Long-term outcomes after pre-emptive liver transplantation in primary hyperoxaluria type 1.

BACKGROUND: Primary hyperoxaluria type 1 (PH1) is an autosomal recessive disease caused by the liver defect of oxalate metabolism, which leads to kidney failure and systemic manifestations. Until recently, liver transplantation was the only definitive treatment. The timing of liver transplantation can be early, while kidney function is still normal (pre-emptive liver transplantation-PLT), or when the patient reaches stage 5 chronic kidney disease (CKD) and needs combined liver-kidney transplantation. We aimed to determine the long-term kidney outcomes of PLT in PH1 patients. METHODS: A retrospective single-center study of PH1 patients who were followed in our center between 1997 and 2017. We compared the kidney outcomes of patients who underwent PLT to those who presented with preserved kidney function and did not undergo PLT. RESULTS: Out of 36 PH1 patients, 18 patients were eligible for PLT (eGFR > 40 mL/min/1.73 m2 at the time of diagnosis). Seven patients underwent PLT (PLT group), while 11 continued conservative treatments (PLTn group). In the PLT group, the median eGFR at the time of PLT and at the end of the follow-up period (14-20 years) was 72 (range 50-89) and 104 (range 86-108) mL/min/1.73 m2, respectively, and no patient died or reached stage 5 CKD. In the PLTn group, eight patients (72.7%) reached stage 5 CKD (median time to kidney replacement therapy was 11 years), and two patients died from disease complications (18.2%). CONCLUSIONS: Pre-emptive liver transplantation preserved kidney function in patients with PH1 in our cohort. Early intervention can prevent kidney failure and systemic oxalosis in PH1. A higher resolution version of the Graphical abstract is available as Supplementary information.

Maternal and Cord Anti-SARS-CoV-2-Spike IgG following COVID-19 Vaccination versus Infection during Pregnancy: A Prospective Study, Israel October 2021-March 2022.

OBJECTIVE: Defining how pregnant women respond to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection and vaccination is critical to optimize vaccination strategies that protect mother and infant at the epidemic. This study aimed to compare anti-SARS-CoV-2-spike immunoglobulin G (IgG) of vaccinated versus infected women and to determine the optimal timing of maternal vaccination during pregnancy at the time of epidemic. STUDY DESIGN: We collected maternal/cord blood at delivery (October 2021-March 2022) and measured anti-SARS-CoV-2-spike IgG geometric mean concentrations (IgG-GMCs) using a quantitative immunoassay. We compared groups according to timing and number of doses and correlated maternal and fetal IgG levels. We described the proportion of women with IgG levels above the 150 AU/mL positivity threshold according to the timing of infection/vaccination and performed a subanalysis for maternal IgG-GMC levels pre- and during the Omicron wave. RESULTS: We included 238 vaccinated women, 125 who received two doses and 113 three doses, and 48 unvaccinated infected women. All groups infected/vaccinated in the second or third trimester had an IgG-GMC above the positivity threshold. Third-trimester vaccination (second/third dose) resulted in higher maternal and cord-blood IgG-GMC compared to the second trimester (maternal-IgG: 102,32 vs. 4,325 AU/mL, p < 0.001; cord-IgG: 12,113 vs. 8,112 AU/mL, p < 0.001). Compared with infected-only women, a higher proportion of vaccinated women with ≥2 doses and their newborns had IgG levels above the positivity threshold at all time points. In vaccinated women, there were higher maternal IgG-GMC levels during the Omicron wave than pre-Omicron. CONCLUSION: At the time of epidemic, receiving an additional COVID-19 vaccine dose in the third trimester resulted in a higher IgG-GMC compared to the second trimester. Relatively higher levels of maternal and cord IgG-GMC were achieved following vaccination than infection. Women infected during or before the first trimester might benefit from an additional third-trimester dose to prevent peripartum infection and to passively immunize their newborn. The higher levels of maternal IgG-GMC in the Omicron period are suggestive of hybrid immunity. KEY POINTS: · Higher maternal anti-SARS-IgGs in vaccinated → infected.. · Higher cord anti-SARS-IgGs in vaccinated → infected.. · Third-trimester vaccine resulted in high-cord IgG levels..

