Israel Medical Association Journal

Nail-patella syndrome (NPS, OMIM: #161200), also known as Fong disease, hereditary osteo-onychodysplasia (HOOD), and Turner-Kieser syndrome, is a rare pleiotropic, multisystemic condition with an estimated incidence of 1 per 50,000. It is characterized mainly by developmental defects of dorsal limb structures due to symmetrical mesodermal and ectodermal abnormalities. It manifests as a classic clinical tetrad of distal digital abnormalities and fingernail dysplasia, which are typically bilateral and symmetrical, hypoplasia or absence of the patella, presence of iliac horns, and elbow deformities. It can also affect other structures (e.g., tendons, ligaments, and muscles), and may impact ophthalmic (glaucoma, increased ocular pressure and subsequent blindness), renal (nephropathy), neurological, orthopedic, and gastrointestinal systems. NPS can lead to sensorineural hearing loss and vasomotor problems [1,2]. Clinical manifestations vary greatly in frequency and severity. The prognosis is relatively good when clinical features are mild and cause no disability. However, serious and even life-threatening complications can occur. NPS is usually clinically diagnosed based on physical examination and radiological imaging. Genetic testing and renal biopsy can also assist in diagnosis confirmation.

Background: Immunoglobulin 4 (IgG4) is the least abundant immunoglobulin in the sera of healthy individuals; however, its levels can vary in different diseases such as IgG4-related disease (high) or Sjögren's syndrome (low). While previous studies have suggested the importance of IgG4 in autoimmune diseases, the clinical and biological significance of high or low levels remains unclear.

Objectives: To investigate the association between IgG4 antibody levels and systemic sclerosis (SSc), as well as the clinical features of the disease.

Methods: We measured IgG4 levels in the sera of 74 SSc patients from the years 2000 to 2019 and compared them to IgG4 levels in 80 healthy donors from the Israeli national blood bank. We performed correlation analyses between IgG4 levels and various factors, including age, sex, disease subtype, disease duration, organs involved, and medications taken by the patients.

Results: Our findings revealed significantly lower IgG4 levels in SSc patients compared to healthy participants. SSc patients receiving steroid treatment exhibited prominently lower IgG4 levels. In addition, SSc patients with Raynaud's phenomenon tended to have lower IgG4 levels compared to those without Raynaud's phenomenon.

Conclusions: Our study demonstrates that IgG4 levels are lower in SSc patients. Further research is needed to elucidate whether this observation contributes to the etiology of the disease or if it represents a common manifestation among other autoimmune diseases.

Among the advancements in echocardiography, the introduction of two-dimensional strain marked a pivotal moment in the quantitative evaluation of cardiac function. In fact, Global longitudinal strain (GLS) has emerged as a key focus due to its robust validation and evidence base. GLS provides a comprehensive assessment of left ventricular function, which offers greater sensitivity in detecting subtle changes compared to traditional metrics such as ejection fraction. In this position paper, we elucidate the rationale behind routine global longitudinal strain calculation, offering practical recommendations and insights for its implementation in clinical echocardiography. By bridging technical nuances with clinical relevance, the calculation of longitudinal strain aims to optimize patient care and enhance the diagnostic precision of echocardiographic examinations.

Background: Prognostication is complex in patients critically ill with coronavirus disease 2019 (COVID-19).

Objectives: To describe the natural history of ventilated critical COVID-19 patients.

Methods: Due to our legal milieu, active withdrawal of care is not permitted, providing an opportunity to examine the natural history of ventilated critical COVID-19 patients. This retrospective cohort included COVID-19 ICU patients who required mechanical ventilation. Respiratory and laboratory parameters were followed from initiation of mechanical ventilation for 14 days or until extubation, death or tracheostomy.

Results: A total of 112 patients were included in the analysis. Surviving patients were younger than non-survivors (62 years [range 54–69] vs. 66 years [range 62–71], P = 0.01). Survivors had a shorter time to intubation, shorter ventilation duration, and longer hospital stay. Respiratory parameters at intubation were not predictive of mortality. Nevertheless, on ventilation day 10, many of the ventilatory parameters were significantly better in survivors. Regarding laboratory parameters, neutrophil counts were significantly higher in non-survivors on day 1 and C-reactive protein levels were significantly lower in survivors on day 10. Modeling using a generalized estimating equation showed small dynamic differences in ventilatory parameters predictive of survival.

