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Olga Kostrzewa, Paweł Kołodziej, Maria Majdan, Robert Zwolak, Anna Górak, Krzysztof Bar
In this article we have presented a case of 31-year-old male with thickening of the skin and tender cord of superficial veins of the penis and laboratory findings of the high titer of PM/Scl-100 antibodies in the serum. The patient was referred to the Rheumatology Department due to suspected systemic sclerosis. The HRCT scan of his lungs revealed fibrous degeneration and ground-glass opacity in the lower lobes. Capillaroscopy showed abnormalities, which were not typical for systemic sclerosis. In Doppler ultrasound examination of penis, superficial dorsal and circumflex veins thrombosis and inflammatory infiltration were observed...
2018: Wiadomości Lekarskie: Organ Polskiego Towarzystwa Lekarskiego
Aleksandra Szuster, Justyna Tyburek, Karolina Widłak, Piotr Wilkołek, Karol Wiśniewski, Maria Majdan
The coexistence of systemic lupus erythematosus (SLE) and psoriasis is rarely observed in everyday clinical practice. Apart from providing a correct diagnosis, a major difficulty is to provide a proper treatment. In this case, for example, the use of systemic glucocorticosteroids may have a negative effect on the course of psoriasis, whereas phototherapy, which is widely practiced in psoriasis, may cause SLE exacerbation. The aim of the paper is to present the difficulty along the diagnostic process and in choosing the best type of treatment for patients with a coexistence of SLE and psoriasis and also a review of the subject-related literature...
2018: Wiadomości Lekarskie: Organ Polskiego Towarzystwa Lekarskiego
Monika Turska, Jolanta Parada-Turska
Cutaneous polyarteritis nodosa is a rare disease that affects vessels of the deep skin and the subcutaneous tissue. Its etiopathology remains unknown. It predominantly affects skin, and the main cutaneous symptoms are subcutaneous nodules, livedo reticularis, and ulcerations that are mainly located on legs. Cutaneous polyarteritis nodosa can also cause extracutaneous symptoms (fever, malaise, myalgias, arthralgias, neuropathy). It is a chronic benign disease with a relapsing course. Diagnostic criteria for this disease were recently proposed and both clinical and typical histological features must be present to confirm the diagnosis...
2018: Wiadomości Lekarskie: Organ Polskiego Towarzystwa Lekarskiego
Mohd Faizal Ahmad, Muhammad Azrai Abu, Kah Teik Chew, Kun Leng Sheng, Mohd Asyraf Zakaria
A positive urine pregnancy test (UPT) with adnexal mass in ectopic pregnancy is not the ultimate diagnosis. The incidence of ectopic pregnancy is about 27 per 1000 pregnancies [1]. On average, about 6-16% will present to an emergency department with first-trimester bleeding and abdominal pain [2]. On presenting with these symptoms with the simultaneous presence of an adnexal mass and an empty uterus, a UPT is of paramount importance to determine whether the symptoms are pregnancy related or not. When the UPT is positive, an ectopic pregnancy is not the only diagnosis as the rare entity of non-gestational ovarian choriocarcinoma (NGOC) should be considered...
March 20, 2018: Hormone Molecular Biology and Clinical Investigation
Julie D Yeterian, Krisanne Bursik, John F Kelly
BACKGROUND: A growing literature on adults with substance use disorders (SUD) suggests that religious and spiritual processes can support recovery, such that higher levels of religiosity and/or spirituality predict better substance use outcomes. However, studies of the role of religion and spirituality in adolescent SUD treatment response have produced mixed findings, and religiosity and spirituality have rarely been examined separately. METHODS: The present study examined religiosity and spirituality as predictors of outcomes in an outpatient treatment adolescent sample (N = 101) in which cannabis was the predominant drug of choice...
March 20, 2018: Substance Abuse
Oğuz Ahmet Hasdemir, Serhat Tokgöz, Fulya Köybaşıoğlu, Harun Karabacak, Cüneyt Yücesoy, Gökşen İnanç İmamoğlu
BACKGROUND: Metaplastic carcinoma of the breast (MpBC) is defined as a group of heterogeneous malignant neoplasms that contain glandular and non-glandular components with mixed epithelial and mesenchymal differentiations. OBJECTIVES: The aim of this study was to research the clinical and pathological characteristics of MpBC determining its rank among all breast cancers. MATERIAL AND METHODS: Metaplastic carcinoma of the breast was found in 7 out of 1,164 patients who had been diagnosed with breast cancer within the period of 12 years in our hospital...
