[Treatment strategy for patients with thrombotic thrombocytopenic purpura].

Thrombotic thrombocytopenic purpura (TTP) is a rare and potentially life-threatening disease that is characterized by microangiopathic hemolytic anemia, consumptive thrombocytopenia, and ischemic organ damage resulting from the formation of platelet-rich thrombi in the microvasculature. It is especially related to a severe deficiency of ADAMTS13, the specific von Willebrand factor-cleaving protease. Thus, <10% of the normal activity of ADAMTS13 is an essential diagnostic marker for establishing a diagnosis of TTP. TTP can be of congenital form that is termed the Upshaw-Schulman syndrome (USS) that is caused by genetic abnormality of ADAMTS13 and acquired form that is caused by autoantibodies against ADAMTS13. The congenital form is treated with the infusion of fresh frozen plasma, and the treatment of the acquired form involves plasma exchange combined with immunosuppressive therapy that uses corticosteroids and rituximab. Recently, the efficacy and safety of new drugs caplacizumab, single-domain antibody against ADAMTS13, and recombinant ADAMTS13 products have been reported in large-scale clinical trials conducted in other countries. These results suggested the better outcome in the treatment for the patients with TTP in the near future.

An assessment of sensitivity biomarkers for urinary cadmium burden.

BACKGROUND: Excess cadmium (Cd) intake poses a general risk to health and to the kidneys in particular. Among indices of renal dysfunction under Cd burden measures are the urinary N-acetyl-ß-D-glucosidase (UNAG) and urinary ß2-microglobulin (Uß2-MG) enzymes. However, the end-pointed values and the Cd burden threshold remain controversial because the scopes fluctuate widely. METHODS: To ascertain the clinical benchmark dose of urinary Cd (UCd) burden for renal dysfunction, 1595 residents near a Cd site were surveyed. Urine was sampled and assayed. A benchmark dose low (BMDL) was obtained by fitting UCd levels and index levels. RESULTS: We found that over 50% of the subjects were suffering from Cd exposure as their UCd levels far exceeded the national standard threshold of 5.000 µg/g creatinine (cr). Further analysis indicated that Uß2-MG was more sensitive than UNAG for renal dysfunction. The BMDL for UCd was estimated as 3.486 U/g cr (male, where U is unit of enzyme) and 2.998 U/g cr (female) for UNAG. The BMDL for Uß2-MG, which is released into urine from glomerulus after Cd exposure, was found to be 2.506 µg/g cr (male, where µg is the unit of microglobulin) and 2.236 µg/g cr (female). CONCLUSIONS: Uß2-MG is recommended as the sensitivity index for renal dysfunction, with 2.2 µg/g cr as the threshold for clinical diagnosis. Our findings suggest that Uß2-MG is the better biomarker for exposure to Cd.

Can common serum biomarkers predict complicated appendicitis in children?

PURPOSE: As appendicitis in children can be managed differently according to the severity of the disease, we investigated whether commonly used serum biomarkers on admission could distinguish between simple and complicated appendicitis. METHODS: Admission white blood cell (WBC), neutrophil (NEU), and C-reactive protein (CRP) levels were analysed by ROC curve, and Kruskal-Wallis and contingency tests. Patients were divided according to age and histology [normal appendix (NA), simple appendicitis (SA), complicated appendicitis (CA)]. RESULTS: Of 1197 children (NA = 186, SA = 685, CA = 326), 7% were <5 years, 55% 5-12, 38% 13-17. CA patients had higher CRP and WBC levels than NA and SA (p < 0.0001). NEU levels were lower in NA compared to SA or CA (p < 0.0001), but were similar between SA and CA (p = 0.6). CA patients had higher CRP and WBC levels than SA patients in 5-12- (p < 0.0001) and 13-17-year groups (p = 0.0075, p = 0.005), but not in <5-year group (p = 0.72, p = 0.81). We found CRP >40 mg/L in 58% CA and 37% SA (p < 0.0001), and WBC >15 × 109/L in 58% CA and 43% SA (p < 0.0001). CONCLUSIONS: Admission CRP and WBC levels may help the clinician predict complicated appendicitis in children older than 5 years of age. Early distinction of appendicitis severity using these tests may guide caregivers in the preoperative decision-making process.