Conclusions: In ventilated COVID-19 patients when there is no active care withdrawal, prognostication may be possible after a week; however, differences between survivors and non-survivors remain small.

Background: One of the major causes of reversible visual impairment is a refractive error, which can be corrected through refractive surgery. Data regarding the outcomes and complications of these procedures exist; however, there is a notable gap in understanding the factors leading to patient rejection, particularly in diverse populations like Israel.

Objectives: To detect clinical risk factors of patients who intend to undergo LASIK procedure and to improve the conversion rates of LASIK procedure in this population.

Methods: The study included a retrospective analysis of patients who were rejected from having refractive surgery in two Enaim Refractive Surgery Centers: Jerusalem and Tel Aviv. Comparisons between centers were conducted using the t-test for continuous variables and Fisher's exact test for categorical data.

Results: Our study included 337 patients who were rejected from having refractive surgery, including 152 (45.1%) who were rejected permanently and 185 (54.9%) rejected temporarily. The most common reasons for permanent rejection were corneal irregularity (n=81, 53.3%), keratoconus (n=27, 17.8%), thin corneas (n=13, 8.6%), and amblyopia (n=10, 6.6%). The most common temporary reasons were unstable refraction (n=96, 51.9%), prolonged use of contact lenses (n=54, 29.2%), and corneal irregularity (n=16, 8.6%).

Conclusions: The leading permanent cause of rejection for refractive surgery was a corneal irregularity, whereas the primary temporary cause was unstable refraction. In a time of rapid technological advancements and growing demand for freedom from glasses, there is an increasing need for more informed and patient-focused refractive correction approaches.

Background: Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a rare autoimmune demyelinating disorder of the central nervous system. Optic neuritis (ON) is the most common clinical manifestation of MOGAD in adults. In 2023, new MOGAD diagnostic criteria were proposed, highlighting the importance of supplemental criteria when MOG-immunoglobulin G (IgG) titers are unavailable.

Objective: To investigate the applicability of the 2023 MOGAD criteria in patients diagnosed with MOGAD and treated before the availability of MOG-IgG titers.

Methods: We conducted a retrospective chart review of patients classified as MOGAD between 2010 and 2023 at Rabin Medical Center. Patient demographics as well as clinical and imaging data were collected, including visual acuity, expanded disability status score, core demyelinating events, antibody status, and brain and optic nerve magnetic resonance imaging data. Patients fulfilling the 2023 MOGAD criteria were reported as definite MOGAD.

Results: Fifteen patients met the 2023 MOGAD diagnostic criteria despite lack of MOG-IgG titer. The most common supplemental criterion meeting the 2023 MOGAD criteria was optic disc edema (n=12, 80%), followed by longitudinal optic nerve involvement (53%), bilateral ON (40%), and perineural optic sheath enhancement (33%).

Conclusions: All patients with a clinical diagnosis of MOG-ON in our cohort fulfilled the 2023 MOGAD criteria despite the lack of antibody titers. The 2023 MOGAD criteria can be reliably applied to Israeli cohorts, prior to availability of MOGAD IgG titers, with particular attention to additional supplemental criteria. Since the 2023 MOGAD criteria were published, MOGAD IgG titers have been added to routine testing at our facility.

The issue of genital ulcers (GUs) in Israeli Arab female patients with Behçet's disease (BD is important as GUs are considered a unique feature of BD and stand as diagnostic criterion for the diagnosis in the International Study Group (ISG) criteria [1] and the International Criteria for Behcet Disease (ICBD) [2]. According to the ISG, GUs can be detected by a physician or by the patient [1]. While oral ulcers are easily observable, the identification of more hidden GUs pose a significant challenge.

Background: Hidradenitis suppurativa (HS) is a chronic inflammatory skin disease involving apocrine gland-bearing regions. There is an under-representation of non-Caucasians in epidemiologic studies of HS. The characteristics of HS in Israeli Arabs have not yet been studied.

Objectives: To investigate the demographic and clinical profile of HS in the Israeli Arab population.

Methods: A retrospective analysis was conducted in two cohorts of patients with HS in Israel. The patients were derived from the database of a large health management organization (n=4191, 639 Arabs; population-based) and a major tertiary medical center (n=372, 49 Arabs). Demographic and clinical data were compared between ethnic groups.

