Israel Medical Association Journal

Inflammatory myopathies include polymyositis, necrotizing autoimmune myositis, dermatomyositis, juvenile inflammatory myopathy, and inclusion body myositis. These diseases are classified based on the different clinical and pathological characteristics unique to each of them [1]. Dermatomyositis is a rare disease with an incidence of 6–10 cases/1,000,000 a year with the highest incidence in the 7th decade of life as reported by a Norwegian cohort in a Caucasian population [2].

Diagnosis of dermatomyositis is based on typical signs and symptoms combined with laboratory results, imaging, and electromyography findings and muscle biopsy. Historically, the diagnosis of dermatomyositis was based on the classification criteria named after Bohan and Peter published in 1975. Many other classification criteria were proposed subsequently, the latter by the European League Against Rheumatism/American College of Rheumatology (EULAR/ACR), which were published in 2020 [3].

The clinical features of dermatomyositis are diverse. Skin manifestations can accompany or precede muscle weakness. Classical skin findings include periorbital heliotrope rash and a rash of the upper chest, back, and shoulders, known as the V sign and shawl sign respectively, as well as the Gottron's papules on the knuckles. Another skin appearance is subcutaneous calcifications that break periodically through the skin causing ulcerations. Dermatomyositis usually manifests as a symmetrical proximal muscle weakness but can present with preserved strength called amyopathic dermatomyositis [1].

Background: Sperm banks initially focused on providing sperm donation (SD) to heterosexual couples grappling with severe male infertility. Notable advancements in fertility treatments and sociological trends have broadened the scope of SD toward single women and same sex female couples.

Objectives: To evaluate SD recipient characteristics over the last three decades in Israel according to demographic parameters.

Methods: This retrospective cohort study included 5489 women who received SD between January 1992 and December 2021 from a tertiary referral center. We divided the overall period into six groups of five years each. A comparison of demographic characteristics of women who received SD in different periods was performed according to age at the beginning of the treatment, marital status (single women and same sex female couples, heterosexual couples), and ethnic origin.

Results: The average age of women who received SD was 37.02 ± 5.36 years. The average patient age rose from 35.08 years in 1992–1997 to 37.43 years in 2017–2021 (P-value < 0.01). The use of SD was more common among single women and same sex female couples compared to heterosexual couples in later years. Regarding single and same sex female couple, the percentage of SD recipients increased radically from 33% to 88.1% (P-value < 0.01).

Conclusions: Modern sperm banks treat older patients in non-heterosexual relationships. These trends encompass not only medical implications (e.g., in vitro fertilization vs. intrauterine insemination) but also delve into the personal and sociological impact experienced by both patients and offspring.

Background: Younger patient age and relatively good prognosis have been described as factors that may increase caregiver motivation in treating patients with septic shock in the intensive care unit (ICU).

Objectives: To examine whether clinical teams tended to achieve unnecessarily higher map arterial pressure (MAP) values in younger patients.

Methods: We conducted a population-based retrospective cohort study of patients presenting with septic shock who were treated with noradrenaline and hospitalized in a general ICU between 2006 and 2018. The patients were classified into four age groups: 18-45 (n=129), 46-60 (n=96), 61-75 (n=157), and older than 75 years (n=173). Adjusted linear mixed models and locally weighted scatterplot smoothing (LOWESS) curves were used to assess associations and potential non-linear relationships, respectively, of age group with MAP and noradrenaline dosage.

Results: The cohort included 555 patients. An inverse relation was observed between average MAP value and age. Among patients aged 18–45 years, the average MAP was 4.7 mmHg higher (95% confidence interval 3.4–5.9) than among patients aged > 75 years (P-value <0.001) after adjustment for sex, death in the intensive care unit, and Sequential Organ Failure Assessment scores.

Conclusions: Among patients with septic shock, the titration of noradrenaline by staff led to a higher average MAP for younger patients. Although the MAP target is equal for all age groups, staff may administer noradrenaline treatment according to a higher target of MAP due to attitudes toward patients of different ages, despite any evidence that such practice is beneficial.

In the acute settings of generalized myasthenia gravis (MG) treatment options include plasma exchange (PLEX), intravenous immunoglobulin (IVIG), and pyridostigmines. A thymoma is associated with the disease in up to 20% of cases [1,2].

In cases where a thymoma is detected, surgical treatment to remove the tumor is recommended in certain age groups. At present, there are no clear guidelines regarding the optimal time to perform thymectomy after diagnosis of acute crisis or from the last treatment to thymectomy. Treatment is at the clinician's discretion.

Acute or chronic aortic dissection is considered a rare emergency, with an estimated rate of 2.9 to 5 cases per 100,000 patients each year. This condition is most prevalent in males older than 65 years of age with a history of hypertension, atherosclerosis, and previous cardiac surgery [1,2]. To confirm the diagnosis, imaging is used, often by computed tomography angiography (CTA) of the chest. Although prompt treatment is required, patients often present with non-specific symptoms, such as abdominal pain or neurologic deficits, resulting in the early diagnosis of less than 20% and a high mortality rate [1].

Retroperitoneal infiltration, also known as retroperitoneal fibrosis (RPF), is a rare condition, which mostly occurs in men over the age of 40 years. This condition involves inflammation of the soft tissue of the retroperitoneal cavity, most commonly around the infrarenal abdominal aorta, iliac arteries, ureters, and abdominal organs. Clinical manifestations consist of severe pain in the lower back, abdomen, or flank, which may radiate to the inguinal region. Pain may be acute at the onset and can be mistaken for renal colic. Renal and ureteral involvement is common and can develop into acute kidney injury and hypertensive crises.

Background: Asthma is a common respiratory disease, which is linked to air pollution. However, little is known about the effect of specific air pollution sources on asthma occurrence.