Is There a Place for the Introduction of Colposcopy Quality Standards?

OBJECTIVES: The aims of the study were to examine the degree of compliance with international quality measures for colposcopy in Israel, which does not currently have formal guidelines and to compare the achievement of quality measures between clinical settings. METHODS: This is a retrospective cohort study, in a hospital, a community clinic, and 2 private colposcopy clinics in Israel, including women aged 18-70 years presenting for colposcopy after abnormal Pap results. Compliance was compared between clinical sites regarding 6 international standards: documentation of reason for referral, type of transformation zone, biopsy location, and grade; proportion of women with high-degree cytological abnormalities (atypical squamous cells - cannot exclude high grade squamous intraepithelial lesion and above) receiving a colposcopy within 4 weeks; and the positive predictive value of colposcopy to detect cervical intraepithelial neoplasia 2 and above. RESULTS: Documentation of reason for referral (1.3% of target), transformation zone type (22.6% of target), biopsy location (18% of target), and lesion grade (31% of target) all failed to meet international standards, as did the proportion of patients with high-degree cytological abnormalities who underwent colposcopy within 4 weeks (32.9% of the target). The positive predictive value of colposcopy exceeded standards (30% above target). Differences existed between clinical settings. CONCLUSIONS: In Israel, there is a considerable shortfall in performance and documentation of most international quality measures for colposcopy. Quality measures for cervical examinations and colposcopy should be considered for inclusion in the National Program for Quality Measures.

Blood Myths and Ethnic-Cultural Background as Impediments in Patient Blood Management Education.

A significant percentage of red blood cell transfusions (RBCTs) are the result of overuse. The implementation of patient blood management (PBM) is challenging. We examined whether blood-linked myths and ethnic-cultural background factors are impediments to PBM education and implementation. Data about the influence of blood myths and diverse ethnic-cultural communities were collected from physicians in our medical center via an anonymous questionnaire which contained questions about myths as well as knowledge of blood transfusion. No statistical differences were found between ethnic and cultural groups regarding blood myths and cultural background influence, although the Jewish participants were less influenced by myths than their Arab colleagues. The influence of blood myths concerning the decision to transfuse exists in both studied ethnic groups. With regard to the association between knowledge and myths influence, we found that the greater the knowledge of the participant, the lower was the myths influence. In a significant proportion of our physician cohort, blood myths and cultural-ethnic status influenced their approach toward RBCT and can be considered an impediment in PBM education. A high knowledge level is associated with less myths influence. The myths and cultural-ethnic background may play a role in PBM education.

Localized Provoked Vulvodynia: Association With Nerve Growth Factor and Transient Receptor Potential Vanilloid Type 1 Genes Polymorphisms.

OBJECTIVE: The aim of the study was to study the associations between localized provoked vulvodynia (LPV) and several single-nucleotide polymorphisms (SNPs) in the transient receptor potential vanilloid type 1 (TRPV1), nerve growth factor (NGF), and the heparanase (HPSE) genes. MATERIALS AND METHODS: Prevalence of SNPs among 65 women with moderate or severe primary LPV (initial symptoms occur with first provoking physical contact) and 126 healthy, ethnically matched controls was analyzed in an observational case-control study. Each participant answered a questionnaire addressing familial LPV occurrence and comorbid pain conditions. RESULTS: Familial occurrences of LPV, temporomandibular joint (TMJ) symptoms, recurrent vaginitis, and irritable bowel syndrome were significantly higher among LPV women than healthy controls. Genotyping analyses revealed a novel, statistically significant high prevalence of polymorphism c.945G>C (rs222747) of TRPV1 and a SNP in the promoter region of NGF (rs11102930) in LPV women compared with controls. A logistic regression model for rs222747 and rs11102930 frequent alleles indicates significant LPV association within the entire study group and Ashkenazi Jewish women, respectively. Comparison of pain conditions with frequent alleles showed the rs222747 "CC" genotype of TRPV1 associated with women with TMJ, recurrent vaginitis, and LPV. CONCLUSIONS: Our results suggest novel genetic susceptibility to primary LPV associated with specific alleles in genes TRPV1 and NGF and propose the rs222747 "C" allele of TRPV1 as a common genetic predisposition for other pain syndromes.