Results: The prevalence of HS in Israeli Arabs was found to be 0.5%, fivefold higher than in Jews. Arab patients were younger (35.3 vs. 40.5 years, P < 0.001) and mostly male (52% vs. 35.7%, p < 0.001), with lower rates of co-morbidities, including smoking (40.8% vs. 55.7%, p < 0.001), hyperlipidemia, and depression as well as a higher rate of dissecting cellulitis (10.2% vs. 1.9%, p = 0.008). HS was more severe in Arabs, but of shorter duration, with mainly axillary involvement (79.6% vs. 57.9%, p = 0.004). Treatment with hormones was more common in Jews, and with biologic agents in Arabs.

Conclusions: The findings suggest a different phenotype of HS in Arabs, warranting further study.

Since the introduction of the international study group (ISG) criteria for the diagnosis of Behçet's disease (BD) in the early 1990s by Yazici and colleagues [1] and the international criteria for BD (ICBD) by Davatchi and colleagues in 2014 [2], great progress has been achieved in the diagnosis of BD with fairly high sensitivity and specificity rates. However, a small, but very challenging and unique minority might not fulfill these criteria, at least at presentation. These patients are most challenging as they may present with life-threatening vascular or neurological manifestations. If the diagnosis BD is delayed, the risk for morbidity and even mortality might be increased. Therefore, we should aim for early diagnosis and prompt treatment.

Background: According to Hamas sources, many Israeli hostages in Gaza were killed by indiscriminate Israeli airstrikes, together with a large number of Palestinian citizens.

Objectives: To verify whether the estimated death rate of Israeli hostages was similar to the estimated death rate of Gaza citizens from these acts of war.

Methods: We used two estimates of hostage death rates, one obtained from Israeli intelligence sources, and one published by a Hamas spokesperson. We used the Palestinian casualty rates published by the Palestinian Ministry of Health. We compared death rates using Fisher's exact test.

Results: By 30 December 2023, the rate of Israeli hostage death was 23/238 (9.7%) according to Israeli intelligence sources, and 60/238 (25.2%) according to Hamas. Both figures are strikingly and significantly higher than the death rate among Palestinians, estimated to be 19,667/2.2 million (0.89%) by 19 December 2023 (P < 0.0001).

Conclusions: Israeli airstrikes as the cause of death of Israeli hostages are implausible unless they were specifically exposed to these strikes more than Palestinian citizens.

Background: Survivors of critical illness are at increased risk of long-term impairments, referred to as post-intensive care unit (ICU) syndrome (PICS). Post-traumatic stress disorder (PTSD) is common among ICU survivors with reported rates of up to 27%. The prevalence of PTSD among Israeli ICU survivors has not been reported to date.

Objectives: To evaluate the prevalence of new onset PTSD diagnosed in a post-ICU clinic at a tertiary center in Israel.

Methods: We conducted a retrospective, single center, cohort study. Data were collected from medical records of all patients who visited the Tel Aviv Sourasky Medical Center post-ICU clinic between October 2017 and June 2020. New onset PTSD was defined as PTSD diagnosed by a certified board psychiatrist during the post-ICU clinic visit. Data were analyzed using descriptive statistics.

Results: Overall, 39 patients (mean age 51 ± 17 years, 15/39 females [38%]) attended the post-ICU clinic during the study period. They were evaluated 82 ± 57 days after hospital discharge. After excluding 7 patients due to missing proper psychiatric analysis, 32 patients remained eligible for the primary analysis. New PTSD was diagnosed in one patient (3%).

Conclusions: We found lower incidence of PTSD in our cohort when compared to existing literature. Possible explanations include different diagnostic tools and low risk factors rate. Unique national, cultural, and/or religious perspectives might have contributed to the observed low PTSD rate. Further research in larger study populations is required to establish the prevalence of PTSD among Israeli ICU survivors.

Background: Completing internal medicine specialty training in Israel involves passing the Israel National Internal Medicine Exam (Shlav Aleph), a challenging multiple-choice test. multiple-choice test. Chat generative pre-trained transformer (ChatGPT) 3.5, a language model, is increasingly used for exam preparation.

Objectives: To assess the ability of ChatGPT 3.5 to pass the Israel National Internal Medicine Exam in Hebrew.

