Rambam Maimonides Medical Journal最新文献

On 7 October, 2023 Israel was attacked over the Gaza border by Hamas terrorists. Mostly civilians, approximately 1,200 people were killed, with an additional 251 taken hostage (in addition to 4 abducted before October 7 for a total of 255 hostages), many of whom have since died. Of the total abducted, 13% were older than 65, a third of whom were octogenarians. Brief case histories of three abductees over 80 years of age are presented: two released and one still in captivity. The extreme "pre-morbid" vulnerability of these older hostages is described along with the additional data on their clinical situation and the extreme stresses to which they are being subjected. The situation described constitutes one of the most severe examples of elder abuse documented in the modern era.

IgG4-related disease (IgG4-RD) is a rare illness with inflammatory and fibrotic changes in affected organs such as pancreas, thyroid, salivary or lacrimal glands, and retroperitoneal space; rarely other organs may be involved. IgG4-related breast disease (IgG4-BD) is very rare and generally presents as a lump or mastitis. IgG4-BD as a presenting feature of IgG4-RD is extremely rare. Hence, this paper reviews the known (n=48) IgG-BD cases reported in the literature to date. The majority of cases were diagnosed on routine mammography or during assessment for other clinically significant features. The absence of a lump border, and especially the absence of calcifications on ultrasonography, mammography, or computed tomography, is typical for IgG4-BD. Characteristic IgG4-BD pathological findings were dense lymphoplasmacytic infiltration with stromal fibrosis, and more than 10% IgG4⁺ plasma cells/high-power field (HPF); the mean percentage of IgG4⁺/IgG⁺ plasma cells was 54.2%, and only one-third of the patients had all "classical" signs of IgG4-BD including storiform fibrosis and obliterative phlebitis. Most of the cases had a benign course and responded to surgical excision with or without steroid therapy.

The appointment of a new chancellor in 1933 marked the beginning of the Third Reich in Germany. The ideology of the Nazi Party focused on establishing a pure Aryan state characterized by nationalism and racial superiority. Their goals would be achieved through a totalitarian form of government that enforced the subjugation, exclusion, and elimination of those they defined as inferior minorities, particularly Jews, who were depicted as non-human. Implementation of the Nazi ideology required the exclusion of Jewish people and other dissenters, particularly Jewish physicians, from their professions. The exclusion of Jewish physicians, referred to herein as a "Medical Professional Elimination Program," was gradually imposed on other Jewish professions in nations absorbed by the Third Reich, and particularly enforced by incorporated Austria. Why did German and Austrian doctors support the Nazi racial ideology, the removal of Jewish physicians from every possible sphere of influence, and subsequently participate in criminal medical research and experimentation, as well as euthanasia of perceived non-contributors to society, and become involved in refining the effectiveness of the death camps? Was the Medical Professional Elimination Program an opportunistic political concept, or was it part of an entrenched ideology? With these questions in mind, the lives of four key Nazi physicians and two institutions are examined.

Pancytopenia is defined as a reduction in red blood cells, white blood cells, and platelets, and can pose as a diagnostic challenge due to the multitude of causes. Myxedema coma is the manifestation of severe untreated hypothyroidism. This case report presents a rare instance of myxedema coma-associated pancytopenia in a 53-year-old man with a history of untreated hypothyroidism. The patient presented with altered mental status and vital instability, and on further workup was found to have pancytopenia. During his hospital stay his symptomatic hypothyroidism was identified, and he was treated with intravenous levothyroxine, hydrocortisone, and supportive care. The patient's clinical status improved gradually, with normalized blood counts upon discharge. This case underscores the significance of considering myxedema coma in the differential diagnosis of pancytopenia, especially in older patients with limited healthcare access. Increased awareness of this association can aid clinicians in timely diagnosis and management, preventing potential complications associated with untreated hypothyroidism.

