Phosphoglucomutase 1 (PGM1) deficiency is a rare genetic disorder that affects glycogen metabolism and manifests as a multisystem disease. Most patients present with oropharyngeal malformations, cardiomyopathies, elevated liver enzymes, and hypoglycemia. Treatment with D-galactose has been shown to improve symptoms. Our patient presented with PGM1 deficiency. She conceived spontaneously. Throughout her pregnancies, our patient was monitored by a multidisciplinary team of cardiologists, endocrinologists, and physicians with experience in high-risk pregnancy. She delivered twice by cesarean section. Our case highlights the importance of a multidisciplinary approach and individualized management of prenatal and postpartum care of a patient with a PGM1 deficiency. To the best of our knowledge, there have been no reports about a PGM1-deficient patient who conceived and delivered.

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].

Intranasal corticosteroids (INCS) are frequently prescribed for allergic rhinitis but can also be used for other indications, such as sinusitis and nasal congestion. INCS are considered effective in controlling nasal symptoms with a similar safety profile among the different INCS formulations. In this review, we presented all available INCS formulations marketed in Israel while emphasizing the differences among them with a practical approach for medical providers in selecting a specific INCS agent. We conducted a literature review using PubMed, Medline, and Google Scholar to identify articles related to INCS, triamcinolone acetonide, fluticasone propionate, and fluticasone furoate. Currently, five brands of INCS are available in Israel. While they all have similar efficacy in treating nasal symptoms, only fluticasone furoate consistently demonstrated a reduction in ocular symptoms compared to placebo. Other differences included sensory attributes, recommended regimens, approved age for use, and cost. When selecting INCS agent, a personalized approach is advised. Factors such as age, co-morbidities, concurrent medications, pregnancy, and patient preferences should be considered.

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.

Similar to ageism, which is defined as discrimination against older people due to negative and inaccurate stereotypes [1], geriatrism, a new concept in medical field, reflects a negative attitude and bias toward the study of geriatrics, especially observed among students specializing in therapeutic fields such as nursing, physiotherapy, occupational therapy, speech therapy, psychology, and medicine. However, it implies a reluctance of all professionals in the therapeutic fields, including young medical doctors, to specialize in treating individuals aged 65 years and older.

The concept of starvation osteopathy is an old and an investigated one, which is well established in many ways. Studies were conducted on famine survivors during World War I, in the Ukraine in the early 1930s, throughout Europe during World War II, and in Asia and Africa soon after. However, the main topic of this article is the effect of starvation inflicted during the Holocaust.

Background: On 7 April 1933, the Nazi Law for the Restoration of the Professional Civil Service was enacted. The law triggered the dismissal of most Jewish medical staff from German universities. A few Jewish professors in Berlin were permitted to continue their academic activity with restrictions. Those professors were gradually dismissed as laws and restrictions were enforced.

Objectives: To identify the last Jewish medical professors who, despite severe restrictions, continued their academic duties and prepared students for their examinations in Berlin after the summer of 1933.

Methods: We reviewed dissertations written by the medical faculty of Berlin from 1933 to 1937 and identified Jewish professors who mentored students during those years.

Results: Thirteen Jewish tutors instructed dissertations for the medical examinations after the Nazi regime seized power. They were employees of different university hospitals, including the Jewish hospitals. We did not identify Aryan students instructed by Jewish professors. The professors were active in different medical disciplines. Half of the reviewed dissertations were in the disciplines of surgery and gynecology. The last Jewish tutors were dismissed in October 1935. However, some of their studies were submitted for examination after that date.

Conclusions: After the Nazi regime seized power, academic activities and medical research by Jewish professors declined but did not stop. However, these professors worked with only Jewish students on their theses. Most dissertations were approved and examined after the Jewish academics were dismissed by the university, in some cases even after they left Germany.

Background: Pediatric urolithiasis is relatively uncommon and is generally associated with predisposing anatomic or metabolic abnormalities. In the adult population, emergency department (ED) admissions have been associated with an increase in ambient temperature. The same association has not been evaluated in the pediatric population.

Objectives: To analyze trends in ED admissions due to renal colic in a pediatric population (≤ 18 years old) and to assess the possible effect of climate on ED admissions.

Methods: We conducted a retrospective, multicenter cohort study, based on a computerized database of all ED visits due to renal colic in pediatric patients. The study cohort presented with urolithiasis on imaging during their ED admission. Exact climate data was acquired through the Israeli Meteorological Service (IMS).

