Robert Hostoffer

• DO,FACOP,FAAP,FACOI,FCCP
• Professor (Associate) at Case Western Reserve University

Several genetic mutations have been linked to recurrent infections that vary in severity from mild to life threatening. A mutation of the hyou1 gene has been associated with a defective protein resulting in poor cellular homeostasis from stress related to lack of necessary substrates for energy production, which is known to lead to a unique pattern...

Bloom syndrome is an autosomal recessive disease caused by loss-of-function mutations in the BLM gene. Mutations in this gene cause deformations in the DNA helicase protein, which is functional in DNA repair. DNA repair is known to have an important role in the development of antigen receptors on B and T cells; therefore, DNA repair disturbances ma...

Context Chronic rhinosinusitis (CRS) is a prevalent inflammatory disease of the paranasal sinuses that may significantly impair quality of life. CRS may also benefit from the application of manual techniques through osteopathic manipulative treatment (OMT), which aims to improve venous and lymphatic circulation, sympathetic and parasympathetic outf...

Atopic dermatitis, more commonly known as atopic eczema, is a chronic, relapsing inflammatory skin disorder characterized by dry skin, localized erythematous rash, and intense pruritus. The clinical manifestations are variable and age dependent. As one of the most common skin disorders globally, atopic dermatitis poses a significant clinical and ec...

Background The CDC and ACIP recommend COVID-19 vaccination for patients with inborn errors of immunity (IEI). Not much is known about vaccine safety in IEI, and whether vaccination attenuates infection severity in IEI. Objective To estimate COVID-19 vaccination safety and examine effect on outcomes in patients with IEI. Methods We built a secure...

The Cleveland Academy of Osteopathic Medicine's (CAOM)

Context Anecdotal evidence suggested that osteopathic manipulative treatment (OMT) may have imparted survivability to patients in osteopathic hospitals during the 1918 influenza pandemic. In addition, previous OMT research publications throughout the past century have shown evidence of increased lymphatic movement, resulting in improved immunologic...

X-linked agammaglobulinemia (XLA) is a genetic disorder with mutation in Bruton’s tyrosine kinase (BTK). Defects in B cell development and immunoglobulin production lead to recurrent infections following loss of maternal IgG at 6 months of age. A 55-year-old male with a longstanding common variable immunodeficiency diagnosis on infusion therapy pre...

Background Selective anti-polysaccharide antibody deficiency (SPAD) with CD5 B-cell predominance and autoimmune phenomena was identified in a male cohort first reported by Antall et al in 1999. The phenotypically likewise and genotypically identical X-linked immunodeficiency with magnesium defect, Epstein–Barr Virus infection, and neoplasia (XMEN)...

Chronic granulomatous disease (CGD) is characterized by recurrent bacterial and fungal infections due to reduced nicotinamide adenine dinucleotide phosphate oxidase resulting in impaired production of reactive oxidant species, and aberrant inflammation. X-linked CGD (XL-CGD) is associated with a mutation in the CYBB gene. XL-CGD affects more males,...

Pneumonia is a common complaint in the US, especially among the elderly. Osteopathic manipulative treatment (OMT) is utilized for pneumonia. This study attempts to answer questions on effectiveness of OMT in terms of hospital length of stay (LOS) in the management of pneumonia in the elderly.

Vaccinations for pathogenic organisms have been utilized for decades in both the protection and diagnosis of immunodeficiency patients. Some of these immunodeficient patients may not create an adequate response to vaccination, although some who have significant aberrancies in their immune system may surprisingly create antibodies to immunizations....

IgM deficiency is characterized by remarkably low serum levels of IgM with normal IgG and IgA levels. These patients clinically present with recurrent infections, autoimmune disorders, and malignancies. While unknown, the proposed mechanisms explain the pathophysiology as an issue due to impaired IgG antibody response. The connexin genes encode for...

Introduction In a suburban allergy outpatient office, the practice of epinephrine rinse of subcutaneous allergen immunotherapy (SCIT) syringes prior to injection has been employed for over 30 years with documented favorable clinical outcomes. A retrospective study was conducted to explore the effectiveness of epinephrine rinsing of SCIT syringes in...