March 19, 2018: Advances in Clinical and Experimental Medicine: Official Organ Wroclaw Medical University
Ulkem Kocoglu Barlas, Hasan Serdar Kıhtır, Nilufer Goknar, Melike Ersoy, Nihal Akcay, Esra Sevketoglu
BACKGROUND: Hemolytic uremic syndrome (HUS) is a clinical syndrome characterized by hemolytic anemia, thrombocytopenia, and acute kidney injury. Atypical hemolytic uremic syndrome (aHUS) is a devastating disease with significant mortality and high risk of progression to end-stage kidney disease. It is mostly caused by dysregulation of the alternative complement pathway. Cobalamin C (Cbl C) defect is a genetic disorder of cobalamin metabolism and is a rare cause of HUS. CASE-DIAGNOSIS/TREATMENT: We present a 6-month-old male infant who was admitted to the pediatric intensive care unit (PICU) due to restlessness, severe hypertension, anemia, respiratory distress, and acute kidney injury...
March 20, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Waltraud Friesenbichler, Angela Schumich, Ingrid Simonitsch-Klupp, Renate Panzer-Grümayer, Oskar Haas, Georg Mann, Michael Dworzak
Myelofibrosis is associated with a wide variety of neoplastic and non-neoplastic bone marrow diseases, predominately myeloproliferative neoplasms and acute myeloid leukemia. The following case documents an unusual patient presenting with pancytopenia and acute myelofibrosis accompanied by precursor B-cell acute lymphoblastic leukemia. This very rare clinical presentation raises questions concerning the relationship between concurrent occurrence of acute myelofibrosis and acute lymphoblastic leukemia.
March 19, 2018: Journal of Pediatric Hematology/oncology
Yoon-Sic Han
Sinus augmentation is a routine procedure performed in patients presenting with severe atrophy of the posterior maxillary alveolar ridge who seek dental implant surgery. Although this strategy is successful in large part, complications such as maxillary sinusitis, antral bleeding, resorption of graft material, infection, failure of implant installation, and oroantral fistula formation are documented on occasion. However, reports of postoperative maxillary cyst (POMC) arising after sinus augmentation are rare...
March 19, 2018: Implant Dentistry
Claudio Agostinelli, Agnese Agostinelli, Marco Berardini, Paolo Trisi
PURPOSE: The aim of the present study was to analyze the alveolar bone morphology of the upper first and second molars. This analysis aims to evaluate the morphology of a hypothetical postextractive site in the upper molar area to diagnose the possibility of immediate postextraction implant placement. MATERIALS AND METHODS: Cone-beam CT scans of 100 patients were examined. The measurements were made using a dedicated 3D software. Reference points were identified to allow clear and repeatable measurements...
March 19, 2018: Implant Dentistry
Alex C Stabell, Nicholas R Meyerson, Rebekah C Gullberg, Alison R Gilchrist, Kristofor J Webb, William M Old, Rushika Perera, Sara L Sawyer
Human dengue viruses emerged from primate reservoirs, yet paradoxically dengue does not reach high titers in primate models. This presents a unique opportunity to examine the genetics of spillover versus reservoir hosts. The dengue virus 2 (DENV2) - encoded protease cleaves human STING, reducing type I interferon production and boosting viral titers in humans. We find that both human and sylvatic (reservoir) dengue viruses universally cleave human STING, but not the STING of primates implicated as reservoir species...
March 20, 2018: ELife
Marco Baldrighi, Pier Paolo Sainaghi, Mattia Bellan, Ettore Bartoli, Luigi Mario Castello
INTRODUCTION: Although hypovolemia remains the most relevant problem during acute decompensated diabetes in its clinical manifestations (diabetic ketoacidosis, DKA, and hyperglycemic hyperosmolar state, HHS), the electrolyte derangements caused by the global hydroelectrolytic imbalance usually complicate the clinical picture at presentation and may be worsened by treatment itself. AIM: This review article is focused on the management of dysnatremias during hyperglycemic hyperosmolar state with the aim of providing clinicians a useful tool to early indentify the sodium derangement in order to address properly its treatment...
March 19, 2018: Current Diabetes Reviews
Pablo Roa Rojas, Hernán Arango Fernández, Martha Rebolledo Cobos, Jonathan Harris Ricardo
Beckwith-Wiedemann syndrome is a rare congenital condition, characterized by presenting macroglossia, defects of the abdominal wall, hemihypertrophy, omphalocele, neonatal hypoglycemia, umbilical hernia, hepatomegaly, cardiac abnormalities, among others. Macroglossia occurs in 90% of cases, causing a problem in chewing, swallowing, phonation and breathing, resulting in a closure of the upper airway. The therapeutic option of choice is partial glossectomy. We present a 2-month-old pediatric patient with Beckwith-Wiedemann syndrome and area blockage due to severe macroglossia; in the medical history, congenital heart disease, interatrial communication, persistent ductus arteriosus, symptomatic epilepsy, renal failure, hypoglycemia, tracheotomy and gastrostomy, due to airway collapse and dysphagia...
