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Sample records for erdheim-chester disease case

  1. Erdheim-Chester disease: a two-case report

    International Nuclear Information System (INIS)

    Hexsel, Fernando Fernandez; Suwa, Eiji; Aguiar, Paula Musa; Maciel, Antonio Carlos; Hospital de Clinicas de Porto Alegre, RS

    2009-01-01

    Erdheim-Chester disease is a rare non-Langerhans cell histiocytosis of unknown etiology, affecting multiple organ system, involving bones, central nervous system, eyes, lungs, mediastinum, kidneys and retroperitoneum. The authors report two cases that progressed with the typical presentation of the disease. Radiological findings were in agreement with literature and guided the diagnosis, confirmed by immunohistochemistry. (author)

  2. Bone scintigraphy in Erdheim-chester disease: a case report

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    Siqueira, V.L.; Soares, L.M.M.; Ribeiro, V.P.B.; Coura Filho, G.B.; Sapienza, M.T.; Ono, C.R.; Watanabe, T.; Costa, P.L.A.; Hironaka, F.; Buchpiguel, C.A. [Universidade de Sao Paulo (FM/USP), SP (Brazil). Fac. de Medicina. Hospital das Clinicas

    2008-07-01

    Full text: Introduction: Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis, of unknown etiology, characterized by infiltration of foamy histiocytes. Clinically, patients usually present with bone pain, and various extraskeletal manifestations. ECD differs from Langerhans cell histiocytosis (LCH) by radiologic and immunohistochemistry features. Case report: A 57-year-old woman presented with a history of intense pain on her left hand, besides eyelid xanthelasmas and xanthoms on frontal area ten years ago. Four years late she presented with pain on hips, legs and feet. Xanthoms spread to perioral area, mento and neck. Radiographs of the hands showed osteolysis of carpal bones bilaterally, osteolysis of fifth left metacarpal bone, osteosclerosis of all metacarpal bones bilaterally, except the fifth, and osteosclerosis of the second and third proximal falanges bilaterally. The legs showed bilateral diaphyseal and metaphyseal osteosclerosis. Bone scintigraphy demonstrated increased uptake on face bone (maxilla), and symmetric intense uptake on elbows, distal radii and ulnae, hands, distal area of femurs, tibias particularly on proximal and distal area, and feet. A tibia biopsy and a biopsy of neck lesion were made. The analysis of histology and immunohistochemistry were consistent with ECD. She has been treated with a-interferon for 1,5 year, and she reports delay in xanthoms progression and bone pain remission. Discussion: ECD is an adult multisystemic xanthogranulomatous infiltrative disease of unknown etiology. It may be confused with LCH, however ECD have distinctive immunohistochemistry and radiologic findings. LCH shows typically lytic bone lesions on axial skeleton, whereas symmetrical long-bone osteosclerosis is the radiologic sign for ECD. LCH stain positive for CD1a and S-100 protein, and the electron microscopy of cytoplasm discloses Biberck granules. ECD stain positive for CD68, negative for CD1a and S-100 protein, shows absent of

  3. Erdheim-Chester disease: a comprehensive review.

    Science.gov (United States)

    Abdelfattah, Ahmed Maher; Arnaout, Karim; Tabbara, Imad A

    2014-07-01

    Erdheim-Chester disease is a rare form of non-Langerhans' cell histiocytosis characterized by multi-system infiltration by xanthogranulomas composed of foamy histiocytes surrounded by fibrosis. Approximately 400 cases have been reported in the literature, and the recent increase in the number of cases is likely due to the increased awareness of its associated morbidity and mortality. The etiology of this disease remains unknown, the clinical course is variable and treatment is still not well-established. The objective of this review is to describe the pathogenesis, clinical manifestations, and diagnosis of this rare disorder, and to review its prognosis and treatment. Erdheim-Chester disease (ECD) is a rare form of non-Langerhans' cell histiocytosis. It was first described in 1930. Approximately 400 cases have been reported in the literature. Copyright© 2014 International Institute of Anticancer Research (Dr. John G. Delinassios), All rights reserved.

  4. Erdheim Chester disease with appendicular skeletal, renal and pleural involvement responding to Zelboraf (BRAF inhibitor) treatment: case report

    International Nuclear Information System (INIS)

    Borys, Dariusz; Nystrom, Lucas; Song, Albert; Lomasney, Laurie M.

    2016-01-01

    Erdheim Chester disease is a rare non-Langerhans cell histiocytosis which may involve multiple organs including bone, soft tissue, lungs, cardiovascular system, kidneys (retroperitoneum), skin, and central nervous system. Bone involvement is most common followed by other organs. This case report describes a 58-year-old man who presented with progressive renal dysfunction presumed due to obstruction. The patient failed multiple urinary tract interventions, and clinical course was complicated by recurrent low-grade fevers, and bilateral knee pain. Advanced imaging and histopathological features on bone biopsy were consistent with Erdheim Chester disease. Molecular studies of tissue showed BRAF V600 mutation. This patient was treated with Zelboraf (vemurafenib) BRAF inhibitor with subsequent improvement in renal and pleural dysfunction as well as decreased histiocytic soft tissue masses on CT. (orig.)

  5. Erdheim Chester disease with appendicular skeletal, renal and pleural involvement responding to Zelboraf (BRAF inhibitor) treatment: case report

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    Borys, Dariusz [Loyola University Medical Center Chicago, Department of Pathology, Maywood, IL (United States); Loyola University Medical Center Chicago, Departmet of Orthopaedic Surgery, Maywood, IL (United States); Loyola University Medical Center, Maywood, IL (United States); Nystrom, Lucas [Loyola University Medical Center Chicago, Departmet of Orthopaedic Surgery, Maywood, IL (United States); Song, Albert [Loyola University Medical Center Chicago, Department of Radiology, Maywood, IL (United States); Lomasney, Laurie M. [Loyola University Medical Center Chicago, Departmet of Orthopaedic Surgery, Maywood, IL (United States); Loyola University Medical Center Chicago, Department of Radiology, Maywood, IL (United States)

    2016-10-15

    Erdheim Chester disease is a rare non-Langerhans cell histiocytosis which may involve multiple organs including bone, soft tissue, lungs, cardiovascular system, kidneys (retroperitoneum), skin, and central nervous system. Bone involvement is most common followed by other organs. This case report describes a 58-year-old man who presented with progressive renal dysfunction presumed due to obstruction. The patient failed multiple urinary tract interventions, and clinical course was complicated by recurrent low-grade fevers, and bilateral knee pain. Advanced imaging and histopathological features on bone biopsy were consistent with Erdheim Chester disease. Molecular studies of tissue showed BRAF V600 mutation. This patient was treated with Zelboraf (vemurafenib) BRAF inhibitor with subsequent improvement in renal and pleural dysfunction as well as decreased histiocytic soft tissue masses on CT. (orig.)

  6. A Rare Case of Erdheim-Chester Disease and Langerhans Cell Histiocytosis Overlap Syndrome

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    Shahzaib Nabi

    2015-01-01

    Full Text Available A 48-year-old woman with a past medical history of seizures and end-stage renal disease secondary to obstructive uropathy from retroperitoneal fibrosis presented to the emergency department with seizures and altered mental status. A Glasgow Coma Scale of 4 prompted intubation, and she was subsequently admitted to the intensive care unit. Magnetic resonance imaging of the brain performed to elucidate the aetiology of her seizure showed a dural-based mass within the left temporoparietal lobe as well as mass lesions within the orbits. Further imaging showed extensive retroperitoneal fibrosis extending to the mediastinum with involvement of aorta and posterior pleural space. Imaging of the long bones showed bilateral sclerosis and cortical thickening of the diaphyses. Imaging of the maxillofacial structures showed osseous destructive lesions involving the mandible. These clinical and radiological features were consistent with a diagnosis of Erdheim-Chester disease; however, the patient’s skin biopsy was consistent with Langerhans cell histiocytosis.

  7. Peculiar Distribution of Tumorous Xanthomas in an Adult Case of Erdheim-Chester Disease Complicated by Atopic Dermatitis

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    Yukako Murakami

    2011-05-01

    Full Text Available Erdheim-Chester disease is a rare non-Langerhans form of histiocytosis with multiple organ involvement. Approximately 20% of patients have xanthoma-like lesions, usually on the eyelids. We report a case of Erdheim-Chester disease in a 32-year-old male who showed peculiar xanthomatous skin lesions and also had atopic dermatitis. His skin manifestations included ring-like yellowish tumors on his periorbital regions, rope necklace-like tumors on his neck, and spindle-shaped tumors on his right preauricular region and cubital fossas. He also had exophthalmos and diabetes insipidus. Chronic eczematous lesions were present on the flexor aspect of his extremities, and his serum eosinophil numbers and immunoglobulin E levels were elevated. A histological examination of his right neck tumor showed foamy macrophages and touton-type giant cells, which were positive for CD68 and CD163 and negative for S-100 and CD1a. We suggest that the complication of atopic dermatitis may have contributed to the uncommon clinical features in this case.

  8. Epiphyseal involvement in Erdheim-Chester disease: radiographic and scintigraphic findings in a case with lytic lesions

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    Ruiz-Hernandez, G.; Tajahuerce-Romera, G.M.; Latorre-Ibanez, M.D.; Lara-Pomares, A. [Servicio de Medicina Nuclear, Hospital Provincial de Castellon (Spain); Vila-Fayos, V. [Servicio de Reumatologia, Hospital Comarcal de Vinaroz (Spain)

    2000-08-01

    We reported a symmetric increase of activity in lower links secondary to Erdheim-Chester disease and demonstrated by bone scans and radiographs. An inusual scintigraphic and radiographic appearance with epiphyseal involvement and lytic lesions is described. Differential diagnosis of bone scan and radiographic findings is discussed. (orig.)

  9. Multisystem Radiologic Manifestations of Erdheim-Chester Disease

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    Umairullah Lodhi

    2016-01-01

    Full Text Available Erdheim-Chester Disease is a rare form of multiorgan non-Langerhans’ cell histiocytosis that affects individuals between the ages of 50 and 70 with an equal distribution among males and females. It is associated with significant morbidity and mortality that is mostly due to infiltration of critical organs. Some of the sites that Erdheim-Chester Disease affects include the skeletal system, central nervous system, cardiovascular system, lungs, kidneys (retroperitoneum, and skin. The most common presenting symptom of Erdheim-Chester Disease is bone pain although a large majority of patients are diagnosed incidentally during a workup for a different disease process. Diagnosing Erdheim-Chester Disease is challenging due its rarity and mimicry to other infiltrative processes. Therefore, a multimodality diagnostic approach is employed with imaging being at the forefront. As of date, a comprehensive radiologic review of the manifestations of Erdheim-Chester Disease has rarely been reported. Here we present radiologic findings of an individual suffering from Erdheim-Chester Disease.

  10. [Pseudo-tumoral and ischemic encephalic Erdheim-Chester disease].

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    Amezyane, T; Abouzahir, A; Bassou, D; Zoubeir, Y; Hammi, S; Mahassin, F; Ohayon, V; Archane, M-I

    2009-01-01

    Erdheim-Chester disease (ECD) is a rare non-langerhans cell histiocytosis of unknown etiology. It is a multi-systematic xanthogranulomatous infiltration with almost constant bone involvement; the neurological manifestations are not specific and occur in 15-20% of cases. We report the case of a 59-year-old woman hospitalized for a frontal syndrome and right hemiparesis. Imaging revealed a left caudate nucleus process with recent infarct. Cardiovascular involvement and bilateral osteosclerosis of long bones strongly suggested ECD, confirmed after biopsies of the pericardium and bone. Pseudo-tumor encephalic ECD is very rare; the caudate nuclei is an unusual localization; ischemic stroke has been exceptionally described. Prognosis depends largely on the involvement of the central nervous and cardiovascular systems.