Structural skeletal abnormalities are associated with primary immunodeficient (PID) patients. These abnormalities have not been well studied in PID with reference to osteopathic medicine tenets. Osteopathic structural examinations of PID patients with respect to these tenets and the diagnosis of somatic dysfunctions preventing the free flow of lymp...

Introduction Selective IgM deficiency (SIGMD) is characterized by remarkably low serum levels of IgM with normal IgG and IgA levels. These patients clinically present with recurrent infections, autoimmune disorders, and malignancies. While unknown, the proposed mechanisms explain the pathophysiology as an issue due to impaired IgG antibody response...

Introduction Signal transducer and activator of transcription 3 (STAT3) is a transcriptional regulator that plays an important role in controlling the innate and adaptive immune system. Hyper IgE syndrome, caused by a STAT3 deficiency, is a rare disorder that commonly presents with connective tissue, skeletal, and vascular abnormalities. Known vasc...

Introduction Interleukin-21 (IL21) and its receptor (IL21R) are involved in activation of the JAK/STAT pathway and are necessary for B-Cell differentiation. Common variable immunodeficiency (CVID) is a disorder where the patient has hypogammaglobulinemia. There is a growing number of mutations associated with this disease, and we present a novel he...

Introduction Common variable immunodeficiency (CVID) is a clinically heterogeneous disorder with a growing list of genetic causes. The LPS Responsive Beige-Like Anchor (LRBA) gene encodes for a protein that is part of the CTLA-4 pathway important in immune regulation. Heterozygote mutations of this gene have been described in the literature in pati...

The clinical components of asthma are composed of three characteristics which include edema, mucous production, and airway contraction. These characteristics have been treated with various modalities which include beta-agonists, inhaled corticosteroids, muscarinic agonists, and leukotriene modifiers. Advances in the phenotyping and endotyping of as...

Autoimmune disorders arise from a failure of self-tolerance, or immunologic unresponsiveness to an individual’s own antigens, in genetically susceptible individuals. The mechanisms that result in tissue damage in autoimmune diseases parallel the normal responses of adaptive immunity and may include autoantibodies, immune complexes and/or autoreacti...

Background: Angiotensin-converting enzyme inhibitors are first-line drugs for patients suffering from hypertension. These drugs have common adverse effects including bronchospasm, cough, and angioedema. Drug-induced bullous eruptions have been reported previously with numerous drugs including lisinopril, an angiotensin-converting enzyme inhibitor....

Introduction Pneumonia is an infection of the lungs that can be fatal. In elderly the average length of stay for patients over 65 is reported as 5.6 days. Multiple efforts have been made to decrease the length of stay of hospitalized patients to reduce the economic burden as well as improve the overall quality of life for patients. Through a meta-a...

Purpose The rapid spread of SARS-CoV-2, the virus that is responsible for causing COVID-19, has presented the medical community with another example of when convalescent plasma (CP) is still used today. The ability to standardize CP at the onset of a pandemic is unlikely to exist in a reliable and uniformly reproducible way. We hypothesized that CP...

Background The coronavirus 2019 disease (COVID-19) has infected many individuals worldwide and continues to pose a significant threat to those with weakened immune systems. The data evaluating the clinical outcomes of patients with humoral immunodeficiencies that contract COVID-19 is limited and conflicting. Objective To describe the clinical outc...

Introduction Lime phytodermatitis, also known as margarita dermatitis, is a condition that results in a skin rash after sunlight exposure when handling certain plants. Misdiagnosis is common due to its resemblance to skin burns or allergic contact dermatitis. Detailed history and disease recognition is important to provide accurate treatment recomm...

Uniformity in a teaching hospital's pediatric outpatient clinic is often inconsistent because of the varying numbers of housestaff involved in patient evaluation. We have developed a flow sheet that defines the appropriate standards of care to be delivered at the Doctors Hospital Pediatric Outpatient Clinic. This flow sheet encompasses a method of...

Background: Novel messenger RNA vaccines against severe acute respiratory syndrome coronavirus (SARS-CoV-2) have been vital in resolving the coronavirus disease-2019 (COVID-19) pandemic. Detection of neutralizing antibodies (NAbs) against the SARS-CoV-2 spike protein (S) confirms immunogenicity with high sensitivity and specificity. Few recent stu...

