Clinical Characteristics in Neonates with Alloimmune Neutropenia: Significance of the Detection of Antineutrophil Antibodies

Abstract Neonate alloimmune neutropenia (NAIN) is caused by the transplacental transfer of maternal alloantibodies directed against antigens on the infant’s neutrophils. To date, there are scant studies about its clinical characteristics and characterization of antineutrophil antibodies though some case reports are found. In this study we analyzed 11 cases with NAIN from January 2005 to December 2007. The diagnosis of NAIN was confirmed by the transient neutropenia less than 500/μl of absolute neutrophil count (ANC), the detection of maternal antineutrophil antibody, the incompatibility of neutrophil antigens between parents, and their mothers without autoimmune diseases. Antineutrophil antibodies were detected by granulocyte indirect immunofluorescence test using flow cytometry. To quantify the strength of the antibodies, the ratio of the mean fluorescence channel of each sample to that of control serum was expressed as relative fluorescence intensity according to the method reported previously (Blood99: 3468, 2002). The median age at diagnosis in NAIN patients was 8 days after birth ranged from 0 to 30 days. The average of ANC at the presentation was 170/μl ranged from 0 to 500/μl. All antineutrophil antibodies detected in sera of both neonates and their mothers were against HNA-1 antigens. The alloantibody against HNA-1a was found in 2 cases, that against HNA-1b was in 6 cases, and that against FcγR IIIb was found in 3 cases. The fact that the frequencies of homozygote of HNA-1a and HNA-1b in Japanese population were approximately 50% and 12%, respectively may reflect the frequency of alloantibody specificity in NAIN. During the neutropenic period, 7 cases with NAIN showed mild to moderate infections associated with neutropenia, such as pyrexia and pyodermia. In contrast, 4 of 11 cases with NAIN did not have any infectious episodes in their clinical course. In all patients, the spontaneous recovery of neutropenia with the disappearance of alloantibody was observed within 6 months (median 85 days ranged from 3 weeks to 6 months). The duration until spontaneous resolution of neutropenia was dependent on the strength of alloantibody found in sera of the mothers and neonates. Two patients with significantly high strength of alloantibodies had the relatively long duration to restore the neutropenia(4 months and 6 months). In conclusion, the specificity of antineutrophil antibodies in patients with alloimmune neutropenia is dependent on the frequencies of neutrophil antigens in Japanese population. The quantification of alloantibodies in neonates and their mothers may be useful in considering the clinical course of neutropenia in neonates.

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Myositis-associated Interstitial Lung Disease: Predictors of Failure of Conventional Treatment and Response to Tacrolimus in a US Cohort

The Journal of Rheumatology ◽

10.3899/jrheum.161217 ◽

2017 ◽

Vol 44 (11) ◽

pp. 1612-1618 ◽

Cited By ~ 15

Author(s):

Niharika Sharma ◽

Michael S. Putman ◽

Rekha Vij ◽

Mary E. Strek ◽

Anisha Dua

Keyword(s):

Interstitial Lung Disease ◽

Lung Disease ◽

Clinical Characteristics ◽

Conventional Treatment ◽

Case Reports ◽

Response To Therapy ◽

Disease Modifying Antirheumatic Drugs ◽

Antirheumatic Drugs ◽

Small Series ◽

The Mean

Objective.Patients with myositis-associated interstitial lung disease (MA-ILD) are often refractory to conventional treatment, and predicting their response to therapy is challenging. Recent case reports and small series suggest that tacrolimus may be useful in refractory cases.Methods.A retrospective cohort study of patients with MA-ILD comparing clinical characteristics between those who responded to or failed conventional treatment. In those who failed conventional treatment and received adjunctive tacrolimus, response to tacrolimus was measured by the improvement in myositis, ILD, and change in the dose of glucocorticoids.Results.Thirty-one of 54 patients (57%) responded to conventional treatment based on the predefined variables of improvement in myositis and/or ILD. Patients with polymyositis (PM)-ILD were more likely to respond to conventional treatment than those with dermatomyositis (DM)-ILD (67% vs 35%, p = 0.013). Twenty-three patients failed conventional treatment, 18 of whom subsequently received adjunctive tacrolimus. Ninety-four percent had improvements in ILD and 72% showed improvement in both myositis and ILD. The mean doses of prednisone decreased from baseline by 65% at 3–6 months (p = 0.002) and 81% at 1 year (p < 0.001).Conclusion.Patients with PM-ILD were more likely to respond to conventional treatment than patients with DM-ILD, but clinical characteristics and serology did not otherwise predict response to therapy. A majority of patients with MA-ILD refractory to conventional therapy improved while receiving tacrolimus and were able to decrease their dose of both glucocorticoids and other disease-modifying antirheumatic drugs.