[Quality of life as an effectiveness criterion of surgical treatment for anterior urethral strictures].

RELEVANCE: Recent trends in the contemporary medicine have shown a growing request for the personalized choice of treatment and evaluation of its results. PROM-USS questionnaire has been designed for patients undergoing surgical correction of anterior urethral strictures to measure the treatment success using patient reported outcomes in numerical terms. AIM: To test the effectiveness of different surgical modalities for anterior urethral strictures using patient reported outcomes. MATERIAL AND METHODS: Ninety men self-completed the PROM-USS questionnaire after surgical treatment of anterior urethral strictures. Mean and median follow-up was 6-132 months and 72 months, respectively. Measures included lower urinary tract symptom score, overall quality of life (QOL) and overall health, and patient satisfaction with treatment. RESULTS: At the median follow-up of 72 months after surgery for anterior urethral stricture, the survey findings showed LUTS total score 7.4+/-1.2 and urination score on VAS scale 2.3+/-1.1. Sixty-five (72%) of the men believed that residual LUTS did not significantly affect their quality of life. Fifteen (17%) and 10 (11%) patients reported a slight and moderate/strong negative impact of urinary symptoms on their quality of life, respectively. The EQ-5D score of overall health state on VAS was 73+/-4.2 out of 100, the EQ-5D score of the overall quality of life was 0.79+/-0.2. Seventy-eight (86.7%) of 90 men were satisfied (32.2% very satisfied and 54.5% satisfied) with the surgery results, and 12 (13.3%) were dissatisfied. Reasons for dissatisfaction were the emergence of new problems (post-micturition dribbling) with an overall improvement of urinary symptoms (5) and the lack of improvement in urinary symptoms (7). Among patients treated with OIU, 79% were satisfied, which was significantly less than in groups of men who underwent anastomotic urethroplasty - 89% (p<0.05) and substitution urethroplasty using a buccal mucosal grafting - 95% (p<0.05). CONCLUSION: The patient-reported outcomes showed that surgery for anterior urethral stricture is highly effective and characterized by a long term patient satisfaction and quality of life.

Hereditary TTP/Upshaw-Schulman syndrome: the ductus arteriosus controls newborn survival.

Hereditary TTP (hTTP), termed Upshaw-Schulman syndrome, is an ultra-rare disorder caused by a severe deficiency of plasma ADAMTS13 activity that allows circulation of ultra-large von Willebrand factor (UL-VWF) multimers. The greatest risk for hTTP is in their first days after birth, when 35-50% of patients will have severe hemolysis, jaundice, and thrombocytopenia. It is often fatal without effective treatment. In utero, fetal blood flowing from the pulmonary artery through the ductus arteriosus (DA) to the aorta is under low-shear-force. At birth, blood flow through the DA reverses to a left-to-right shunt, and the diameter of the DA begins to decrease due to hyper-oxygenated blood and decreased plasma prostaglandin E2. This causes turbulent circulation that unfolds UL-VWF, allowing platelet aggregation. If the DA closes promptly, hTTP newborns survive, but if it remains patent, turbulent circulation persists, triggering microvascular thrombosis. hTTP is commonly diagnosed as hemolytic disease of the fetus and newborn (HDFN) caused by anti-red cell antibodies and treated with exchange blood transfusion, which prevents kernicterus even when the diagnosis of hTTP is missed. The diagnosis of newborn-onset hTTP should be considered because HDFN does not cause severe thrombocytopenia, which might be effectively treated with recombinant ADAMTS13.

Novel ADAMTS13 mutations in an obstetric patient with Upshaw-Schulman syndrome.