Background STAT1 gain‐of‐function (GOF) is an immune dysregulatory disorder with poorly studied genotype‐phenotype correlation, impeding prognostication and early intervention. Given previous mechanistic studies, as well as anecdotal clinical reports, we sought to systematically determine whether DNA‐binding domain (DBD) mutations in STAT1 result i...

Introduction Different subtypes of vaccines have been developed to help protect populations from COVID-19. Currently, three vaccines have been authorized by the United States Food and Drug Administration for emergency use to combat the COVID-19 pandemic. With COVID-19 vaccination rates increasing, it is important to know whether immunodeficient pat...

Background: The coronavirus 2019 disease (COVID-19) has infected many individuals worldwide and continues to pose a significant threat to those with weakened immune systems. A paucity of data exists evaluating the clinical outcomes of patients with humoral immunodeficiencies that contract COVID-19. Objective: To describe the clinical outcomes of CO...

Background Anaphylaxis is a life-threatening allergic reaction that is often inadequately treated in the hospital setting, leading to adverse outcomes. We hypothesize that a brief educational intervention will enhance knowledge of community-based medical professionals evaluated by pre- and post-questionnaires, leading to improved recognition and ma...

Chronic spontaneous urticaria (CSU, chronic idiopathic urticaria) is a clinical diagnosis characterized by recurrent urticaria of unknown origin, with or without angioedema, that occurs for six weeks or longer. Management of CSU includes a second-generation H1 antihistamine and/or elimination of exacerbating factors. If initial treatment is unsucce...

Context Research is emphasized as a critical component of Accreditation Council for Graduate Medical Education (ACGME) Osteopathic Recognition (OR) criteria, yet there remains a deficit of osteopathic contributions to the literature. Scholar 12 combines discrete research development tools into an interactive application and blog forum that guides s...

Introduction Anaphylaxis to jackfruit (Artocarpus heterophyllus) is rare. Two previously reported cases have been published in two healthcare workers from jackfruit endemic regions. Latex allergy and birch pollen cross reactivity have both been associated with jackfruit anaphylaxis, providing two separate mechanisms of sensitization. We present a c...

Introduction The coronavirus disease 2019 (COVID-19) pandemic due to the novel severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) causes worldwide devastation. We describe the course of a patient with COVID-19 in the setting of an acquired humoral deficiency as a result of chemotherapeutic treatment for rheumatologic conditions. Case Rep...

Background: Nasal polyps (NPs) are inflammatory outgrowths of paranasal sinus mucosa that occur in one to four percent of the population and most commonly cause congestion, obstruction, or hyposmia. Intranasal corticosteroids, along with short courses of oral corticosteroids, are most often recommended for symptomatic nasal polyposis, prior to cons...

Introduction: Posner-Schlossman syndrome (PSS) is a rare glaucomatocyclitic crisis with clinical features including recurrent episodes of unilateral elevated intraocular pressure. Autoimmune and infectious causes have been proposed as potential etiologies of PSS. We report the first case of PSS in the setting of common variable immunodeficiency (C...

Asthma is among of the first ailments documented in the existing academic literature as being successfully managed with osteopathic manipulative treatment (OMT) techniques. Time-efficient and well-tolerated OMT techniques have been gradually added to the literature to manage this increasingly prevalent disease. In this narrative review, the authors...

Introduction Interleukin-1 (IL-1) antagonists have been successful in the management of monogenic auto-inflammatory diseases, notably classic hereditary fever syndromes, such as Familial Mediterranean Fever (FMF). Anakinra (Kineret®), a human recombinant IL-1 receptor antagonist (IL-1Ra), has been clinically effective in the management of persisten...

Familial cold inflammatory syndrome (FCAS) is a rare, inherited inflammatory disease characterized by episodes of fever, rash, and arthralgias after exposure to cold stimuli. Previous literature has established FCAS linked to autosomal dominant mutations in the NLRP3 (CIAS1) and NLRP12 genes. Moreover, there has been recent evidence of NLRC4-inflam...

