High Immunoglobulin Research Articles

Sesquiterpene-Loaded Co-Polymer Hybrid Nanoparticle Effects on Human Mast Cell Surface Receptor Expression, Granule Contents, and Degranulation.

Biodegradable polymeric nanoparticles (NPs) such as poly(lactic-co-glycolic acid) (PLGA) and polyvinyl alcohol (PVA) have been used as drug delivery systems for natural and synthetic compounds and are designed to control the loading and release of biodegradable materials to target cells, tissues, and organs. Eremophilane-type sesquiterpenes have anti-inflammatory properties but are lipophilic, cytotoxic, and not biocompatible with many cells. To determine whether biodegradable PLGA/PVA could improve the biocompatibility of sesquiterpenes, sesquiterpene-loaded NPs were synthesized and their effects on human mast cells (LAD2), the major effector cells of allergic inflammation, were determined. NPs composed of PLGA/PVA and two types of sesquiterpenes (fukinone, PLGA/PVA-21 and 10βH-8α,12-epidioxyeremophil-7(11)-en-8β-ol, PLGA/PVA-22) were produced using a microfluidic synthesis method. The NPs’ size distribution and morphology were evaluated by dynamic light scattering and cryogenic transmission electron microscopy (TEM). PLGA/PVA-21 and PLGA/PVA-22 were 60 to 70 nm and were readily internalized by LAD2 as shown by flow cytometry, fluorescence microscopy, and TEM. While unencapsulated sesquiterpenes decreased LAD2 cell viability by 20%, PLGA/PVA-21 and PLGA/PVA-22 did not alter LAD2 viability, showing that encapsulation improved the biocompatibility of the sesquiterpenes. PLGA/PVA-21 and PLGA/PVA-22 decreased the expression of genes encoding the subunits of the high affinity immunoglobulin E receptor (FcεR1α, FcεR1β, FcεR1γ) and the stem cell factor receptor (Kit,), suggesting that hybrid NPs could alter mast cell responses to antigens and shift their maturation. Similarly, PLGA/PVA-21 and PLGA/PVA-22 inhibited tryptase expression but had no effect on chymase expression, thereby promoting a shift to the tryptase-positive phenotype (MCT). Lastly, PLGA/PVA-21 and PLGA/PVA-22 inhibited mast cell degranulation when the LAD2 cells were activated by IgE crosslinking and FcεRI. Overall, our results suggest that PLGA/PVA-21 and PLGA/PVA-22 alter human mast cell phenotype and activation without modifying viability, making them a more biocompatible approach than treating cells with sesquiterpenes alone.

Successful mild brain hypothermia therapy followed by targeted temperature management for pediatric hemorrhagic shock and encephalopathy syndrome.

Hemorrhagic shock and encephalopathy syndrome (HSES) is the most severe form of acute encephalopathy that progresses rapidly, often resulting in death or severe neurological sequelae. We report the case of a 4-year-old girl with HSES with shock and impaired consciousness. Blood test results showed hypercytokinemia, and the 4-year-old patient was immediately admitted to the intensive care unit. Within 4 h of symptom onset, she received mild brain hypothermia therapy with a target body temperature of 35°C. Methylprednisolone pulse, high dose immunoglobulin, and large doses of circulatory drugs were administered. After 72 h of brain hypothermia therapy, targeted temperature management with a target body temperature between 36°C and 37°C was continued for 96 h. The patient was diagnosed with HSES based on acute encephalopathy with shock, hypercytokinemia, low platelet count, coagulation disorder, renal damage, and intestinal bleeding. Magnetic resonance imaging results revealed no signs of any specific acute encephalopathy. She was discharged without neurological sequelae 28 days after symptom onset. Mild brain hypothermia therapy initiated in the early stages followed by targeted temperature management may be an effective way to improve neurological outcomes in children suffering from HSES.