April 1, 2018: Archivos Argentinos de Pediatría
Elisa Vaiani, Cintia Morales, Florencia Soto, Yeny Blanco, Carlos Rugilo, Marta Ciaccio, Alicia Belgorosky
Giant prolactinomas are rare pituitary adenomas characterized by their great local invasion. In this paper, we report a 15-year-old male with left retro-ocular pain and ipsilateral exophthalmos of 4 months of evolution, secondary to a tumour in the base of the skull that invaded the orbit. Hormonal studies revealed serum prolactin of 6913,7 ng/ml (normal value < 20), confirming the diagnosis of giant prolactinoma. The patient started treatment with the dopaminergic agonist cabergoline in increasing doses...
April 1, 2018: Archivos Argentinos de Pediatría
Tamer Sekmenli, Metin Gündüz, Hikmet Akbulut, H Haldun Emiroglu, Mustafa Koplay, Ilhan Ciftci
Percutaneous endoscopic gastrostomy (PEG) is used as an alternative to enteral/nasoenteral feeding in situations where long-term oral feeding is ineffective or not tolerated. It is mostly preferred in patients with neurological conditions and also to support nutrition in patients with congenital heart diseases, cystic fibrosis, inflammatory bowel disease, and various oropharyngeal diseases. Although it is easily applicable compared to many invasive procedures, it has complications ranging from wound infection to death...
April 1, 2018: Archivos Argentinos de Pediatría
Claudia Salomone B, Isabel Ogueta C, Carlos Reyes V, Gloria Durán S, Noemí Aguirre, Angélica Wietstruck
Congenital erythropoietic porphyria is an extremely rare, autosomal recessive, non-acute cutaneous porphyria, caused by uroporphyrinogen III synthase deficiency, codificated by UROS gene on the chromosome 10q26.2. Porphyrins deposit in cornea, bones and teeth. The first symptoms could be manifested in early childhood, with skin fragility, vesicles and bullae. Severe course produces acral tissues mutilation, eye involvement, hemolytic anemia and hypersplenism. The treatment is complex and it is based in the photoprotection...
April 1, 2018: Archivos Argentinos de Pediatría
Alma V Huidobro-Chávez, Gianmarco D Vigo Pareja, Carlos Pachas-Peña, Karina Patiño-Calla
The Langerhans cell histiocytosis is a rare disease characterized by the clonal proliferation of CD1a + myeloid dendritic cells associated with a significant inflammatory component. The localized form of the disease is called eosinophilic granuloma. Bone involvement is common; in children, lytic lesions are most frequently found in the cranial dome being rare in the orbit. We present an 18-month-old infant who consulted due to periorbital edema and proptosis of the right eye, with two months of evolution. The computed tomography and the magnetic resonance imaging showed a maxillary sinus tumor mass of expansive growth and erosion of the roof of the orbit...
April 1, 2018: Archivos Argentinos de Pediatría
Evaggelina Papakanderaki, Konstantinos Kanakakis, Sotirios Goule, Maria Chounti, Panagiotis Hountis
Raoultella Ornithinolytica (RO) is an encapsulated, Gram- negative, nonmotile aerobic rob which was reclassified from Klepsiella genus belonging in the family of Enterobacteriaceae. It is a rare human infection and few cases have been reported in post thoracotomy patients. Here we present a case of a left lower lobectomy patient that was complicated by pleural effusion and high fever with positive sputum cultures of Raoultella Ornithinolytica and positive pleural fluid cultures of Staphylococcus hominis. It is related with aquatic life poisoning...
February 26, 2018: Monaldi Archives for Chest Disease, Archivio Monaldi Per le Malattie del Torace
A Ulate-Campos, J Petanas-Argemi, M Rebollo-Polo, C Jou, C Sierra, J Armstrong, M C Fons-Estupina
INTRODUCTION: X-linked adrenoleukodystrophy (X-ALD) is the most frequent peroxisomal disease. It is due to a mutation in the ABCD1 gene. The loss of functioning of ABCD1 triggers ineffective beta oxidation of very long-chain fatty acids, which gives rise to an accumulation of these fatty acids. The typical alteration revealed in neuroimaging scans in the cerebral form is symmetrical periventricular demyelination with posterior location. CASE REPORT: We report the case of a 10-year-old boy with right spastic hemiparesis and subacute cognitive impairment...
April 1, 2018: Revista de Neurologia
Alina Dima, Ciprian Jurcut, Anca Manolache, Daniel Vasile Balaban, Alina Popp, Mariana Jinga
BACKGROUND AND AIMS: Celiac disease (CD) presents with a wide spectrum of extra-digestive symptoms, including hemorrhagic manifestations. The aim of this review was to conduct an extensive analysis of the hemorrhagic events reported in adult CD patients. METHODS: Case report and review of the literature. Pubmed (MEDLINE) database search from January 1970 onwards was performed using the medical subject headings [MeSH] terms "celiac disease" AND "blood coagulation disorders", "hemorrhage", "hematoma", "hematuria", "hemoptysis", "epistaxis", "hemosiderosis"...
March 2018: Journal of Gastrointestinal and Liver Diseases: JGLD
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