Nonalcoholic Fatty Liver Disease and Ethnicity: Lessons Learned from the Arab Population in Israel.

Non-alcoholic fatty liver disease (NAFLD) is the most common liver disease. We aimed to investigate the potential similarities and differences regarding the disease among Arabs and Jews. Retrospective study included all patients older than 18 years with NAFLD diagnosis according to ICD-10 codes. Data regarding demographics, comorbidities, and outcomes were retrieved using the MdClone platform from "Clalit" in Israel. Data concerning 34,090 Arab patients and 173,500 Jewish patients with NAFLD were included. Arab patients were significantly younger at diagnosis (35.0 ± 13 years vs. 43.6 ± 15 years, p < 0.001) and had higher rates of obesity and diabetes mellitus (69.5% vs. 56.5% and 27.0% vs. 22.7%, p < 0.001, respectively). Arab patients had higher rates of cirrhosis and portal hypertension-related complications (2.5% vs. 2.0%, p < 0.001), esophageal varices (0.9% vs. 0.5%, p < 0.001), spontaneous bacterial peritonitis (0.3% vs. 0.1%, p < 0.001), and hepatorenal syndrome (0.3% vs. 0.1%, p < 0.001). There was no significant difference in the prevalence of hepatocellular carcinoma between study groups (0.4% vs. 0.5%, p = 0.156). Liver transplantation was performed in 0.2% of Arab NAFLD patients compared to 0.07% of Jewish NAFLD patients (p < 0.001). Lower rates of all-cause mortality were found among the Arab NAFLD patients versus Jewish NAFLD patients (7.7% versus 11.5%, p < 0.001). According to the Cox regression model, Arab ethnicity is a risk factor for death with OR of 1.36. Significant differences regarding comorbidities, complications, liver transplantations rates, and all-cause mortality were found among NAFLD patients of different ethnicities, hence specific population need specific consideration in prevention, early diagnosis and follow up.

Cochlear implantation compliance among minorities at high risk for hearing impairment following universal newborn hearing screening.

INTRODUCTION: The universal newborn hearing screening (UNHS) allows for early detection of hearing impairment (HI). The goal of this current study was to evaluate the impact of cultural background involving consanguineous marriage on newborn HI diagnosed using UNHS, and compliance with hearing rehabilitation. MATERIALS AND METHODS: This is a retrospective cohort study that included all children born at a single tertiary medical center between 2011 and 2017 who did not pass the UNHS (oto-acoustic emission and auditory brainstem response), and were diagnosed with HI. The study group included children from consanguineous marriage cultural background which were compared to a control group-all other children. Data were retrieved from the computerized medical charts and included epidemiological, audiological, and pregnancy/delivery-related data, and known risk factors for congenital HI. RESULTS: A total of 238 (196 study and 42 control) neonates were diagnosed with HI. Family history of HI was significantly more prevalent in the study group in mild-severe and profound HI subgroups (p = 0.03 and 0.01, respectively). Study group demonstrated lower rates of cochlear implantation (CI) compliance (p = 0.079), performed at a significantly older age (23 months (IQR 17-36.5) vs. 16 (IQR 12-26) months, p = 0.021). When recommended, bilateral CI compliance was significantly lower in the study group (94.1 vs.48.9%, p < 0.001). CONCLUSION: UNHS allows for early HI detection among minority populations at higher risk for CI, yet compliance rates remain lower compared with control. Familiarity of families with the importance of early detection and HI risk may result in higher compliance rates for cochlear implantation. Health providers should aim to improve education and communication with this unique group of patients and consider health promotion programs.

Liver Cirrhosis, Etiology and Clinical Characteristics Disparities Among Minority Population.

