Israel Medical Association Journal

The incidence of aortic valve stenosis is growing rapidly in the elderly. Nonetheless, many symptomatic patients are not referred for surgery usually because of high surgical risk. Unfortunately, percutaneous balloon valvuloplasty is unsatisfactory due to high recurrence rates. In 2002, Cribier and colleagues were the first to describe percutaneous aortic valve implantation, opening a new era of aortic stenosis management. In the present review we report a patient treated by this novel method, discuss and assess how it is implanated, report the findings of studies conducted to date, and suggest future directions for percutaneous treatment of aortic valve disease.
 

Objectives: To evaluate the incidence and comparative frequency of clinical manifestations in different etiologic-pathogenic variants of RTD[1] in Israel and in summarized published data.

Methods: Stillborn and neonatal autopsy material from nine medical centers in northern and central Israel was studied. Information concerning pregnancy, labor and postnatal status and autopsy findings of cases with histologically, histochemically and immunohistochemically confirmed RTD were obtained from corresponding reports and from published material.

Results: From the 1538 autopsies of fetuses (≥ 20 weeks gestation) and neonates that were performed between 1976 and 2007 we identified 12 cases of RTD (0.78%). Abnormality occurred more often (1.4%) in the Upper and Western Galilee than in Israel as a whole.

Conclusions: Our study and a review of the literature showed that the autosomal recessive variant of RTD was more frequent than twin-twin transfusion induced. Most symptoms were similar in all variants of RTD, but their frequency was different in each of them.

Organophosphate and carbamate are mainly used to kill insects, thereby protecting livestock, crops, homes and communities. Yet, these compounds also convey great danger. OP[1] and CRB[2] poisoning is an important clinical problem, often life-threatening, especially in the pediatric population in rural areas where reaching a physician or hospital on time is difficult. We present a summary of accumulated toxicological knowledge as well as clinical and laboratory experience from a medical center serving a relatively vast rural area and pediatric population. We stress the importance of knowing how to recognize the classic signs of OP and CRB poisoning and when it is appropriate to investigate for such poisoning even in the absence of those signs. Like any medical emergency, OP and CRB poisoning requires prompt resuscitation and use of antidotes. Atropine, oxygen and fluids are the mainstay of therapy. Oximes, which were found useful in some cases of OP poisoning and useless in some cases of CRB poisoning, are absolutely safe as empiric treatment, which is often needed since the major differential diagnosis of OP poisoning is CRB poisoning that is clinically indistinguishable. We hope that continuing research will offer further insights into the management of such events, and we are confident that improved medical management of OP and CRB poisoning will result in a reduction of morbidity and other complications associated with intensive care procedures and hospitalization. 

The frequency of pregnancy-associated breast cancer, a rare but serious occurrence, may increase in light of the secular trends for lower parity in general and for an older age at first full-term delivery in particular. Data on PABC[1] in individuals who are at high risk for breast cancer are limited. A computerized search of PUBMED showed that the reported incidence of PABC is 1:3000 pregnancies; it is often diagnosed at an advanced stage and its prognosis is inferior compared to non-PABC. Carriers of mutations in the genes BRCA1/2 may present a specific high risk group for PABC especially at younger ages. Women treated with fertility treatment drugs may be at a higher risk for PABC as well.  

Background: The diagnosis of gastrointestinal stromal tumors is based on documentation of c-KIT and platelet-derived growth factor-alpha receptors or specific c-KIT mutations. Before the diagnosis of GIST[1] was possible, all cases had been classified as sarcomas or benign tumors.

Objectives: To identify cases of GIST formerly diagnosed as abdominal or retroperitoneal mesenchymal tumors.

Methods: We reviewed the archive material on all surgical cases diagnosed as gastrointestinal related malignant mesenchymal tumors or GIST in our medical center during the last decade (1995–2004).

Results: Sixty-eight cases of retroperitoneal soft tissue sarcoma were identified. Thirty-eight were reconfirmed to be GIST, 19 were newly diagnosed as GIST (the hidden cases), 8 cases were re-diagnosed as mesenchymal tumors, and 3 cases of sarcoma remained sarcomas. Of all the GIST tumors, c-KIT-positive and PDGFRα[2]-positive tumors were more characteristic of primary gastric tumors, while c-KIT-positive and PDGFRα-negative tumors were found in the colorectal area. The c-KIT-negative and PDGFRα-positive cases were of gastric origin.

Conclusions: Any c-KIT-negative malignant mesenchymal mass located near the proximal gastrointestinal tract should also be stained for PDGFRα to differentiate between GIST and other soft tissue sarcomas. Practically, formerly diagnosed abdominal or retroperitoneal soft tissue sarcomas should be reviewed to identify patients with misdiagnosed GIST and thereby avoid future unnecessary and ineffective chemotherapy.

Objectives: To review the value of mortality-case discussions in primary care clinics, particularly teaching clinics.

Methods: The clinic death register, instituted in 1998, includes age, gender, cause of death, place of death, relevant illnesses, and support provided to the patient before the death. In the half-yearly sessions, the data are reviewed, and individual cases that had an emotional impact on the staff, or information that can bring about changes in future care are discussed by the clinic staff and trainees.

