אלכסנדר רוזין, דניאל שפירא, אליסיה נכטיגל ומנחם נהיר

מובאת במאמר זה, פרשת חולה בן 21 שנה שלקה באוסטאוארתרוזיס (או"א) קשה של מיפרקי הירכיים. נוכח המימצא המפתיע, נדונה האפשרות של מחלת קשין-בק (מק"ב) – מחלה אנדמית לאזור מגוריו הקודם של החולה (מזרח-סיביר).

מובאת במאמר זה בקצרה האבחנה המבדלת של פגיעה דו-צדדית במיפרקי ירכיים מישנית למחלות אחרות, כולל דלקת מיפרקים שיגרונתית של גיל הנעורים סטיל (דמשנ"ס).

(1) הדסה גולדברג-שטרן, (1) רונן מוסרי, (2) בן-ציון גרטי, (3) נתן גדות

(1) המרכז לכיפיון, (2) מח' ילדים ב', מרכז רפואי שניידר לרפואת ילדים, (3) המח' לנירולוגיה, בית-חולים מאיר, כפר-סבא

בשנים האחרונות הצטבר מידע על הקשר בין הפרעות פירכוסיות (epilepsies) לוויטאמין B6 (פיטרידוקסין). פירידוסקין עובר בגוף תהליך זירחון לפירידוקסל-פוספאט, שהוא קו-פקטור חיוני במספר תהליכים אנזימיים, ביניהם הפיכת חומצה גלוטמית לנירוטרנסמיטור מעכב במוח – חומצה אמינו-בוטירית (GABA - aminobutyric acid) על ידי האנזיםglutamic acid decarboxylase (GAD) (תרשים 1). הדרישה היומית המיקטית של פירידוקסין עד גיל 6 חודשים היא 0.3 מ"ג/יום ובמבוגר – 2 מ"ג/יום. כאשר קיים חוסר בנירוטרנסמיטור המעכב (GABA), עלולים להתרחש פירכוסים. פירכוסים הקשורים לפירידוקסין מסווגים ל-3 קבוצות.

חיים בסן ואורי קרמר

המכון להתפתחות הילד והיחידה לנירולוגית ילדים, החטיבה לילדים, בי"ח דנה, מרכז רפואי ת"א, הפקולטה לרפואה סאקלר, אוניברסיטת ת"א

הליגה הבינלאומי למניעת כיפיון מגדירה פירכוסי חום (פ"ח) כפירכוסים המתרחשים בילדים מעל גיל חודש, נלווים למחלת חום, אינם נובעים מזיהום במערכת העצבים המרכזית, בהעדר אנאמנזה של פירכוסים ללא חום ואשר אינם במיתאם עם ההגדרה של פירכוסים תסמיניים חדים אחרים. פ"ח מתרחשים ברוב החולים בשלב עליית החום, בד"כ אך לא תמיד ביממה הראשונה של מחלת החום, ובלי קשר למידת החום. שיעור הפ"ח בארה"ב ובמערב אירופה הוא 4%-2%, ביפן 10%-9% ובגואם 14%. תופעה זו היא הסיבה השכיחה ביותר לפירכוסים בילדות.

Mortality in Fractures of the Hip 

Y. Titiun, Z. Eshkol, Z. Horesh, M. Soudry

Dept. of Orthopedic Surgery, Beilinson Campus, Rabin Medical Center, Petah Tikva

Prevalence of hip fractures is increasing world-wide, as the mean age of populations increases. Despite advances in anesthesia, nursing care, and surgical techniques, hip fractures remain a significant cause of morbidity and mortality in the elderly.

We operated on 65 cases of hip fractures from 1995 to the end of 1997: average age was 82.9, 72% were women, average waiting time for operation was 1.6 days, perioperative mortality was 3.5% and postoperative mortality 26.2%.

Alpha-Amino Adipic Aciduria: a Rare Psycho-Motor Syndrome

Ben-Ami Sela, Tamar Massos, Dov Fogel, Joseph Zlotnik

Pathological Chemistry and Child Development Institutes, Sheba Medical Center, Tel Hashomer and Sackler Faculty of Medicine, Tel Aviv University

A 3.5-year-old boy with developmental motor retardation, hypotonicity, and severe speech disturbance had alpha-amino adipic acid in his blood and very high levels in his urine. In only 20 cases has this catabolite of lysine and hydroxylysine been found in high concentrations in urine, due to enzymatic block.

The clinical features associated with alpha-amino adipic aciduria may include mental retardation, developmental and motor delay, learning difficulties, convulsions, speech problems and ataxia. 3 siblings had milder symptoms of psychomotor delay and intermediate degrees of alpha amino-adipic aciduria, suggesting that the described developmental deficits could be related to this metabolite or its derivatives.