Serum-free immunoglobulin E: A useful biomarker of atopy and type 2 asthma in adults with asthma

BackgroundIt has been known that a high serum total immunoglobulin E (IgE) level is a predisposing factor of allergic asthma; however, there are considerable limitations to apply it in clinical practice. ObjectiveTo determine the clinical significance of the serum-free IgE level in patients with adult asthma. MethodsWe measured free IgE levels using our homemade enzyme-linked immunosorbent assay by applying a novel IgE TRAP protein (GI innovation, Seoul, Republic of Korea) in sera of adults with asthma (n = 116) compared with healthy controls (n = 32); enzyme-linked immunosorbent assay inhibition test was performed to validate its binding specificity. Associations between asthma-related clinical and laboratory parameters were analyzed. The diagnostic value and cutoff point for detecting atopy and type 2 asthma were determined using receiver operating characteristic curve analysis. ResultsThe serum-free IgE levels were significantly higher in adults with asthma than in healthy controls and were significantly associated with atopic status and type 2 asthma (all P < .001). In the receiver operating characteristic analysis, serum-free IgE had a significantly greater area under the curve (AUC) than serum total IgE for assessing asthma, especially type 2 asthma (AUC, 0.810 vs 0.743; P = .006 and AUC, 0.729 vs 0.572; P < .001). The optimal cutoff points for predicting atopy and type 2 asthma were 82.8 and 120.8 ng/mL, respectively. ConclusionIt is suggested that a higher serum-free IgE level may be a useful biomarker of atopy and type 2 asthma in adults with asthma.

Treating Pediatric Myocarditis with High Dose Steroids and Immunoglobulin

Purpose Though many pediatric centers use immunotherapy including immunoglobulin (IVIG) and corticosteroids to treat patients with acute myocarditis, given a lack of evidence supporting its efficacy there is considerable variability in practice. We report our approach using high dose steroids with IVIG to treat acute myocarditis. Methods This is a single center retrospective observational study of children diagnosed with myocarditis treated with high dose steroids and IVIG from January 2004-April 2020. Diagnostic criteria for myocarditis included positive endomyocardial biopsy, MRI meeting Lake Louise criteria, or clinical diagnosis including an elevated troponin, LVEDD Z score Results ty one patients met inclusion criteria. Median age at diagnosis was 10.7 years (1 wk-20 yrs). The majority were male (65%). Diagnosis was made clinically in 68% of cases (N=21), MRI in 16% (N=5) and biopsy in 16% (N=5). Ten patients had positive respiratory viral PCR, 6 had positive plasma viral PCR and 0 had positive biopsy viral PCR. Median EF at diagnosis was 41% (IQR 10-65). Mean duration of steroids received was 74 days (SD 13) with IV steroids for 7 days (IQR 3-13) prior to transition to an enteral regimen. The mean cumulative dose of steroids for first 7 days was 38 mg/kg. All patients received IVIG; median cumulative dose was 2 g/kg. Seven patients (23%) required mechanical circulatory support (5 ECMO, 1 Centrimag LVAD and 1 Centrimag BiVAD). There were no serious secondary bacterial. In-hospital mortality was 0%. Mean follow up was 2 years (SD=2yr); 1 patient died 2 years after discharge; 1 patient underwent transplant during initial hospitalization; no others were listed. Five required re-admission for cardiovascular reasons. Six patients (19%) had steroid course altered due to suspected side effects such as weight gain or hypertension which resolved at follow up. By 3 months from diagnosis, 84% (N=26) of patients regained normal LV function. Conclusion Our study demonstrates that use of high dose steroids with IVIG to treat myocarditis can be safe without significant infections or side effects. Our cohort had excellent recovery of ventricular function and survival without transplant (94%). Prospective comparison of a combination of high dose steroids with IVIG versus either therapy alone is needed.

The potential significance of high avidity immunoglobulin G (IgG) for protective immunity towards SARS-CoV-2

BackgroundAvidity is defined as the strength of binding between immunoglobulin G (IgG) and its specific target epitope. IgG of high avidity is established during affinity maturation. Failure to achieve high avidity IgG may result in a lack of protective immunity towards infection and disease. It is known that the interaction between SARS-CoV-2 spike protein and its cellular receptor is driven by high affinity. Therefore, it is predictable that protective antibodies towards SARS-CoV-2 should show high affinity/avidity. Avidity after SARS-CoV-2 infectionRecent findings by several groups demonstrate that the serological response towards infection with SARS-CoV-2 and seasonal coronaviruses is characterized by incomplete avidity maturation, followed by a decline of the serological response. This response might facilitate reinfection, prevent herd immunity and potentially allow repeated cycles of infection. Consequences for vaccination towards SARS-CoV-2Therefore, the sole focus on antibody titers reached after vaccination towards SARS-CoV-2 might not be sufficient to evaluate the degree of achieved protection. Rather, it is suggested to include avidity determination to optimize vaccination protocols and achieve high avidity IgG directed towards SARS-CoV-2 through vaccination. Avidity determination might also be useful to control for truly protective immunity towards SARS-CoV-2 in individual cases.

