Israel Medical Association Journal

Background: The external approach is the golden standard for sinonasal tumor removal but it is associated with several side effects, including facial scars, intracranial and extracranial complications, a long hospitalization period and high costs. Endoscopic sinus surgery enables resection of benign and selected malignant sinonasal tumors and has the advantages of no facial scars, better functional and structural preservation of the sinonasal complex, minimal trauma to surrounding tissue, a shorter hospitalization stay and lower costs.

Objectives: To evaluate the advantages and limitations of endoscopic resection of benign and malignant sinonasal tumors, their recurrence and complication rates.

Methods: The medical and radiology records of 56 patients who underwent endonasal endoscopic resection of benign and malignant sinonasal tumors between 1996 and 2003 were retrospectively reviewed. Tumors located in the center of the nose and sinuses were endoscopically resected.

Results: Six cases of malignant tumor and 50 cases of benign tumor underwent resection by ESS[1]. One of the patients with malignant tumor died, the remainder showing no evidence of disease on follow-up of 3–60 months (mean 26.8 months). Inverted papilloma was the most common benign tumor (40 patients). Seven patients (18%) had recurrence followed by endonasal resection. No major complications were recorded. Hospitalization stay was 2–7 days (mean 3.6 days).

Conclusions: Endoscopic resection of benign sinonasal tumors that are centrally located in the nose and sinuses should be considered before the external approach is used. In very carefully selected cases of malignant tumors ESS is oncologically acceptable, but more experience is needed for discerning the indications for endoscopic resection of malignant tumors. The complication rate for endoscopic resection is low, there are no facial scars, hospitalization stay is short, and costs are low.

Background: Granulocyte colony-stimulating factor has had a major impact on the management of severe chronic neutropenia – a collective term referring to congenital, idiopathic, or cyclic neutropenia. Almost all patients respond to G-CSF[1] with increased neutrophils, reduced infections, and improved survival. Some responders with congenital neutropenia (termed Kostmann’s syndrome herein) and Shwachman-Diamond syndrome have developed myelodysplastic syndrome and acute myeloid leukemia, which raises the question of the role of G-CSF in pathogenesis. The issue is complicated because both disorders have a propensity for MDS[2] or AML[3] as part of their natural history.

Objective and Methods: To address this, the Severe Chronic Neutropenia International Registry used its large database of chronic neutropenia patients treated with G-CSF to determine the incidence of malignant myeloid transformation in the two disorders, and its relationship to treatment and to other patient characteristics.

Results: As of January 2001, of the 383 patients with congenital forms of neutropenia in the Registry, 48 had MDS or AML (crude rate, about 12.5%). No statistically significant relationships were found between age at onset of MDS or AML and patient gender, G-CSF dose, or duration of G-CSF therapy. What was observed, however, was the multistep acquisition of aberrant cellular genetic changes in marrow cells from Kostmann’s syndrome patients who transformed, including activating ras oncogene mutations, clonal cytogenetic abnormalities, and G-CSF receptor mutations. The latter in murine models produces a hyperproliferative response to G-CSF, confers resistance to apoptosis, and enhances cell survival.

Conclusions: Since Kostmann’s syndrome and Shwachman-Diamond syndrome are inherited forms of bone marrow failure, G-CSF may accelerate the propensity for MDS/AML in the genetically altered stem and progenitor cells, especially in those with G-CSF receptor and ras mutations (82% and 50% of Kostmann’s syndrome patients who transform, respectively). Alternatively, and equally plausible, G-CSF may simply be an innocent bystander that corrects neutropenia, prolongs patient survival, and allows time for the malignant predisposition to declare itself. Only careful long-term follow-up of the cohort of patients receiving G-CSF will provide the answer.

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[1] G-CSF = granulocyte colony-stimulating factor

[2] MDS = myelodysplastic syndrome

[3] AML = acute myeloid leukemia

Background: Although Listeria monocytogenes is widely distributed in nature, it rarely causes clinical infection in previously healthy people. This microorganism. however, may cause severe invasive disease in pregnant women and newborns.

Objectives: To investigate – in our pregnant population – the impact, severity and outcome of listeriosis on both mother and fetus.

Method: The study was carried out at a level III, university two-hospital complex, In a retrospective chart review of 65,022 parturients during a 10 year period (1990-1999), we identified and: evaluated 11 pregnant patients and their offspring with Listeria infection;

Results: Chorioamnionitis with multiple. placental abscesses were observed in all five placentae examined. Clinically 4 of 11 parturients had a cesarean section for fetal distress (36.3%), as compared to the 14% mean CS rate in our general population. Two of 11 had a fate abortion (18.1%), as compared with the 4% rate in our hospital. Four of 11 had premature labor (36%), which was about four times the rate in our population. Finally, although no intrauterine feta1 death was recorded in our series, there was one neonatal death of a term infant. (1/11, 9%), which is about 10 times higher than our corrected perinatal mortality rate.

Conclusions: If not promptly and adequately treated, listeriosis in pregnancy may present serious hazards to the fetus and newborn through direct infection-of the placenta and chorioamnionitis.
 

Background: Head louse infestations are prevalent worldwide. Over the past 20-25 years, 15-20% of all children in Israel between 4 and 13 years of age have been infested with head lice; This is mainly due to the existence of ineffective pediculicides on the market.

