Eosinophilic ureteritis is a rare cause of ureteral obstruction, and to date the diagnosis can only be made on pathologic examination. The true underlying cause is not well understood, but there may ...be some association with eosinophilia, atopy and/or trauma. We present a case of a two-year-old boy with ureteropelvic junction obstruction (UPJO) and ipsilateral vesicoureteral reflux (VUR) found to have eosinophilic ureteritis. To our knowledge, this is the youngest reported patient with this finding, and the only patient with eosinophilic ureteritis causing UPJO with concomitant VUR.
Abstract An extremely preterm neonate developed a progressively worsening right axillary ulcerative lesion at the previous site of a temperature probe. Despite treatment with broad-spectrum ...antibiotic and antifungal therapy, the lesion worsened and required extensive surgical debridement. Histologic examination of the debrided tissue demonstrated the presence of invasive mucormycosis. With prompt surgical intervention and proper antifungal therapy, the patient survived. Here we present a unique case of invasive cutaneous mucormycosis in a preterm neonate, along with a review of the current literature on this rare infection.
Background: In pediatrics, acquired aplastic anemia (AA) is most commonly due to infection, particularly viruses, when a cause can be identified. Coronavirus disease 2019 (COVID-19) has affected more ...than 197 million people worldwide, and children typically experience a less severe disease course. COVID-19 is known to cause transient hematologic abnormalities, including leukopenia, lymphopenia, anemia and thrombocytosis or thrombocytopenia in severe cases.
Objectives: Describe three cases of COVID-19 associated acquired aplastic anemia in immunocompetent pediatric patients.
Design/Methods: Case series established by retrospective review of the electronic medical record.
Results: Case 1: An 8-year-old Hispanic male presented with a three-week history of increased bruising and a one-week history of progressive exercise intolerance, shortness of breath, pallor and fatigue. Labs showed pancytopenia. Bone marrow aspirate and biopsy was markedly hypocellular at 5-10% consistent with aplastic anemia (Figure 1). Work-up for the etiology of his aplastic anemia was only significant for positive SARS-COV-2 antibodies and a SEC23B variant of unknown significance on a comprehensive bone marrow failure (BMF)/myelodysplastic syndrome (MDS)/leukemia panel from the Children's Hospital of Philadelphia (CHOP). He was treated with eltrombopag olamine and then proceeded to immunotherapy with cyclosporine (CsA) and horse antithymocyte globulin (ATG) when a sibling match was not identified for hematopoietic stem cell transplant (HSCT). Three months later, his peripheral blood counts have improved, and he is no longer transfusion-dependent. Repeat bone marrow aspirate and biopsy continues to show markedly hypocellularity at <5%.
Case 2: A 5-year-old non-Hispanic white female presented with a two-week history of easy bruising, petechial rash, fatigue and bone pain. Labs showed pancytopenia, and bone marrow aspirate and biopsy showed marked hypocellularity at 5-10% consistent with aplastic anemia (Figure 2). Her aplastic anemia work-up was significant for positive SARS-COV-2 antibodies and subclinical RBC and WBC paroxysmal nocturnal hemoglobinuria (PNH) clones. She was started on eltrombopag olamine and then proceeded to immunotherapy with CsA and ATG when a matched sibling donor was not identified. Three months later, she continues to be severely neutropenic, anemic and thrombocytopenic requiring multiple transfusions. Repeat bone marrow aspirate and biopsy showed variable cellularity with some areas 10-20% and others 70% with an overall cellularity of 50%.
Case 3: An 8-year-old non-Hispanic white female presented with a 10-day history of fatigue, bilateral leg pain and pallor. Labs showed pancytopenia, elevated inflammatory markers and elevated hemoglobin F. Bone marrow aspirate and biopsy demonstrated mild-moderate hypocellularity at 40-50%, left-shifted myelopoiesis and dyspoiesis in the erythroid and megakaryocytic cell lines (Figure 3). MDS and acute lymphoblastic leukemia (ALL) fluorescence in situ hybridization (FISH) panels were negative. Additional work-up revealed positive SARS-COV-2 antibodies. Her pancytopenia resolved within two weeks of her initial hospitalization. Four months later, she presented with increased bruising and fatigue. Labs showed leukocytosis, thrombocytopenia, anemia and circulating peripheral blasts. Bone marrow aspirate and biopsy was consistent with B-cell ALL. She is receiving chemotherapy on study COG AALL1732.
Conclusion: Severe aplastic anemia (SAA) has high morbidity and mortality, and timely diagnosis is needed for appropriate treatment. Multiple different viral infections have been known to cause acquired aplastic anemia. Data on all the sequelae of COVID-19 infection is still emerging, but it is plausible that COVID-19 infection may cause SAA. All three patients were found to have positive COVID-19 antibodies but did not have any evidence of previous COVID-19 infection. Further research and follow-up is needed to determine if previous COVID-19 infection is indeed a risk factor for development of SAA.
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No relevant conflicts of interest to declare.
We present a case of a 3-year-old male originally diagnosed with a CD30+ anaplastic cutaneous T-cell lymphoma with no evidence of systemic disease after CT scan, PET scan, and bone marrow aspiration. ...Sentinel lymph node biopsy (SLNB) was performed as an additional step in the workup and showed microscopic disease. Current management/recommendations for cutaneous T-cell lymphoma do not include SLNB. Medical and surgical management of cutaneous malignancies is dramatically different for local versus advanced disease. Therefore adequate evaluation is necessary to properly stage patients for specific treatment. Such distinction in extent of disease suggests more extensive therapy including locoregional radiation and systemic chemotherapy versus local excision only. Two international case reports have described SLNB in cutaneous T-cell lymphoma with one demonstrating evidence of node positive microscopic disease despite a negative metastatic disease workup. This case is being presented as a novel case in a child with implications including lymphoscintigraphy and SLNB as a routine procedure for evaluation and staging of cutaneous T-cell lymphoma if the patient does not demonstrate evidence of metastatic disease on routine workup.