Methods: Using the 2023 Shlav Aleph exam questions, ChatGPT received prompts in Hebrew. Textual questions were analyzed after the appeal, comparing its answers to the official key.

Results: ChatGPT 3.5 correctly answered 36.6% of the 133 analyzed questions, with consistent performance across topics, except for challenges in nephrology and biostatistics.

Conclusions: While ChatGPT 3.5 has excelled in English medical exams, its performance in the Hebrew Shlav Aleph was suboptimal. Factors include limited training data in Hebrew, translation complexities, and unique language structures. Further investigation is essential for its effective adaptation to Hebrew medical exam preparation.

Background: The Gaza–Israeli conflict poses challenges for unbiased reporting due to its complexity and media bias. We explored recent scientific publications to understand scholarly discourse and potential biases surrounding this longstanding geopolitical issue.

Objectives: To conduct a descriptive bibliometric analysis of PubMed articles regarding the recent Gaza–Israeli conflict.

Methods: We reviewed 1628 publications using keywords and medical subject headings (MeSH) terms related to Gaza, Hamas, and Israel. We focused on articles written in English. A team of researchers assessed inclusion criteria, resolving disagreements through a third researcher.

Results: Among 37 publications, Lancet, BMJ, and Nature were prominent journals. Authors from 12 countries contributed, with variety of publication types (46% correspondence, 32% news). Pro-Gaza perspectives dominated (43.2%), surpassing pro-Israel (21.6%) and neutral (35.1%) viewpoints. Pro-Gaza articles exhibited higher Altmetric scores, indicating increased social media impact. Pro-Israel publications were predominantly authored by Israelis.

Conclusions: The prevalence of pro-Gaza perspectives underscores challenges in maintaining impartiality. Higher social media impact for pro-Gaza publications emphasizes the need for nuanced examination. Addressing bias is crucial for a comprehensive understanding of this complex conflict and promoting balanced reporting.

Background: Helicobacter pylori (H. pylori) prevalence varies according to both geographical region and ethnicity. The interplay between these two factors has been poorly studied.

Objectives: To determine the positivity rate of H. pylori infection among Jewish and Arab patients who live in a mixed urban center in Israel.

Methods: Between November 2009 and September 2014, dyspeptic patients referred to a gastroenterology clinic in Lod, Israel, were enrolled in a prospective study. For each patient, clinical and epidemiological data were collected and a noninvasive or endoscopy-based test for H. pylori was performed.

Results: A total of 429 consecutive patients (322 Jewish and 107 Arabs), mean age 45 years (range 15–91 years) were included; 130 males. Overall positivity for H. pylori was 42.4% (182/429). The positivity rate of H. pylori was 38.8% for Jews (125/322) and 53.2% for Arabs (57/107) in Lod (P < 0.01). When immigrants were excluded, the difference in H. pylori positivity did not reach statistical significance (45.0% [77/171] vs. 53.2% [57/107], P = 0.217, in Jews and Arabs, respectively).

Conclusions: H. pylori infection was more common in Arabs that Jews in the mixed city of Lod, Israel. This finding may suggest that non-environmental factors were responsible for the observed difference in H. pylori positivity.

Background: Websites serve as a source of medical information for a large part of the public, some claim to be a substitute for a physician’s consultation. Many patients meet a physician after conducting internet research. Medical staff are concerned that internet sources of information are unreliable and may lead to erroneous decisions by patients.

Objectives: To examine the reliability of web-based sources of information (through the Google™ search engine) regarding five common orthopedic complaints and injuries.

Method: We performed a search of five common orthopedic complaints and injuries using the Google search engine. The reliability of web-based information was measured by the DISCERN tool, which is a valid and verified tool for examining the reliability of medical information sources to the public. The reliability of 47 websites was examined by two orthopedic surgeons and two senior residents.

Results: The overall average score given to the sites was 2.8, on a scale of 1 to 5. We found that the higher the site appeared in the search results, the higher the quality of its information. Commercial sites scored higher than general internet information sources.

Conclusions: The internet network is a very broad source of information. For those who lack scientific education and training it is not easy to distinguish between reliable and unreliable or biased sources. The trend of searching for medical information and self-healing is increasing. We must strengthen the network with reliable sources by creating official scientific position papers by medical teams and promoting them online.