Predictive Value of Radiologic Central Compartment Atopic Disease for Identifying Allergy and Asthma in Pediatric Patients.

Central compartment atopic disease (CCAD) has recently been suggested as a phenotype of chronic rhinosinusitis (CRS). This study aims to investigate the prevalence of the radiologic CCAD phenotype in CRS within a pediatric population and identify its ability to predict comorbid allergy and asthma. Computed tomography and endoscopic examination were conducted on pediatric patients with CRS either with or without nasal polyps. Allergen sensitization was determined with the multiple-allergen simultaneous test and skin prick test. Serum total immunoglobulin E (IgE), peripheral blood eosinophil percentage, and presence of asthma were also evaluated. A total of 82 pediatric patients were enrolled. Overall, 55 (67.1%) of the participants demonstrated aeroallergen sensitization, and 31 (18.9%) of the 164 sides of sinuses were radiologically defined to fit the CCAD phenotype. Patients having CRS with the CCAD phenotype had a higher prevalence of aeroallergen sensitization (87.1% vs 62.4%, P = .008), particularly house dust mite (74.2% vs 53.4%, P = .035), and a higher incidence of asthma (16.1% vs 3.8%, P = .010). Additionally, patients having CRS with the CCAD phenotype demonstrated a high serum total IgE levels (51.6% vs 30.1%, P = .023) in comparison to patients having CRS without CCAD. In pediatric CRS, the radiological CCAD phenotype was associated with allergen sensitization and asthma. Furthermore, the CCAD phenotype was associated with high serum total IgE levels, suggesting allergy etiology should be considered with this type of pediatric patients with CRS.

IL-35 and IL-37 are negatively correlated with high IgE production among children with asthma in Saudi Arabia

Background: Asthma is one of the most prevalent inflammatory disorders among children in Saudi Arabia.Objective: This study aimed to determine the correlation between the serum levels of vitamin D, immunoglobulin E (IgE), and cytokine (interferon-gamma (IFN-γ), interleukin (IL)-1β, IL-4, IL-6, IL-10, IL-13, IL-35, and IL-37) in relation to the severity of disease in patients with asthma. Methods: This case-control study was carried out at King Abdullah Specialist Children’s Hospital, Saudi Arabia, and included 48 patients with asthma and 47 matched controls, aged 6–14 years. A validated questionnaire was administered to the participants, after which each patient with asthma underwent pulmonary function tests. The serum levels of vitamin D, IgE, IFN-γ, IL-1β, IL-4, IL-6, IL-10, IL-13, IL-35, and IL-37 of each participant were also measured.Results: Patients with asthma demonstrated significantly higher IgE and cytokine (IL-1β, IL-4, IL-6, IL-10, IL-13, IL-35, and IL-37) levels compared to the control group (p value < .001). The levels of IL-1β, IL-4, IL-10, and IL-13 were consistently positively correlated with the serum levels of IgE among patients with asthma. However, the IgE levels in patients with asthma were consistently negatively correlated with IL-35 and IL-37. Conclusions: We found significantly higher levels of eosinophils, IgE, IL-1β, IL-4, IL-6, IL-10, IL-13, IL-35, and IL-37 in patients with asthma compared to the controls, but no relationship between vitamin D and asthma.

Mast Cells Are the Trigger of Small Vessel Disease and Diastolic Dysfunction in Diabetic Obese Mice.

[Figure: see text].

