Israel Medical Association Journal

Inborn errors of immunity (IEI) encompass a rapidly growing spectrum of genetic disorders affecting immune development and function and resulting in susceptibility to infection, immune dysregulation, autoimmunity and malignancy.

The therapeutic approach to IEI has evolved considerably over recent decades. The cornerstones of management include antimicrobial prophylaxis, immunoglobulin replacement, and treatment of immune dysregulation and lymphoproliferative complications. For patients with severe IEI, hematopoietic stem cell transplantation offers potentially curative therapy, with early transplantation associated with improved outcomes in appropriately selected patients. The therapeutic landscape is expanding through the development of gene therapy and an increasing number of disease-specific, targeted treatments directed at defined molecular and immunological pathways.

These advances are shifting the paradigm from predominantly supportive management toward increasingly definitive and precision-based therapies. As therapeutic opportunities continue to evolve, broadening awareness of these advances across medical disciplines will be critical to ensuring that patients with IEI can benefit from the full range of available therapies and achieve the best possible outcomes.

Background: During Operation Roaring Lion, Carmel Medical Center, a 500-bed tertiary hospital, relocated nearly half of its inpatient activity to the fortified underground facility of Rambam Health Care Campus while maintaining independent clinical and managerial responsibility. Returning patients after hostilities subsided represented a complex organizational transition requiring preservation of patient safety and continuity of care.

Objectives: To describe a multidisciplinary operational leadership model and evaluate the use of failure mode and effects analysis (FMEA) to support safe wartime hospital relocation.

Methods: A prospective quality improvement study used multidisciplinary FMEA before patient relocation. The relocation pathway was divided into four phases, and potential failure modes were scored by severity, occurrence, and detectability to prioritize mitigation strategies. Interventions included centralized command, real-time patient tracking, structured clinical handoffs, standardized transfer checklists, medication reconciliation, risk stratification, and enhanced post-transfer monitoring. Outcomes included patient safety events, falls, resuscitation events, unplanned intensive care unit (ICU) admissions within 24 hours, multidrug-resistant organism (MDRO) cross-transmission, and departmental feedback.

Results: During 55 days of wartime activity, 843 patients were hospitalized in the underground facility. In total, 84 medically complex patients were relocated in three operational waves. The highest-risk domains involved communication, documentation, operational coordination, staffing, and clinical monitoring. No predefined patient safety events, falls, resuscitation events, unplanned ICU admissions within 24 hours, or documented MDRO cross-transmission were identified. Department leaders reported high organizational preparedness and improved operational control.

Conclusions: A multidisciplinary operational leadership model integrating proactive FMEA enabled coordinated management of clinical, informational, operational, and infection-related risks during complex wartime hospital relocation.

Background: Pain following arthroscopic partial meniscectomy (APM) varies considerably among patients. Previous studies have suggested that gender and ethnicity may influence postoperative pain after orthopedic procedures. However, limited evidence exists regarding pain differences between Arab and Jewish patients after APM.

Objectives: To evaluate the association of gender and ethnicity with postoperative pain and analgesic consumption during the first month after APM.

Methods: Patients who underwent primary APM between 2018 and 2019 were retrospectively identified and stratified by gender and ethnicity. Intraoperative findings, including meniscal tear type and cartilage injury, were recorded. Postoperative pain was assessed using the Knee injury and Osteoarthritis Outcome Score (KOOS), analgesic consumption was quantified in grams, and pain sensitivity was evaluated with the Pain Sensitivity Questionnaire (PSQ). Outcomes were analyzed weekly during the first postoperative month.

Results: In total, 99 patients were included (65 men, 34 women; 39 Arabs, 60 Jews), comprising 22 Arab men, 43 Jewish men, 17 Arab women, and 17 Jewish women. Women reported lower KOOS scores and consumed more analgesics during weeks 3 and 4. Arab patients also demonstrated lower KOOS scores, higher PSQ scores, and greater analgesic consumption at selected postoperative intervals. Combined analysis showed an additive effect of gender and ethnicity, with Arab women reporting the poorest pain outcomes.

Conclusions: Female gender and Arab ethnicity were associated with greater postoperative pain and increased analgesic requirements after APM. These factors should be considered when tailoring postoperative pain management, although baseline differences and cultural influences may partially explain the associations.

Background: Pepper spray and tear gas are chemical irritants causing ocular, dermal, and respiratory injury. Data specific to pediatric emergency department (PED) presentations remain limited.

Objectives: To characterize the epidemiology, clinical presentation, and short-term outcomes of chemical irritant exposure in children presenting to a tertiary PED.