[DERMATOLOGICAL DISEASES IN ELDERLY HOSPITALIZED PATIENTS IN THE GALILEE MEDICAL CENTER - COMPARISON BETWEEN TWO AGE GROUPS].

INTRODUCTION: Although dermatologic conditions bring relatively few people to the Emergency Department, hospitalized patients, especially older people, often suffer from skin problems that contribute to their morbidity. AIMS: We wanted to identify the frequency, clinical course, treatment and influence on hospitalization of dermatologic conditions in patients hospitalized in internal and geriatric departments in Galilee Medical Center. We concentrated on two groups of adults, aged 40-65 years (adult group) and above 65 years (elderly group), in order to understand differences in the cause of referral, type of diagnosis and mode of treatment. METHODS: We performed a retrospective review of 82 hospitalized patients who were referred for dermatological consultation between May-September 2013. Of the 82 patients, 47.6% made up the 'adult' group and 52.4% the 'elderly' group; 62.2% of patients were independent, 18.3% partially independent and 19.5% needed nursing care. RESULTS: Skin infections (38.3%), allergy (mostly drug induced) (23.5%) and trophic disorders (18.5%) were the most common diagnoses. 'Elderly' were less often referred to dermatological consultation than 'adults' (44.3% vs. 55.7%, respectively); skin infections were more common in the 'elderly' (44.8% vs. 55.7%). Nursing care patients (19.5%) were least referred to dermatological consultation, but severity of skin condition (the number of diagnoses and number of treatments per patient) was greater in nursing care patients. CONCLUSIONS: The clinical course between the independent and nursing care patients varies in the number of requests, the different type of diagnoses, the severity of the conditions and the number of treatments provided. DISCUSSION: Our study emphasizes the importance of skin examination by a dermatologist, considering the high number of referrals for dermatological consultations. On the other hand, there was a significant difference between the 'elderly' and 'adult' groups, with fewer referrals for dermatological consultations by the medical staff in the 'elderly' group. Our results resemble those in the literature, having identified the most common skin problems in two groups of hospitalized patients.

Periorbital Ecchymosis Post Closed Rhinoplasty: Natural History, Risk Factors, and Validation of a New Scoring System.

Postrhinoplasty periorbital ecchymosis is an inevitable side effect contributing to patients' psychological aspect and early postoperative morbidity. Efforts are constantly being made to reduce ecchymosis using different methods with varying success. To evaluate treatment response, it is mandatory to have a reliable score. Several studies suggest other scoring systems, but none has been postrhinoplasty-specific, validated, and accepted. This study aimed to demonstrate the natural history of postrhinoplasty ecchymosis, find potential risk factors for worsening patterns, and suggest a useful and reliable periorbital ecchymosis scoring system for postrhinoplasty follow-up. Methods: This prospective study included 183 patients who underwent closed rhinoplasty by the same surgeon and the same principle method. Photographs of the periorbital ecchymosis were taken on postoperative days 1, 2, and 7. The periorbital area was divided into quarters, and three independent physicians assigned the dominant color of each quarter. Results: There were no significant variations between the three physicians' scoring. The interobserver consistency defined as an excellent scoring system reliability, according to our statistical analysis. The postoperative ecchymosis demonstrated a consistent pattern of spread over time, dominating the medial quarters on early postoperative days 1 and 2, following into the lower lateral quarters in postoperative day 7. We found no correlation between patient demographics and clinical characteristics to ecchymosis patterns and temporal spread. Conclusions: Our study suggests a reliable and easy-to-use postrhinoplasty ecchymosis scoring system. This scoring method can be used for postrhinoplasty ecchymosis assessment and as a research-validated tool to quantify different perioperative treatments to reduce ecchymosis and estimate mid-face trauma.