Upshaw-Schulman syndrome (USS) is a rarely reported congenital form of thrombotic thrombocytopenic purpura (TTP) that results from mutations in the ADAMTS13 gene. Many USS patients are diagnosed during the second or third trimester of their first pregnancy. We present a patient diagnosed with USS following retinal detachments and intrauterine fetal demise at 34 weeks of gestation. The patient's plasma was tested for ADAMTS13 activity, inhibitor, and antibody. Subsequently, she and her first-degree relatives had ADAMTS13 gene sequencing. Initially, the patient was found to have an ADAMTS13 activity of <5% in the absence of an ADAMTS13 inhibitor (FRETS assay) or antibody (immunoassay). Repeat studies in the months following hospital discharge showed persistent, undetectable ADAMTS13 activity and she was given a clinical diagnosis of USS. Molecular sequencing demonstrated two novel missense mutations in the ADAMTS13 gene: one in the maternal exon 17 (p.Ala690Thr due to nucleotide substitution c.2068 G>A) and another in the paternal exon 22 (p.Arg915Cys due to nucleotide substitution c.2746 C>T). In addition to being compound heterozygous for two ADAMTS13 mutations, the patient also had two maternally inherited single nucleotide polymorphisms: p.P618A (exon 16) and p.A732V (exon 18). Her parents and only sister had normal or near-normal ADAMTS13 activity. Each was heterozygous for one of the novel missense mutations. This case highlights the importance of molecular analysis of the ADAMTS13 gene in patients and family members when the severe ADAMTS13 deficiency does not appear to be autoimmune in nature.

Advances in commingled human remains analysis between 2014 and 2023.

This study reviews recent advances in osteometric, genetic, geochemical, and digital modeling applications since 2014 to demonstrate the expanded range of analyses and skeletal elements that can be used to separate individuals from commingled contexts. While traditional methods remain foundational to commingling resolution, new advances allow increased individuation, identification of human versus non-human remains, and an amplified scale of assemblages that can be analyzed. This summary offers ways for practitioners to consider the juxtaposition of analytical goals, time, financial concerns, and methods when managing commingled assemblages. Forensic anthropology and bioarchaeology case studies illustrate differences related to application of methods in terms of recovery environments, project goals, and recovered materials. Whether the goal is to isolate and individuate only major elements or as many bones as possible, in nearly all cases, it is best practice to combine several types of methods to fulfill the project scope within the established parameters. This review can help practitioners identify the most appropriate analytical protocols and methods for their projects.

Antenatal detection of a thyrocervical teratoma in the third trimester - A case report.

Introduction: Congenital teratomas are rare with less than 10% found in the cervical region. When they do occur in the neck, they are usually identified during the third trimester and, due to their location, they are associated with polyhydramnios. Case report: Cervical teratoma was identified at 32 weeks gestation during a routine scan of a 33-year-old female expecting dichorionic diamniotic twins. Only one twin was affected and referral to a specialist hospital allowed a care plan to be arranged to ensure safe delivery followed by immediate treatment and surgery. To date, both twins are thriving. Discussion: Cervical teratomas usually occur in the third trimester and are not always detected prenatally. Although usually benign, their size and location may cause polyhydramnios as well as increasing the risk of neonatal asphyxiation and death. It is therefore essential to assess the foetal neck when polyhydramnios is noted at any third trimester scan, to maximise detection and ensure appropriate multidisciplinary healthcare can be organised to optimise postnatal survival. Conclusion: Antenatal detection of a rare cervical teratoma was pivotal in the management and survival of this infant. Ultrasound practitioners must consider the possibility of a neck teratoma in the presence of third trimester polyhydramnios and therefore evaluate carefully foetal head, neck and chest anatomy as part of their examination.

No recovery of a large-scale anthropogenic sediment disturbance on the Pacific seafloor after 77 years at 6460 m depth.

Habitat restoration and recolonisation of benthic communities after physical perturbation in the deep sea has long been thought to be extremely slow. This study reports on a serendipitous opportunity to survey the current state of a large mechanical disturbance of sediments at 6460 m in the Pacific Ocean. The impact was caused 77 years ago by the sinking of the USS Johnston. The surrounding debris field had little impact on the sedimentary habitat, other than in the provision of artificial hard substrates, while the troughs that formed as the ship impacted the seafloor and slid down the slope of the Philippine Trench were still completely void of animal tracks and burrows, or any observable epifauna, and in some areas subsurface stratification was still exposed at the surface. This suggests that mechanical perturbations of sediments in the deep Pacific may remain ecologically significant for, at the very least, 100 years.

Novel ADAMTS13 mutation in a family with three recurrent neonatal deaths: a case report and literature review.