Methods: We conducted a retrospective cohort study of patients aged 0–18 years of age presenting to a single tertiary PED between 2016 and 2024 following civilian-grade chemical irritant exposure. Cases were identified using ICD-9 codes and free-text electronic medical records search. Demographic characteristics, clinical findings, treatment, and disposition were analyzed using descriptive statistics.

Results: We included 84 pediatric patients (median age 12.5 years, IQR 6.0–15.6; 69.0% male). Exposures occurred most commonly at home (44.0%) and school (36.9%). Ocular involvement was common. Superficial punctate keratitis was identified in 54.5% of patients who underwent fluorescein examination by ophthalmologist (21.5% of the total cohort). Respiratory and dermal symptoms were mild and self-resolving. No severe complications were documented; 96.4% were discharged home.

Conclusions: Civilian-grade chemical irritant exposures in children were predominantly self-limited and managed successfully with conservative ED care, supporting decontamination-based protocols while highlighting home and school settings as priorities for prevention.

Background: Diabetic foot (DF) is a dangerous complication of diabetes mellitus (DM). Modern treatment modalities and good organization of care can improve prognosis.

Objectives: To analyze results of DF treatment in a tertiary care setting and the degree of implementation of international guidelines in practice.

Methods: We conducted a retrospective analysis of 90 patients treated for DF ulcers/wounds in our center (May–December 2023). Thirty-five patients received only outpatient care. The median age was 65 (37–90) years, 96% had type 2 DM.

Results: Median wound duration was 29 (1–1461) days. Previous treatment was conducted by a family doctor in 30%, at a local wound clinic in 7%, by other medical specialists in 27%, or by the patient/relatives in 29%. At this stage off-loading devices were prescribed for 37%, and 35% were referred to a vascular surgeon. Limb ischemia occurred in 44 patients (49%), revascularization was conducted for 16 patients. At 6 months, healing rates were 48%. Minor or major amputation rates were 4% and 5%, respectively; 6% died, 29% stayed unhealed, and 8% were lost to follow-up. Multivariable analysis revealed independent predictors of non-healing (ethnic group and impossibility of revascularization) and of amputation (previous major amputation, presence of osteomyelitis, and white blood cell count).

Conclusions: To prevent diabetes-related amputations, healthcare professionals must prioritize preventive care, provide regular podiatry services for high-risk patients, ensure early specialist treatment for any foot ulcer, implement international guidelines at all levels of DF care, and increase the capacity of secondary wound clinics and access to revascularization.

Background: Proton pump inhibitors (PPIs) are a common and effective management strategy for eosinophilic esophagitis (EoE) leading to successful remission in most cases. However, there is uncertainty regarding the ideal follow-up pharmacological treatment to maintain remission. We performed a cost-effectiveness and quality-adjusted life year (QALY) analysis.

Objectives: To compare various approaches of pharmacologic treatments for EoE.

Methods: A Markov model was constructed to evaluate the cost-effectiveness and QALY of maintenance therapies for eosinophilic esophagitis, including PPI, topical corticosteroids (tCS), and no treatment strategy, with crossover in treatments for primary and secondary nonresponse. The primary outcomes were incremental cost-effectiveness and QALY at a 5-year time horizon.

Results: In the base-case scenario (5-year time horizon), the average costs were no treatment:₪ 73,075, PPI:₪ 81,455, and tCS:₪ 145,669 as maintenance therapy, with PPI being the dominant strategy (more effective/less costly), and a QALY of 45543, 4.5538, and 4.2283, respectively.

Conclusion: PPI was the most effective/least costly maintenance therapy. PPI is also the most cost-effective pharmacologic strategy. The relatively low adverse effects of this treatment, in addition to its effectiveness and low cost, make it an ideal choice following successful PPI induction treatment.

Background: Greater occipital nerve (GON) blocks are used in migraine management. While ultrasound-guided proximal GON blocks at the C2 level (GONC2) have been suggested to provide more sustained benefit, distal landmark-guided GON blocks are used due to their simplicity and accessibility.

Objectives: To evaluate distal GON blocks as an initial procedural approach for migraine; to assess the effect of subsequent proximal GONC2 blocks in patients who did not respond to an initial distal block.

Methods: We conducted a retrospective cohort study of adult migraine patients treated at a tertiary pain clinic between May 2022 and July 2023. Patients received either distal landmark-guided GON blocks or ultrasound-guided proximal GONC2 blocks as part of routine clinical care. Monthly migraine days (MMD) were assessed at baseline, 1 month, and 3 months following treatment.