Liver cirrhosis (LC) is a common disease with varied primary causes and ethnic disparities. Clinical characteristics and outcomes of Arab Bedouin (AB) and Jewish patients with LC were retrospective collected and compared. We included 1048 patients, 95 (9%) Arab Bedouin and 953 (91%) Jewish patients. The incidence of cirrhosis was much lower among AB. Age at diagnosis was 47 ± 18 years among Bedouins compared to 61 ± 13 years (p < 0.001) among Jews. The most frequent causes of cirrhosis among Bedouin patients were fatty liver 21.1%, cryptogenic 20%, hepatitis B 17.9% and autoimmune hepatitis 15.8%, while hepatitis C (39.2%), fatty liver (17.2%) and alcoholic liver disease (14.4%) were most common among Jewish patients. An all-cause mortality of 48.4% was observed in AB patients compared to 66.4% in Jewish patients (p < 0.001). Significant disparities regarding incidence, clinical characteristics and outcomes of cirrhosis among Arab Bedouin compared with Jewish population were found.

Accelerated Corneal Cross-linking as an Adjunct Therapy in the Management of Presumed Bacterial Keratitis: A Cohort Study.

PURPOSE: To compare the outcomes of accelerated photoactivated chromophore for keratitis corneal cross-linking (PACK-CXL) as an adjunct treatment for bacterial keratitis (PACK-CXL plus standard antibiotic therapy) for patients receiving only standard antibiotic therapy. METHODS: Retrospective cohort study of outcomes of patients with moderate infectious presumed bacterial keratitis (ulcer diameter 2 to 7 mm and stromal depth < 300 µm) were compared before and after initiation of a new treatment protocol of PACK-CXL in addition to standard antibiotic treatment. RESULTS: A total of 70 eyes of 70 patients were included: 39 eyes in the PACK-CXL plus antibiotic (PACK-ABX) group and 31 eyes in the antibiotic only (ABX) control group. The PACK-ABX group showed shorter times to complete reepithelialization (9.3 ± 6.0 vs 16.0 ± 12.7 days, P = .01) and did not require tectonic emergency keratoplasty (0% versus 19.4%, P = .006). The PACK-ABX group also showed a higher percentage of eyes with complete reepithelialization in 6 days or less (46.2% vs 6.5%, P < .001) and a trend for shorter hospitalizations (6.3 ± 5.0 vs 8.5 ± 4.5 days, P = .06). A multivariate analysis controlling for age showed that PACK-ABX treatment remained significantly associated with early ulcer reepithelialization (odds ratio = 0.09, 95% confidence interval = 0.02 to 0.48, P = .005). CONCLUSIONS: This study validates previous findings regarding the use of accelerated PACK-CXL in the treatment of bacterial keratitis. Adding PACK-CXL improved clinical outcomes (reducing healing time) when compared to antibiotics alone. [J Refract Surg. 2020;36(4):258-264.].

INCREASED SERUM CALCIUM IS NOT COMMON IN HOSPITALIZED PATIENTS WITH PRIMARY HYPERPARATHYROIDISM: A RETROSPECTIVE, OBSERVATIONAL STUDY.

Objective: Serum calcium levels often decrease during acute illness in patients with an intact calcium-regulating system. However, the dynamics of serum calcium levels in hospitalized patients with primary hyperparathyroidism (PHPT) have not yet been described. Methods: Clinical and laboratory data were retrospectively retrieved from the electronic medical records of patients with PHPT before, during, and after hospitalization for various reasons (excluding parathyroid surgery). Results: There were 99 nonselected patients with asymptomatic, hypercalcemic PHPT, hospitalized for various reasons; 42% were admitted for apparent infectious or septic conditions, and 58% were admitted for noninfectious conditions. Total serum calcium increased >0.5 mg/dL in 7.4% of the patients: 10.9% and 2.5% of the patients with noninfectious and infectious conditions, respectively. In 65.7% of the patients, the mean total serum calcium (TsCa), but not albumin-corrected calcium (corrCa), decreased significantly during hospitalization, down to below the upper limit of the reference range. Although prehospitalization TsCa and corrCa were similar in patients with infectious and noninfectious conditions, during hospitalization, TsCa was lower in patients with infectious conditions (P = .02). Both TsCa and albumin returned to prehospitalization levels after recovery. Conclusion: TsCa increases in a minority of hospitalized PHPT patients. In the majority of hospitalized patients with PHPT, TsCa, but not corrCa, decreases to within the normal reference range, more so in patients with infectious conditions, obscuring the major characteristic of PHPT. Therefore, it is prudent to follow calcium and corrCa during hospitalization in patients with PHPT. Abbreviations: corrCa = albumin-corrected serum calcium; IQR = interquartile range; PHPT = primary hyperparathyroidism; PTH = parathyroid hormone; TsCa = total serum calcium.