Results: In our clinic 233 deaths occurred during a 6 year period (1998–2003). The crude all-cause mortality rate was 7.1/1000. The median age was 80 years old. Neoplastic causes were slightly more frequent than cardiovascular causes of death. Only 15% died at home; 20% lived alone and 70% lived with a spouse or family members before the death. Topics discussed in the mortality review meetings include identifying pre-suicidal patients, when to hospitalize the sick elderly, dealing with the anger of bereaved families, and ensuring proper home care for terminal patients.

Conclusions: We recommend keeping a death register and conducting mortality review sessions in order to improve the quality of care, emotional support of the staff, and training students and residents about the complex issues surrounding the death of patients.

Adjustment disorder is a common diagnosis in psychiatric settings and carries a significant rate of morbidity and mortality. However, both current and previous diagnostic criteria are vague and lead to many difficulties in terms of validity and reliability. This review is based on a thorough literature search and a systematic evaluation of the empiric and theoretic data. The various pitfalls inherent in the process of diagnosing this disorder are discussed in light of the diagnostic criteria for the disorder.

Objectives: To provide data on fatal traumatic heart wounds in autopsied cases.

Methods: We reviewed 2,487 medico-legal autopsy records of the morgue department of the Bursa branch of the Turkish Council of Forensic Medicine for the period 1997–2001.

Results: Of these cases, 160 (6.4%) had cardiac injury; 13.8% were females and 86.2% males, and the mean age was 35.9 years old (range 4–65). The most common cause of heart wounds was penetrating trauma (87.5%), namely sharp injuries (48.1%) and firearm injuries (39.4%). The two most common causes of blunt heart wounds were traffic accidents (5.6%) and falls from a height (5%). Rupture was present in 96.9% of the cases, and isolated left ventricle and isolated right ventricle were ruptured in 31.3% and 23.8%, respectively. In penetrating injury the risk of ventricle rupture was higher than of atrium rupture. Alcohol was detected in 16.3% of cases. Only 3.5% of the penetrating cardiac injury cases and 5% of the blunt cardiac injury cases were admitted to hospital.

Conclusions: Given that only a very low percentage of the patients who sustain cardiac injury reach hospital alive, population-based studies, especially autopsy results, should be conducted to define the characteristics of cardiac injuries.

Objective: To analyze the clinical features of cutaneous mastocytosis in a large series of children.

Methods: We conducted a file review of all children clinically diagnosed with cutaneous mastocytosis in our department over the last 20 years. We evaluated gender, age at onset, character and distribution of the lesions, associated symptoms, and course of the disease.

Results: Altogether, 180 patients with cutaneous mastocytosis were identified. The male to female ratio was 1.5:1. About one-third of patients had a mastocytoma, which was present at birth in over 40% and appeared during the first year of life in most of the remainder. Urticaria pigmentosa was noted in 65% of the patients, presenting at birth in 20% and during the first year in most of the remainder. The majority of lesions was distributed over the trunk and limbs. Different kinds of associated symptoms were noted. Prognosis, in general, was good. Only 11% of the cases, all urticaria pigmentosa, were familial.

Conclusions: Most cases of pediatric mastocytosis are sporadic and appear during the first 2 years of life, especially on the trunk. Urticaria pigmentosa is the most frequent variant. The prognosis of pediatric mastocytosis, in general, is good. 

Background: Current clinical guidelines restrict catheterization laboratory activity without on-site surgical backup. Recent improvements in technical equipment and pharmacologic adjunctive therapy increase the safety margins of diagnostic and therapeutic cardiac catheterization.

Objective: To analyze the reasons for urgent cardiac surgery and mortality in the different phases of our laboratory’s activity in the last 11 years, and examine the impact of the new interventional and therapeutic modalities on the current need for on-site cardiac surgical backup.

Methods: We retrospectively reviewed the mortality and need for urgent cardiac surgery (up to 12 hours post-catheterization) through five phases of our laboratory’s activity: a) diagnostic (years 1989–2000), b) valvuloplasties and other non-coronary interventions (1990–2000), c) percutaneous-only balloon angioplasty (1992–1994), d) coronary stenting (1994–2000), and e) use of IIb/IIIa antagonists and thienopiridine drugs (1996–2000).

Results: Forty-eight patients (0.45%) required urgent cardiac surgery during phase 1, of whom 40 (83%) had acute coronary syndromes with left main coronary artery stenosis or the equivalent, and 8 (17%) had mechanical complications of acute myocardial infarction. Two patients died (0.02%) during diagnostic procedures. In phase 2, eight patients (2.9%) were referred for urgent cardiac surgery due to either cardiac tamponade or severe mitral regurgitation, and two patients (0.7%) died. The combined need for urgent surgery and mortality was significantly lower in phase 4 plus 5 as compared to phase 3 (3% vs. 0.85%, P = 0.006).

Conclusion: In the current era using coronary stents and potent antithrombotic drugs, after gaining experience and crossing the learning curve limits, complex cardiac therapeutic interventions can safely be performed without on-site surgical backup.
 

Southern Sinai, a mountainous desolated arid area, is inhabited by Bedouin nomad tribes composed of Arabic-speaking Moslems. Until the Six Day War between Egypt and Israel in 1967, healthcare services in the region were based on traditional medicine performed by the Darvish, a local healer. Over the course of Israeli rule (1967-1982) an elaborate healthcare service was established and maintained, providing modern, up to date, comprehensive medical services that were available to all free of charge.