Background and objective: Chronic low back pain disability (CLBPD) is a syndrome that includes pain, disability, emotional impairments, and social problems. The study was conducted to examine the effect of an inpatient rehabilitation program on the performance of everyday life tasks (daily activities), and report on pain in CLBPD patients with primary activities of daily living (ADL) deficits.

Methods: In a retrospective cohort study, demographic and clinical data were retrieved from records of inpatients admitted consecutively to the program. Scores of the Spinal Pain Independence Measure (SPIM) and of changes in reported pain levels were used to assess improvement in the performance of daily activities and pain reduction. T-tests were used to assess the significance of score changes. Spearman's correlations and analysis of variance were used to assess relationships of SPIM gain and affecting factors.

Results: Ninety-nine patients were included. Daily task performance improved in 71 patients (71.7%). The SPIM score increased from 48.7 (SD 16.3) at admission to the rehabilitation program to 57.8 (SD 12.5) at discharge (P<0.001). The SPIM score at admission negatively affected SPIM gain (P<0.001). The SPIM gain was significant for admission SPIM scores of 50 or lower (P<0.05), but differences in SPIM scores were not as noticeable for patients with admission SPIM scores above 50. Relief in pain was reported in 59 patients (59.6%) and was not associated with function.

Conclusions: The multidisciplinary rehabilitation program, which improved function and provided limited pain relief in inpatients with CLBPD primary ADL deficits, can be effective for the most severe CLBPD cases.

Objective: A single layer of tightly spaced cells, known as the endothelium, rests on the posterior side of the cornea. This endothelium regulates the stroma's relative dehydration, which is essential for corneal clarity. Cell therapy is an innovative method being used to repair various corneal abnormalities. Mesenchymal stem cells (MSCs) are now one of the most significant types of stem cells scientists have studied. This study aimed to evaluate the role of MSCs for corneal endothelial regeneration.

Methods: A systematic review was performed by searching for articles from reputable databases with many study-type references, including PubMed, Cochrane Library, Science Direct, and Google Scholar, up to January 2024. The resulting data were displayed using the 2020 PRISMA flowchart and evaluated using the PRISMA 2020 checklist. Most of the included studies were in vivo and used topical application and anterior chamber injection as the administration routes.

Results: Based on the findings of this review, MSCs increased corneal endothelial cell density, improved the defect area and corneal transparency, facilitated endothelial cell regeneration and wound healing, and decreased neovascularization and corneal pro-inflammatory cytokines as compared to controls.

Conclusion: Administration of MSCs into the anterior chamber could increase regeneration and proliferation of corneal endothelial tissue.

Background: Resection of oral cavity carcinoma often leads to complex defects causing functional and aesthetic morbidity. Providing optimum reconstruction with free flaps becomes challenging in a high-volume center setting with constrained resources. Hence, understanding the local flap technique for reconstructing oral cancer defects is prudent.

Materials and methods: This study is a retrospective analysis of prospectively operated cases of oral cavity resections which were subsequently reconstructed using local flaps from 2019 to 2022. Patients who underwent reconstruction with either melolabial flap, islanded facial artery myomucosal (FAMM) flap, submental flap, supraclavicular artery island flap, infrahyoid flap, or platysma myocutaneous flap (PMF) were included in this analysis. Eligible patients were followed up to evaluate functional outcomes like oral feeding and to analyze the Performance Status Scale for Head and Neck Cancer.

Results: The study included 104 patients. The tongue was the most common subsite, resulting in most hemiglossectomy defects, which were reconstructed using the melolabial flap procedure. Buccal mucosa defects in our series were reconstructed using the supraclavicular flap, whereas the submental flap procedure was the choice for lower lip-commissure defects. Complications such as partial and total flap loss, deep neck infection, and donor site complications like infection and gaping, oral cutaneous fistula, parotid fistula, and seroma were analyzed; the supraclavicular flap presented with a majority of complications.

Conclusion: Local flaps are an alternative to free flap reconstruction in select cases with optimum functional outcomes and minimal donor site morbidity. This article comprehensively reviews the surgical steps for various local flap procedures in oral cancer defects.