ACO2 homozygous missense mutation associated with complicated hereditary spastic paraplegia.

OBJECTIVE: To identify the clinical characteristics and genetic etiology of a family affected with hereditary spastic paraplegia (HSP). METHODS: Clinical, genetic, and functional analyses involving genome-wide linkage coupled to whole-exome sequencing in a consanguineous family with complicated HSP. RESULTS: A homozygous missense mutation was identified in the ACO2 gene (c.1240T>G p.Phe414Val) that segregated with HSP complicated by intellectual disability and microcephaly. Lymphoblastoid cell lines of homozygous carrier patients revealed significantly decreased activity of the mitochondrial aconitase enzyme and defective mitochondrial respiration. ACO2 encodes mitochondrial aconitase, an essential enzyme in the Krebs cycle. Recessive mutations in this gene have been previously associated with cerebellar ataxia. CONCLUSIONS: Our findings nominate ACO2 as a disease-causing gene for autosomal recessive complicated HSP and provide further support for the central role of mitochondrial defects in the pathogenesis of HSP.

BONE TURNOVER IN OSTEOPOROTIC WOMEN DURING LONG-TERM ORAL BISPHOSPHONATES TREATMENT: IMPLICATIONS FOR TREATMENT FAILURE AND "DRUG HOLIDAY" IN THE REAL WORLD.

OBJECTIVE: Little data exist to support concerns over bone turnover suppression during prolonged oral bisphosphonate treatment and on consequences of the recommended "drug holiday." This study was performed to assess bone resorption rates in postmenopausal osteoporotic women on prolonged oral bisphosphonate treatment and in response to switching to "drug holiday" intravenous bisphosphonate, or continuation of oral bisphosphonates. METHODS: The frequency distribution of the bone resorption marker urinary deoxypyridinoline crosslinks (uDPD), was obtained retrospectively from 211 osteoporotic women attended at an academic hospital endocrine clinic, treated for >2 years with oral bisphosphonates. In some patients, uDPD was re-assessed following modification or continuation of treatment. RESULTS: The mean duration of oral bisphosphonates treatment was 7.2 ± 3.1 years. uDPD was within reference range for premenopausal women in 61.6% of the patients, below in 7.6% of the patients, and above upper limit in 30.8%. uDPD decreased significantly following intravenous zoledronic acid, increased significantly during "drug holiday," and slightly decreased in those continued on oral bisphosphonate treatment. CONCLUSION: In this real-world study, the majority of women on prolonged oral bisphosphonates maintained bone resorption rates within the normal reference range for premenopausal women. The likelihood for inadequate suppression was considerably greater than that of over-suppression. Implementing a "drug holiday" resulted in a marked increase in bone resorption rates. Additional studies should explore the potential role of bone turnover markers in the evaluation of patients on prolonged oral bisphosphonates and during "drug holiday" in different settings and using additional markers. ABBREVIATIONS: BMD = bone mineral density; IQR = interquartile range; uDPD = urinary deoxypyridinoline crosslinks.

Prospective study of the impact of diabetes mellitus newly diagnosed by glycated hemoglobin on outcomes in patients undergoing percutaneous coronary intervention.