  11. Erdheim-Chester disease in a child with MR imaging showing regression of marrow changes

    International Nuclear Information System (INIS)

    Joo, Chan Uhng; Go, Yang Sim; Kim, In Hwan; Kim, Chul Seong; Lee, Sang Yong

    2005-01-01

    Erdheim-Chester disease is a disseminated xanthogranulomatous infiltrative disease of unknown origin that generally presents in adulthood. A review of the English-language literature demonstrated that pediatric cases were extremely rare, and to our knowledge, only two cases, a 7- and 14-year-old, have been published. We report a case of Erdheim-Chester disease in a 10-year-old girl evaluated with MR imaging. Radiographs revealed typical bilateral, symmetric osteosclerosis of the metaphyseal regions of long bones of the upper and lower extremities. A histologic examination demonstrated foamy histiocytes in bone marrow smears. Bilateral symmetric low signal intensities of both proximal tibiae and distal femurs were demonstrated on T1-weighted MR images. After oral steroid therapy for 8 months, follow-up MR imaging showed remarkable restoration of normal high signal intensity in both the tibial and femoral metaphyses. To our knowledge, this may be the first case of Erdheim-Chester disease that showed normal restoration of the abnormal signal intensities in the metaphyses of long bones after steroid therapy. (orig.)

  12. Erdheim-Chester Disease: Comprehensive Review of Molecular Profiling and Therapeutic Advances.

    Science.gov (United States)

    Haroun, Faysal; Millado, Kristen; Tabbara, Imad

    2017-06-01

    The revised 2016 World Health Organization classification introduced Erdheim-Chester disease (ECD) as a provisional entity within the histiocytic and dendritic cell neoplasms separate from the juvenile xanthogranuloma family based on distinct molecular features. However, evolving knowledge regarding the molecular and genetic aberrations in addition to common clinical features of ECD support the classification of ECD together with Langerhans cell histiocytosis (LCH). Accordingly, ECD can be thought of as an inflammatory myeloid clonal disorder based on the detection of various activating mutations along the mitogen activated protein kinase-extracellular signal regulated kinase (MAPK-ERK) pathway with most notable variant being a valine to a glutamic acid substitution at amino acid 600 in the B-rapidly accelerated fibrosarcoma protein (BRAFV600E). In this group, targeted therapy with a B-Raf inhibitor alone or combined with a MAPK-ERK (MEK) inhibitor has shown promising results based on several case reports. Currently, two phase II trials with BRAF inhibitors are underway and could potentially change the standard of care. MEK inhibitors may also be efficacious in ECD harboring mutations in MAP2K1; other potential targetable aberrations include programed cell death receptor 1 and mutations in phosphoinositide 3-kinase. Copyright© 2017, International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved.

  13. Erdheim-Chester disease with cutaneous features in an Indian patient.

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    Garg, Taru; Chander, Ram; Gupta, Tanvi; Mendiratta, Vibhu; Jain, Manjula

    2008-01-01

    the fifth lumbar vertebrae, bilateral sacroiliac bone, and left iliac bone and areas of photopenia in sacrum. Bone marrow aspiration from the sternum showed normal cells with a normal erythroid-to-myeloid ratio. Ultrasonographic B scanning of the eyes showed no retro-orbital involvement. Erdheim-Chester disease was diagnosed in this patient on the basis of diagnostic histopathology, radiologic features involving bones, and ILD and treatment with 40 mg oral prednisolone daily was started. Surgical debulking of her skin lesions was planned, but the patient refused due to her worsening ILD.

  14. Successful treatment with cladribine of Erdheim-Chester disease with orbital and central nervous system involvement developing after treatment of langerhans cell histiocytosis

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    Perić Predrag

    2016-01-01

    Full Text Available Introduction. Erdheim-Chester disease (ECD is a rare, systemic form of non-Langerhans cell histiocytosis of the juvenile xantho-granuloma family with characteristic bilateral symmetrical long bone osteosclerosis, associated with xanthogranulomatous extras-keletal organ involvement. In ECD, central nervous system (CNS and orbital lesions are frequent, and more than half of ECD patients carry the V600E mutation of the proto-oncogene BRAF. The synchronous or metachronous development of ECD and Langerhans cell histiocytosis (LCH in the same patients is rare, and the possible connection between them is still obscure. Cladribine is a purine substrate analogue that is toxic to lymphocytes and monocytes with good hematoencephalic penetration. Case report. We presented a 23-year-old man successfully treated with cladribine due to BRAF V600E-mutation-negative ECD with bilateral orbital and CNS involvement. ECD developed metachronously, 6 years after chemotherapy for multisystem LCH with complete disease remission and remaining central diabetes insipidus. During ECD treatment, the patient received 5 single-agent chemotherapy courses of cladribine (5 mg/m2 for 5 consecutive days every 4 weeks, with a reduction in dose to 4 mg/m2 in a fifth course, delayed due to severe neutropenia and thoracic dermatomal herpes zoster infection following the fourth course. Radiologic signs of systemic and CNS disease started to resolve 3 months after the end of chemotherapy, and CNS lesions completely resolved within 2 years after the treatment. After 12-year follow-up, there was no recurrence or appearance of new systemic or CNS xanthogranu-lomatous lesions or second malignancies. Conclusion. In accordance with our findings and recommendations provided by other authors, cladribine can be considered an effective alternative treatment for ECD, especially with CNS involvement and BRAF V600E-mutation-negative status, when interferon-α as the first-line therapy fails.

  15. Síndrome coronario agudo y enfermedad de Erdheim-Chester. Patogénesis e implicaciones terapéuticas

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    José Carlos Alarcón-García

    2017-05-01

    Full Text Available La enfermedad de Erdheim-Chester es una histiocitosis celular diferente a la histiocitosis de Langerhans, de origen incierto. Se caracteriza por una implicación multi-orgánica debida a la infiltración de los histiocitos CD68+/CD1a-, en forma de xantogranulomas, que afectan principal y comúnmente a la metáfisis y diáfisis de huesos largos. El diagnóstico se realiza mediante biopsia, donde se revelan histiocitos CD68+/CD1a-, carencia de proteína S, y presencia de gránulos de Birbeck. Se ha subestimado la implicación cardiovascular. Reportamos un caso de un varón de 67 años con la enfermedad de Erdheim-Chester e infarto de miocardio agudo, debido a implicación coronaria, además de enfermedad ósea, vascular, pituitaria y retroperitoneal. Revisamos la literatura relevante y describimos el tratamiento clínico de estos pacientes.

  16. Ribbing disease

    International Nuclear Information System (INIS)

    Mukkada, Philson J; Franklin, Teenu; Rajeswaran, Rangasami; Joseph, Santhosh

    2010-01-01

    Ribbing disease is a rare sclerosing dysplasia that involves long tubular bones, especially the tibia and femur. It occurs after puberty and is reported to be more common in women. In this article we describe how Ribbing disease can be differentiated from diseases like Engelmann-Camurati disease, van Buchem disease, Erdheim-Chester disease, osteoid osteoma, chronic osteomyelitis, stress fracture, etc

  17. International Rare Histiocytic Disorders Registry (IRHDR)

    Science.gov (United States)

    2018-04-18

    Rare Histiocytic Disorders (RHDs); Juvenile Xanthogranuloma (JXG); Reticulohistiocytoma (Epithelioid Histiocytoma); Xanthoma Disseminatum (XD); Multicentric Reticulohistiocytosis (MRH); Systemic Juvenile Xanthogranuloma; Erdheim-Chester Disease (ECD); Multi-system Rosai-Dorfman Disease (RDD)

  18. MR evaluation of diabetes insipidus

    International Nuclear Information System (INIS)

    Tien, R.D.; Newton, T.H.; Kucharczyk, J.

    1989-01-01

    The authors have used MR imaging to evaluate the hypothalamic/pituitary region in 21 patients with central diabetes insipidus. Diagnoses included histiocytosis-X, germ cell tumors trauma, metastases, tuberculosis, pituitary abscess, and Erdheim-Chester disease. Gd-DTPA was administered in eight patients. Seventeen of 21 patients had a pituitary infundibular stalk that was thicker than that of normal subjects, but the signal intensity of the abnormal stalk was normal. The pituitary stalk was uniformly thickened in the histiocytosis-X and tuberculosis patients. Asymmetric thickening was seen in the patients with germ cell tumors, metastases, and Erdheim-Chester disease. Pituitary stalk was disrupted in the posterior pituitary lobe was absent in all patients

  19. LEBER'S DISEASE. CLINICAL CASE

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    N. N. Maslova

    2013-01-01

    Full Text Available Violation of visual functions and oculomotor frustration develop at 90% of patients with MS. More than a half of patients are transferred by the numerous recurrence of an optical neuritis which is coming to an end with a partial atrophy of optic nerve. For well-timed purpose of pathogenetic treatment differential diagnostics with other diseases, being accompanied an atrophy of an optic nerve, in particular with Leber's disease

  20. Wilson Disease: Case Report

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    Esra Tuğ

    2007-01-01

    Full Text Available Wilson Disease (WD is an autosomal recessive hereditary disease of human copper metabolism, which causes hepatic and neuropsychiatric diseases. Estimated prevalence is 1: 30.000. In WD, ATP7B gene located on chromosome 13 (13q14.3-q21.1, coding the protein for hepatic copper transport and, having an important role in copper metabolism has been affected. Clinical findings in WD are complex and, neurological symptoms such as tremor, disartria and psychiatric disorders, acute liver deficiency, chronic hepatit or cirrhosis may develop. For the last year, 27 years old female patient observed in other medical centre owing to benign positional vertigo applied to our department. Her sister and brother have been diagnosed as WD. No peripheral syptoms of hepatic disease or hepatosplenomegaly existed in our patient. Neurological examination was normal to except for positional tremor. Because our patient had rare clinical features for WD and bad prognosis, presented by us to emphasize necessity of the researched of the most frequent mutations seen in Turkiye.

  1. Pompe disease: A case report

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    Abdullah Çim

    2015-12-01

    Full Text Available Pompe disease is inherited in an autosomal recessive manner, and is usually observed in the children of asymptomatic carriers. Pompe disease, known as Glycogen Storage Disorder type II, is caused by pathogenic mutations in the gene encoding lysosomal acid alpha-glucosidase (GAA. There are three types of Pompe disease: classical infantile form, non-classical infantile form and late-onset Pompe disease. Age of onset and severity of the disease determine the type of Pompe disease. We aimed to identify a mutation in GAA gene in parents who were first cousins and their baby girl was passed away due to the Pompe disease. The baby girl had reduced acid alpha-glucosidase activity, but genetic analysis had not been performed. Mutation analysis of parents was performed using high-throughput DNA sequencing method. Heterozygous mutation of c.896 T>C in exon 5 was found in parents, and prenatal diagnosis was performed for their next pregnancy. In conclusion, c.896 T>C substitution in GAA gene may lead to the severe type of Pompe disease. Using a relatively fast and reliable molecular genetic analysis method to confirm the early diagnosis of the Pompe disease is important for the management of the disease.

  2. Reported cases of selected diseases.

    Science.gov (United States)

    1994-06-01

    The number of reported cases of measles, poliomyelitis, tetanus, diphtheria, and whooping cough for the period of January 1, 1994 to the date of the last report is presented in tabular form by country with a comparison for the same epidemiological period in 1993. The countries included are Bolivia, Colombia, Ecuador, Peru, Venezuela, Argentina, Chile, Paraguay, Uruguay, Brazil, Belize, Costa Rica, El Salvador, Guatemala, Honduras, Nicaragua, Panama, Mexico, Cuba, Haiti, the Dominican Republic, Antigua and Barbuda, the Bahamas, Barbados, Dominica, Grenada, Guyana, Jamaica, Saint Kitts and Nevis, Saint Vincent, Saint Lucia, Suriname, Trinidad and Tobago, Canada, and the US. The figures for measles are given as reported and as confirmed. In some countries, the reported number of cases of measles decreased from 1993 figures (Venezuela 5275 vs. 6060, Paraguay 26 vs. 958, Brazil 272 vs. 958, Canada 30 vs. 38), but, in others, the figure increased from 1993 (Mexico 47 vs. 21, the US 155 vs. 86). There were no reported cases of poliomyelitis for either year in any country. The figures for tetanus are divided into nonneonatal and neonatal. In Brazil the number of nonneonatal cases decreased to 360 from 371 in 1993, and the number of neonatal cases decreased to 28 from 65. In Mexico, nonneonatal cases decreased to 28 from 45, but neonatal cases increased to 23 from 20 in 1993. The number of cases of diphtheria cases in Brazil decreased to 28 from 65 in the same period of 1993. The number of cases of whooping cough decreased to 431 from 1651 in Brazil and to 51 from 70 in Mexico. However, the number of cases in Canada increased to 1047 from 784.