Tension-Free "Plug and Patch" Inguinal Hernia Repair

Y. Mintz, D. Goitein, M. Muggia-Sullam

Dept. of General Surgery, Hadassah-Hebrew University Hospital, Jerusalem

Inguinal hernias are common and have highly variable clinical presentations, ranging from the asymptomatic to surgical emergencies when incarcerated, sometimes necessitating bowel resection. Several techniques have been described for repair of inguinal hernias based on the unique anatomic structure of the inguinal region. In 1989 Lichtenstein and co-workers described a "tension free" repair based on using a synthetic mesh patch to reinforce the inguinal canal floor and as a plug to prevent recurrence through the internal inguinal ring. This technique has been used in many centers worldwide with good rates of success.

Vacuum Phenomenon in the Hip Joint: Diagnostic Value

S. Iordache, E. Rath, D. Atar, A. Vindzberg

Orthopedics Dept., Soroka Medical Center, Beer Sheba

Vacuum phenomenon is well known in degenerative spinal disease in the elderly, but is seldom seen in other joints, especially in children. The phenomenon does not represent a pathological finding, and can be used for imaging of the articular facets, mainly in the hip and knee joints. We report a patient with this phenomenon in the hip joint.

Conversion Total Hip Replaceafter Failed Internal Fixation of Intertrochantric Fracture

M. Kligman, M. Roffman

Dept. of Orthopedic Surgery, Carmel Medical Center, Haifa

Between 1933-1995, 16 patients underwent conversion-total hip replacement after internal fixation of an intertrochanteric fracture failed. Clinical results were unsatisfactory compared to primary total hip replacement. There was a high incidence of intra- and postoperative complications, including femoral fracture, wound infection, and aseptic loosening. This study should increase the surgeon's awareness of the difficulties encountered in conversion of failed intertrochanteric fractures to total hip replacement.

Ketogenic Diet for Intractable Epilepsy in Adults

Yaffa Schiff, Tally Lerman-Sagie

Nutrition and Diet Unit, Tel Aviv Medical Center; and Pediatric Neurology Unit, Wolfson Medical Center, Holon; and Sackler Faculty of Medicine, Tel Aviv University

The ketogenic diet is an accepted alternative for children with intractable generalized or multi-focal seizures not amenable to surgery. It is not commonly used in adults because of the impression that the diet is less effective after childhood, when it is more difficult both to achieve ketosis and to change dietary habits. We present a 20-year-old man with intractable epilepsy since early childhood who is being treated with great success by a medium-chain triglyceride ketogenic diet. It has not only controlled the seizures but has also improved quality of life. We recommend a therapeutic trial of the ketogenic diet in intractable epilepsy for all ages.

Orthopedic Ward Policy in Introduction of New Types of Total Hip Implants

U. Givon, M.I. Siebzhener, M. Salai, A. Chechick, H. Horoszowski, J. Shemer

Orthopedic Staff, Medical Technology Unit, Medical Dept. F, and Gertner Institute for Study of Health Services, Chaim Sheba Medical Center, Tel Hashomer

The use of different types of total hip implants in medical centers in Israel was surveyed. Questionnaires were sent to all orthopedic ward directors in Israel requesting information on the number of total hip arthroplasties performed between the years 1984-1993, the types of implants used, and whether attending physicians or residents perform the operations. 22 of 24 orthopedic wards responded but 1 ward was excluded because only the results for 1993 were reported. 5 wards reported more and 16 fewer than 50 operations a year. 15 different types of implants were in use in Israel in that period, and in 5 wards 5 or more types of implants were used. Only 1 of the wards performed more than 50 operations a year. We conclude that the indiscriminate use of multiple technologies in wards performing few operations can lead to the long "learning curves" previously associated with poor results. Orthopedic surgeons should resist the impulse to introduce new implants, thus improving results and lowering expenditure. The need for regulating the introduction of new implants is emphasized.

Magnetic Resonance Imaging for Suspected Femoral Neck Fractures

M. Kligman, M. Roffman

Dept. of Orthopedic Surgery, Carmel Medical Center, Haifa

Painful hip as a result of injury, with or without a history of trauma, is a common reason for referring elderly patients to the emergency room. The diagnosis of femoral neck fracture requires the combination of a physical examination, X-rays, and in problematic cases, a bone scan. However, even this combination does not always provide a diagnosis. We present 50 patients with painful hip who complained of limp and reduced hip joint motion, but had no evidence of fracture, either on X-ray or bone scan. After conservative treatment, 5 patients with no history of trauma underwent hemiarthroplasty of the hip for displaced subcapital fracture. In addition, we present a case of subcapital fracture which was diagnosed only by MRI, in whom both X-rays and bone scan were considered normal.

Femoral Fracture following Total Hip Replacement

M. Kligman, M. Roffman

Dept. of Orthopedic Surgery, Carmel Medical Center, Haifa

Between January 1990 and May 1996 we performed 500 total hip replacements, 6 of which were complicated by ipsilateral femoral fracture. Treatment was either by skeletal traction or by internal fixation, with or without revision-total hip replacement. Results of surgical treatment were superior to those of conservative treatment. This study supports use of Mennen plate-fixation. Further studies are necessary for final evaluation of the efficacy of this method.