Sudden Chest Pain Due to Esophageal Intramural Dissection

Sudden Chest Pain Due to Esophageal Intramural Dissection

Human peripheral basophils extended phenotype shows a high expression of CD244 immuno-regulatory receptor

IntroductionBasophils play a major physio-pathological role in hypersensitivity related diseases. Basophils express high affinity Immunoglobulin (Ig) E receptors (FcεRI), IgG and complement regulatory. Basophils also have immunoregulatory activity through interaction with T cells. The aim of this study was to look for the expression of markers reflecting the activation status of peripheral Basophil in healthy donors. Methodthe study was performed on 29 healthy donors, 62% females with a mean age of 50.1 + 17.0 years. Basophils were identified on their expression of CD123 without HLA-DR and/or CD193 in two 8 colors panels including CD46, CD55, CD59, CD203c, CD32 (FcγRII), CD64 (FcγRIII), CD163, CD137L (4-1BBL), CD252 (OX40L), CD244 (2B4) and CD3 on whole blood. Basophil activation with anti IgE was performed on 14 donors. Results and discussionOur results confirmed the Basophil expression of CD123, CD193 and CD203 (the latter is strongly increased under stimulation). Complement regulatory proteins (CD46, CD55, CD59) were expressed at the same levels as on other leukocytes; CD46, CD59 expression being slightly increased under stimulation. CD32 and CD163 scavenger were slightly higher than on lympho and not influenced by activation. CD252 or CD137L were expressed at low levels and significantly induced by stimulation. Most of all, CD244 was highly expressed on Basophils as compared to any other leukocytes in fresh peripheral blood. ConclusionsOur study shows that human resting Basophils express IgE and IgG Fc receptors and check point receptor CD244 that could potentially play a role in their previously reported immunoregulatory activity in sensitization and even in tumor immune escape.

Recurrent necrotizing cellulitis, multi-organ autoimmune disease and humoral immunodeficiency due to a novel NFKB1 frameshift mutation

BackgroundMutations in NFKB1(nuclear factor of kappa light polypeptide gene enhancer in B-cells 1) are associated with a variety of clinical symptoms, including lymphadenopathy, splenomegaly, hepatomegaly, autoimmune haemolytic anaemia, arthralgia, recurrent respiratory tract infections and post-operative necrotizing cellulitis. Case presentationWe describe a case of a 47-year-old man, who presented with deep necrotizing cellulitis after incision of a submucous abscess by a dentist. Surgical intervention led to a massive progress. Pyoderma gangraenosum (PG) was diagnosed clinically and confirmed histopathologically. High dose corticosteroids and intravenous immunoglobulins (IVIG) improved wound healing dramatically. Until now, immune mediated inflammation events not only affected the skin, but also multiple inner organs, i.e. the heart, lungs and gut. Sequencing of all coding exons of NFKB1 revealed a heterozygous 1bp deletion in exon 23 predicting a frameshift starting at codon Ala891 and resulting in a subsequent stop codon at position 6 in the new reading frame: NM_003998.4: c.2671del; p.(Ala891Glnfs*6) Acute episodes were always successfully treated with corticosteroids, IVIG and concomitant antibiotics. To prevent further exacerbations, the patient receives IVIG once a month, low-dose corticosteroids and methotrexate. ConclusionThis is the first case of a patient with recurrent necrotizing cellulitis and immune mediated multi-organ involvement (heart, lungs, intestine) carrying the novel frameshift mutation c.2671del (p.Ala891Glnfs*6) in NFKB1 effectively treated with IVIG, low-dose corticosteroids and methotrexate.

Engineering a reporter cell line to mimic the high oligomannose presenting surface immunoglobulin of follicular lymphoma B cells

Subtypes of B cell non-Hodgkin’s lymphomas, including follicular lymphomas, have shown a unique high oligomannose presentation on their immunoglobulins that will interact with natural receptors of the innate immunity, reportedly causing stimulation and proliferation. From deep sequencing of the variable heavy and light chain sequences of follicular lymphoma involved tissue sections, we identified the consensus variable sequences possessing glycosylation sites at the complementarity determining region. Using this information, we developed a cell line, referred to here as BZ, which displays the consensus variable segments as part of a surface antibody (IgM) and confirmed its presentation of high oligomannose on the heavy chain both in vitro and in vivo. An mCherry expressing variant provided a reporter cell line displaying the high oligomannose surface biomarker while affording clear fluorescent signals for FACS screening as well as for fluorescent in vivo imaging of ectopic xenograft tumors. In developing this reporter cell line that displays the biomarker glycan of follicular lymphoma, we provide a tool that may be used for future screening and validation of receptive moieties for selectively binding high oligomannose for development of targeted diagnostics or therapeutics to such B cell malignancies that display this unique glycan.