BACKGROUND: We sought to determine the prevalence of diabetes mellitus (DM) newly diagnosed by elevated glycated hemoglobin (HbA1c) in patients undergoing percutaneous coronary intervention (PCI) and its association with 1-year clinical outcomes. METHODS: We prospectively enrolled consecutive patients undergoing PCI (2011-2013). HbA1c levels were assessed during the index hospitalization and newly diagnosed DM was defined as HbA1c≥6.5% in the absence of the previous diagnosis. The primary outcome was MACCE (Major Adverse Cerebro- and Cardiovascular Events) defined as death, stroke, PCI or acute myocardial infarction at 1year. RESULTS: Diabetes was previously diagnosed in 391 (34%) patients (DM group), 221 (19%) had newly diagnosed DM based on the HbA1c level and 539 (47%) did not have diabetes (Non-DM). In DM group HbA1c was 7.80±1.36% as compared with 7.62±1.30% in patients with newly diagnosed DM (p<0.001). These patients were younger (62.0±11.3years) compared to DM (67.9±10.4years) and non-DM (63.7±13.0) patients, p<0.001. 1-year MACCE rates were 14.8%, 19.5% and 27.96% in the non-DM, newly diagnosed DM and DM groups, respectively (p<0.001). Multivariate analysis showed that compared to non-DM, the adjusted one-year hazard ratios for MACCE were 1.75 and 1.40 in patients with known DM and newly diagnosed DM, respectively (p<0.05 for both). CONCLUSION: Newly diagnosed DM based on peri-procedural HbA1c is common and associated with increased short and long term risk for adverse outcomes. Our results may warrant routine screening for DM in all patients undergoing PCI.

Epidemiological, diagnostic, clinical, and therapeutic aspects of Brucella bacteremia in children in southern Israel: a 7-year retrospective study (2005-2011).

BACKGROUND: Data on the various aspects of brucellosis in children living in southern Israel are missing. OBJECTIVES: Our aim was to study the epidemiological, microbiological, diagnostic, clinical, therapeutic and outcome features of brucellosis in children <19 years of age in southern Israel during 2005-2011. PATIENTS AND METHODS: The study population included all patients hospitalized with a diagnosis of brucellosis, established according to a clinical presentation compatible with brucellosis+presence of Brucella bacteremia (BB). RESULTS: A total of 128 (50.8%) of 252 patients admitted with a diagnosis of brucellosis had BB (all with Brucella melitensis). All patients were of Muslim Bedouin ethnicity. The mean incidence of BB in southern Israel was 16 cases/100,000 Bedouin, with no significant changes during the study period. Overall duration of symptoms before diagnosis was 10.1±10.9 days. Fever at diagnosis was recorded in <20% of the patients. The most frequent symptoms were arthralgia (61.7%), weakness (32.8%), gastrointestinal disturbances (27.3%), myalgia (25%), and headache (18.8%). The main clinical findings included monoarthritis (36.7%), hepatosplenomegaly (25%), lymphadenopathy (17.2%), heart murmur (11.7%), and skin rash (9.4%), respectively. Anemia, leukopenia, thrombocytopenia, and pancytopenia were reported in 17.6%, 29.6%, 12.8%, and 2.3% of the patients, respectively. Twenty-nine (30.5%) patients with BB had serum agglutinin titers ≤1/160 (13, 13.7%%, had titers <1/160). Twenty-seven (93%) of the 29 patients aged 0-4 years were treated with gentamicin and trimethoprim-sulfamethoxazole; a total of 77 (60.2%) patients received gentamicin and doxycycline. CONCLUSIONS: Childhood brucellosis remains an important public health problem in southern Israel. BB was diagnosed in >50% of the children with brucellosis, and B. melitensis was identified in all cases. Arthralgia, weakness, and gastrointestinal complaints were the most common symptoms, and monoarthritis, hepatosplenomegaly, and lymphadenopathy were the most common clinical findings. A considerable number of patients with BB had undetectable/low serum agglutinin titers, suggesting insufficient reliability on serology alone in diagnosis of brucellosis.