Neonatal Hemochromatosis, Renal Tubular Dysgenesis, and Hypocalvaria in a Neonate

We report a neonate with neonatal hemochromatosis (NH), renal tubular dysgenesis (RTD), and hypocalvaria. NH is a fatal condition of the newborn, characterized by severe idiopathic liver failure of intrauterine onset and siderosis, intra- and extrahepatic, with sparing of the reticuloendothelial system. RTD is characterized by short, abnormally developed cortical tubules that lack proximal tubule differentiation. Although both NH and RTD have been reported as entities with a genetic component, similar findings can be secondary to in utero insults. Hypocalvaria has been reported in association with fetal hypoxia including that secondary to angiotensin converting enzyme inhibitors. This 38-week-old infant died at 8.5 h. The small nodular liver weighed 44 g. Grossly, the kidneys were normal. Hypocalvaria was present. Microscopically, the hepatic parenchyma was distorted by fibrous tracts, proliferation of bile ducts, and abundant iron deposition in hepatocytes. Extrahepatic siderosis in the pancreas, myocardium, and other organs was consistent with NH. Proximal convoluted tubules were not seen on routine stains and markers for proximal tubules were negative. Previous reports have linked NH with RTD and RTD with hypocalvaria. This infant had all three of these rare conditions, which have been hypothesized or shown to be due to genetic factors, hypoxia, or drugs. The etiology in this case is unknown. Received May 20, 1997; accepted August 15, 1997.     

Anti-SARS CoV-2 IgG in COVID-19 Patients with Hematological Diseases: A Single-center,Retrospective Study in Japan

Objective Coronavirus disease (COVID-19), caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), has spread globally. Although the relationship between anti-SARS-CoV-2 immunoglobulin G (IgG) antibodies and COVID-19 severity has been reported, information is lacking regarding the seropositivity of patients with particular types of diseases, including hematological diseases. Methods In this single-center, retrospective study, we compared SARS-CoV-2 IgG positivity between patients with hematological diseases and those with non-hematological diseases. Results In total, 77 adult COVID-19 patients were enrolled. Of these, 30 had hematological disorders, and 47 had non-hematological disorders. The IgG antibody against the receptor-binding domain of the spike protein was detected less frequently in patients with hematological diseases (60.0%) than in those with non-hematological diseases (91.5%; p=0.029). Rituximab use was significantly associated with seronegativity (p=0.010). Conclusion Patients with hematological diseases are less likely to develop anti-SARS-CoV-2 antibodies than those with non-hematological diseases, which may explain the poor outcomes of COVID-19 patients in this high-risk group.     

Freeze-dried primate sperm retains early reproductive potential after intracytoplasmic sperm injection

Oocytes inseminated by intracytoplasmic sperm injection using fresh ejaculated or freeze-dried rhesus macaque sperm showed similar activation, sperm aster assembly, and male-female pronuclear apposition rates.     

Rheumatoid factor in acute bacterial endocarditis

Sera from 55 parenteral drug abusers with endocarditis due to Staphylococcus aureus were assayed for the presence and titer of rheumatoid factor. Thirteen (24%) of the 55 patients with endocarditis had sera positive for rheumatoid factor at one point or another in their courses; only 2 (7%) of 30 noninfected drug users were found to be positive. It appeared that more severe cases, as evidenced by duration of fever after initiation of antibiotic therapy, were more likely to develop rheumatoid factor.     

Impact of an In-Person Versus Web-Based Practice Standardized Patient Examination on Student Performance on a Subsequent High-Stakes Standardized Patient Examination

Background: Optimal methods of preparing students for high-stakes standardized patient (SP) examinations are unknown. Purposes: The purpose is to compare the impact of two formats of a formative SP examination (Web-based vs. in-person) on scores on a subsequent high-stakes SP examination and to compare students' satisfaction with each formative examination format. Methods: Clustered randomized trial comparing a Web-based module versus in-person formative SP examination. We compared scores on a subsequent high-stakes SP examination and satisfaction. Results: Scores on the subsequent high-stakes SP examination did not differ between the two formative formats but were higher after the formative assessment than without (p < .001). Satisfaction was higher with the in-person than Web-based formative assessment format (4.00 vs. 3.62 on a 5-point scale, p = .01). Conclusions: Two formats of a formative SP examination led to equivalent improvement in scores on a subsequent high-stakes examination. Students preferred an in-person formative examination to online but were satisfied with both.     

Fractionated proton beam radiotherapy for acoustic neuroma

OBJECTIVE: This study evaluated proton beam irradiation in patients with acoustic neuroma. The aim was to provide maximal local tumor control while minimizing complications such as cranial nerve injuries. METHODS: Thirty-one acoustic neuromas in 30 patients were treated with proton beam therapy from March 1991 to June 1999. The mean tumor volume was 4.3 cm(3). All patients underwent pretreatment neurological evaluation, contrast enhanced magnetic resonance imaging, and audiometric evaluation. Standard fractionated proton radiotherapy was used at daily doses of 1.8 to 2.0 cobalt Gray equivalent: patients with useful hearing before treatment (Gardner-Robertson Grade I or II) received 54.0 cobalt Gray equivalent in 30 fractions; patients without useful hearing received 60.0 cobalt Gray equivalent in 30 to 33 fractions. RESULTS: Twenty-nine of 30 patients were assessable for tumor control and cranial nerve injury. Follow-up ranged from 7 to 98 months (mean, 34 mo), during which no patients demonstrated disease progression on magnetic resonance imaging scans. Eleven patients demonstrated radiographic regression. Of the 13 patients with pretreatment Gardner-Robertson Grade I or II hearing, 4 (31%) maintained useful hearing. No transient or permanent treatment-related trigeminal or facial nerve dysfunction was observed. CONCLUSION: Fractionated proton beam therapy provided excellent local control of acoustic neuromas when treatment was administered in moderate doses. No injuries to the Vth or VIIth cranial nerves were observed. A reduction in the tumor dose is being evaluated to increase the hearing preservation rate.     

Open Versus Minimally Invasive Resection of Gastric GIST: A Multi-Institutional Analysis of Short- and Long-Term Outcomes

Background

Gastrointestinal stromal tumors (GIST) are the most common mesenchymal tumors of the gastrointestinal tract. Overall surgical experience with minimally invasive surgery (MIS) has increased; however, published reports on MIS resection of GIST are limited to small, single-institution experiences.

Methods

A total of 397 patients who underwent open surgery (n = 230) or MIS (n = 167) for a gastric GIST between 1998 and 2012 were identified from a multicenter database. The impact of MIS approach on recurrence and survival was analyzed using propensity-score matching by comparing clinicopathologic factors between patients who underwent MIS versus open resection.

Results

There were 19 conversions (10 %) to open; the most common reasons for conversion were tumor more extensive than anticipated (26 %) and unclear anatomy (21 %). On multivariate analysis, smaller tumor size and higher body mass index (BMI) were associated with receipt of MIS. In the propensity-matched cohort (n = 248), MIS resection was associated with decreased length of stay (MIS, 3 days vs open, 8 days) and fewer ≥ grade 3 complications (MIS, 3 % vs open, 14 %) compared with open surgery. High rates of R0 resection and low rates of tumor rupture were seen in both groups. After propensity-score matching, there was no difference in recurrence-free or overall survival comparing the MIS and the open group (both p > 0.05).

Conclusions

An MIS approach for gastric GIST was associated with low morbidity and a high rate of R0 resection. The long-term oncological outcome following MIS was excellent, and therefore the MIS approach should be considered the preferred approach for gastric GIST in well-selected patients.