Objective: To assess individual asthma risk in three urban areas in Israel characterized by different primary sources of air pollution: predominantly traffic-related air pollution (Tel Aviv) or predominantly industrial air pollution (Haifa bay area and Hadera). 

Methods: The medical records of 13,875, 16- 19-year-old males, who lived in the affected urban areas prior to their army recruitment and who underwent standard pre-military health examinations during 2012–2014, were examined. Nonparametric tests were applied to compare asthma prevalence, and binary logistic regressions were used to assess the asthma risk attributed to the residential locations of the subjects, controlling for confounders, such as socio-demographic status, body mass index, cognitive abilities, and education.

Results: The asthma rate among young males residing in Tel Aviv was 8.76%, compared to 6.96% in the Haifa bay area and 6.09% in Hadera. However, no statistically significant differences in asthma risk among the three urban areas was found in controlled logistic regressions (P > 0.20). This finding indicates that exposure to both industrial- and traffic-related air pollution is associated with asthma prevalence.

Conclusions: Both industrial- and traffic-related air pollution have a negative effect on asthma risk in young males. Studies evaluating the association between asthma risk and specific air pollutants (e.g., sulfur dioxide, particulate matter, and nitrogen dioxide) are needed to ascertain the effects of individual air pollutants on asthma occurrence. 

 

Background: Current guidelines for choosing between revascularization modalities may not be appropriate for young patients.

Objectives: To compare outcomes and guide treatment options for patients < 40 years of age, who underwent percutaneous coronary intervention (PCI) or coronary artery bypass grafting (CABG) between 2008 and 2018.

Methods: Outcomes were compared for 183 consecutive patients aged < 40 years who underwent PCI or CABG between 2008 and 2018, Outcomes were compared as time to first event and as cumulative events for non-fatal outcomes.

Results: Mean patient age was 36.3 years and 96% were male. Risk factors were similar for both groups. Drug eluting stents were implemented in 71% of PCI patients and total arterial revascularization in 74% of CABG patients. During a median follow-up of 6.5 years, 16 patients (8.6%) died. First cardiovascular events occurred in 35 (38.8%) of the PCI group vs. 29 (31.1%) of the CABG group (log rank P = 0.022), repeat events occurred in 96 vs. 51 (P < 0.01), respectively. After multivariate adjustment, CABG was associated with a significantly reduced risk for first adverse event (hazard ratio [HR] 0.305, P < 0.01) caused by a reduction in repeat revascularization. CABG was also associated with a reduction in overall repeat events (HR 0.293, P < 0.01). There was no difference in overall mortality between CABG and PCI.

Conclusions: Young patients with coronary disease treated by CABG showed a reduction in the risk for non-fatal cardiac events. Mortality was similar with CABG and PCI.

Background: Young women concerned about a breast cancer diagnosis will visit breast care centers and request breast cancer screening, including imaging studies, on their initial visit.

Objectives: To explore the role of breast examination and breast ultrasound in self-referred asymptomatic women under the age of 40 years.

Methods: We identified 3524 women under the age of 40 at our medical clinic from 1 January 2010 until 1 June 2014. Of this group, 164 women with above average breast cancer risk were excluded and 233 were excluded because of breast complaints. Of 3127 women, 220 underwent breast ultrasound following the initial visit to the clinic and formed the study group.

Results: Of 220 women evaluated with ultrasound, 68 had prior positive clinical findings. Of this group 8 women had no sonographic findings, and in the remaining 60, a total of 30 simple cysts, 15 fibroadenomas, and 15 suspicious solid masses were identified. One infiltrating ductal carcinoma and one ductal carcinoma in situ were found in a biopsy. The remaining 152 of the 220 total women who underwent breast ultrasound without showing prior physical findings did not require follow-up.

Conclusions: In the absence of clinical findings during physical breast examination, the addition of breast ultrasonography does not provide additional information to supplement the physical examination in self-referred women under age 40 who do not have any major risk factors for developing breast cancer.

Background: Contemporary data on clinical profiles and long-term outcomes of young adults with coronary artery disease (CAD) are limited.

Objectives: To determine the risk profile, presentation, and outcomes of young adults undergoing coronary angiography.

Methods: A retrospective analysis (2000–2017) of patients aged ≤ 35 years undergoing angiography for evaluation and/or treatment of CAD was conducted.

Results: Coronary angiography was performed in 108 patients (88% males): 67 acute coronary syndrome (ACS) and 41 non-ACS chest pain syndromes. Risk factors were similar: dyslipidemia (69%), positive family history (64%), smoking (61%), obesity (39%), hypertension (32%), and diabetes (22%). Eight of the ACS patients (12%) and 29 of the non-ACS (71%) had normal coronary arteries without subsequent cardiac events. Of the 71 with angiographic evidence of CAD, long-term outcomes (114 ± 60 months) were similar in ACS compared to non-ACS presentations: revascularization 41% vs. 58%, myocardial infarction 32% vs. 33%, and all-cause death 8.5% vs. 8.3%. Familial hypercholesterolemia (FH) was diagnosed in 25% of those with CAD, with higher rates of myocardial infarction (adjusted hazard ratio [HR] 2.62, 95% confidence interval [95%CI] 1.15–5.99) and revascularization (HR 4.30, 95%CI 2.01–9.18) during follow-up. Only 17% of patients with CAD attained a low-density lipoprotein cholesterol treatment goal < 70 mg/dl.

Conclusions: CAD in young adults is associated with marked burden of traditional risk factors and high rates of future adverse cardiac events, regardless of acuity of presentation, especially in patients with FH, emphasizing the importance of detecting cardiovascular risk factors and addressing atherosclerosis at young age.