Background: Upshaw-Schulman syndrome (USS) is rare, autosomal recessive, hereditary thrombotic thrombocytopenic purpura (TTP) caused by variants in a disintegrin-like and metalloprotease with thrombospondin type 1 motif (ADAMTS13). USS has a heterogeneous clinical course, and most symptoms overlap with other diseases. Early diagnosis may have important implications for the patients. We found novel ADAMTS13 mutation and explored the clinical features and prognosis of newborn-onset USS to increase awareness of the disease. Case Description: The same, non-consanguineous couple had three unexplained neonatal deaths. The symptoms of the three infants were mainly severe jaundice, anemia and thrombocytopenia after birth, which was consistent with the reported USS symptoms of neonates and died rapidly suddenly in the during rescue efforts. By using whole-exome sequencing (WES) for the study family, we found a novel heterozygous compound in ADAMTS13 (c.1187 (exon10) G>A (p.C396Y)/c.1595 (exon14) G>T (p.C532F)) that was carried by the three newborns originating from father and mother respectively. We reviewed nine published studies of newborn-onset USS and compared our cases for clinical symptoms and laboratory testing. All nine published cases were diagnosed by ADAMTS13 activity; in seven cases gene mutation analysis was performed and eight cases were still alive at the time of publication. Conclusions: The case has added clinicians' awareness of the diagnosis and treatment of USS. A novel rare mutation in ADAMTS13 broadens the spectrum of genetic causes of this rare disorder and expands the phenotypic spectrum.

Sensitivity and specificity of the Alvarado Score for the timely differential diagnosis of acute appendicitis for a case series in Samoa.

BACKGROUND: Timely diagnosis of acute appendicitis (AA) is essential for optimal management of abdominal pain emergency presentations. Improved diagnostic procedures should minimise progression to complicated appendicitis, as well as reduce the number of unwanted surgical interventions. The Alvarado Score is an important tool to assist with the management of AA based on clinically predictive values. The aim of the present study was to evaluate the sensitivity and specificity of the Alvarado Score as an instrument for the diagnosis of AA in our low-resourced context of Samoa. MATERIALS AND METHODS: The lead investigator performed a retrospective clinical data review design of all patients admitted to the Tupua Tamasese Meaole Hospital Surgical Unit for AA from January 2015 to December 2016. Exclusion criteria were appendiceal abscess and/or mass, and final diagnosis 'not acute appendicitis'. Age, sex, village, Alvarado Score, Ultrasound Scan Result, surgical management, and histopathology result were the main variables entered into the SPSS statistical software package for analysis. RESULTS: Of the 225 patients (2-86 years) admitted for AA, 67.1% (N = 151) underwent appendicectomy, and the remaining 32.9% (N = 74) were treated with antibiotics. An Alvarado Score (≥5) showed 91.7% sensitivity and 50% specificity for AA. There was a significant relationship between the Alvarado Score and the Ultrasound Scan for AA (p < 0.05). Histology results showed complicated AA in 68.6% (N = 94) of cases. The negative appendicectomy rate was 2.6%. CONCLUSIONS: The Alvarado Score is an effective tool for the timely differential diagnosis of AA in our Samoan context. The high rate of complicated acute appendicitis warrants investigation to further improve the management of AA in the Pacific Island setting.

Cervical leiomyosarcoma in a teenage girl: A rare form of uterine leiomyosarcoma.

Sarcomas arising from the cervix are rare, and the reported prevalence is 0.20-0.55%. A 15-year-old Para 0+0 secondary school student presented to the emergency department in shock with a 1-year history of painless vaginal protrusion, vaginal bleeding, foul-smelling vaginal discharge, occasional passage of blood clots, fatigue, fainting episodes, and weight loss. She was resuscitated with intravenous fluids and blood transfusions. General examination revealed a young girl with a 16-week sized abdominal mass. Vaginal examination revealed a large mobile fleshy mass 14 cm by 10 cm with an offensive discharge and odour. It was externally friable, bled actively on contact, had areas of tissue necrosis, and was oedematous. It was difficult to determine the adnexa structures because of tenderness. Examination under anaesthesia showed that the mass was continuous with the cervix and was not attached to the vagina or vulva. The histology report of the biopsied specimens showed features consistent with cervical leiomyosarcoma (LMS). Cervical LMS was confirmed by immunohistochemistry and a total abdominal hysterectomy and bilateral salpingo-oophorectomy were performed as definitive treatment. Postoperative hormone replacement therapy was initiated. The patient's postoperative condition was stable and there was no tumour recurrence for >2 years on follow-up. Making a diagnosis and instituting surgical and adjuvant treatments for LMS in a low-resource setting are challenging. This is due to lack of access to universal healthcare coverage. A multidisciplinary approach with early diagnosis and complete surgical resection of the tumour provides the most favourable possibility of an improved survival and quality of life.