Results: Between January 2010 and December 2020, 609 patients, ≤ 18 years, were admitted to EDs in five medical centers with renal colic: 318 males (52%), 291 females (48%). The median age was 17 years (IQR 9–16). ED visits oscillated through the years, peaking in 2012 and 2018. A 6% downward trend in ED admissions was noted between 2010 and 2020. The number of ED admissions in the different seasons was 179 in autumn (30%), 134 in winter (22%), 152 in spring (25%), and 144 in summer (23%) (P = 0.8). Logistic regression multivariable analysis associated with ED visits did not find any correlation between climate parameters and ED admissions due to renal colic in the pediatric population.

Conclusions: ED admissions oscillated during the period investigated and had a downward trend. Unlike in the adult population, rates of renal colic ED admissions in the pediatric population were not affected by seasonal changes or rise in maximum ambient temperature.

Background: Ankylosing spondylitis (AS) and inflammatory bowel disease (IBD) are chronic conditions with overlapping pathogenic mechanisms. The genetic predisposition and inflammatory pathways common to both diseases suggest a syndemic relationship. While some evidence points to a connection between the two conditions, other reports do not support this link.

Objectives: To investigate the association between AS and the subsequent incidence of IBD. To identify potential risk factors and effect modifiers that contribute to this relationship.

Methods: Utilizing the Chronic Disease Registry of Clalit Health Services, we conducted a retrospective cohort study of individuals diagnosed with AS between January 2002 and December 2018. We compared these patients with age- and sex-matched controls, excluding those with a prior diagnosis of IBD. Statistical analyses included chi-square and t-tests for demographic comparisons, and Cox proportional hazards models for evaluating the risk of IBD development, with adjustments for various co-morbidities and demographic factors.

Results: The study included 5825 AS patients and 28,356 controls. AS patients demonstrated a significantly higher incidence of IBD with hazard ratios of 6.09 for Crohn's disease and 2.31 for ulcerative colitis, after multivariate adjustment. The overall incidence of IBD in the AS cohort was significantly higher compared to controls.

Conclusions: AS patients exhibit a markedly increased risk of developing IBD. These findings advocate for heightened clinical vigilance for IBD symptoms in AS patients and suggest the need for a multidisciplinary approach to patient care. Further research into the shared pathogenic pathways is needed to develop personalized treatment strategies and improve patient management.

Background: The prevalence of Group A streptococcus (GAS) carriage among adults is studied less than in children. The variability of reported carriage rates is considerably large and differs among diverse geographic areas and populations.

Objectives: To evaluate the prevalence of GAS carriage among adults in Israel.

Methods: In this prospective study, conducted in a large healthcare maintenance organization in Israel, we obtained pharyngeal cultures from adults attending the clinic without upper respiratory tract complaints or fever. Patient data included sex, age, number of children, and religious sectors.

Results: From May to December 2022, eight family physicians collected a total of 172 throat swabs (86% response rate). The median age was 37 years (range 18–65); 72.7% were females, 22.7% were ultra-Orthodox Jewish, and 69.2% had children. The prevalence of GAS carriage was 6.98%, 95% confidence interval (95%CI) 3.7%–11.9%. GAS carriers were younger (31.7 vs. 39.3 years, P = 0.046), and the majority were ultra-Orthodox Jews (58.3% vs. 20%, P = 0.006). All GAS carriers were from lower socioeconomic status. When assessing risk factors for GAS carriage using multivariate analysis, only being an ultra-Orthodox Jew was positively related to GAS carriage (adjusted odds ratio 5.6, 95%CI 1.67–18.8).

Conclusion: Being an ultra-Orthodox Jew was the single variable associated with a GAS carriage, which may be related to having many children at home and living in overcrowded areas. Primary care physicians in Israel should recognize this situation when examining patients with sore throats, mainly ultra-Orthodox Jews.

Background: The cavum septi pellucidi (CSP) is a brain-enclosed cavity located on the midline between the two leaflets of the septum pellucidum that separates the lateral ventricles. This structure develops in the fetus from week 18 and can be seen up to week 37 in almost all cases and then begins to disappear.

Objectives: To measure and determine the normative values of the CSP volume in fetuses between 20 to 40 weeks of gestation.

Methods: The study comprised 161 consecutive pregnant women between 20 to 40 weeks of gestation with single viable fetuses. All patients had normal, disease-free pregnancies. Transvaginal or transabdominal ultrasound was used according to the fetal presentation. The fetal head was assessed in mid-sagittal sections. Once the CSP was visualized, its volume was measured using three-dimensional ultrasound with Virtual Organ Computer-aided Analysis software. The width of the CSP was also measured at the biparietal diameter (BPD) plane.