High level of IgE in acute low-tone sensorineural hearing loss: A predictor for recurrence and Meniere Disease transformation

BackgroundAutoimmunity may play an important role in sudden onset sensorineural hearing loss. However, little is known about the relationship between immunoglobulin E (IgE) and acute low-tone sensorinerual hearing loss (ALHL). ObjectivesTo investigate the relationship between IgE level and endolymphatic hydrops and outcomes of ALHL. MethodsA total of 242 subjects with sudden onset hearing loss, including 115 with ALHL and 127 with idiopathic sudden sensorineural hearing loss (ISSHL), were included in this study. Peripheral venous blood samples of 242 subjects were collected for detection. Clinical data, IgE level, and distribution of allergens were compared between the ALHL and ISSHL groups. The ALHL group received an electrocochleogram (ECochG) test and a follow-up in the outpatient unit or by telephone to evaluate outcomes. ResultsCompared to the values in the ISSHL group, a significantly younger onset age (42.30±14.33 years old), higher female onset proportion (72/115, 62.61%), increased total IgE level (median: 66.47, interquartile range: 24.56, 180.96, IU/mL) and specific IgE level (median: 9.42, interquartile range: 1.42, 22.23 IU/mL) were noted in the ALHL group. A clear difference in allergen distribution was noted between the ALHL and ISSHL groups (p=.001). Total IgE and specific IgE levels were factors that contributed to the SP/AP ratio in the electrocochleogram (ECochG) (R2=0.413) in ALHL group. Finally, during the follow-up (17.61±3.46 months) for the ALHL group, 37 subjects recurred, and 17 subjects developed Meniere Disease. In the ROC curve for ALHL recurrence, the area under the curve (AUC) of total IgE was 0.709 and that of specific IgE was 0.679. For MD transformation, the AUC of total IgE was 0.736 and that of specific IgE was 0.716. ConclusionsHigh IgE levels correlated with an enhanced SP/AP ratio in ALHL. High IgE levels could be used as a predictor of ALHL recurrence and MD transformation.

Total IgE as a Marker for Chronic Spontaneous Urticaria.

ObjectiveImmunoglobulin E (IgE) and its receptor, FcɛRI, importantly contribute to the pathophysiology of chronic spontaneous urticaria (CSU). Recent findings point to a possible role of total IgE as a marker of CSU disease activity, endotypes, and responses to treatment. The evidence in support of total IgE included in the diagnostic workup of patients with CSU has not yet been reviewed.MethodsPublications were searched via PubMed. The search terms used were “chronic urticaria” and “total IgE.” Studies were screened by titles and abstracts, and 141 were used in the review.ResultsCSU patients frequently had elevated total IgE serum levels (up to 50%), but normal or very low total IgE levels also occurred. High total IgE may represent high disease activity, longer disease duration, high chance of responding to omalizumab treatment, quick relapse after stopping omalizumab, and lower chance of responding to cyclosporine. Low IgE, in contrast, may suggest Type IIb autoimmune CSU, poor response to treatment with omalizumab and a better chance to benefits from cyclosporine treatment. Furthermore, IgE in different CSU cohorts may have different physicochemical properties that could explain differences in treatment responses to IgE-directed therapies.ConclusionThe results of our review suggest that total IgE is a valuable marker for CSU, and we recommend its assessment in the routine diagnostic workup of CSU patients.

