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1.
BMJ Case Rep ; 16(12)2023 Dec 07.
Article in English | MEDLINE | ID: mdl-38061856

ABSTRACT

Behcet's disease (BD) is a rare autoimmune and autoinflammatory disease characterised by recurrent oral and genital aphthous ulcers as well as gastrointestinal, ocular, neurological, articular and vascular inflammation. Patients are at risk for both thrombotic events and bleeding, so providers are often challenged in deciding whether to start patients on anticoagulation. We report a rare, complex case of a patient with BD who presented with massive gastrointestinal bleeding. This case report highlights the management of recurrent thrombosis due to protein C and S deficiency in a patient with BD who also presents with acute gastrointestinal bleeding.


Subject(s)
Behcet Syndrome , Thrombosis , Humans , Behcet Syndrome/complications , Behcet Syndrome/diagnosis , Thrombosis/diagnostic imaging , Thrombosis/drug therapy , Thrombosis/etiology , Gastrointestinal Hemorrhage , Acute Disease
2.
J Investig Med High Impact Case Rep ; 8: 2324709620949293, 2020.
Article in English | MEDLINE | ID: mdl-32806974

ABSTRACT

Lipomas are the most common benign soft tissue tumor. Yet, strikingly simple tumors can become problematic when compounded by odd characteristics such as size and location. We report the case of a 53-year-old male who developed complete right lung collapse secondary to a large right-sided chest wall lipoma with accelerated growth in the past 6 months. Bronchoscopy revealed extrinsic compression of the right mainstem bronchus. Histopathology of the soft tissue mass was suggestive of a lipoma. The mass was not amenable to surgery due to a high risk of mortality from his underlying comorbidities. His hospital stay was complicated by progressive end-stage restrictive lung disease necessitating intubation and eventually a tracheostomy, recurrent pneumonias, multiorgan dysfunction, and his eventual demise. We highlight a rare presentation of an unchecked lipoma, which ultimately led to the death of our patient. Simple lipomas show insidious growth and can remain asymptomatic until they reach a large size. Chest wall tumors should be considered malignant until proven otherwise by excisional biopsy. This reiterates the need to treat all chest wall tumors with wide resection in order to provide the best chance for cure.


Subject(s)
Lipoma/complications , Lung Diseases/etiology , Renal Insufficiency/etiology , Thoracic Neoplasms/complications , Thoracic Wall/pathology , Bronchoscopy , Disease Progression , Fatal Outcome , Humans , Lipoma/pathology , Male , Middle Aged , Thoracic Neoplasms/pathology , Tomography, X-Ray Computed
3.
Ann Transl Med ; 8(14): 887, 2020 Jul.
Article in English | MEDLINE | ID: mdl-32793731

ABSTRACT

Here, we present a 22-year-old female patient with adult-onset Still's disease (AOSD) who was newly diagnosed in the setting of secondary macrophage activation syndrome (MAS), a rare, life-threatening inflammatory disease with 50% mortality due to multi-organ failure. She met the diagnostic criteria of AOSD and MAS, while genetic testing excluded primary causes of MAS. She had high fevers, anemia, thrombocytopenia, splenomegaly, hematophagocytosis, and elevated serum ferritin (37,950 ng/mL) and CD25 levels (11,870 pg/mL), which remained unresponsive to corticosteroids and anakinra. Her serum interferon gamma (IFN-γ) levels were elevated (7 pg/mL). She was markedly responsive to IFN-γ blockade with emapalumab that eliminated her fevers and all MAS-associated laboratory abnormalities. This report provides initial evidence for therapeutic efficacy for IFN-γ blockade in AOSD and secondary MAS.

4.
J Investig Med High Impact Case Rep ; 8: 2324709620950113, 2020.
Article in English | MEDLINE | ID: mdl-32787461

ABSTRACT

A 42-year-old woman with juvenile idiopathic arthritis was treated with anakinra, corticosteroids, and hydroxychloroquine when she developed chronic hypoxic respiratory myopathy. She was admitted to the intensive care unit for acute hypercapnic respiratory failure and required prolonged intubation, subsequent tracheostomy, and long-term ventilatory support due to multiple failed spontaneous breathing trials after discontinuation of anakinra and steroids. Muscle biopsy revealed type II fiber atrophy with the accumulation of autophagosomes and vacuoles presenting as curvilinear bodies, elevated MHC class I antigen expression, and infiltration by CD68+ macrophages and CD8+ T cells. Type II fiber atrophy was attributed to corticosteroid use and curvilinear bodies due to blockade of autophagy by hydroxychloroquine. After hydroxychloroquine was discontinued, the patient recovered to her prehospitalization baseline.


Subject(s)
Hydroxychloroquine/adverse effects , Muscle Weakness/chemically induced , Muscular Diseases/chemically induced , Respiration, Artificial , Respiratory Insufficiency/etiology , Adrenal Cortex Hormones , Adult , Antirheumatic Agents/adverse effects , Female , Humans , Interleukin 1 Receptor Antagonist Protein , Muscular Diseases/complications , Muscular Diseases/pathology , Quadriceps Muscle/pathology , Respiratory Insufficiency/therapy , Tomography, X-Ray Computed , Tracheostomy
5.
Proc (Bayl Univ Med Cent) ; 34(1): 93-94, 2020 Sep 28.
Article in English | MEDLINE | ID: mdl-33456157

ABSTRACT

Popular probiotics contain Lactobacillus, Bifidobacterium, and Streptococcus. Lactobacillus is a gram-positive rod-shaped bacterium that colonizes human oropharyngeal, gastrointestinal, and female urogenital tracts. Although lactobacilli are generally nonpathogenic, they have been implicated in uncommon cases of infection and have the potential to cause bacteremia, endocarditis, endometritis, pulmonary abscesses, and orogastrointestinal abscesses, especially in the immunocompromised. We report a case of Lactobacillus infection in a diabetic patient that occurred 2 weeks after wisdom tooth extraction. The only bacterium found was Lactobacillus, possibly related to her diet of yogurt and smoothies after the tooth extraction.

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