Baseline moderate-range albuminuria is associated with protection against severe COVID-19 pneumonia.

BACKGROUND: Diabetes mellitus is considered a leading contributor to severe coronavirus disease 2019 (COVID-19). AIM: To characterize differences between hospitalized diabetic patients with vs without COVID-19, and parameters associated with COVID-19 severity for prediction. METHODS: This case-control study included 209 patients with type 2 diabetic mellitus hospitalized at the Galilee Medical Center (Nahariya, Israel) and recruited between September 2020 and May 2021, 65 patients with COVID-19 infection in dedicated wards and 144 COVID-19-negative patients in internal medicine wards hospitalized due to other reasons. Clinical parameters - including age, type of antiglycemic medications, presence of retinopathy, smoking history, body mass index (BMI), glycosylated hemoglobin, maximum neutrophil:lymphocyte ratio (NLRmax), C-reactive protein (CRP), estimated glomerular filtration rate (eGFR), and albumin (blood and urine) - were compared between the two primary patient groups, and then between COVID-19-negative patients hospitalized due to infectious vs non-infectious disease. Finally, we explored which parameters were associated with severe COVID-19 pneumonia. RESULTS: COVID-19-negative patients were older (63.9 ± 9.9 vs 59.8 ± 9.2, P = 0.005), and had longer duration of diabetes (P = 0.031), lower eGFR (P = 0.033), higher albumin (P = 0.026), lower CRP (P < 0.001), greater smoking prevalence (P < 0.001), and more baseline albuminuria (54.9% vs 30.8%, P = 0.005) at admission; 70% of COVID-19 patients with albuminuria had moderate-range albuminuria (albumin:creatinine 30-300 mg/g). Most of the patients with albuminuria had chronic kidney disease stage II (CKD II). Oral antiglycemic therapies were not significantly different between the two groups. Multivariable logistic regression showed that higher BMI was significantly associated with severe COVID-19 (OR 1.24, 95%CI: 1.01-1.53, P = 0.04), as was higher NLRmax (OR 1.2, 95%CI: 1.06-1.37, P = 0.005). Surprisingly, pre-hospitalization albuminuria, mostly moderate-range, was associated with reduced risk (OR 0.09, 95%CI: 0.01-0.62, P = 0.015). Moderate-range albuminuria was not associated with bacterial infections. CONCLUSION: Moderate-range albuminuria in COVID-19-positive diabetic patients with CKD II is associated with less severe COVID-19. Further studies should explore this potential biomarker for risk of COVID-19-related deterioration and early interventions.

Pre-infection 25-hydroxyvitamin D3 levels and association with severity of COVID-19 illness.