  3. Hydatid disease localized in mesorectum: Case report

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    Abdullah Oğuz

    2015-03-01

    Full Text Available Hydatid disease is a parasitic disease, which is caused by echinococcus and often located in the liver and lung but occasionally found in other organs. Only one previous study reported localization in the mesorectum. In this case report, we present a 27-year-old male, as a second case in the literature, with a hydatid cyst located in the mesorectum. Abdominopelvic computed tomography revealed cystic masses localized in the mesorectum with no pulmonary or hepatic involvement. Preoperative cyst hydatid IgG (1/1000 was positive, and the preliminary diagnosis was hydatid disease. The patient underwent partial cystectomy. Macroscopic and microscopic examination of the specimens confirmed the hydatid cyst. This case report demonstrates that hydatid disease should be taken into consideration in the differential diagnosis of a cystic mass in any anatomic localization, especially in endemic areas. J Clin Exp Invest 2015; 6 (1: 75-77

  4. Menetrier's disease in childhood: a case report

    International Nuclear Information System (INIS)

    Souza, Luis Ronan Marques Ferreira de; Nogueira Filho, Jairo Ramos; Ferme, Andrea Langone; D'Ippolito, Giuseppe; Szejnfeld, Jacob; Goldman, Suzan Menasce

    2005-01-01

    Hypertrophic gastritis was described by Menetrier in 1888, and can be divided into two anatomical categories: occurrence of polyps and gastric mucosa hypertrophy. Menetrier's disease of the stomach affects mostly adults and occurs less frequently in children (less than 100 cases in the literature). The authors report the imaging findings in a child with Menetrier's disease, and present a brie review of the literature. (author)

  5. Timeliness of notification in infectious disease cases.

    OpenAIRE

    Domínguez, A; Coll, J J; Fuentes, M; Salleras, L

    1992-01-01

    Records of notification in cases of eight infectious diseases in the "Servei Territorial de Salut Publica" of the Province of Barcelona, Spain, between 1982 and 1986 were reviewed. Time from onset of symptoms to notification, time from notification to completion of data collection, and time from onset to completion of the case investigation were analyzed. For the period from onset to notification, the shortest mean was registered for meningococcal infection (6.31 days) and the longest was for...

  6. Disease severity in familial cases of IBD.

    Science.gov (United States)

    Andreu, M; Márquez, L; Domènech, E; Gisbert, J P; García, V; Marín-Jiménez, I; Peñalva, M; Gomollón, F; Calvet, X; Merino, O; Garcia-Planella, E; Vázquez-Romero, N; Esteve, M; Nos, P; Gutiérrez, A; Vera, I; Cabriada, J L; Martín, M D; Cañas-Ventura, A; Panés, J

    2014-03-01

    Phenotypic traits of familial IBD relative to sporadic cases are controversial, probably related to limited statistical power of published evidence. To know if there are phenotype differences between familial and sporadic IBD, evaluating the prospective Spanish registry (ENEIDA) with 11,983 cases. 5783 patients (48.3%) had ulcerative colitis (UC) and 6200 (51.7%) Crohn's disease (CD). Cases with one or more 1st, 2nd or 3rd degree relatives affected by UC/CD were defined as familial case. In UC and CD, familial cases compared with sporadic cases had an earlier disease onset (UC: 33 years [IQR 25-44] vs 37 years [IQR 27-49]; p<0.0001); (CD: 27 years [IQR 21-35] vs 29 years [IQR 22-40]; p<0.0001), higher prevalence of extraintestinal immune-related manifestations (EIMs) (UC: 17.2% vs 14%; p=0.04); (CD: 30.1% vs 23.6%; p<0.0001). Familial CD had higher percentage of ileocolic location (42.7% vs 51.8%; p=0.0001), penetrating behavior (21% vs 17.6%; p=0.01) and perianal disease (32% vs 27.1%; p=0.003). Differences are not influenced by degree of consanguinity. When a sufficiently powered cohort is evaluated, familial aggregation in IBD is associated to an earlier disease onset, more EIMs and more severe phenotype in CD. This feature should be taken into account at establishing predictors of disease course. © 2013.

  7. Case series of Stargardt's disease: Our experience

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    Syed Abdul Wadud

    2016-08-01

    Full Text Available Stargardt disease is the most common form of juvenile macular degeneration. Clinically, it is characterized by pisciform flecks at lhe level of the retinal pigment epithelium and a bull's-eye maculopathy. Inheritance is usually autosomal recessive, although dominantly inherited case have been described. Both sexes are affected equally. We reported here three cases of Stargardt's macular dystrophy, who are siblings and daughters of non consanguineous parents. In case-1,2 and 3 we found the typical presentation with almost same findings.

  8. Thoracoabdominal actinomycosis mimicking metastatic disease: case report

    International Nuclear Information System (INIS)

    Ros, L.H.; Villacampa, V.M.; Torres, G.M.; Ros, P.R.

    1999-01-01

    Actinomycosis is a chronic suppurative infection with bacteria of the Actinomycetaceae family, characterized by the formation of abundant granular tissue and multiple abscesses. It is a rare entity, and clinical and radiological findings are similar to those in other inflammatory and in neoplastic processes. Actinomycosis should be considered in the differential diagnosis in high-risk patients with predisposing factors, such as alcoholism, poor oral hygiene, maxillofacial trauma, tuberculosis, chronic obstructive pulmonary disease, steroid ingestion or immunodeficiency, and in patients in whom the disease history does not correlate with widespread metastatic involvement. Early diagnosis is important, to prevent disease progression and unnecessary surgery, since the response to drug treatment is very good. We present a case of diffuse actinomycosis involving multiple organs (liver, kidneys, colon, and lungs) that simulated metastatic disease on radiography and computed tomography (CT). (author)

  9. Thoracoabdominal actinomycosis mimicking metastatic disease: case report

    Energy Technology Data Exchange (ETDEWEB)

    Ros, L.H.; Villacampa, V.M. [Hospital Miguel Servet, Dept. of Radiology, Zaragoza (Spain); Torres, G.M. [Univ. of Florida, Dept. of Radiology, Gainesville, Florida (United States); Ros, P.R. [Harvard Medical School, Brigham and Women' s Hospital, Dept. of Radiology, Boston, Massachusetts (United States)

    1999-12-01

    Actinomycosis is a chronic suppurative infection with bacteria of the Actinomycetaceae family, characterized by the formation of abundant granular tissue and multiple abscesses. It is a rare entity, and clinical and radiological findings are similar to those in other inflammatory and in neoplastic processes. Actinomycosis should be considered in the differential diagnosis in high-risk patients with predisposing factors, such as alcoholism, poor oral hygiene, maxillofacial trauma, tuberculosis, chronic obstructive pulmonary disease, steroid ingestion or immunodeficiency, and in patients in whom the disease history does not correlate with widespread metastatic involvement. Early diagnosis is important, to prevent disease progression and unnecessary surgery, since the response to drug treatment is very good. We present a case of diffuse actinomycosis involving multiple organs (liver, kidneys, colon, and lungs) that simulated metastatic disease on radiography and computed tomography (CT). (author)

  10. Tay Sachs disease: an autopsy case report.

    Science.gov (United States)

    Jadhav, Meenal Vitthal; Landge, Meenal P; Sawaimoon, Satyakam K; Harke, Arun B; Deshmukh, Sanjay D

    2005-10-01

    This report describes a case report of a postmortem performed on a 5-year old patient of Tay-Sachs disease, presenting with failure to thrive, muscular flaccidity, and cherry-red spots on macula on fundoscopy. There was no history of similarly affected sibling or any other family member. The diagnosis was confirmed by enzyme studies. At postmortem, there was no organomegaly. The brain, on microscopy, showed vacuolated swollen neurons.

  11. Creutzfeldt-Jacob Disease: Two Case Reports

    Directory of Open Access Journals (Sweden)

    Aysu Şen

    2006-02-01

    Full Text Available Creutzfeldt-Jakob Disease (CJD is characterised by subacute progressive dementia, cerebellar ataxia, myoclonic jerks together with pyramidal and extrapyramidal signs. It is a rare prion disease and definitive diagnosis can only be made by biopsy. It becomes progressively worse and the death is the rule. We presented two CJD cases because of their demonstrative characteristics. A 43 year-old female and a 52 year-old male patient was suspected to be CJD due to presence of subacute severe cognitive deterioration, neuropsychiatric disturbances, myoclonic jerks, ataxia, pyramidal and extrapyramidal signs and also periodic spike and wave complexes in EEG. Patients were lost in a short period of time because of the complications of disease process. Medical autopsy were done in both cases for definitive diagnosis and autopsy results displayed characteristic pathologic findings of CJD. Patients were diagnosed as definitive sporadic CJD according to Master’s, French and European criterias. CJD should be considered in patients with rapidly progressive dementia, that starts with various neuropsychiatric symptoms. Although seen very rare, CJD is a untreatable, fatal disease. Therefore we emphasize that, preventive precaution should be taken when a CJD diagnosis is suspected

  12. Case definition terminology for paratuberculosis (Johne's disease).

    Science.gov (United States)

    Whittington, R J; Begg, D J; de Silva, K; Purdie, A C; Dhand, N K; Plain, K M

    2017-11-09

    Paratuberculosis (Johne's disease) is an economically significant condition caused by Mycobacterium avium subsp. paratuberculosis. However, difficulties in diagnosis and classification of individual animals with the condition have hampered research and impeded efforts to halt its progressive spread in the global livestock industry. Descriptive terms applied to individual animals and herds such as exposed, infected, diseased, clinical, sub-clinical, infectious and resistant need to be defined so that they can be incorporated consistently into well-understood and reproducible case definitions. These allow for consistent classification of individuals in a population for the purposes of analysis based on accurate counts. The outputs might include the incidence of cases, frequency distributions of the number of cases by age class or more sophisticated analyses involving statistical comparisons of immune responses in vaccine development studies, or gene frequencies or expression data from cases and controls in genomic investigations. It is necessary to have agreed definitions in order to be able to make valid comparisons and meta-analyses of experiments conducted over time by a given researcher, in different laboratories, by different researchers, and in different countries. In this paper, terms are applied systematically in an hierarchical flow chart to enable classification of individual animals. We propose descriptive terms for different stages in the pathogenesis of paratuberculosis to enable their use in different types of studies and to enable an independent assessment of the extent to which accepted definitions for stages of disease have been applied consistently in any given study. This will assist in the general interpretation of data between studies, and will facilitate future meta-analyses.

  13. Kikuchi-Fujimoto Disease: A Case Report

    Directory of Open Access Journals (Sweden)

    Chih-Hung Lee

    2003-05-01

    Full Text Available We describe a rare but typical case of Kikuchi-Fujimoto disease (KFD. Two subcutaneous nodules appeared suddenly on the right of the neck of a 15-year-old girl. Microscopy of a surgical specimen of the larger nodule showed necrotizing lymphadenitis. Prompt treatment with mepirizole resulted in the disappearance of the smaller nodule. No recurrence was noted during 6 months of follow-up. KFD is a rare, self-limiting, necrotizing histiocytic lymphadenitis that needs to be differentiated from malignant lymphoma. Any nodal biopsy showing fragmented nuclei, necrosis, and karyorrhexis without prominent neutrophils should alert the physician to consideration of KFD, especially in a young woman presenting with cervical lymphadenopathy.

  14. Seasonal ataxia: a case report of a disappearing disease

    African Journals Online (AJOL)

    Seasonal ataxia: a case report of a disappearing disease. Adebiyi Ayoade ... ological profiles of the disease. ... low serum albumin levels [8] act as a potentiating factor to trigger the ... 65% neutrophils, 25% lymphocytes,eosinophils 5% and.