The global carrier frequency and genetic prevalence of Upshaw-Schulman syndrome.

BACKGROUND: Upshaw-Schulman syndrome (USS) is an autosomal recessive disease characterized by thrombotic microangiopathies caused by pathogenic variants in ADAMTS13. We aimed to (1) curate the ADAMTS13 gene pathogenic variant dataset and (2) estimate the carrier frequency and genetic prevalence of USS using Genome Aggregation Database (gnomAD) data. METHODS: Studies were comprehensively retrieved. All previously reported pathogenic ADAMTS13 variants were compiled and annotated with gnomAD allele frequencies. The pooled global and population-specific carrier frequencies and genetic prevalence of USS were calculated using the Hardy-Weinberg equation. RESULTS: We mined reported disease-causing variants that were present in the gnomAD v2.1.1, filtered by allele frequency. The pathogenicity of variants was classified according to the American College of Medical Genetics and Genomics criteria. The genetic prevalence and carrier frequency of USS were 0.43 per 1 million (95% CI: [0.36, 0.55]) and 1.31 per 1 thousand population, respectively. When the novel pathogenic/likely pathogenic variants were included, the genetic prevalence and carrier frequency were 1.1 per 1 million (95% CI: [0.89, 1.37]) and 2.1 per 1 thousand population, respectively. CONCLUSIONS: The genetic prevalence and carrier frequency of USS were within the ranges of previous estimates.

CO2/CH4 and He/N2 Separation Properties and Water Permeability Valuation of Mixed Matrix MWCNTs-Based Cellulose Acetate Flat Sheet Membranes: A Study of the Optimization of the Filler Material Dispersion Method.

The main scope of this work is to develop nano-carbon-based mixed matrix cellulose acetate membranes (MMMs) for the potential use in both gas and liquid separation processes. For this purpose, a variety of mixed matrix membranes, consisting of cellulose acetate (CA) polymer and carbon nanotubes as additive material were prepared, characterized, and tested. Multi-walled carbon nanotubes (MWCNTs) were used as filler material and diacetone alcohol (DAA) as solvent. The first main objective towards highly efficient composite membranes was the proper preparation of agglomerate-free MWCNTs dispersions. Rotor-stator system (RS) and ultrasonic sonotrode (USS) were used to achieve the nanofillers' dispersion. In addition, the first results of the application of the three-roll mill (TRM) technology in the filler dispersion achieved were promising. The filler material, MWCNTs, was characterized by scanning electron microscopy (SEM) and liquid nitrogen (LN2) adsorption-desorption isotherms at 77 K. The derivatives CA-based mixed matrix membranes were characterized by tensile strength and water contact angle measurements, impedance spectroscopy, gas permeability/selectivity measurements, and water permeability tests. The studied membranes provide remarkable water permeation properties, 12-109 L/m2/h/bar, and also good separation factors of carbon dioxide and helium separations. Specifically, a separation factor of 87 for 10% He/N2 feed concentration and a selectivity value of 55.4 for 10% CO2/CH4 feed concentration were achieved.

Serological Biomarker Panel in Diagnosis of Atrophic Gastritis and Helicobacter pylori Infection in Gastroscopy Referral Patients: Clinical Validation of the New-Generation GastroPanel® Test.