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Neurologic Involvement in Scleroderma en Coup de Sabre

Autoimmune Diseases ◽

10.1155/2012/719685 ◽

2012 ◽

Vol 2012 ◽

pp. 1-6 ◽

Cited By ~ 4

Author(s):

Tiago Nardi Amaral ◽

João Francisco Marques Neto ◽

Aline Tamires Lapa ◽

Fernando Augusto Peres ◽

Caio Rodrigues Guirau ◽

...

Keyword(s):

Magnetic Resonance Imaging ◽

Clinical Characteristics ◽

Case Reports ◽

Localized Scleroderma ◽

Resonance Imaging ◽

Disease Pathogenesis ◽

Neurologic Involvement ◽

Parry Romberg Syndrome ◽

Sclerotic Lesions

Localized scleroderma is a rare disease, characterized by sclerotic lesions. A variety of presentations have been described, with different clinical characteristics and specific prognosis. In scleroderma en coup de sabre (LScs) the atrophic lesion in frontoparietal area is the disease hallmark. Skin and subcutaneous are the mainly affected tissues, but case reports of muscle, cartilage, and bone involvement are frequent. These cases pose a difficult differential diagnosis with Parry-Romberg syndrome. Once considered an exclusive cutaneous disorder, the neurologic involvement present in LScs has been described in several case reports. Seizures are most frequently observed, but focal neurologic deficits, movement disorders, trigeminal neuralgia, and mimics of hemiplegic migraines have been reported. Computed tomography and magnetic resonance imaging have aided the characterization of central nervous system lesions, and cerebral angiograms have pointed to vasculitis as a part of disease pathogenesis. In this paper we describe the clinical and radiologic aspects of neurologic involvement in LScs.

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Characterization of Slurry-Cast Layer Compounds for 3D Printing of High Strength Casting Cores

Materials ◽

10.3390/ma14206149 ◽

2021 ◽

Vol 14 (20) ◽

pp. 6149

Author(s):

Patricia Erhard ◽

Jan Angenoorth ◽

Joachim Vogt ◽

Johannes Spiegel ◽

Florian Ettemeyer ◽

...

Keyword(s):

3D Printing ◽

Bending Strength ◽

State Of The Art ◽

Temperature Stability ◽

High Strength ◽

Process Window ◽

Drying Process ◽

The Mean ◽

Drying Conditions

Additive manufacturing of casting cores and molds is state of the art in industrial application today. However, improving the properties of chemically bonded casting cores regarding temperature stability, bending strength, and surface quality is still a major challenge. The process of slurry-based 3D printing allows the fabrication of dense structures and therefore sinterable casting cores. This paper presents a study of the slurry-based fabrication of ceramic layer compounds focusing on the drying process and the achievable properties in slurry-based 3D printing of casting cores. This study aims at contributing to a better understanding of the interrelations between the drying conditions in the 3D printing process and the properties of sintered specimens relating thereto. The drying intensity influenced by an IR heater as well as the drying periods are varied for layer thicknesses of 50, 75, and 100 µm. Within this study, a process window applicable for 3D printing of sinterable casting cores is identified and further indications are given for optimization potentials. At layer heights of 75 µm, bending strengths between ~8 and 11 MPa as well as densities of around 50% of the theoretical density were achieved. Since the mean roughness depth Rz is determined to be <30 µm in plane, an application of slurry-based 3D printing in investment casting is conceivable.

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COVID-19 infection with asymptomatic or mild disease severity in young patients: Clinical course and association between prevalence of pneumonia and viral load

PLoS ONE ◽

10.1371/journal.pone.0250358 ◽

2021 ◽

Vol 16 (4) ◽

pp. e0250358

Author(s):

Cherry Kim ◽

Wooil Kim ◽

Ji Hoon Jeon ◽

Hyeri Seok ◽

Sun Bean Kim ◽

...