  15. Addison's Disease Mimicking as Acute Pancreatitis: A Case Report.

    Science.gov (United States)

    Chaudhuri, Sayani; Rao, Karthik N; Patil, Navin; Ommurugan, Balaji; Varghese, George

    2017-04-01

    Over past two decades there has been significant improvement in medical field in elucidating the underlying pathophysiology and genetics of Addison's disease. Adrenal insufficiency (Addison's disease) is a rare disease with an incidence of 0.8/100,000 cases. The diagnosis may be delayed if the clinical presentation mimics a gastrointestinal disorder or psychiatric illness. We report a case of Addison's disease presenting as acute pain in abdomen mimicking clinical presentation of acute pancreatitis.

  16. Lhermitte-duclos Disease: A Case Report.

    OpenAIRE

    Dr Varun Chaudhary; Dr Ami Jani

    2016-01-01

    Lhermitte-Duclos disease (LDD) is a benign neoplasm of posterior fossa, involving cerebellum. It is also known as dysplastic cerebellar gangliocytoma. It is not a true neoplasm but a hamartoma. It can be either isolated finding or associated with Cowden disease (multiple hamartoma syndrome). Lhermitte-Duclos disease typically presents in young adults.

  17. Kawasaki disease in Ghana: Case reports from Korle Bu Teaching ...

    African Journals Online (AJOL)

    Kawasaki disease, an acute febrile vasculitis, predominantly affects children under the age of 5 years and is thought to be a rare disease in the developing world. . It has previously never been reported in Ghana. We report 3 cases from February, 2007 to February, 2008. This potentially serious disease has no definitive ...

  18. Two cases of Kawasaki disease presented with acute febrile jaundice.

    Science.gov (United States)

    Kaman, Ayşe; Aydın-Teke, Türkan; Gayretli-Aydın, Zeynep Gökçe; Öz, Fatma Nur; Metin-Akcan, Özge; Eriş, Deniz; Tanır, Gönül

    2017-01-01

    Kawasaki disease is an acute, systemic vasculitis of unknown etiology. Although gastrointestinal involvement does not belong to the classic diagnostic criteria; diarrhea, abdominal pain, hepatic dysfunction, hydrops of gallbladder, and acute febrile cholestatic jaundice are reported in patients with Kawasaki disease. We describe here two cases presented with fever, and acute jaundice as initial features of Kawasaki disease.

  19. Eye and rare genetic diseases: Case series and literature review ...

    African Journals Online (AJOL)

    Genetic diseases are generally characterised by a multi visceral pathogenesis. Although orphan, these diseases interest many disciplines due to their clinical expression. Eye is sometimes part of the clinical polymorphism of some rare genetic diseases. Ocular signs are in some cases leading to the diagnosis of these ...

  20. Diaphragm disease of the small intestine: an interesting case report.

    Science.gov (United States)

    Ullah, Sana; Ajab, Shereen; Rao, Rajashekhar; Raghunathan, Girish; DaCosta, Philip

    2015-06-01

    Diaphragm disease of small intestine usually presents with nonspecific clinical features. Radiological investigations often fail to differentiate it from small intestinal tumors and inflammatory bowel disease. It is therefore diagnosed on final histology after surgical resection. We hereby report an interesting case of a suspected small bowel tumor later diagnosed as diaphragm disease on histology. © The Author(s) 2014.

  1. Kimura's disease: A case presentation of postauricular swelling ...

    African Journals Online (AJOL)

    Kimura's disease: A case presentation of postauricular swelling. A Rajesh, T Prasanth, V.C. Naga Sirisha, M.D.S. Azmi. Abstract. Kimura's disease (KD) is a rare chronic inflammatory disease of subcutaneous tissues and occurs predominantly in head and neck region. It is seen primarily in young Asian males. Typical clinical ...

  2. KIKUCHI-FUJIMOTO DISEASE (KFD): A Rare Case Report

    OpenAIRE

    Dr. Gunja Jain; Dr. Mayank Gupta; Dr. Laxmikant Goyal; Dr. Jai Purohit; Dr. Sudhir Mehta

    2017-01-01

    Abstract— Kikuchi-Fujimoto disease (KFD) is a rare disease. It has a worldwide distribution with a higher prevalence in Asians. KFD is usually a self limiting disease and benign in nature. Clinically it presents as regional cervical lymphadenopathy and sometimes may presents as generalized lymphadenopathy as well. Night sweats and low grade fever may also be associated in some cases. A case of a 36 year old female had attended in SMS Hospital. She presented with fever, weight loss and tender ...

  3. A case of primary osseous pelvic hydatid disease (echinococcus granulosus)

    Energy Technology Data Exchange (ETDEWEB)

    Sinner, W.N. von (King Faisal Specialist Hospital and Research Centre, Riyadh (Saudi Arabia). Dept. of Radiology)

    1991-07-01

    Primary bone involvement in hydatid disease (HD) is rare. Sporadic reports estimate its prevalence to 1%. Only a few cases have been reported where CT has been used. Magnetic resonance imaging (MRI) of primary pelvic skeletal hydatid disease has, to my best knowledge, previously not been published. This report deals with a case of primary hydatid disease of the right ilium and the sacrum examined by plain film radiography. CT and MRI confirmed by pathology. (orig.).

  4. A case of primary osseous pelvic hydatid disease (echinococcus granulosus)

    International Nuclear Information System (INIS)

    Sinner, W.N. von

    1991-01-01

    Primary bone involvement in hydatid disease (HD) is rare. Sporadic reports estimate its prevalence to 1%. Only a few cases have been reported where CT has been used. Magnetic resonance imaging (MRI) of primary pelvic skeletal hydatid disease has, to my best knowledge, previously not been published. This report deals with a case of primary hydatid disease of the right ilium and the sacrum examined by plain film radiography. CT and MRI confirmed by pathology. (orig.)

  5. Addison's disease and pregnancy: case report.

    Science.gov (United States)

    Cosimo, Caterina; Franco, Ciro

    2009-10-01

    Addisonian crises, a rare but life-threatening event in pregnant women, may accompany stressful conditions such as labor, puerperium, infection, hyperemesis gravidarum or surgery. A 36-year-old woman, primigravida, with Addison's disease, diagnosed when she was 10 year-old and treated with cortisone and fludrocortisone. The therapy was regulated to avoid adrenal crisis during pregnancy. The woman underwent to caesarean section at 38th week and gave birth to a normal baby. The successful management of pregnant women with Addison's disease, regarding her state and that of the foetus, reassures those women that nowadays Addison's disease and pregnancy are not incompatible when proper monitoring and management is provided.

  6. Kawasaki disease: review of 21 cases

    Directory of Open Access Journals (Sweden)

    Cem Arat

    2016-03-01

    Conclusion: Kawasaki disease has difficulty in diagnosis because of having broad spectrum of presenting symptoms. Early diagnosis and treatment is very important in preventing coronary artery abnormalities. [Cukurova Med J 2016; 41(1.000: 97-104

  7. [Venous thromboembolic disease: presentation of a case].

    Science.gov (United States)

    Mirpuri-Mirpuri, P G; Álvarez-Cordovés, M M; Pérez-Monje, A

    2013-01-01

    Venous thromboembolic disease in its clinical spectrum includes both deep vein thrombosis and pulmonary thromboembolism, which is usually a complication of deep vein thrombosis. It is a relatively common disease with significant morbidity and requires an accurate diagnosis. They are numerous risk factors for venous thromboembolism, and there is evidence that the risk of thromboembolic disease increases proportionally to the number of predisposing risk factors present. The primary care physician should know the risk factors and suspect the presence of venous thromboembolic disease when there is a compatible clnical picture. The treatment for this pathology is anticoagulation. We report a patient with cardiovascular risk factors who was seen with pain in the right leg and shortness of breath and referred to the hospital with suspected venous thromboembolism, atrial fibrillation and pleural effusion. Copyright © 2012 Sociedad Española de Médicos de Atención Primaria (SEMERGEN). Publicado por Elsevier España. All rights reserved.

  8. A case of osteomyelitis of mandibular bone in Kimura's disease

    Energy Technology Data Exchange (ETDEWEB)

    Kinugawa, Hiroshi; Shimada, Fumihiko; Sai, Yoshikazu; Amakata, Yoshikuni; Yoshitake, Kazusada [Shiga Univ. of Medical Science, Otsu (Japan)

    1991-07-01

    We experienced a case of osteomyelitis of mandibular bone in Kimura's disease. The patient received radiation therapy to head and neck area against the same disease. Bone tissue which received radiation therapy had developed malnutrition as side effect of radiation, and osteomyelitis was induced by infection from the teeth. Even in the benign soft tissue disease like Kimura's disease, especially after radiation therapy, pathologic fracture of bone may happen due to bone damage caused by radiation. (author).

  9. Kawasaki disease following Rocky Mountain spotted fever: a case report

    Directory of Open Access Journals (Sweden)

    Bal Aswine K

    2009-07-01

    Full Text Available Abstract Introduction Kawasaki disease is an idiopathic acute systemic vasculitis of childhood. Although it simulates the clinical features of many infectious diseases, an infectious etiology has not been established. This is the first reported case of Kawasaki disease following Rocky Mountain spotted fever. Case presentation We report the case of a 4-year-old girl who presented with fever and petechial rash. Serology confirmed Rocky Mountain spotted fever. While being treated with intravenous doxycycline, she developed swelling of her hands and feet. She had the clinical features of Kawasaki disease which resolved after therapy with intravenous immune globulin (IVIG and aspirin. Conclusion This case report suggests that Kawasaki disease can occur concurrently or immediately after a rickettsial illness such as Rocky Mountain spotted fever, hypothesizing an antigen-driven immune response to a rickettsial antigen.

  10. Intraoral Morgellons disease or delusional parasitosis: a first case report.

    Science.gov (United States)

    Dovigi, Allan J

    2010-08-01

    Morgellons disease is a new emerging disease that is still controversial and believed to be, by some practitioners, as nothing more than delusional parasitosis. The Center for Disease Control has recently launched an epidemiological investigation into this disease due to the increased number of reports. A first case is reported of an oral lesion and symptoms consistent with Morgellons disease. The nature of the characteristic fibers associated with the intraoral lesion is investigated. Research has started at a number of institutions to elucidate the nature of this emerging disease.

  11. Addison's disease secondary to connective tissue diseases: a report of six cases.

    Science.gov (United States)

    Zhang, Zhuo-li; Wang, Yu; Zhou, Wei; Hao, Yan-jie

    2009-04-01

    Addison's disease is an autoimmune process. However, Addison's disease associated with connective tissue diseases (CTD) is only occasionally reported. Here, we report six cases of Addison's disease secondary to a variety of CTD, which include systemic lupus erythematosus, Takayasu arteritis, systemic sclerosis, ankylosing spondylitis (AS) and antiphospholipid antibody syndrome. The association of Addison's disease with Takayasu arteritis and AS is reported for the first time. We also found high prevalence of hypothyroidism as concomitant autoimmune disorder. Our case series highlight the autoimmune features of Addison's disease. Therefore, we suggest considering adrenal dysfunction in patients with CTD.

  12. Hypnosis in the treatment of Morgellons disease: a case study.

    Science.gov (United States)

    Gartner, Ashley M; Dolan, Sara L; Stanford, Matthew S; Elkins, Gary R

    2011-04-01

    Morgellons Disease is a condition involving painful skin lesions, fibrous growths protruding from the skin, and subcutaneous stinging and burning sensations, along with symptoms of anxiety, depression, fatigue, and memory and attention deficits. The etiological and physiological bases of these symptoms are unclear, making the diagnosis controversial and challenging to treat. There are currently no established treatments for Morgellons Disease. The following case example depicts treatment of a woman with Morgellons Disease using hypnotherapy. Data from this case example suggest that hypnotherapy is a promising intervention for the physical and psychological symptoms associated with Morgellons Disease.

  13. Anaesthesiological implications of Kimura's disease: a case report

    Directory of Open Access Journals (Sweden)

    Sorbello Massimiliano

    2009-06-01

    Full Text Available Abstract Introduction Kimura's disease is a chronic inflammatory condition belonging to the angio-lymphatic proliferative group of disorders, usually affecting young men of Asian race, but is rare in Western countries. It is a benign but locally injurious disease, of unknown aetiology, whose classical clinical features are a tumour-like swelling, usually in the head and neck, with or without satellite lymphadenopathy, often accompanied by eosinophilia and elevated serum IgE. Case presentation We report the case of a 33-year-old Caucasian woman with an atypical localization of Kimura's disease, discussing the anaesthesiological implications and reviewing the current literature on Kimura's disease. Conclusions The diagnosis of Kimura's disease can be difficult and misleading, and anaesthesiological precautions could be ignored. Patients with this disease are often evaluated for other disorders: unnecessary diagnostic tests and investigations, or even surgery, may be avoided by just being aware of Kimura's disease.

