetic inheritance, ocular involvement is common to all of them. Manifestations include bilateral progressive blepharoptosis with or without extraocular muscle malfunction.

During surgical repair of the ptotic eyelid, consideration must be given to eyeball movements, in addition to maximal eyelid elevation, and to avoiding overcorrection and consequent corneal overexposure, leading to dryness and visual impairment. With these muscular dystrophic disorders, resection of the levator muscle or blepharoplasty alone does not suffice. Follow-up shows that most patients need a secondary repair after a short while. Operative correction uses a frontalis sling for eyelid elevation and support.

A series of 8 patients with these diseases, operated on by various surgical techniques during the past 7 years, is presented.

A Molecular Method of Diagnosis of Congenital Adrenal Hyperplasia

Shoshana Israel, Chaim Brautbar

Tissue Typing Unit, Hadassah Medical Center, Jerusalem

Congenital adrenal hyperplasia (CAH) is caused mainly by deficiency of the 21-hydroxylase enzyme. The disease may appear in the classical salt-losing, simple virilizing forms or as a mild, nonclassical form. 21-hydroxylase is encoded by the CYP21B gene on the short arm of chromosome 6, in the midst of the human leukocyte antigen (HLA) complex, between HLA Class I and Class II regions.

We describe a method for identifying mutations in the CYP21B gene. It is based on amplification of the gene using the polymerase chain reaction and identification of mutations with sequence-specific oligo-probes. The mutations identified were: V281 and P30L responsible for nonclassical CAH, and I2 splice, Q318X, I172N, cluster E6, and a deletion including 8bP in the third exon (8bP del) responsible for the classical form of CAH.

We also analyzed 2 families affected with the classical form of CAH which demonstrate possible complications in genotyping. Typing for HLA haplotypes can be helpful in certain cases, as demonstrated in 1 of the families presented. In this case it was necessary to distinguish between 2 possible genotypes: 1 with the mutations in tandem on 1 chromosome and the other with the mutated genes on both chromosomes. HLA haplotyping enabled the assignment of the mutations to the relevant chromosomes and thus allowed correct genetic counseling.

The other family demonstrated the importance of CYP21B genotyping in individuals with the nonclassical form of CAH. This form may consist of 1 mild and 1 severe mutation, representing a serious potential for transmitting the classical form of CAH.

Benign Paroxysmal Positional Vertigo: Diagnostic Pitfalls 

C.R. Gordon, O. Zur, R. Furas, E. Kott, N. Gadoth

Depts. of Neurology and of Physical Therapy, Meir General Hospital, Kfar Saba, and Sackler Faculty of Medicine, Tel Aviv University

Benign paroxysmal positional vertigo (BPPV) is a common but often unrecognized cause of treatable vertigo. Possible causes of misdiagnosis of BPPV were studied by review of the records of 191 patients referred to our neurology clinic; 36 were identified as having BPPV not previously diagnosed. On referral the patients carried the following diagnoses: unspecified dizziness/vertigo (33%), transient ischemic attacks (28%), cervicogenic vertigo (19%), psychogenic dizziness/vertigo (11%), and others (8%).

The paroxysmal nature of the vertigo and position-precipitating factors were not spontaneously reported by 31%. Atypical, even bizarre, symptoms including dizzy sensations were reported by 19%, and neck pain and headache were cardinal symptoms that accompanied vertigo.

The Dix-Hallpike maneuver, which is essential for the diagnosis of BPPV, was not performed in any of the patients prior to referral. 30 (83%) experienced complete resolution of signs and symptoms after the first physical treatment session.

We conclude that non-paroxysmal, non-positional vertigo does not rule out BPPV. Atypical and even bizarre complaints of dizziness, as well as neck pain and headache could be cardinal symptoms of BPPV. The Dix-Hallpike maneuver is mandatory in those complaining of dizziness and vertigo.

Progressive Ptosis in Children as a Presenting Sign of Kearns-Sayre Syndrome 

Ron Gal, Eli Lahat

Pediatric Neurology Unit, Assaf Harofeh Medical Center and Sackler Faculty of Medicine, Tel Aviv University

Mitochondrial encephalopathies represent a heterogeneous group of various neurological syndromes caused by defects in mitochondrial metabolism. All clinical syndromes can be subdivided by type of biochemical defect into 3 subgroups: defective oxidation, defects in pyruvate metabolism and various defects in the respiratory chain.