BACKGROUND/AIM: Prompted by the increasing demand of non-invasive diagnostic tools for screening of gastric cancer (GC) risk conditions, i.e., atrophic gastritis (AG) and Helicobacter pylori (Hp) infection, the GastroPanel® test (GP: biomarker panel of PGI, PGII, G-17, Hp IgG ELISA) that was developed in the early 2000's, was recently updated to a new-generation (unified GP) test version. This clinical validation study evaluated the diagnostic accuracy of the new-generation GP test in detection of AG and Hp among gastroscopy referral patients in a University Clinic. PATIENTS AND METHODS: Altogether, 522 patients were enrolled among the patients referred for gastroscopy at the Gastro Center, Oulu University Hospital (OUH). All patients underwent gastroscopy with biopsies classified using the Updated Sydney System (USS), and blood sampling for GP testing. RESULTS: Biopsy-confirmed AG was found in 10.2% (53/511) of the patients. The overall agreement between the GP and the USS classification was 92.4% (95%CI=90.0-94.6%), with the weighted kappa (κw) of 0.861 (95%CI=0.834-0.883). In ROC analysis using moderate/severe AG of the corpus (AGC2+) as the endpoint, AUC=0.952 (95%CI=0.891-1.000) and AUC=0.998 (95%CI=0.996-1.000) for PGI and PGI/PGII, respectively. Hp IgG antibody ELISA detected biopsy-confirmed Hp-infection with AUC=0.993 (95%CI=0.987-0.999). CONCLUSION: The new generation GastroPanel® is a precise test for non-invasive diagnosis of atrophic gastritis and Hp-infection in dyspeptic patients referred for diagnostic gastroscopy.

Daily concordance between ecological stressors and sleep in young minority children during the pre-COVID-19 outbreak period.

Objective: As the COVID-19 pandemic brings widespread changes in families, the sociology of sleep becomes noticeable. Yet, the socio-contextual determinants of a biopsychosocial phenomenon as sleep are poorly investigated. We examine changes concomitantly occurring in the child's sleep per familial and community stressors. Methods: During the pre-COVID-19 outbreak period, in 24 minority children (5.4 ± 1.7 years old, 54.2% girls), sleep was objectively measured 24 h for two consecutive weeks, and this was repeated three times over the study period of three months. The caregiver filled out questionnaires surveying sociodemographic, community and family aspects. Results: Children went to bed at 22:26 and woke up at 07:04, with each a variability of about 50 min. Money and time were revealed as related key stressors to sleep. Five dimensions best fitted their association. In general, concurrent changes within the individual child indicate that mean sleep variables seem to relate to predominantly features of the stressors (explained variance of 34.7 to 56.7%), while variability of sleep tends to associate to situational aspects of the stressors (explained variance of 30.4 to 61.8%). Associations were best explained in terms of the 24 h dimension, particularly exposing sleep variability. Conclusion: Individual variabilities in a child's sleep are associated with familial resources, such as caregiver's time to self, money and basic needs. Time spent in bed, a modifiable factor by society and shaper of sleep quantity and quality, plays a key role in stressor-sleep associations. Insights from biopsychosocial perspectives may be valuable for understanding COVID-19 sleep studies, and the development of (post-) COVID-19 sleep recommendations.

Right-sided obstructed hemivagina ipsilateral renal agenesis (OHVIRA): A case report.

INTRODUCTION: Obstructed hemivagina ipsilateral renal agenesis (OHVIRA) is a rare anomaly of the urogenital system. The characteristic triad of this syndrome, which was initially reported in 1950, is didelphys uterus, obstructed hemivagina, and ipsilateral renal agenesis (Embrey, 1950 [1]). CASE: A 17-year-old girl was referred with a 6-month history of offensive vaginal discharge. Magnetic resonance imaging (MRI) established the diagnosis of OHVIRA. She underwent surgery for drainage of the hematocolpos and excision of the vaginal septum followed by an uncomplicated recovery and the patient had normal menstrual cycles after surgery. CONCLUSION: There should be a high suspicion of OHVIRA syndrome when encountering adolescent patients with non-specific abdominal or pelvic symptoms.

A comparative study between the Universal Spinal System® (USS) and the CD Horizon® Legacy™ (CDH) in the management of thoracolumbar fractures.