  14. Kienböck’s Disease: Case Report - Case Report

    Directory of Open Access Journals (Sweden)

    Hayal Güler

    2009-04-01

    Full Text Available Osteonecrosis is defined as the death of bone due to interruption of the blood supply to the bone. Kienböck’s disease is osteonecrosis of lunate bone as a result of heavy repetitive loading on wrist. We planned to present a 39-year-old patient having pain, swelling and limitation of range of motion (ROM in right wrist, because Kienböck’s disease should be into account in differential diagnosis of such patients. X-ray of the right wrist of patient demonstrated sclerosis, cystic appearance and irregularity in lunate bone. Irregularities, loss of volume and diffuse low-intensity appearance were observed in lunate bone on T1-weighted MR images. T2-weighted fat-saturated MR images demonstrated diffuse hyperintensity in the same areas which is suggested bone-marrow edema. These findings were determined as the changes with related osteonecrosis of the lunate bone. Resting splint and antiinflammatory treatment were given to the patient. The patient was consulted to the department of orthopedics and traumatology. Patient refused operation. Pain, swelling and limitation of ROM of the patients improved after 3 weeks. In conclusion, Kienböck’s disease should be into account in the differential diagnosis of patients having pain, swelling and limitation of ROM in wrist. (From the World of Osteoporosis 2009;15:26-8

  15. Graves Disease And Down Sindrome : Clinical Case

    Directory of Open Access Journals (Sweden)

    Scrinic Olesea

    2015-08-01

    Full Text Available Introduction: Pacients with Down’s syndrome present an increase revalence of autoimune endocrine disorders. We communicate the case of 14 years and 6 months old pacient known with Down syndrome admitted in Endocrinology department with suspicion of hyperthyroidism, the diagnosis being confirmed by hormonal dosage. The particularity of the case consists in: symptomatology onset during puberty, clinical evolution with mild symptoms, without ocular involvement, morphological and functional remission obtained relatively soon after the initiation of antithyroid therapy, lack of posttherapy side effects, favorabile evolution under the “block and replace” therapy

  16. CASE STUDY – HIV AND LUNG DISEASE

    African Journals Online (AJOL)

    2011-04-02

    Apr 2, 2011 ... pathology deep to the paraseptal bullae. An intercostal drain tip is seen in the left lateral pleural space. Fig. 2. Axial computed tomography scan on lung windows. Large bilateral paraseptal bullae are demonstrated with residual antero-medial pneumothorax. 37. CASE STUDY – HIV AND LUNG DISEASE ...

  17. Prader-Willi Disease: A Case Study.

    Science.gov (United States)

    Forbus, William R., III

    A case study focuses on the characteristics and physical management of a 15-year-old with Prader-Willi Syndrome, a birth defect associated with hypotonia, insatiable appetite, hypogonadism, central nervous system dysfunction, and abnormal growth and development . A literature review addresses studies dealing with behavior modification of obesity…

  18. [Mondor's disease of penis: A case report].

    Science.gov (United States)

    Marsaudon, E; Legal, C; Gayoux, D; Weber, O

    2016-09-01

    Mondor's disease is a rare superficial thrombophlebitis, historically involving the thoracic venous system of women. However, it can occur in both gender and all over the skin. We report a 40-year-old man with type one diabetes who presented with a thrombosis of the superficial dorsal vein of the penis that he treated as a fungal infection. Treatment with non-steroidal anti-inflammatory drugs and low molecular weight heparin contributed to a favorable outcome in 2 weeks. Pathogenesis and treatment of Mondor's disease remain incompletely dominated. Some predisposing factors have been highlighted in the literature. It might be interesting to add diabetes, due to its frequent pelvic problems leading to a potential venous inflammatory trigger. Copyright © 2015 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier SAS. All rights reserved.

  19. Canine Degenerative Valve Disease: A Case Report

    Directory of Open Access Journals (Sweden)

    Carmenza Janneth Benavides Melo

    2014-07-01

    Full Text Available Degenerative valvular disease or endocardiosis is the most common cardiovascular pathology in dogs. It is characterized by regurgitation of blood into the atria with decreased cardiac output, leading to volume overload with eccentric hypertrophy and congestive heart failure. This report describes the clinical and autopsy findings of a dog, suggestive of valvular endocardiosis. The patient was admitted to the outpatient Veterinary Clinic “Carlos Martínez Hoyos” at the University of Nariño (Pasto, Colombia. His owner said the dog was sick for two months, with signs of respiratory disease, weight loss, and decay. Clinical examination showed very pale mucous membranes, inspiratory dyspnea, rale, split S2, grade 4 mid-systolic murmur of regurgitation, and abdominal dilatation with sign of positive shock wave. Necropsy evidenced plenty of translucent watery material in the abdominal, chest and pericardium cavity, severely enlarged and rounded heart with thickened atrioventricular valves, moderate reduction in liver size and signs of lobulation, severely diminished and pale kidneys with irregular surface showing the presence of multiple cystic areas in corticomedullary region. Samples were taken from these tissues and fixed in 10% buffered formalin to be processed for histopathological analysis at the Laboratory of Pathology at the University of Nariño, using hematoxylin and eosin stain. This way, degenerative valvular disease was diagnosed.

  20. Cases of Lyme disease reported in a military community.

    Science.gov (United States)

    Underwood, P K; Armour, V M

    1993-02-01

    Lyme disease, a growing public health problem in the United States, is also an increasing threat in Europe. Cases identified in a military community in West Germany are presented and problems of diagnosis and treatment discussed.

  1. CASE REPORT : GRAVE'S DISEASE PRESENTING AS PARANOID SCHIZOPHRENIA

    OpenAIRE

    Singh, S.K.; Hatwal, A.; Agarwal, J.K.; Bajpai, H.S.; Sharma, I.

    1989-01-01

    SUMMARY The case of a 37 year old male is described who initially presented as paranoid schizophrenia unresponsive to anti-psychotic drug treatment and subsequently developed features of Grave's disease. Treatment with carbimazole alone improved his psychiatric symptoms.

  2. Kawasaki disease following Rocky Mountain spotted fever: a case report.

    Science.gov (United States)

    Bal, Aswine K; Kairys, Steven W

    2009-07-06

    Kawasaki disease is an idiopathic acute systemic vasculitis of childhood. Although it simulates the clinical features of many infectious diseases, an infectious etiology has not been established. This is the first reported case of Kawasaki disease following Rocky Mountain spotted fever. We report the case of a 4-year-old girl who presented with fever and petechial rash. Serology confirmed Rocky Mountain spotted fever. While being treated with intravenous doxycycline, she developed swelling of her hands and feet. She had the clinical features of Kawasaki disease which resolved after therapy with intravenous immune globulin (IVIG) and aspirin. This case report suggests that Kawasaki disease can occur concurrently or immediately after a rickettsial illness such as Rocky Mountain spotted fever, hypothesizing an antigen-driven immune response to a rickettsial antigen.

  3. Ocular manifestations of Gaucher disease: case report and literature review

    Directory of Open Access Journals (Sweden)

    Mejía-Turizo, Juan Carlos

    2017-07-01

    Full Text Available We report the case of a patient with Gaucher disease (GD type 3b, with a homozygous GBA gene mutation (c.1448T > C p.L483P (L444P. Ocular findings characteristic of this mutation are described, including vitreous condensation and macular edema. To our knowledge this is the first case reported in Colombia with these characteristics. A review of the ocular manifestations of this disease is also presented.

  4. A case of Lyme disease in a Japanese woman

    Directory of Open Access Journals (Sweden)

    Seki M

    2018-04-01

    Full Text Available Masafumi Seki,1 Yuji Watanabe,2 Hiroki Kawabata3 1Division of Infectious Diseases and Infection Control, Tohoku Medical and Pharmaceutical University Hospital, Sendai City, Miyagi, Japan; 2Laboratory for Clinical Microbiology, Tohoku Medical and Pharmaceutical University Hospital, Sendai City, Miyagi, Japan; 3Department of Bacteriology-1, National Institute of Infectious Diseases, Tokyo, Japan Abstract: This report presents the case of a Japanese patient who developed Lyme disease. The patient was a 44-year-old woman who had general fatigue and the inability to open her mouth. She was initially suspected of having mild tetanus with lockjaw; however, she reported a past history of a tick bite while camping in the USA and had erythema migrans 2 months before this visit. Finally, Lyme disease was serologically confirmed. A few cases of Lyme disease are annually reported in Japan; however, this infectious disease should also be suspected. Keywords: Borrelia burgdorferi, Clostridium tetani, tetracycline, facial nerve palsy, erythema migrans

  5. Unilateral segmental Darier disease following Blaschko lines: A case report

    Directory of Open Access Journals (Sweden)

    César Bimbi

    2017-07-01

    Full Text Available Darier disease is an autosomal-dominant disorder of keratin production which leads to a loss in epithelial adhesion and abnormal keratinization. The clinical correspondence is keratotic papules grouped in sebaceous areas of trunk, scalp, forehead and flexures. It is a rare disease and the variant focused on here of unilateral segmental distribution following the lines of Blaschko is rarer still, considering the fact that this presentation counts for only 10% of this already uncommom disease and with only 40 cases being reported in English medical literature. Mutation in this gene is expressed in the skin and brain. The treatment of Darier disease can be challenging and is often difficult and sometimes unsatisfactory. Systemic retinoids are considered the drug of choice for treating Darier disease. However, their use is limited by potential side effects. We described the case a metalworker male with unilateral segmental Darier disease following Blaschko lines and we review the literature on this subject.

  6. Addison’s Disease: A rare case report

    Directory of Open Access Journals (Sweden)

    Sanjay N. Agrawal

    2015-04-01

    Full Text Available A female patient presented with progressive weakness, asthenia and generalized hyperpigmentation. The characteristic hyperpimentation pointed towards possibility of Addison’s disease which was proved by markedly decreased plasma cortisol levels, hyponatremia and hyperkalemia. This could be one of the very few cases of Addison’s Disease reported.

  7. Auditory and optic neuropathy in Kjer's disease : case report

    NARCIS (Netherlands)

    Haaksma-Schaafsma, S. C.; van Dijk, P.; Dikkers, F. G.