Specific antibody immunodeficiency (SAD) is a primary immunodeficiency disorder characterized by normal levels of serum immunoglobulins (IgG, IgA, and IgM) associated with a dysfunctional immune response. SAD is associated with recurrent infections in the setting of an insufficient response to polysaccharide vaccinations. Streptococcus pneumoniae i...

Introduction Atopic dermatitis (AD, eczema) is familial chronic inflammatory skin disease of complex etiology and increasing prevalence. Dupilumab is an IL-4 receptor subunit alpha (IL-4Rα) antagonist that is the first Food and Drug Administration-approved biological therapy for moderate-to-severe adult AD inadequately controlled with topical thera...

Historically, osteopathic principles have focused on the appropriate drainage of cranial structures to relieve symptoms of rhinitis, which include nasal congestion, anterior/posterior rhinorrhea, sneezing, and itching. Allergic rhinitis is primarily an aberrant immunologic reaction caused by cytokines secreted from lymphocytes that traverse the lym...

The increasing availability of genetic testing for modern immunologists in the evaluation of immune diseases could provide a definite diagnosis in elusive cases. A 27-year-old white male patient presented to the clinic with recurrent sinopulmonary and cutaneous infections since childhood. The patient’s mother had seronegative polyarthritis, and one...

There have been few studies illustrating the post immunological phenotype of patients receiving autologous bone marrow transplant (ABMT) for the treatment of diffuse large B-cell lymphoma. High-dose chemotherapy and autologous bone marrow transplantation have been shown to be the only potential curative treatment modalities for B-cell lymphoma. Aut...

Context: Inefficiencies in care coordination-specifically, the lack of an effective method of communication among multiple health care professionals-often leads to an unnecessary increase in length of hospital stay. Objective: To determine whether daily integrated care conferences (ICCs) would significantly reduce the length of stay for patients...

Background: Common variable immunodeficiency (CVID) is a heterogeneous group of primary immune deficiency disorders that may be characterized by heightened susceptibility to gastrointestinal (GI) infection. GI conditions manifest in 20 to 50% of CVID patients but rarely include cholangitis. Methods/Results: This is a 61-year-old female, with a hist...

This protocol for rapid desensitization to intravenous radiographic contrast material (RCM) improves the strategy first reported by Uppal et al. Desensitization is a validated preventative measure for medical emergencies, such as cardiac catheterization, when patients present with histories of anaphylactoid reactions to the allergen of concern. The...

Introduction: Alpha (⍺)-gal syndrome (AGS) is an immunoglobulin E (IgE) antibody response against the glycoprotein carbohydrate galactose-⍺-1,3-galactose-β-1-(3)4-N-acetylglucosamine-R (Gal⍺-1,3Galβ1-(3)4GlcNAc-R or ⍺-gal) that is present in Ixodida (tick) saliva and noncatarrhine mammals as well as cetuximab, antivenom, and the zoster vaccine. Th...

Introduction: Allergic rhinitis (AR) is a widely prevalent immunoglobulin E-mediated inflammatory nasal condition resulting from reexposure to an allergen in a sensitized individual. The genetic associations behind AR and other allergic conditions have been studied. However, familial success with AR therapies, specifically allergen desensitization...

Stimulation of the immune response after vaccination can occasionally result in unwanted side effects, including demyelination of the central nervous system. The most common presentation of post-vaccination demyelination is acute disseminated encephalomyelitis, but cases of optic neuritis, transverse myelitis, and multiple sclerosis relapses have b...

Asthma is one of the most common chronic syndromes worldwide (Moorman et al., Vital Health Stat 3(35), 2012). It is not a diagnosis but a clinical syndrome based on a constellation of signs and symptoms (Li et al., Ann Allergy Asthma Immunol 81:415–420(IIa), 1998). The classic symptoms of asthma include chest tightness, wheeze, cough, and dyspnea (...

Asthma is one of the most common chronic syndromes worldwide (Moorman et al., Vital Health Stat 3(35), 2012). It is not a diagnosis but a clinical syndrome based on a constellation of signs and symptoms (Li et al., Ann Allergy Asthma Immunol 81:415–420(IIa), 1998). The classic symptoms of asthma include chest tightness, wheeze, cough, and dyspnea (...