OBJECTIVE: Studies have demonstrated a potential correlation between low vitamin D status and both an increased risk of infection with SARS-CoV-2 and poorer clinical outcomes. This retrospective study examines if, and to what degree, a relationship exists between pre-infection serum 25-hydroxyvitamin D (25(OH)D) level and disease severity and mortality due to SARS-CoV-2. PARTICIPANTS: The records of individuals admitted between April 7th, 2020 and February 4th, 2021 to the Galilee Medical Center (GMC) in Nahariya, Israel, with positive polymerase chain reaction (PCR) tests for SARS-CoV-2 (COVID-19) were searched for historical 25(OH)D levels measured 14 to 730 days prior to the positive PCR test. DESIGN: Patients admitted to GMC with COVID-19 were categorized according to disease severity and level of 25(OH)D. An association between pre-infection 25(OH)D levels, divided between four categories (deficient, insufficient, adequate, and high-normal), and COVID-19 severity was ascertained utilizing a multivariable regression analysis. To isolate the possible influence of the sinusoidal pattern of seasonal 25(OH)D changes throughout the year, a cosinor model was used. RESULTS: Of 1176 patients admitted, 253 had records of a 25(OH)D level prior to COVID-19 infection. A lower vitamin D status was more common in patients with the severe or critical disease (<20 ng/mL [87.4%]) than in individuals with mild or moderate disease (<20 ng/mL [34.3%] p < 0.001). Patients with vitamin D deficiency (<20 ng/mL) were 14 times more likely to have severe or critical disease than patients with 25(OH)D ≥40 ng/mL (odds ratio [OR], 14; 95% confidence interval [CI], 4 to 51; p < 0.001). CONCLUSIONS: Among hospitalized COVID-19 patients, pre-infection deficiency of vitamin D was associated with increased disease severity and mortality.

Comparison of early outcome from transperitoneal versus transhepatic percutaneous cholecystostomy.

BACKGROUND/AIMS: To determine whether there is a statistically significant difference in the short-term clinical outcome in patients undergoing percutaneous cholecystostomy based on the anatomic route of gallbladder puncture that is, transhepatic versus transperitoneal. METHODOLOGY: Our population consisted of 132 patients who: 1) presented with acute cholecystitis, 2) were at high risk for surgery because of comorbid conditions, 3) underwent percutaneous cholecystostomy either using computed tomography guidance or ultrasound guidance and whose anatomic route was known: the transhepatic percutaneous cholecystostomy group comprised 59 patients, the transperitoneal group 73 patients. Demographic characteristics and clinical parameters of the groups were compared statistically, as were postprocedure hospital course, complications and time to hospital discharge. RESULTS: The two groups were similar in demographic characteristics. There was a statistically significant tendency for computed tomography-guided percutaneous cholecystostomy to be transhepatic, and for ultrasound-guided percutaneous cholecystostomy to be transperitoneal. There were no differences in short-term postprocedure complications between the two groups. There was a tendency for shorter time to hospital discharge following transperitoneal percutaneous cholecystostomy. CONCLUSIONS: Transperitoneal and transhepatic percutaneous cholecystostomy are similar in short-term safety, with no significant difference in complication rate. The interventional radiologist can feel secure in performing percutaneous cholecystostomy using either approach.

Physicians' lack of knowledge - a possible reason for red blood cell transfusion overuse?

BACKGROUND: A significant percentage of red blood cell transfusions are inappropriately overused. This study investigated physicians from the western Galilee in terms of their knowledge of transfusion medicine as a potential reason for red blood cell overuse, and assessed the influence of personal background characteristics on their knowledge. METHODS: Data were collected via anonymous questionnaires. The questionnaires included a personal background section and a professional section. Study participants were grouped according to field of specialty, seniority, and location of medical school graduation, in order to correlate participant characteristics with knowledge. RESULTS: Scores were calculated on a 0-100 scale. The overall knowledge of the study population was low (mean score 47.8 ± 18.6). Knowledge regarding basic physiology of red blood cell transfusion was also low. Internal medicine physicians and senior physicians had significantly greater overall knowledge scores and were more familiar with a restrictive blood management policy than were surgeons and residents, respectively. Comparing knowledge scores, no difference was found regarding indications for transfusion. CONCLUSION: General and fundamental knowledge in transfusion medicine is lacking among physicians in the non-operating room setting, which may play a role in red blood cell transfusion overuse. Field of specialty and professional status influenced knowledge of transfusion medicine. Educational programs and increased physicians' awareness might help decrease unnecessary transfusions. TRIAL REGISTRATION: Not applicable.