We present a 12-year-old girl admitted for evaluation of progressive ptosis over a period of 3 years, diagnosed as having the rare mitochondrial encephalopathy, Kearns-Sayre syndrome.

Sacrospinous Ligament Fixation for Vaginal Vault Prolapse

M. Neuman, B. Zuckerman, O. Lavie, U. Beller

Division of Gynecological Surgery and Oncology, Shaare Zedek Medical Center, Jerusalem

Sacrospinous ligament fixation is a well-known method for correction of vaginal vault prolapse. The procedure is primarily indicated after hysterectomy and as a prophylactic measure for total uterine prolapse.

8 women with post-hysterectomy vaginal vault prolapse aged 48-72 years, were referred 1-25 years following primary surgery. Sacrospinous ligament fixation was elected to enable simultaneous correction of cystocele and rectocele, and to preserve sexual function. All operations were completed without significant complications.

In 6 of the 8 patients located for long-term interview, 1 reported mild bulging of the introitus, another mild urinary stress incontinence, urgency and frequency and 1 reported frequency only. Sexual function in 4 was without complaints. Defecation in all patients was normal.
We conclude that this operation is safe and effective. We encourage gynecologic surgeons to consult an experienced tutor prior to performing this procedure, as this type of surgery is quite dangerous and many gynecologists are not familiar enough with it.  

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.

Familial Hemiplegic Migraine of Childhood

A. Livne, E. Lahat

Pediatric Division and Pediatric Neurology Unit, Assaf Harofeh Medical Center, and Sackler Faculty of Medicine, Tel Aviv University

Familial hemiplegic migraine is a rare autosomal, dominant, migraine subtype. It is characterized by acute episodes of hemiplegia and hemisensory deficits, and other neurological abnormalities occurring either before or together with severe headache, nausea and vomiting; episodes last several hours and then spontaneously subside. Intervals between episodes are relatively prolonged. Unless there is a relevant family history suggesting this syndrome, the diagnosis is usually delayed. Recently the gene for the syndrome was identified on chromosome 19. We report 3 boys and 1 girl, 11-15 years old with hemiplegic migraine.

Sacral Insufficiency Fractures - A Frequent Cause of Low Back Pain in Elderly Women

H. Semo, Z. Zwas, A. Goshen, S. Levenkrohn, A. Adunsky

Depts. of Geriatric Medicine and Nuclear Medicine, Sheba Medical Center, Tel Hashomer

Sudden low back pain is common in elderly women. It causes physical and mental stress, and results in deterioration of functional movement and in activities of daily living. Awareness of possible sacral insufficiency fracture is important; they may be demonstrated by imaging modalities, mainly radionuclide bone scan. Prognosis is good and accurate diagnosis serves to exclude malignancy and relieve fear of chronic pain and disability. We describe 4 women, aged 84, 82, 71 and 77 who illustrating the clinical and imaging findings of this disorder.

Plagiocephaly in Children: Etiology, Differential Diagnosis and Helmet Treatment

A. Margulis, V. Hatuel, A. Weinberg, A. Neuman, Z. Israel, M.R. Wexler

Depts. of Plastic Surgery, Occupational Therapy, and Neurosurgery, Hadassah University Hospital, Jerusalem

Plagiocephaly in a head-and-neck irradiated rat model or rhomboid-shaped head, occurs in at least 1 in 300 live births. In most cases such asymmetry is not caused by synostosis of the unilateral coronal or lambdoid sutures, but is rather a deformity produced by intrauterine and/or postnatal deformational forces. Categorization and diagnosis of plagiocephaly as synostotic or deformational is reliably made by physical examination and computerized tomography. Its differential diagnosis is extremely important because prompt surgical correction is usually indicated for the synostotic type. In contrast, infants with deformational frontal or occipital plagiocephaly generally respond to helmet treatment.

10 infants with significant deformational plagiocephaly were treated with individual plastic helmets during the past 2 years and 4 other infants with plagiocephaly are currently being treated. In each instance, cranial asymmetry dramatically improved as the brain grew and the head filled out the helmet. There were no significant complications. Awareness of deformational plagiocephaly allows more accurate diagnosis and appropriate treatment, avoiding unnecessary surgical intervention in patients with positional molding.