Keyword(s):

Viral Load ◽

Retrospective Study ◽

Disease Severity ◽

Clinical Characteristics ◽

Clinical Course ◽

Young Patients ◽

Viral Loads ◽

Mild Disease ◽

Recovery Times ◽

The Mean

Few studies have focused on clinical courses or viral loads in young asymptomatic or mild patients with COVID-19 infection. We sought to better understand the clinical course and association between viral load and prevalence of pneumonia in young COVID-19 patients with asymptomatic or mild disease severity. In this retrospective study, 106 COVID-19 young patients with asymptomatic or mild disease severity were analyzed for clinical characteristics, clinical course, prevalence of radiologically proven pneumonia and viral load. The cut-off value of viral load for presence of pneumonia was also investigated. The mean age was 28.0±9.3 years. Eleven patients (10.4%) experienced viral remission within one week of diagnosis, but one (0.9%) transferred to the hospital due to aggravation of pneumonia. Patients with pneumonia had significantly higher viral load than those without, and the cut-off value of the Ct value for presence of pneumonia were 31.38. The patients with pneumonia had significantly slower recovery times than those without. Diarrhea was significantly more common in patients with pneumonia than patients without pneumonia. In conclusion, most young asymptomatic and mildly symptomatic patients showed stable clinical course. There were significant differences in viral load and recovery times between patients with and without pneumonia.

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Erythema Multiforme and COVID-19: What Do We Know?

Medicina ◽

10.3390/medicina57080828 ◽

2021 ◽

Vol 57 (8) ◽

pp. 828

Author(s):

Luigi Bennardo ◽

Steven Paul Nisticò ◽

Stefano Dastoli ◽

Eugenio Provenzano ◽

Maddalena Napolitano ◽

...

Keyword(s):

Erythema Multiforme ◽

Clinical Characteristics ◽

Web Of Science ◽

Clinical Course ◽

Case Reports ◽

Case Series ◽

Drug Induced ◽

Cutaneous Eruption ◽

Isi Web Of Science ◽

Juvenile Type

(1) Background: Erythema multiforme (EM) is an acute cutaneous eruption often associated with infections and more rarely with drugs. This review aimed to evaluate the association between erythema multiforme and coronavirus disease 2019 (COVID-19). (2) Methods: A systematic search of PubMed/MEDLINE, Scimago Scopus, and ISI/Web of Science was performed. Original articles, case series, or case reports were evaluated and selected. (3) Results: Fourteen articles were selected, describing a total of 70 patients. EM is a cutaneous eruption rarely occurring in COVID-19 and is, in most cases, associated with a hypersensitivity reaction to the virus. In these cases, EM seems to affect patients younger than 30 years or older than 55 years. Infrequently, some drugs used in the management of COVID-19 may induce EM, especially hydroxychloroquine. The three groups of patients seem to have different clinical characteristics and courses. (4) Conclusions: From these data, it is possible to preliminarily propose that EM or EM-like eruptions linked to COVID-19 might be divided into three types: the virus-related juvenile type (affecting patients <30-year-old), the virus-related older type (affecting patients >55 years), and the drug-induced type. The occurrence of a skin rash does not seem to be related to the severity and clinical course of COVID-19.

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Characterization of water availability in a hydrographic basin

Engenharia Agrícola ◽

10.1590/s0100-69162012000300018 ◽

2012 ◽

Vol 32 (3) ◽

pp. 591-601 ◽

Cited By ~ 9

Author(s):

Fabiane K. Arai ◽

Silvio B. Pereira ◽

Geula G. G. Gonçalves

Keyword(s):