    Objective: Description of a female patient with diagnosed Kjer's disease and sensorineural hearing loss, who specifically complained of a progressive inability to understand speech in noisy situations. Design: Case report. Subject: A 30-year-old, Caucasian woman with Kjer's disease. Results:

  8. Hard metal lung disease: a case series.

    Science.gov (United States)

    Mizutani, Rafael Futoshi; Terra-Filho, Mário; Lima, Evelise; Freitas, Carolina Salim Gonçalves; Chate, Rodrigo Caruso; Kairalla, Ronaldo Adib; Carvalho-Oliveira, Regiani; Santos, Ubiratan Paula

    2016-01-01

    To describe diagnostic and treatment aspects of hard metal lung disease (HMLD) and to review the current literature on the topic. This was a retrospective study based on the medical records of patients treated at the Occupational Respiratory Diseases Clinic of the Instituto do Coração, in the city of São Paulo, Brazil, between 2010 and 2013. Of 320 patients treated during the study period, 5 (1.56%) were diagnosed with HMLD. All of those 5 patients were male (mean age, 42.0 ± 13.6 years; mean duration of exposure to hard metals, 11.4 ± 8.0 years). Occupational histories were taken, after which the patients underwent clinical evaluation, chest HRCT, pulmonary function tests, bronchoscopy, BAL, and lung biopsy. Restrictive lung disease was found in all subjects. The most common chest HRCT finding was ground glass opacities (in 80%). In 4 patients, BALF revealed multinucleated giant cells. In 3 patients, lung biopsy revealed giant cell interstitial pneumonia. One patient was diagnosed with desquamative interstitial pneumonia associated with cellular bronchiolitis, and another was diagnosed with a hypersensitivity pneumonitis pattern. All patients were withdrawn from exposure and treated with corticosteroid. Clinical improvement occurred in 2 patients, whereas the disease progressed in 3. Although HMLD is a rare entity, it should always be included in the differential diagnosis of respiratory dysfunction in workers with a high occupational risk of exposure to hard metal particles. A relevant history (clinical and occupational) accompanied by chest HRCT and BAL findings suggestive of the disease might be sufficient for the diagnosis. Descrever aspectos relacionados ao diagnóstico e tratamento de pacientes com doença pulmonar por metal duro (DPMD) e realizar uma revisão da literatura. Estudo retrospectivo dos prontuários médicos de pacientes atendidos no Serviço de Doenças Respiratórias Ocupacionais do Instituto do Coração, localizado na cidade de S

  9. Multiple symmetrical lipomatosis (Madelung's disease) - a case report

    International Nuclear Information System (INIS)

    Vieira, Marcelo Vasconcelos; Abreu, Marcelo de; Furtado, Claudia Dietz; Silveira, Marcio Fleck da; Furtado, Alvaro Porto Alegre; Genro, Carlos Horacio; Grazziotin, Rossano Ughini

    2001-01-01

    Multiple symmetrical lipomatosis (Madelung's disease) is a rare disorder characterized by deep accumulation of fat tissue, involving mainly the neck, shoulders and chest. This disease is associated with heavy alcohol intake and it is more common in men of Mediterranean origin. This disease can cause severe aesthetic deformities and progressive respiratory dysfunction. We report a case of a patient with multiple symmetrical lipomatosis and describe the clinical and radiological features of this disorder. (author)

  10. IMPULSIVE-COMPULSIVE DISORDERS IN PARKINSON'S DISEASE. CLINICAL CASES

    OpenAIRE

    N. V. Fedorova; A. V. Nikitina

    2015-01-01

    Objective – a description of clinical cases of impulsive­compulsive disorders in Parkinson,s disease. The first clinical case. Patient N., 75 years old, suffering for 15 years from Parkinson,s disease, akinetic­rigid form of the disease, stage 4 by Hyun–Yar. Since 2009, he received levodopa/carbidopa 250/25 mg 5 times/day (daily dose of 1250 mg of levodopa); pramipexole 3.5 mg per day (daily dose 3.5 mg), amantadine sulfate 100 mg 5 times/day (daily dose 500 mg). While taking antiparkinsonian...

  11. The neuropsychiatric aspect of Addison's disease: a case report.

    Science.gov (United States)

    Abdel-Motleb, Mohamed

    2012-10-01

    Chronic adrenal insufficiency, known as Addison's disease, presents with a constellation of symptoms and signs. The neuropsychiatric aspect of this condition is not fully understood and not much has been documented about it in the English literature. This article presents a case of a 41-year old male patient who presented initially with depression after a recent life stressor. After his condition escalated and therapy continued to fail, the medical team revised its diagnosis to Addison's disease. Neuropsychiatric symptoms could be the first presentation of Addison's disease, and thus should be kept in mind whenever such a case presents to the physician.

  12. Graves' disease presenting as pseudotumor cerebri: a case report

    Directory of Open Access Journals (Sweden)

    Freitas Cláudia

    2011-02-01

    Full Text Available Abstract Introduction Pseudotumor cerebri is an entity characterized by elevated intracranial pressure with normal cerebrospinal fluid and no structural abnormalities detected on brain MRI scans. Common secondary causes include endocrine pathologies. Hyperthyroidism is very rarely associated and only three case reports have been published so far. Case presentation We report the case of a 31-year-old Luso-African woman with clinical symptoms and laboratory confirmation of Graves' disease that presented as pseudotumor cerebri. Conclusion This is a rare form of presentation of Graves' disease and a rare cause of pseudotumor cerebri. It should be remembered that hyperthyroidism is a potential cause of pseudotumor cerebri.

  13. Unilateral Darier’s diseasecase report

    Directory of Open Access Journals (Sweden)

    Jolanta Węgłowska

    2017-11-01

    Full Text Available Introduction . Darier’s disease (dyskeratosis follicularis, keratosis follicularis, Darier-White disease, Darier disease is a rare genodermatosis inherited in autosomal dominant manner, caused by a mutation in the ATP2A2 gene located on chromosome 12. The gene encodes SERCA2 ATPase, an enzyme responsible for the metabolism of calcium ions in the endoplasmic reticulum. Darier’s disease is characterized by small brown hyperkeratotic papules which appear already in childhood or early adolescence. Skin lesions are located primarily in seborrhoeic areas. In addition to typical skin eruptions, the clinical picture may include involvement of the nails and mucous membranes. The histopathological pattern is characterized by the coexistence of two abnormalities: acantholysis and dyskeratosis. Objective . To present and discuss a case of unilateral Darier’s disease diagnosed and treated at our medical centre. Case report . We present the case of a 46-year old woman with Darier’s disease manifesting as skin lesions in the form of typical hyperkeratotic papules localized on the left part of the trunk and on left-side limbs. The diagnosis was made on the basis of clinical features and histopathological findings. A good therapeutic effect was achieved after introducing treatment with acitretin at a dose of 25 mg/day. Conclusions . The case presented is interesting because of the rarity of unilateral Darier’s disease.

  14. Carotid body tumor imitator: An interesting case of Castleman's disease.

    Science.gov (United States)

    Shakir, Hakeem J; Diletti, Sara M; Hart, Alexandra M; Meyers, Joshua E; Dumont, Travis M; Siddiqui, Adnan H

    2015-01-01

    There are very few reports in the literature of Castleman's disease affecting the carotid artery and a single previous report of a case of Castleman's disease of the neck originally mistaken as a carotid body tumor. We describe a rare case of Castleman's disease, manifesting with classic radiographic hallmarks of a carotid body tumor. The postoperative pathologic examination identified the resected mass as Castleman's lymphadenopathy. The management of this particular case is discussed, and the findings are highlighted. We present a unique case of a tumor initially and incorrectly diagnosed as a carotid body tumor. However, after comprehensive treatment with endovascular and surgical modalities and subsequent pathologic examination, the diagnosis of this rare entity was made.

  15. Periodontal disease and anemias associated with Crohn's disease. A case report.

    Science.gov (United States)

    Nagpal, Swati; Acharya, Anirudh B; Thakur, Srinath L

    2012-03-01

    Crohn's disease (CD) is an inflammatory bowel disease with oral findings, including periodontal manifestations. Anemias, such as iron deficiency and anemia of chronic disease (ACD), are the most common hematologic complications of CD. Periodontitis has systemic effects, and may tend toward anemia, which can be explained by depressed erythropoiesis. In the report presented here, the authors review a case of Crohn's disease diagnosed 10 years previous to the patient presenting with a changing anemic profile and periodontal disease. A discussion of patient and disease management is included.

  16. A rare case of Weil's disease with alveolar haemorrhage

    Directory of Open Access Journals (Sweden)

    Abhiram Chakrabarti

    2014-05-01

    Full Text Available Leptospirosis, a disease of protean manifestations occurs sporadically throughout the year with a peak seasonal incidence during the rainy season mimicking other febrile viral illness. In the rare case, the disease leads to renal and hepatic involvement with hemorrhage which may be associated with multisystem organ dysfunction in form of pulmonary, cardiac and central nervous system, when it is known as Weil's disease. Rarely haemorrhagic manifestations are assosciated. Early diagnosis is important as sometimes the disease may be life threatening. Proper antibiotics results in dramatic improvement. We hereby presented a case that had clinical features of Weil's disease with cough, dyspnoea and haemoptysis. Leptospirosis was detected on ELISA testing. Patient was cured rapidly with antibiotics.

  17. A rare case of Weil's disease with alveolar haemorrhage.

    Science.gov (United States)

    Chakrabarti, Abhiram; Nandy, Manab; Pal, Dipankar; Mallik, Sudesna

    2014-05-01

    Leptospirosis, a disease of protean manifestations occurs sporadically throughout the year with a peak seasonal incidence during the rainy season mimicking other febrile viral illness. In the rare case, the disease leads to renal and hepatic involvement with hemorrhage which may be associated with multisystem organ dysfunction in form of pulmonary, cardiac and central nervous system, when it is known as Weil's disease. Rarely haemorrhagic manifestations are assosciated. Early diagnosis is important as sometimes the disease may be life threatening. Proper antibiotics results in dramatic improvement. We hereby presented a case that had clinical features of Weil's disease with cough, dyspnoea and haemoptysis. Leptospirosis was detected on ELISA testing. Patient was cured rapidly with antibiotics.

  18. Extranodal Rosai Dorfman disease in multiple sites: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Shim, Jong Joon; Kim, Ho Kyun; Shim, Jae Chan; Lee, Kyoung Eun; Lee, Ghi Jai; Suh, Jung Ho; Hong, Seong Woo; Lee, Hye Kyung [Seoul Paik Hospital/Inje Univ. College of Medicine, Seoul (Korea, Republic of)

    2012-07-15

    Rosai Dorfman disease involves an abnormal proliferation of histiocytes. This abnormal growth tends to occur within the lymph nodes, with occasional extranodal presentation. Rosai Dorfman disease is a rare disease, and the extranodal cases are even more uncommon. We report a rare case of extranodal Rosai Dorfman disease in multiple sites in a 56 year old male patient. Abdominopelvic CT revealed soft tissue attenuation masses, encasing both the renal pelvis and both ureters, as well as the thoracic vertebra. Following the neck sonography, both submandibular glands had an enlarged honey combed appearance. Although Rosai Dorfman disease is rare, it should be considered as a potential differential diagnosis when multiple sites involving soft tissue attenuation masses are observed with sonogram and CT.

  19. Hematologic manifestations of Crohn's disease: two clinical cases

    Directory of Open Access Journals (Sweden)

    O. V. Taratina

    2017-01-01

    Full Text Available Inflammatory bowel diseases (IBD are commonly associated with extraintestinal manifestations, hematological disorders being the most special among them. In some cases, they dominate the clinical picture masking the intestinal manifestations of the underlying disease. Aplastic anemia is an extremely rare extraintestinal IBD manifestation. There are only two clinical cases of aplastic anemia associated with ulcerative colitis and non with Crohn's disease reported in the literature. Combination of Crohn's disease and В₁₂-deficient anemia is more prevalent, but is seen usually only after more than 20 cm of the ileus has been resected. The first clinical case presented in this paper is a  combination of severe fistula-forming Crohn's disease with a constriction in the terminal part of the ileus and profound pancytopenia as an outcome of aplastic anemia. This profound pancytopenia is associated with an extremely high risk of life-threatening complications both of surgical treatment, as well as of several chemotherapeutic agents, which made the management of this patient difficult. The second clinical case demonstrates the manifestation of Crohn's disease as ileocolitis starting from the symptoms of cobalamin deficiency: severe В₁₂-deficient anemia, funicular myelosis and sensory ataxia, with blunted intestinal symptoms. This made the initial diagnosis and timely treatment difficult. Replacement therapy with cobalamin injections and treatment with glucocorticoids and antibacterials led to endoscopically confirmed remission of Crohn's disease and normalization of hematological parameters, with persistent polyneuropathy. Thus, management of patients with Crohn's disease should be multidisciplinary. In the case of anemia, leucopenia and/or thrombocytopenia in IBD patients it is necessary to exclude potential myelodysplasia and bone marrow aplasia. In the event of megaloblastic anemia and/or progressive polyneuropathy one should bear in mind

  20. A case of enchondromatosis (Ollier's disease)

    International Nuclear Information System (INIS)

    Lee, Chul Se; Lee Seung; Kim, Byung Soo

    1971-01-01

    A case of enchondromatosis confirmed by the x-ray and microscopic findings, was reported, who was 25 year old man with the complaints of the globular mass deformities of both hands and the limping owing to the shortening of right leg and the genu varum deformity of the lower extremities during about 21 years. His family history was non-contributory. The x-ray films of the chest, lumbar spine, and both extremities and hands, showed the various sized cartilaginous globular mass es consisting with some of calcium deposits originating from the bony tissues especially in the metaphyseal areas. We discussed that it should be differentiated from metaphyseal aclasia which similar findings roentgenologically. He was operated for the removal of the masses the left 1st, 2nd and 3rd fingers. After operation the microscopic examination revealed that the specimen consisted of hyperplastic cartilaginous tissue with hyaline degeneration and large areas of myxomatous degeneration, but no evidence of any malignant changes

  1. CT appearance of menetrier's disease (a report of 4 cases)

    International Nuclear Information System (INIS)

    Peng Weijun; Zhang Pei; Zhou Kangrong; Chen Huiming

    2000-01-01

    Objective: To define the CT imaging characteristic of menetrier's disease. Methods: Four patients with proved Menetrier's disease were retrospectively studied by means of CT performed with the water as oral contrast, hypotonic agent and dynamic or spiral CT technique. Results: All cases showed that the enlarged rugae were seen projecting into the gastric lumen, but the gastric wall was otherwise normal. CT detected all lesions and made a confirmed diagnosis before pathology biopsy. Conclusion: The primary result revealed that the CT appearance of Menetrier's disease was quite characteristic, CT is a very valuable tool for the diagnosis of Menetrier's disease

  2. Angioid streaks in a case of Camurati–Engelmann disease

    Directory of Open Access Journals (Sweden)

    Betül Tugcu

    2017-01-01

    Full Text Available Camurati–Engelmann disease (CED is a rare autosomal dominant disease with various phenotypic expressions. The hallmark of the disease is bilateral symmetric diaphyseal hyperostosis of the long bones with progressive involvement of the metaphysis. Ocular manifestations occur rarely and mainly result from bony overgrowth of the orbit and optic canal stenosis. We report a case of CED showing angioid streaks (ASs in both fundi with no macular involvement and discuss the possible theories of the pathogenesis of AS in this disease.