Results: The cohort included 54 patients (27 distal, 27 GONC2). Baseline characteristics were comparable between groups. A significant overall effect of time on MMD was observed (P = 0.017). In the distal group, MMD decreased significantly at 1 month (P < 0.001) but not at 3 months. In the GONC2 group, MMD decreased significantly at both 1 month (P < 0.001) and 3 months (P = 0.001). No significant between-group differences were found at 1 month (P = 0.142) or 3 months (P = 0.509).

Conclusions: Distal GON blocks provide meaningful short-term reduction in migraine frequency and represent an effective, accessible initial interventional option. Proximal GONC2 blocks demonstrate sustained benefit and may be considered in selected patients. These findings support a pragmatic, stepwise approach to procedural migraine management.

Background: Atrial fibrillation (AF) is a common cardiac arrhythmia. Prevalence increases with age. It is a major public health concern due to high risk of thromboembolic complications such as stroke and systemic embolism. Direct oral anticoagulants (DOACs) have been shown to have improved safety and efficacy profiles and are the preferred therapy in all recent guidelines. However, patients are still discharged from internal medicine wards without appropriate DOAC therapy.

Objectives: To investigate the effectiveness of hospitalization in correcting inappropriate therapy for AF in patients who were hospitalized in the internal medicine departments for any reason and discharged without receiving DOAC therapy.

Methods: In this retrospective cohort study, we gathered data from electronic medical records at Sheba Medical Center on 222 patients. The patients were divided into three groups based on the anticoagulation therapy they received.

Results: The patient cohort (mean age of 77.1 years; 42.3% males; 21.6% presented with cognitive impairment. Researchers identified several potential contraindications for DOACs (renal disease in 24.3%, mechanical valves in 14.4%). When comparing patients with and without DOAC contraindications, there were no statistically significant differences regarding cognitive impairment rates or the lack of anticoagulation therapy. We further divided patients who did not receive DOACs into two groups: 134 due to contraindications and 88 mistakenly. Between these groups, baseline factors showed no statistically significant differences.

Conclusions: We found that older patients received less standardized care. Findings emphasize the need to consider individual patient characteristics and co-morbidities to eliminate management errors and improve the quality of clinical care.

Gallstone ileus is an uncommon complication of cholelithiasis. It accounts for less than 1–4% of all small bowel obstructions [1]. The condition involves the passage of a gallstone into the gastrointestinal tract through a biliary-enteric fistula, often resulting in obstruction at the ileum or, less commonly, the duodenum. This condition is known as Bouveret syndrome [1]. Management can be surgical (gastrotomy, intragastric laparoscopy, pyloromyotomy, duodenotomy, enterotomy) or endoscopic (removal with nets/baskets, electrohydraulic lithotripsy [EHL], extracorporeal shockwave lithotripsy [ESWL]). Endoscopic management is preferred due to its lower morbidity and mortality rates [2,3].

Rat-bite fever (RBF) is a rare zoonotic infection caused by Streptobacillus moniliformis or Spirillum minus. It is typically transmitted through contact with rats. In the United States, Streptobacillus moniliformis is the predominant causative agent, whereas in Asia, cases are more commonly attributed to Spirillum minus. We report a case of RBF in Israel, in which the identified pathogen was Streptobacillus moniliformis, a pleomorphic, Gram-negative bacillus. The classic clinical presentation of RBF includes fever, rash, and arthralgia, which may be migratory in nature.

Ovarian dermoid cysts, also known as mature cystic teratomas (MCT), are common benign germ cell tumors that account for approximately 20% of all ovarian neoplasms. They are composed of tissues derived from the three germ layers: ectoderm, mesoderm, and endoderm, and are most frequently diagnosed in women of reproductive age [1]. Diagnosis is typically based on imaging, with ultrasound often revealing characteristic features such as echogenic mural nodules, fat–fluid levels, or calcifications [1].

Human T-cell Leukemia Virus (HTLV) was first described in 1980 as a retrovirus known by its ability to infect CD4 T cells or macrophages, leading to a chronic infection that may develop into adult T-cell leukemia/lymphoma (ATLL) and chronic progressive myelopathy known as HTLV-associated myelopathy/tropical spastic paraparesis (HAM/TSP) [1]. Viral transmission occurs from unprotected sexual intercourse, vertical transmission, and contact with a contaminated blood sample. There is evidence of an association between HTLV-1 infection and rheumatic diseases [2,3].

Chondrodermatitis nodularis helicis (CNH) is a benign, painful, inflammatory condition of the external ear characterized by a solitary, hyperkeratotic, central-crusted nodule primarily affecting the helix or antihelix. We present a case of CNH clinically mimicking basal cell carcinoma, which was successfully identified and definitively cleared using Mohs micrographic surgery, resulting in immediate symptomatic relief and an optimal aesthetic outcome.