Primary amebic meningoencephalitis (PAM) is a rare and lethal infection caused by Naegleria fowleri. We report an epidemiological and environmental investigation relating to a case of PAM in a ...previously healthy boy age 8 years. An interview of the patient's family was conducted to determine the likely exposure site and to assess risk factors. Data from the United States Geological Survey site at Waterloo, NE, on the Elkhorn River were used to estimate water temperature and streamflow at the time and site of exposure. Data from the National Weather Service were used to estimate precipitation and ambient air temperature at the time and site of exposure. Despite conventional treatment, the patient died 2 days after hospital admission. The patient participated in recreational water activities in the Elkhorn River in northeastern Nebraska 5 days before symptom onset. In the week before exposure, water and ambient air high temperatures reached annual highs, averaging 32.4°C and 35.8°C, respectively. The day before infection, 2.2 cm of precipitation was reported. Streamflow was low (407 ft3/s). Infections in several northern states, including Nebraska, suggest an expanding geographic range of N. fowleri transmission, which may lead to increased incidence of PAM in the United States. Similar environmental investigations at suspected exposure sites of future cases will allow data aggregation, enabling investigators to correlate environmental factors with infection risk accurately.
The identification of recurrent chromosomal abnormalities in benign and malignant mesenchymal neoplasms has provided important pathogenetic insight as well as powerful diagnostic adjuncts. Nasal ...chondromesenchymal hamartoma (NCMH), an extremely rare benign tumor arising in the sinonasal tract of infants and children, has not been previously subjected to cytogenetic analysis. Histopathologically composed of mixed mesenchymal elements, NCMH exhibits a relatively wide differential diagnosis to include chondromyxoid fibroma, chondroblastoma, aneurysmal bone cyst, fibrous dysplasia, and osteochondromyxoma. An interesting association with pleuropulmonary blastoma has been reported in a small subset of NCMH patients. In the current study, cytogenetic analysis of a NCMH arising in an 11-year-old boy with a past medical history of pleuropulmonary blastoma revealed a novel 12;17 translocation, t(12;17)(q24.1;q21), as the sole anomaly.
The influence of a Semitic language, Arabic, on Portuguese vocabulary is the subject of this thesis. To contextualise this investigation, the first part of this thesis provides an introduction to ...Arabic, its history, and its linguistic particularities, as well as an overview of the history of Arabic influences upon Portuguese. Particular attention is given to the phonetic transformations of Arabic loan words resulting from the contact and exchange between speakers of these languages. Portuguese was influenced by Arabic in discrete phases, which are described in detail: the period of Muslim rule in Portugal in the Middle Ages and the period of migration of African Muslim slaves and the Syro-Lebanese population to Brazil. Having outlined Arabic words brought into Portuguese usage, the second part of this thesis then turns to the analysis of this corpus of loan words. Thanks to various Portuguese corpora, the frequency of Arabic loan words is analysed. This thesis is completed with an appendix including supplementary information about Arabic and cities of the former Gharb Al-Andalus. Key words: Arabic, Portuguese, lexicology, Arabic loan words, Syro-Lebanese migration, semantic fields
Ewing family of tumors is a group of highly aggressive neoplasias that occur most commonly in the first two decades of life. These tumors are most frequently localized in bones, less frequently in ...soft tissues. They usually appear as undifferentiated small round-cell tumors. With current treatment regiments, 5-year disease-free survival rates exceed 60% in patients with a localized disease. Patients with metastatic disease at the time of their first presentation have a poor prognosis. We describe a rare case of visceral primitive neuroectodermal tumor with the involvement of the kidney in a 9-year-old girl. The tumor was studied with immunohistochemistry, cytogenetics, and molecular biology methods. Strong expression of protein MIC
2 by immunochemistry (antibody HBA 71) with subsequent demonstration of a translocation consistent with t(11;22)(q24;q12) using cytogenetic and reverse transcriptase polymerase chain reaction (RT-PCR) confirmed the histopathological diagnosis of peripheral primitive neuroectodermal tumor. We detected minimal residual disease in bone marrow using RT-PCR.
78 individual milk samples (IS) were taken in the Czech Fleckvieh and Holstein dairy cow herds and 33 bulk milk samples (BS) were taken in 13 Holstein dairy cow herds to compare two methods for the ...determination of fatty acids (FAs) – routine/indirect (infrared (IR) spectroscopy, MIR-FT) and reference/direct (gas chromatography, GC). The IS results show that the fatty acids C16:0 and C18:1 and the group of saturated (SFA) and unsaturated (UFA) FAs can be determined using routine method with a relatively high reliability (correlations (r) 0.7543, 0.7607, 0.8424 and 0.9492; P < 0.001). In case of polyunsaturated FAs is the r lower (0.2891; P < 0.05). In BS the tightness of relations is stronger in the case of the SFA and UFA (r 0.7094; 0.9389; P < 0.001), while for the group of TFA (trans isomers of unsaturated FAs) and PUFA (polyunsaturated FAs) are lower r (0.5897 and 0.5931; P < 0.001 respectively). Routine method for fatty acids profile determination is efficient for selection of raw milk to guarantee the production of specific dairy products with increased content of healthful fatty acids.