Results: Of the 161 fetuses, the CSP volume was measured in 158. In three patients the CSP was not identified. The CSP volume correlated poorly with gestational age (r=0.229) and with the BPD (r=0.295). The mean CSP volume was 0.508 ± 0.372 ml (range: 0.03-1.78 ml). The simple measurement of the CSP width correlated better with gestational age (r=0.535) and the BPD (r=0.484).

Conclusions: The CSP volume had a poor correlation with gestational age; however, the volume did not exceed 2 ml regardless of gestational age. This information can be used to assess pathologies involving the CSP.

 

Background: Syncope is responsible for approximately 1–3% of all emergency department (ED) visits and up to 6% of all hospital admissions in the United States. Although often of no long-term consequence, syncope can be the first presentation of a range of serious conditions such as strokes, tumors, or subarachnoid hemorrhages. Head computed tomography (CT) scanning is therefore commonly ordered in the ED for patients presenting with syncope to rule out any of these conditions, which may present without other associated physical or neurological findings on initial examination. However, the diagnostic yield of head CTs in patients presenting with syncope is unclear.

Objectives: To determine the diagnostic yield of head CT in the ED in patients with syncope.

Methods: We conducted an observational analytical retrospective cross-sectional study on 360 patients diagnosed with syncope who underwent a head CT to determine the diagnostic yield of syncope to determine whether head CT is necessary for every patient presenting with syncope to the ED.

Results: The total of new CT findings was 11.4%. Percentages varied between men (12.8%) and women (9.7%), P = 0.353. There were no significant differences between sexes regarding the findings in head CT, yet the incidence increased, especially among elderly males.

Conclusions: Age had a more significant impact on diagnostic yield of syncope than head CT. The use of a head CT scan as a routine diagnosis tool in patients with syncope is unjustifiable unless there is an indication based on medical history or physical examination.

Hiatal hernia is defined as a protrusion of abdominal contents through the hiatal foramen into the thoracic cavity. Etiology is presumed to be a congenital malformation, trauma, or iatrogenic like prior surgical dissection of the hiatus during surgery for esophageal or gastric etiology. Age, sex, hormonal changes, body habitus (i.e., kyphosis, scoliosis), and increased body weight are key risk factors. Most hiatal hernias are asymptomatic and discovered incidentally. Surgical repair of hiatal hernia is indicated in symptomatic patients with dysphagia, weight loss, respiratory symptoms such as aspirations, and recurrent pneumonia events [1]. Complications arising from laparoscopic repair of hiatal hernia are generally minor and do not typically necessitate surgical intervention. Major complications include pneumothorax, splenic laceration, esophageal rupture, and pericardial injury. Other complications include recurrence of hernia, vagal nerve injury, gastroesophageal reflux disease, and gastroparesis. The utilization of mesh in repair procedures introduces additional complications such as mesh migration and mesh infection. Previously reported recurrence rates following the repair of a hiatal hernia with mesh range from 10–30%. In this case communications, we presented a case involving the early recognition and treatment of postoperative cardiac tamponade.

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: Ischemic stroke is associated with increased risk of morbidity and mortality in future vascular events.

Objectives: To investigate whether CHA₂DS₂-VASc scores aid in risk stratification of middle-aged patients without atrial fibrillation (AF) experiencing ischemic stroke.

Methods: We analyzed data of 2628 patients, aged 40–65 years with no known AF who presented with acute ischemic stroke between January 2020 and February 2022. We explored the association between CHA₂DS₂-VASc scores categorized by subgroups (score 2–3, 4–5, or 6–7) with major adverse cardiac and cerebrovascular events (MACCE) including recurrent stroke, myocardial infarction, coronary revascularization, or all-cause death during a median follow-up of 19.9 months.

Results: Mean age was 57 years (30% women); half were defined as low socioeconomic status. Co-morbidities included hypertension, diabetes, obesity, and smoking in 40–60% of the patients. The incidence rate of MACCE per 100 person-years was 6.7, 12.2, and 21.2 in those with score 2–3, 4–5, and 6–7, respectively. In a multivariate cox regression model, compared to patients with score 2–3 (reference group), those with score 4–5 and 6–7 had an adjusted hazard ratio (95% confidence interval [95%CI]) for MACCE of 1.74 (95%CI 1.41–2.14) and 2.87 (95%CI 2.10–3.93), respectively. The discriminative capacity of CHA₂DS₂-VASc score for overall MACCE was modest (area under curve 0.63; 95%CI 0.60–0.66), although better for myocardial infarction 0.69 (95% CI 0.61–0.77).

Conclusions: CHA₂DS₂-VASc score may predict future MACCE in middle-aged patients with ischemic stroke and no history of AF.