Objective: Medical decision-making is often uncertain. The positive predictive value (PPV) and negative predictive value (NPV) are conditional probabilities characterizing diagnostic tests and assessing diagnostic interventions in clinical medicine and epidemiology. The PPV is the probability that a patient has a specified disease, given a positive test result for that disease. The NPV is the probability that a patient does not have the disease, given a negative test result for that disease. Both values depend on disease incidence or prevalence, which may be highly uncertain for unfamiliar diseases, epidemics, etc. Probability distributions for this uncertainty are usually unavailable. We develop a non-probabilistic method for interpreting PPV and NPV with uncertain prevalence.

Methods: Uncertainty in PPV and NPV is managed with the non-probabilistic concept of robustness in info-gap theory. Robustness of PPV or NPV estimates is the greatest uncertainty (in prevalence) at which the estimate's error is acceptable.

Results: Four properties are demonstrated. Zeroing: best estimates of PPV or NPV have no robustness to uncertain prevalence; best estimates are unreliable for interpreting diagnostic tests. Trade-off: robustness increases as error increases; this trade-off identifies robustly reliable error in PPV or NPV. Preference reversal: sometimes sub-optimal PPV or NPV estimates are more robust to uncertain incidence or prevalence than optimal estimates, motivating reversal of preference from the putative optimum to the sub-optimal estimate. Trade-off between specificity and robustness to uncertainty: the robustness increases as test-specificity decreases. These four properties underlie the interpretation of PPV and NPV.

Conclusions: The PPV and NPV assess diagnostic tests, but are sensitive to lack of knowledge that generates non-probabilistic uncertain prevalence and must be supplemented with robustness analysis. When uncertainties abound, as with unfamiliar diseases, assessing robustness is critical to avoiding erroneous decisions.

Bite mark analysis plays a pivotal role in forensic investigations, by helping to identify suspects and establish links between individuals and crime scenes. However, traditional bite mark methodologies face significant challenges due to issues with reliability and subjectivity. Recent advances in microbiome analysis, which involves identifying and characterizing the microbial communities found in bite marks, have led to the emergence of a promising tool for forensic investigations. The integration of microbiome analysis with conventional DNA profiling enables more accurate interpretation of bite mark evidence in forensic investigations. This review provides an in-depth look at the integration of bite mark microbiome analysis with forensic DNA profiling. It also addresses the challenges and strategies involved in microbiome-based bite mark analysis for forensic purposes.

Alternating hemiplegia of childhood (AHC) is a complex neurodevelopmental disorder characterized by paroxysmal and transient events of unilateral or bilateral paresis, usually occurring before 18 months of age. Mutations in the ATP1A3 gene, mainly p.Asp801Asn, p.Glu815Lys, and p.Gly947Arg at the protein level, are found in around 80% of the individuals with AHC. Interestingly, these mutations reflect the degree of severity of the neurological symptoms (p.Glu815Lys > p.Asp801Asn > p.Gly947Arg). Some channels involved in this disorder are N-type voltage-gated calcium channels, ATP-sensitive potassium channels, and the sodium/calcium exchanger. In this context, the management of AHC should be divided into the treatment of attacks, prophylactic treatment, and management of comorbidities commonly found in this group of individuals, including epilepsy, attention-deficit/hyperactivity disorder, aggressive behavior, cognitive impairment, movement disorders, and migraine. The importance of an integrated approach with a multidisciplinary team, such as neuropsychologists and dietitians, is worth mentioning, as well as the follow-up with a neurologist. In the present study, we propose new diagnostic criteria for AHC, dividing it into clinical, laboratory, supporting, and atypical features. Also, we review the location of the mutations in the ATP1A3 protein of individuals with AHC, rapid-onset dystonia-parkinsonism (RDP) variants, and early infantile epileptic encephalopathy (variants with hemiplegic attack). We also include a section about the animal models for ATP1A3 disorders.