Physiology and miRNA expression in confined sows with different pupillary light reflex

Long-term physical restriction may lead to affective and physiological disorders in sows; pupillary light reflex (PLR) characteristics might be a good indicator to diagnose these symptoms. Accordingly, the physiological and psychological states of sows with different PLR characteristics were investigated in this study. Gestating sows of three parities (parity 0, 2, and 5) were divided into strong reflex (SR) and weak reflex (WR) groups, according to a PLR test. In each group, miRNA expression and serum physiological indices were detected. Compared with the SR group, WR group showed lower 5-hydroxytryptamine levels and higher cortisol, interleukin-6, and beta-endorphin levels (p < .05) in all parities; higher immunoglobulin A and tumour necrosis factor-α levels in the parity 0 and 2 sows (p < .05); and lower miR-335, miR-16, and miR-1202 expression (p < .05) and higher miR-132, miR-504, miR-34a, and miR-30e expression in the parity 2 and 5 sows. Compared with the parity 0 sows, parity 5 sows showed higher cortisol, interleukin-6, tumour necrosis factor-α, and beta-endorphin levels; higher miR-504 and miR-34a expression (p < .05); and lower miR-335, miR-16, miR-134, and miR-1202 expression in both groups. Thus, psychophysiological state differs among sows with different PLR characteristics, and, that of sows with weak PLR characteristics, is consistent with depression disorder, and as parity increases, these psychophysiological differences widen. HIGHLIGHTS PLR characteristics could reflect different physiological states including neurophysiological and immunological states in sows. The miRNA molecular markers and serum physiological indices of sows with weak pupillary light responses were consistent with depression disorder. Long-term physical restriction might worsen physiological and psychological disorders in sows.

Yoga as an Exercise Prescription for the Pregnancy or Postpartum Period: Recent Advances and Perspective

The aim of this study is to examine studies published from 2015 to 2020 and to determine whether yoga can be an efficacious approach during the pregnancy or postpartum period. PubMed, EBSCO, Wiley and Science Direct databases were searched for studies published from January 2015 to June 2020. A total of 14 studies met the inclusion criteria, covering 1116 participants were identified. The results demonstrated that yoga intervention could significantly reduce depression (P < 0.001), anxiety (P = 0.003), labor pain (P = 0.001), back pain and the percentage of cesarean section (P = 0.002). There was significant improvement in psychological well-being (P < 0.5), immune function and the intrauterine fetal growth (P < 0.5). Moreover, the yoga intervention group has lower salivary cortisol (P < 0.001), salivary alpha-amylase and salivary a-amylase levels (P < 0.5) and higher immunoglobulin A (P < 0.001) levels when compared with that in control groups. The findings suggested that yoga is well benefited for either pregnant women or postpartum women. More high-quality and well-controlled randomized controlled trials are required to provide more information regarding the utility of yoga interventions for different stages of pregnancy women.

Cat-NPC2, a Newly Identified Allergen, With High Cross-Reactivity to Can f 7.

PurposePet-derived allergens are the common indoor inhalant allergens. Among them, cat and dog allergens constitute more than 80% of animal allergic patients, which greatly affect the quality-of-life of patients and increase the burden of social health care. The aim of this study was to identify Cat-Niemann pick type C2 (NPC2) protein, a homologue of Can f 7, as a new allergen.MethodsCat-NPC2 complementary DNA (cDNA) was cloned and optimized for amplification and expression in Escherichia coli. Then, recombinant Cat-NPC2 (rCat-NPC2) was purified by Ni2+ affinity chromatography. The allergenicity was assessed by enzyme-linked immunosorbent assay (ELISA), western blot and basophil activation test (BAT). Based on the sequence similarity, the cross-reactivity between Cat-NPC2 and Can f 7 was investigated by inhibition ELISA. Circular dichroism spectroscopy and homology modeling were used to characterize the structure of Cat-NPC2.ResultsThe cDNA sequence of Cat-NPC2 was cloned with a 450-bp open reading frame coding for 149 amino acids (GenBank MN_737596). The condon-optimized NPC2 gene was subcloned and expressed in E. coli with a molecular weight of 18.9 kDa. The native Cat-NPC2 was detected in cat dander extracts. The allergenicity determined by ELISA, western blot and BAT suggested at least 14.5% cat-allergic patients displayed high specific immunoglobulin E (IgE) recognition of Cat-NPC2. The predicted structure of Cat-NPC2 was found to consist of 7 β-strands arranged in 2 β-sheets. An ELISA based assay showed that rCat-NPC2 bound to cholesterol in a dose dependent manner. Based on the structure and sequence similarities, IgE cross-reactivity was demonstrated between Cat-NPC2 and Can f 7/Der f 2.ConclusionsIn the study, a novel cat allergen, belonging to the NPC2 protein family, was identified and characterized at both molecular and immunological levels. The study will offer a deeper understanding of cat allergens and improve a component-resolved diagnosis in pet allergy.