Objective: To examine the pediculicidal efficacy and safety of a natural remedy (”Chick-Chack") and to compare it in an open clinical study with a known pesticide spray.

Methods: The natural remedy, which contains coconut oil, anise ail and ylang ylang oil:, was applied to the hair of infested.children three times at 5 day intervals. Each treatment lasted for 15 minutes. The control pediculicide was a spray, formulation containing permethrin, malathion, piperonyl butoxide, isododecane and propellant gas, which was applied twice for 10 minutes with a 10 day interva1 between applications.

Results: Of 940 Children, aged 6-14 years, from six schools in Jerusalem who were examined for head louse infestastion,199 (21:.2÷/) were infested with lice and eggs, while 164 (17.4% ) were infested only  with nits. Altogether, 119 children were randomly treated with either the natural remedy or the control product. Treatment was successful with the natural remedy  in 60 children (92.3%) and with the control pediculicide in 59 children (92.2%). There were no significant side effects associated with either formulation.

Conclusions: The natural remedy was very effective in controlling  louse infestations under clinical conditions and caused no serious side effects.
 

Background: Long-term follow-up in apical hypertrophic cardiomyopathy is rare.

Objective: To study the natural history of the disease.

Methods: We followed 11 patients, 5 women and 6 men, for 5-20 years.

Results: At presentation all 11 patients had typical features of apical hypertrophic cardiomyopathy, with dyspnea in 3 and chest pains in 8, of whom 5 were typical of angina and 3 had myocardial infarction. R-wave voltage and T-wave negativity progressively decreased in magnitude at serial electrocardiograms in four patients. Perfusion defects were detected on thallium myocardial scintigraphy in three, increased apical uptake in two, and normal in one patient. Apical aneurysm with normal coronary arteries developed in a patient who had sustained ventricular tachycardia. All of the 10 catheterized patients had normal coronaries except for one with significant left anterior descending artery stenosis and another with a minor lesion. Symptomatic sustained ventricular tachycardia was found in two patients, one of whom required the implantation of an internal cardioverter-defibrillator.

Conclusions: Apical hypertrophic cardiomyopathy may develop morphologic and electrocardiographic changes with life-threatening arrhythmias necessitating close follow-up and treatment.

Background: Leptin is a 16 kDa hormone synthesized by adipocytes and involved in body weight regulation.

Objectives: To determine serum leptin concentrations in heart, liver and kidney transplant recipients.

Methods: We investigated 57 patients: 18 male heart transplant recipients (age 25-69 years) at 1-66 months after transplantation, 6 female and 8 male liver transplant recipients (age 33-70) at 11-73 months after transplantation, and 10 female and 15 male kidney transplant recipients (age 20-61) at 3-138 months after transplantation. All recipients were receiving immunosuppressive therapy, including prednisone 0-20 mg/day, azathioprine 75-125 mg/day, cyclosporin 100-250 mg/day or tacrolimus 2-10 mg/day. The results were compared to those of 10 female and 10 male healthy controls. Morning serum concentrations of leptin were measured with a commercial radioimmunoassay (Linco Research Inc., USA), and serum insulin and cortisol levels were measured by radioimmunoassay.

Results: Patients (both men and women) after heart, liver and kidney transplantation exhibited significantly higher serum concentrations of leptin and leptin/body mass index ratios than controls. Serum leptin concentrations were significantly higher in women than in men and correlated very significantly with BMI[1] in all cases. The multivariate stepwise analyses showed that among parameters including BMI, gender, age, time after transplantation, prednisone dose, hematocrit, serum concentrations of glucose, albumin, creatinine, cortisol and insulin, only BMI, gender, cortisol and insulin were significant independent determinants of serum leptin levels in these patients.

Conclusions: This is the first report showing that, in addition to body mass index and gender, basal cortisol and insulin levels affect the hyperleptinemia in transplant patients. The clinical relevance of hyperleptinemia in these patients will require further investigation.

Background: At the start of the Bone and Joint Decade 2000-2010, a paleopathologic study of the physically disabled may yield information and insight on the prevalence of crippling disorders and attitudes towards the afflicted in the past compared to today.

Objective: To analyze “The procession of the Cripples,” a representative drawing of 31 disabled individuals by Hieronymus Bosch in 1500.

Method: Three specialists - a rhumatologist, an orthopedic surgeon and neurologist - analyzed each case by problem-solving means and clinical reasoning in order to formulate a consensus on the most likely diagnosis.

Result: This iconographic study of cripples in the sixteenth century reveals that the most common crippling disorder was not a neural form of leprosy, but rather that other disorders were also prevalent, such as congenital malformation, dry gangrene due to ergotism, post-traumatic amputations, infectious diseases (Pott’s, syphilis), and even simulators. The drawings show characteristic coping patterns and different kinds of crutches and aids.

Conclusion: A correct clinical diagnosis can be reached through the collaboration of a rheumatologist, an orthopedist and neurologist. The Bone and Joint Decade Project, calling for attention and education with respect to musculoskeletal disorders, should reduce the impact and burden of crippling disease worldwide through early clinical diagnosis and appropriate treatment.