Agricultural Development ◽

Spatial And Temporal Variability ◽

Specific Flow ◽

Mato Grosso ◽

Long Duration ◽

Efficient Planning ◽

Major Producer ◽

The Mean ◽

Mato Grosso Do Sul

The quantitative knowledge of hydrological parameters (rainfall and flow) and their spatial and temporal variability on the regions or basins should be understood as essential to the efficient planning and management of water resources. Because the Ivinhema Basin, located in the state of Mato Grosso do Sul, Brazil, represents an important inductor on the region agricultural development, characterized as a major producer of grains and meat, it was used to characterize the hydrological study. Knowing the rainfall, flow and drainage area of each of the studied affluent, it was calculated the proportion of contribution of the affluent. To that end, it was proposed the concepts of potential and real contributions, aiming to identify the proportion of contribution of each of the affluent to the formation of the flow in the Ivinhema Basin. The results revealed that: the highest rainfall in the Ivinhema Basin occurred in the headwater regions; the mean specific flow of long duration reduces from the headwater to the mouth of Ivinhema Basin; the Sub-basin of Dorado's River has the highest potential and real contribution for the formation of the Ivinhema Basin flow; and the drainage areas of the affluent Dourados and Vacaria contribute with 53% flow of the basin.

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Primary Leiomyoma of the Liver: A Review of a Rare Tumour

HPB Surgery ◽

10.1155/2014/959202 ◽

2014 ◽

Vol 2014 ◽

pp. 1-8 ◽

Cited By ~ 9

Author(s):

Ayodeji Oluwarotimi Omiyale

Keyword(s):

Clinical Course ◽

Incidental Finding ◽

Case Reports ◽

Extensive Literature ◽

Liver Tumours ◽

Rare Tumour ◽

Extensive Literature Search ◽

The Mean ◽

Average Size

Context. Primary leiomyoma of the liver is a rare tumour with uncertain pathogenesis with similar presentation with other tumours of the liver. Little is known about its clinical course. Objectives. To review the literature for case reports of primary leiomyoma of the liver. Methods. Extensive literature search was carried out for case reports of primary leiomyoma of the liver. Results. A total of 36 cases of primary leiomyoma of the liver were reviewed. The mean age of presentation is 43 years with slight female sex affectation; females accounted for 55.6% of the cases reported in the literature. The average size of the tumour is 8.7 cm. 34.4% of the cases reviewed were incidental finding with the mean follow-up time of 33 months with most cases reporting no evidence of disease. Conclusions. Primary leiomyoma of the liver is very rare tumour with complex pathogenesis which remains largely unknown. Imaging of the tumour does not allow for a tissue specific diagnosis; hence histological review of the tissue specimen and immunohistochemical stains are imperative for diagnosis. Surgical resection is both diagnostic and curative. The diagnosis of primary leiomyoma of the liver should be considered as a differential in the management of liver tumours.

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Creutzfeldt-Jakob and Vascular Brain Diseases: Their Overlap and Relationships

Frontiers in Neurology ◽

10.3389/fneur.2021.613991 ◽

2021 ◽

Vol 12 ◽

Author(s):

Yacov Balash ◽

Amos D. Korczyn ◽

Nadejda Khmelev ◽

Anda Eilam ◽

Meital Adi ◽

...

Keyword(s):

Clinical Characteristics ◽

Clinical Course ◽

Case Reports ◽

Brain Diseases ◽

Rare Presentation ◽

Laboratory Features ◽

Uncommon Disease ◽

Median Lifespan ◽

Jakob Disease ◽

Τ Protein

Only a few case reports of stroke-like onset of Creutzfeldt-Jakob disease (CJD) have previously been published. We aimed to analyze the neurological, imaging, electroencephalographic (EEG), and laboratory features of patients with this very rare phenomenon. Here, we review the clinical characteristics, onset features, and clinical course variants of stroke-like CJD in 23 such patients. The median age of the patients was 71 years (range: 56–84 years); 12 were women. In 20 patients, CJD was sporadic. Thirteen patients developed apoplexy-like onset of symptoms, whereas the others had prodromal non-specific complaints. Most often the patients manifested with pyramidal signs (n = 13), ataxia (n = 9), and aphasia (n = 8). On MRI DWI sequence, all subjects had abnormal hyperintensities in various parts of the cerebral cortex, striatum, or thalamus, while EEG detected periodic triphasic waves only in 11. CSF 14-3-3 protein and total τ-protein were abnormal in 17 of 23 cases. All patients died, median lifespan being 2 months (range: 19 days−14 months). In conclusion, a complex of clinical, radiological, and laboratory manifestations of stroke-like onset of CJD is outlined. The clinical relationships between CJD and stroke are considered, in an attempt to highlight this rare presentation of an uncommon disease.

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