INTRODUCTION: For the treatment of unstable non-osteoporotic thoracolumbar fractures, the clinical and radiological outcome of short-segment fixation with the USS™ - Universal Spine System (DePuy Orthopedics, Inc., Warsaw, IN, USA) and the CD HORIZON® LEGACY™ 5.5 Spinal System, (Medtronic Sofamor Danek USA, Inc., Memphis, TN, USA) were compared. METHODS: From March 2015 to January 2016, 40 consecutive patients with unstable traumatic thoracolumbar fractures who met our inclusion criteria were treated with either the USS system or CDH Legacy system. Segmental kyphosis angle (SKA) and anterior body height (ABH) of fractured vertebrae, and ASIA Impairment Scale (AIS) were evaluated. Radiological fusion was confirmed with plain X-rays and when indicated with computerized tomography (CT). RESULTS: The mean immediate kyphotic angle correction was 16.6° for the Schanz and 6.4 for the Legacy system, and the immediate mean anterior vertebral body height correction was 0.92 cm for the Schanz and 0.51 cm for the Legacy system. Our study shows a significant statistical difference between Schanz and Legacy systems regarding post-operative segmental kyphosis and height correction immediately postoperatively, at 6 months and at one-year follow-up (p-value < 0.005). The degree of pain reduction and neurological improvement was not influenced by the screw system. CONCLUSION: Usage of USS in thoracolumbar fracture as a short-segment fixation led to a near anatomical reduction when compared to the Legacy system. However, there was no advantage regarding pain reduction and neurological outcome.

Complement activation associated with ADAMTS13 deficiency may contribute to the characteristic glomerular manifestations in Upshaw-Schulman syndrome.

INTRODUCTION: Upshaw-Schulman syndrome (USS) is a congenital form of thrombotic thrombocytopenic purpura (TTP) associated with loss-of-function mutations in the ADAMTS13 gene, possibly leading to aberrant complement activation and vascular injury. However, USS is extremely rare, and there have been no systematic studies correlating histopathological severity with local ADAMTS13 expression and complement activation. MATERIALS AND METHODS: Here, we compared histopathological features, ADAMTS13 immunoreactivity, and immunoreactivity of complement proteins C4d and C5b-9 among renal biopsy tissues from five USS cases, ten acquired TTP cases, and eleven controls. RESULTS: Pathological analysis revealed chronic glomerular sclerotic changes in the majority of USS cases (4 of 5), with minor glomerular pathology in the remaining case. In two of these four severe cases, more than half of the glomerular segmental sclerosis area was localized in the perihilar region. The average number of ADAMTS13-positive cells per glomerulus was significantly lower in USS cases than controls (p < 0.05). Conversely, C4d staining was significantly more prevalent in the glomerular capillary walls of USS cases than controls (p < 0.05), while C5b-9 staining did not differ significantly among groups. CONCLUSIONS: These findings suggest that the severity of glomerular injury in USS is associated with deficient ADAMTS13 expression and local complement activation, particularly in vascular regions with higher endothelial shear stress. We suggest that C4d immunostaining provides evidence for complement-mediated glomerular damage in USS.

Serological biomarker testing helps avoiding unnecessary endoscopies in obese patients before bariatric surgery.

BACKGROUND: To assess the value of serological biomarker testing as a substitute for esophagogastroduodenoscopy (EGDS) in pre-operative assessment of patients referred for bariatric surgery. METHODS: Sixty-five obese patients with a mean age of 43 years (range: 21-65) and a mean body mass index (BMI) of 44 (range: 36-59) were studied. The patients were tested with a four-biomarker panel: pepsinogen I and II, gastrin-17 (basal and stimulated), and Helicobacter pylori (HP) antibodies (GastroPanel®, Biohit Oyj, Finland). On the basis of the biomarker test, the patients were classified into the HS (healthy stomach) group (n = 22) with the normal biomarker profile and the NHS (non-healthy stomach) group (n = 43). The classification of patients into HS and NHS was evaluated against the gold standard, i.e. EGDS with biopsies. RESULTS: The concordance (Cohen's kappa) between the biomarker test and gastric histology was 0.68; 95% CI 0.504-0.854, with an overall agreement of 84.6% (95% CI 73.9-91.4%). In the NHS group, all 43 patients had biopsy-confirmed chronic gastritis: 39 non-atrophic HP-gastritis, 4 atrophic antrum gastritis (AGA) of moderate severity.In the HS group only 6 patients had mild superficial H.pylori negative gastritis. Of the 22 HS subjects with the normal biomarker profile, 20 (31% of all 65) had no complaints either, while the remaining two had reflux symptoms with esophagitis. In the NHS group 10 patients had esophagitis and 8 had also reflux symptoms. CONCLUSIONS: The normal biomarker profile is an excellent surrogate for healthy stomach, implicating that pre-operative EGDS could have been avoided in 31% of our asymptomatic bariatric surgery patients who had the normal biomarker profile.