Idiopathic systemic capillary leak syndrome (Clarkson’s disease) presenting with recurrent hypovolemic shock

A 49-year old male with a past medical history of myocardial infarction and compartment syndromes requiring fasciotomies presented on five occasions with hypovolemic shock. We describe his admissions and presumptive diagnoses which required large volumes of intravenous fluids, admission to intensive care for vasopressors and renal replacement therapy. The presentations were always precipitated by a prodrome of fatigue and pre-syncopal episodes. On his last admission, a diagnosis of Idiopathic systemic capillary leak syndrome (ISCLS), also known as Clarkson’s Disease, was reached. He is currently receiving high dose intravenous immunoglobulins on a monthly basis.

No diffuse intrahepatic biliary stricture after ABO-incompatible adult living donor liver transplantation using tailored rituximab-based desensitization protocol.

BackgroundRituximab (RTx) desensitization protocol offered good outcome in ABO-incompatible (ABOi) living donor liver transplantation (LDLT). However, diffuse intrahepatic biliary stricture (DIHBS) is still inevitable hurdle. We selectively added postoperative high dose intravenous immunoglobulin (IVIG) and/or simultaneous splenectomy if ABO isoagglutinin titer just before liver transplantation after plasma exchange (PE) was higher than 1/16. Herein, we reported the excellent outcome of ABOi LDLT without DIHBS using tailored desensitization protocol and compared it with that of ABO-compatible (ABOc) LDLT.MethodsSixty-five cases (14.8%) of ABOi LDLTs were performed among 438 primary adult LDLTs in our center between March 2012 and June 2017. We performed 1-to-2 propensity score matching (PSM) to extract 60 cases of ABOi LDLTs and 120 cases of ABOc LDLTs.ResultsThere were no significant differences in clinical characteristics between ABOi and ABOc recipients. There were no significant differences in complications and rejection. There was no DIHBS in both groups. The 1-, 3-, and 5-year overall survival rates were 98.3%, 86.7%, and 82.9% in ABOi group and 96.7%, 86.7%, and 85.4% in ABOc group, respectively (P=0.88). Most common cause of deaths of both groups was hepatocellular recurrence. The 1-, 3-, and 5-year biliary complication (anastomosis leakage or stricture) free survival rates were 81.4%, 69.5%, and 67.5% in ABOi group and 83.0%, 81.3%, and 80.0% in ABOc group, with no significant differences (P=0.11).ConclusionsRTx-based tailored (optional IVIG + splenectomy) desensitization protocol for ABOi LDLT was feasible and acceptable.

Strategy of endoscopic skull base reconstruction in endoscopic transsphenoidal approaches

Background Saline Water Conversion Corporation (SWCC) is the main source of water in the Red Sea coast in Jeddah, KSA, the workers and their families are exposed to vapor and chemicals that affect their upper respiratory tract. Aim Our aim was to study the prevalence of unified airway disease [allergic rhinitis (AR) and bronchial asthma] in SWCC Society accommodations, serum immunoglobulin E (IgE) level and its relation to the duration of bronchial asthma, and duration of living in the accommodations. Patients and methods This randomized study included 400 patients with bronchial asthma, the included patients comprised two groups: group I (GI) (n=200) from SWCC Society accommodations and group II (GII) (n=200) patients from outside the accommodations. Both groups were classified according to Allergy Classification of allergic rhinitis and its impact on asthma and blood samples for IgE taken from them. Results According to the classification of allergic rhinitis and its impact on asthma, the persistent and moderate–severe allergy were more common in GI (80%) and the intermittent allergy and mild severity was 20%, while the persistent and moderate–severe allergy were less common in GII (22%) and the intermittent allergy and mild severity was 78%. GI patients experienced a significantly high IgE level compared with GII, higher in males than females, and increases with the duration of living in the accommodations and duration of bronchial asthma. Conclusion Unified airway disease is common in SWCC Society with a high level of IgE compared with outside the accommodations. Keywords: allergic rhinitis, bronchial asthma, environmental diseases, serum total immunoglobulin E.