  3. Legionnaires’ Disease: Clinicoradiological Comparison of Sporadic Versus Outbreak Cases

    Directory of Open Access Journals (Sweden)

    Hafiz Rizwan Talib Hashmi

    2017-06-01

    Full Text Available Background: In 2015, New York City experienced the worst outbreak of Legionnaires’ disease in the history of the city. We compare patients seen during the 2015 outbreak with sporadic cases of Legionella during the past 5 years. Methods: We conducted a retrospective chart review of 90 patients with Legionnaires’ disease, including sporadic cases of Legionella infection admitted from 2010 to 2015 (n = 55 and cases admitted during the 2015 outbreak (n = 35. Results: We saw no significant differences between the 2 groups regarding demographics, smoking habits, alcohol intake, underlying medical disease, or residence type. Univariate and multivariate analyses showed that patients with sporadic case of Legionella had a longer stay in the hospital and intensive care unit as well as an increased stay in mechanical ventilation. Short-term mortality, discharge disposition, and most clinical parameters did not differ significantly between the 2 groups. Conclusions: We found no specific clinicoradiological characteristics that could differentiate sporadic from epidemic cases of Legionella . Early recognition and high suspicion for Legionnaires’ disease are critical to provide appropriate treatment. Cluster of cases should increase suspicion for an outbreak.

  4. Imaging Manifestations in Systemic Cat Scratch Disease: Case report

    International Nuclear Information System (INIS)

    Forero M, Julian F; Perez A, Maria C; Cerquera C, Fredy M

    2011-01-01

    Cat scratch disease is a zoonosis caused by Bartonella henselae, which is transmitted by scratches, bites or exposition to cats saliva (1). The disease typically manifests with local lymphadenitis after bacterial inoculation in the skin, however, there is an atypical systemic presentation in 5 to 10% of patients, which causes unspecific symptoms. There are several imaging findings that lead the radiologist to consider this diagnosis, in order to prevent an invasive procedure, especially if we consider that the majority of cases occur in the pediatric population (2,3). Although in the majority of cases the symptoms and imaging findings resolve spontaneously, there are specific indications like the systemic form of the disease,which requires antibiotic treatment. In the present article we are exposing a case report from Fundacion Cardioinfantil; we will review some epidemiologic aspects, clinical manifestations, diagnostic methods as well as imaging findings in Ultrasonography, Computed Tomography, Magnetic Resonance and Nuclear Medicine.

  5. Imaging Manifestations in Systemic Cat Scratch Disease: Case report

    International Nuclear Information System (INIS)

    Forero Melo, Julian Francisco; Perez Alvarado, Maria Carolina; Cerquera Cabrera, Fredy Martin

    2011-01-01

    Cat scratch disease is a zoonosis caused by Bartonella henselae, which is transmitted by scratches, bites or exposition to cats saliva (1). The disease typically manifests with local lymphadenitis after bacterial inoculation in the skin, however, there is an atypical systemic presentation in 5 to 10% of patients, which causes unspecific symptoms. There are several imaging findings that lead the radiologist to consider this diagnosis, in order to prevent an invasive procedure, especially if we consider that the majority of cases occur in the pediatric population (2,3). Although in the majority of cases the symptoms and imaging findings resolve spontaneously, there are specific indications like the systemic form of the disease, which requires antibiotic treatment. In the present article we are exposing a case report from Fundacion Cardio infantil; we will review some epidemiologic aspects, clinical manifestations, diagnostic methods as well as imaging findings in Ultrasonography, Computed Tomography, Magnetic Resonance and Nuclear Medicine.

  6. EMG analysis in 78 cases with motor neuron disease

    Institute of Scientific and Technical Information of China (English)

    Zhang Qiubin

    2000-01-01

    This paper analysed the FMGs of 78 cases with the motor neuron disease(MND). The EMG of all patients showed following characteristics that the average duration of wave prolonged, the average voltage increased and it was found that fibrillation and fasciculatton potentials appeared spontaneously. The fibrillation potential of ENG waa related to course of disease. In the patients whose course of disease was short, the fibri llation potential increased obviously, while in the cases of chronic MND, It usually decreased. The motor nerve conduction velocity of most pa tients (41%) reduced, however, the sensory nerve conduction velocity was normal but two. We reviewed some references about EMG of the motor neuron disease and discussed their characteristics and mechanism

  7. Bone scintigraphy in a case of Ollier's disease

    Energy Technology Data Exchange (ETDEWEB)

    Otsuka, Nobuaki; Ito, Yasuhiko; Morita, Rikushi [Kawasaki Medical School, Kurashiki, Okayama (Japan)

    1983-11-01

    Bone scintigraphy with sup(99m)Tc-MDP was performed on a case with a Ollier's disease. Compared with Bone X-ray examinations, increased activity is noted in the ribs, hands (esp. left), left humerus, left tibia and left foot. Tumor scintigraphy with /sup 67/Ga-citrate shows slightly increased accumulation in comparison with sup(99m)Tc-MDP findings. However, apparent change was not noted compared with the previous scans. So, malignant change was negative. Malignant bone tumors usually show high activity, but some benign tumors also show high uptake. So, a differential diagnosis of bone disease using sup(99m)Tc-phosphorous compounds is occasionally difficult. In case of Ollier's disease, a follow-up bone scintigraphy is useful for evaluation of tumor growth, because malignant changes were accompanied by intensive uptake of sup(99m)Tc-MDP. Also, /sup 67/Ga-study is necessary for the differentiation of bone disease.

  8. An oral ulceration associated with Morgellons disease: a case report.

    Science.gov (United States)

    Grosskopf, Courtney; Desai, Bhavik; Stoopler, Eric T

    2011-08-01

    Morgellons disease is a psycho-dermatologic condition in which patients report fibers or filaments "growing" out of their skin. This case report highlights an oral ulceration in a young woman associated with Morgellons disease, a condition that has not been previously described in the dental literature. An increasing number of individuals are self-reporting this condition and oral health care providers must be familiar with this disorder. Copyright © 2011 Mosby, Inc. All rights reserved.

  9. Whipple's disease. Report of five cases with different clinical features

    OpenAIRE

    FERRARI,Maria de Lourdes de Abreu; VILELA,Eduardo Garcia; FARIA,Luciana Costa; COUTO,Claudia Alves; SALGADO,Célio Jefferson; LEITE,Virgínia Rios; BRASILEIRO FILHO,Geraldo; BAMBIRRA,Eduardo Alves; MENDES,Claudia Maria de Castro; CARVALHO,Silas de Castro; OLIVEIRA,Celso Affonso de; CUNHA,Aloísio Sales da

    2001-01-01

    Whipple's disease (WD) is a rare systemic disease of infectious etiology which involves the small intestine but can virtually affect any organ. We present here five cases (four males and one female) ranging in age from 20 to 59 years. All patients had intestinal involvement associated or not with clinical manifestations linked to this organ. Vegetation in the tricuspid valve was observed in one patient, suggesting endocarditis caused by Tropheryma whippelii, with disappearance of the echocard...

  10. Osteopetrosis - Albers-Schoenberg disease: a case report

    International Nuclear Information System (INIS)

    Jeronymo, J.R.B.; Borba, L.A.N.

    1989-01-01

    The authors present a brief review of the literature and report a case of osteopetrosis tardo which was diagnosed by chance at the Hospital de Clinicas - Universidade Federal do Parana. This patient had no clinical manifestation of the disease. The radiological findings were characteristic of osteopetrosis and the absence of clinical signs confirm the tardo form of this rare disease, with the patient been well at 74 years of age. (author)

  11. Dental erosion caused by gastroesophageal reflux disease: a case report

    OpenAIRE

    Cengiz, Seda; Cengiz, M ?nan?; Sara?, Y ?inasi

    2009-01-01

    Introduction Chronic regurgitation of gastric acids in patients with gastroesophageal reflux disease may cause dental erosion, which can lead in combination with attrition or bruxism to extensive loss of coronal tooth tissue. Case presentation This clinical report describes treatment of severe tooth wear of a gastroesophageal reflux disease patient who is 54-year-old Turkish male patient. After his medical treatment, severe tooth wear, bruxism and decreased vertical dimensions were determined...

  12. Skeletal Manifestations in Gaucher Disease: A Case Report

    Directory of Open Access Journals (Sweden)

    Altınay Göksel Karatepe

    2005-09-01

    Full Text Available Gauchers disease is the most frequent hereditary lysosomal deposit storage disorder. It is characterized by a deficiency of the enzyme glucocerebrosidase that leads to an accumulation of glucocerebroside in the macrophage lysosomes. It is classified in three types, according to the presence of central nervous system involvement (type 2 and 3 or not (type 1. In the majority of patients there are hepatosplenomegaly, anemia and thrombocytopenia. Skeletal involvement is also important and it is the most disabling manifestation. In this case report, there is presented a case of Gauchers disease with multiple skeletal involvement and the literature is reviewed.

  13. Hydatid disease of the cranium: neuroradiological findings in one case

    International Nuclear Information System (INIS)

    Ruiz, T.; Rivera, A.; Blanco, A.

    1998-01-01

    Hydatid disease is caused by Echinococcus granulosus. The most common locations for hydatid cysts are lung and liver. The central nervous system is involved in only 2% of cases, and these cysts are usually located in the brain parenchyma. Primary involvement of the bones of the skull is very uncommon. We report the case of a child with cranial echinococcosis which presented in the form of an osteolytic lesion with an extradural cyst. This was the unique feature of the disease. The CT and MR findings are described. This presentation has rarely been reported in the literature. (Author) 5 refs

  14. Pediatric surgical diseases. A radiological surgical case study approach

    International Nuclear Information System (INIS)

    Esposito, Ciro; Esposito, Giovanni

    2009-01-01

    Radiologic evaluation of an infant or child suspected of having a surgical disease can be a complex problem. With this volume, the editors have created a book focused on pediatric imaging written by pediatricians, pediatric surgeons and pediatric radiologists. This book is a collection of over 200 case reports. The concept is a case study approach: The reader is given radiologic images (plain radiography, computed tomography, magnetic resonance imaging, ultrasonography, etc.) and the clinical history of the patient. On the basis of this information, the reader is asked to identify a diagnostic and therapeutic strategy. Each case is complemented by information on the disease affecting the patient and the management of the case shown, including therapy and follow-up. This educational text is targeted at all medical professionals faced with a variety of diagnostic and therapeutic problems affecting infants and children. (orig.)

