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Diffuse alveolar hemorrhage - review of an interstitial lung disease clinic

Conclusion: DAH is a severe disorder, with nonspecific presentation, caused by a wide spectrum of disease, as demonstrated by this series. Diagnosis requires high clinical suspicion and a thorough work-up.
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Diffuse Parenchymal Lung Disease Source Type: research

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Abstract: A 5-year-old girl presented with a facial butterfly rash that persisted for 5 months without arthralgia, fever, malaise, photosensitivity, or other symptoms. Lupus erythematosus was clinically suspected. All blood tests were negative or within normal values. Skin biopsy showed a dense nodular superficial and deep inflammatory infiltrate of lymphocytes that reaches subcutaneous tissue. The most striking histopathological finding was plasma cells with some perifollicular accentuation. Borrelia polymerase chain reaction assay of the tissue was positive, and we made the diagnoses of borrelia lymphocytoma mimicking ...
Source: The American Journal of Dermatopathology - Category: Pathology Tags: Extraordinary Case Report Source Type: research
Conclusion: CKD is frequent at PAH diagnosis and is independently associated with increased mortality. Right heart failure may induce renal hypoperfusion and congestion, and is associated with eGFR decrease.Am J Nephrol 2018;47:134 –143
Source: American Journal of Nephrology - Category: Neurology Source Type: research
Am J Nephrol 2018;47:130 –133
Source: American Journal of Nephrology - Category: Neurology Source Type: research
Hereditary spherocytosis is the most frequent congenital hemolytic anemia and is characterized with variable degree of anemia, jaundice, and splenomegaly. In the case of severe hyperbilirubinemia out of proportion with hemolysis, other causes of hyperbilirubinemia must be considered. Gilbert syndrome (GS) is an autosomal dominant disorder characterized with intermittent hyperbilirubinemia without any other sign and symptom of liver disease as a result of reduced activity of uridine diphosphate-glucuronyl transferase 1A1. The calculated rate of coexistence of these 2 diseases is 15 to 35/million births. Here we present a 21...
Source: Journal of Pediatric Hematology Oncology - Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research
Background: Pericardial effusion (PE) is a known complication after hematopoietic stem cell transplant (HSCT). Limited data is currently available regarding the incidence and outcomes of PE in pediatric HSCT. Methods: We conducted a retrospective study on a cohort of patients who underwent HSCT between 2004 and 2015. Risk factors associated with development of PE were evaluated. Results: In 111 HSCT, stem cell source was bone marrow in 37 (33.3%), peripheral blood—42 (37.8%) and cord blood—32 (28.8%). Incidence of PE after HSCT was 37.8%. Insignificant effusion (trivial or small) was noted in 30 (27.0%)...
Source: Journal of Pediatric Hematology Oncology - Category: Hematology Tags: Original Articles Source Type: research
A 4-year-old girl with severe aplastic anemia and 2 previous failed T-depleted haploidentical peripheral blood stem cell transplants developed persistent neutropenic fever and multiple erythematous maculopapular rashes 2 days after her third T-replete haploidentical bone marrow transplant. Skin biopsy confirmed the diagnosis of Trichosporon asahii infection. She was on caspofungin prophylaxis which is not effective against Trichosporon. A high index of suspicion, prompt investigation, and appropriate treatment with voriconazole for 4 months was instrumental in controlling the infection and she remains well presently 9 mont...
Source: Journal of Pediatric Hematology Oncology - Category: Hematology Tags: Clinical and Laboratory Observations Source Type: research
Although vitamin D deficiency (VDD) has been linked to anemia among sickle cell disease (SCD), its relationship with hemolysis is unclear. Serum 25-hydroxyvitamin D and biomarkers of hemolysis (hemoglobin [Hb]/hematocrit, reticulocyte percentage, absolute reticulocyte, and lactate dehydrogenase [LDH] levels) in 36 hydroxyurea-naive SCD children were quantified. Correlations were significantly positive with Hb/hematocrit (r=0.40, P=0.017; r=0.45, P=0.006, respectively); inverse with reticulocyte percentage, absolute reticulocyte, and LDH (r=−0.44, P=0.008; r=−0.47, P=0.007; r=−0.45, P=0.007, respectively)....
Source: Journal of Pediatric Hematology Oncology - Category: Hematology Tags: Clinical and Laboratory Observations Source Type: research
ConclusionsIntranasal bevacizumab injection is an effective treatment for most of the moderate and severe grades of HHT‐associated epistaxis. The duration of the effect of the treatment was variable. Primary and late resistance phenomena to the treatment were quite common. Level of Evidence4. Laryngoscope, 2018
Source: The Laryngoscope - Category: ENT & OMF Authors: Tags: Allergy/Rhinology Source Type: research
Authors: Hamilton JA, Hsu HC, Mountz JD Abstract Type I interferons (IFNs) have a prominent role in many aspects of normal innate and adaptive immunity and autoimmunity. However, cell-type specific information about type I IFN expression and autocrine/paracrine signaling is sparse and mostly focused on non-lymphocyte and non-immune cell populations. A major function of B cells is cytokine production, but surprisingly, type I IFN production by B cells in systemic lupus erythematosus (SLE) has not been thoroughly investigated. This is due, in part, to the established view that plasmacytoid dendritic cells (pDCs) are ...
Source: Discovery Medicine - Category: Research Tags: Discov Med Source Type: research
Authors: Yang BB, Xiao H, Li XJ, Zheng M Abstract OBJECTIVES: To evaluate the safety and efficacy of etanercept (ETN) plus methotrexate (MTX) in patients with rhupus without using corticosteroids. METHODS: Twenty rhupus patients [meeting the criteria for both rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE)] who had never been treated with corticosteroids, DMARDs, or biological agents with a 28-joint Disease Activity Score (DAS28)>3.2 and lupus nephritis determined from histopathological specimens were enrolled. All patients were treated with MTX plus ETN, and monitored for 24 weeks of treatm...
Source: Discovery Medicine - Category: Research Tags: Discov Med Source Type: research
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