  15. Pediatric surgical diseases. A radiological surgical case study approach

    Energy Technology Data Exchange (ETDEWEB)

    Esposito, Ciro [Federico II Univ. of Naples (Italy). Chair of Pediatric Surgery; Esposito, Giovanni (eds.) [Federico II Univ. of Naples (Italy). School of Medicine

    2009-07-01

    Radiologic evaluation of an infant or child suspected of having a surgical disease can be a complex problem. With this volume, the editors have created a book focused on pediatric imaging written by pediatricians, pediatric surgeons and pediatric radiologists. This book is a collection of over 200 case reports. The concept is a case study approach: The reader is given radiologic images (plain radiography, computed tomography, magnetic resonance imaging, ultrasonography, etc.) and the clinical history of the patient. On the basis of this information, the reader is asked to identify a diagnostic and therapeutic strategy. Each case is complemented by information on the disease affecting the patient and the management of the case shown, including therapy and follow-up. This educational text is targeted at all medical professionals faced with a variety of diagnostic and therapeutic problems affecting infants and children. (orig.)

  16. A middle aged man with Caroli's disease: A case report

    Directory of Open Access Journals (Sweden)

    Imran Sarker

    2016-07-01

    Full Text Available Caroli's disease (CD is a rare congenital abnormality characterized by non-obstructive dilatation of intra hepatic bile ducts, which may be complicated by stone formation, recurrent cholangitis, biliary abscess and higher risk for biliary malignancy. We report a 37-year-old man with recurrent bouts of upper abdominal pain, high grade pyrexia, mild icterus with normal liver function tests who was diagnosed as a case of Caroli's disease. The laboratory studies confirmed Caroli' s disease with a SOL in liver suggestive of liver abscess and the patient received broad spectrum antibiotics with anaerobic and amoebicidal coverage. With 14 days course of antibiotics, he gradually recovered from his symptoms.

  17. Presentation of moyamoya disease with occipital hemorrhage: a case report

    Directory of Open Access Journals (Sweden)

    Serkan Demir

    2012-12-01

    Full Text Available Moyamoya disease is a cerebrovascular disease which is characterized with stenosis and occlusions at the distal part of internal carotid artery and at the proximal part of anterior and middle cerebral arteries. It rarely causes temporary or recurrent hemiparesis due to intracranial hemorrhage while symptoms like headache, convulsion, nystagmus, aphasia and ataxia may also occur. In this paper, we present a case of Moyamoya disease which was diagnosed with a 23 year old female patient who was admitted to our emergency department with headache, nausea and vomiting complaints and whose radiological findings showed occipital lobe hemorrhage.

  18. Rapidly aggravated Creutzfeldt-Jacob disease: autopsy-proven case

    International Nuclear Information System (INIS)

    Park, Seung Hyun; Kang, Hyun Koo; Yu, Hyeon; Lee, Sang Chun

    2005-01-01

    Creutzfeldt-Jakob disease (DJD) is one of the transmissible spongiform encephalopathies, which is mediated by what has been known as 'prion'. It is a rare and fatal progressive neurodegenerative disease that affects the middle and old aged. There are a number of subtypes of CJD, one of which is the sporadic type characterized by rapidly progressing clinical symptoms, including progressive dementia, myoclonic jerk, and pyramidal or extrapyramidal syndrome. Patients usually end up dying within 1 to 2 years of contacting the disease. We report an autopsy-proven case of sporadic CJD with clinical symptoms that progressed within several days, along with dramatic changes on diffusion weighted magnetic resonance images

  19. Four Cases of Parkinson Disease Diagnosed During the Postpartum Period.

    Science.gov (United States)

    Maltête, David; Grangeon, Lou; Le Goff, Floriane; Ozel, Gulden; Fetter, Damien; Ahtoy, Patrick; Temgoua, Olivier; Rouillé, Audrey; Lefaucheur, Romain

    2017-07-01

    There is little experience with the effect of pregnancy on Parkinson disease because the number of women with Parkinson disease who are of childbearing age is small. We report four cases beginning during the postpartum period and discuss the potential contribution of different factors that may influence the occurrence of Parkinson disease in this time period. Four women aged 29-35 years developed arm tremor, shoulder pain, dizziness, or decreased dexterity of the hand in the first few days or months after childbirth. They were initially diagnosed with postpartum depression or psychogenic parkinsonism. Finally, dopamine transporter imaging confirmed the diagnosis of young-onset Parkinson disease. Early-onset Parkinson disease may present in postpartum women. In women with atypical motor symptoms in addition to depression, this diagnosis should be considered.

  20. Amyotrophic lateral sclerosis – a motor neuron disease. Case report

    Directory of Open Access Journals (Sweden)

    Maja Rubinowicz-Zasada

    2015-03-01

    Full Text Available Amyotrophic lateral sclerosis, also known as Charcot’s disease and motor neuron disease, is a progressive neurodegenerative disease that causes muscle weakness, paralysis, and ultimately, respiratory failure. The aetiology and the pathogenesis of the syndrome remain unknown. Most people live 2–5 years after their first signs of the disease. There is no cure or effective treatment. We present a case of a female patient affected by progressing Charcot’s disease. On the Amyotrophic Lateral Sclerosis Functional Rating Scale – Revised (ALSFRS-R, the patient obtained 21 points. Atrophy and muscle spasm were very extended. Electromyography revealed features of coexisting denervation and reinnervation in the examined muscles. A growing number of Charcot’s disease cases require multidirectional actions to meet patient’s physical, emotional, and nutritional needs. Amyotrophic lateral sclerosis is an incurable disease. However, it is possible to relieve its symptoms by applying systematic physical rehabilitation.

  1. McArdle disease: a case report and review

    Directory of Open Access Journals (Sweden)

    Leite A

    2012-01-01

    Full Text Available Alberto Leite, Narciso Oliveira, Manuela RochaInternal Medicine Department, Hospital de Braga, PortugalAbstract: McArdle disease (glycogen storage disease type V is a pure myopathy caused by an inherited deficit of myophosphorylase. The disease exhibits clinical heterogeneity, but patients typically experience exercise intolerance, acute crises of early fatigue, and contractures, sometimes with rhabdomyolysis and myoglobinuria, triggered by static muscle contractions or dynamic exercise. We present the case of a 54-year-old man with a lifelong history of fatigability, worsening on exertion. Laboratory evaluation revealed significant elevations in levels of creatine kinase (7924 U/L, lactate dehydrogenase (624 U/L, and myoglobulin (671 ng/mL. A muscle biopsy confirmed the presence of McArdle disease. This case report illustrates how, due to embarrassment, the patient hid his symptoms for many years and was eventually extremely relieved and “liberated” once McArdle disease was diagnosed 40 years later.Keywords: McArdle disease, glycogen storage disease, myophosphorylase

  2. Fox Den Disease: An Interesting Case Following Delayed Diagnosis.

    Science.gov (United States)

    Stehr, Ryan C; Kim, Nicholas; LoGiudice, John A; Ludwig, Kirk

    2015-06-01

    Pyoderma fistulans sinifica, also known as fox den disease, is a rare and poorly understood inflammatory disorder of the skin and subcutaneous tissues. This disorder is often mistaken for other inflammatory skin disorders and treated inappropriately. The authors describe the case of a 53-year-old male who presented to the colorectal surgery service with a longstanding diagnosis of perirectal Crohn's disease. Despite aggressive immunosuppression and numerous surgical procedures, the patient continued to have unrelenting purulent drainage from the skin of his buttocks. Following wide excision of the affected skin and subcutaneous tissues by the colorectal surgeon, the plastic surgery team reconstructed the 30 cm x 55 cm wound using a combination of local flaps and skin grafts. The initial pathology report of the excised specimen confirmed the presence of nonspecific abscesses and inflammation. Upon special request by the plastic surgery team, the sample was resectioned with the specific intent of establishing a diagnosis of fox den disease. The additional slides met the criteria for an unequivocal diagnosis of fox den disease. Immunosuppression was discontinued and the patient healed his wounds without complication. Fox den disease is often overlooked because of the obscurity of the disease and the special histological sectioning needed to establish a diagnosis. In this case, the patient was unnecessarily treated with immunosuppressive drugs for more than 3 decades because of a misdiagnosis. With increased awareness of fox den disease, perhaps its pathophysiology can be better elucidated as more patients are appropriately diagnosed and treated.

  3. Demyelinating disease masquerading as a surgical problem: a case series

    Directory of Open Access Journals (Sweden)

    Awang Saufi M

    2009-08-01

    Full Text Available Abstract Introduction We report three cases of demyelinating disease with tumor-like presentation. This information is particularly important to both neurosurgeons and neurologists who should be aware that inflammatory demyelinating diseases can present as a mass lesion, which is indistinguishable from a tumor, both clinically and radiologically, especially when there is no evidence of temporal dissemination of this disease. Case presentation The first patient was a 42-year-old Malay woman who developed subacute onset of progressive quadriparesis with urinary incontinence. Magnetic resonance imaging of her spine showed an intramedullary lesion at the C5-C7 level. She was operated on and biopsy was suggestive of a demyelinating disease. Retrospective history discovered two episodes of acute onset of neurological deficits with partial recovery and magnetic resonance imaging of her brain revealed demyelinating plaques in the centrum semiovale. The second patient was a 16-year-old Malay boy who presented with symptoms of raised intracranial pressure. A computed tomography brain scan revealed obstructive hydrocephalus with a lesion adjacent to the fourth ventricle. An external ventricular drainage was inserted. Subsequently, a stereotactic biopsy was taken and histopathology was reported as demyelination. Retrospective history revealed similar episodes with full recovery in between episodes. The third case was a 28-year-old Malay man who presented with acute bilateral visual loss and confusion. Magnetic resonance imaging of his brain showed a large mass lesion in the right temporoparietal region. Biopsy was consistent with demyelinating disease. Reexamination of the patient revealed bilateral papillitis and not papilledema. Visual evoked potential was prolonged bilaterally. In all three cases, lumbar puncture for cerebrospinal fluid study was not carried out due to lack of patient consent. Conclusions These cases illustrate the importance of

  4. Report of a case of Hodgkin's nodular sclerosis disease

    International Nuclear Information System (INIS)

    Canizares, Claudio; Araujo, Ivan; Flores, Marlon; Guerrero, Patricia; Sanchez, Mayra

    2004-01-01

    We report a case of Hodgkin's nodular sclerosis disease that presents moderate anemia and lymph node enlargement with a massive infiltrate to the bone marrow. It is rare for these patients to develop such an infiltrate, which give a bad prognosis. The treatment recommended in stage I is Radiotherapy and chemotherapy. (The author)

  5. Neurological Manifestation of Behcet's Disease: A Case Report ...

    African Journals Online (AJOL)

    Behcet's disease causes inflammation of blood vessels but it is of unknown aetiology. When it involves the nervous system, it may present with benign headaches, aseptic meningitis, meningoencephalitis, cranial nerve palsies, seizures, personality changes, and cerebral venous thrombosis. We present the case of A.G is a ...

  6. [Eye and cat scratch disease: A case series].

    Science.gov (United States)

    Deschasse, C; Bielefeld, P; Muselier, A; Bour, J B; Besancenot, J F; Garcher, C C; Bron, A M

    2016-02-01

    Cat scratch disease is a pleiomorphic condition, sometimes with isolated ophthalmic involvement. We report the clinical observations of seven cases with ophthalmologic manifestations of cat scratch disease. There were seven patients, with a median age of 52 years, of whom five were women and three had unilateral involvement. Six exhibited Leber's stellate neuroretinitis, an incomplete syndrome in two cases, and one associated with chorioretinal foci. One patient had isolated retinal infiltrates. The diagnosis of cat scratch disease was confirmed by Bartonella henselae serology, positive in all cases. All patients received treatment with doxycycline. Ocular complications (with optic atrophy and macular retinal pigment epithelial changes) were noted in five cases. Ocular bartonellosis is an atypical clinical form. It requires a directed ancillary work-up with serology or PCR, which has the peculiarity of being highly specific if not very sensitive. Treatment is above all preventive. Antibiotics may be initiated. Cat scratch disease must be excluded in the work-up of posterior uveitis. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

  7. Eales' Disease: Case report | Atipo-Tsiba | East African Medical ...

    African Journals Online (AJOL)

    Eales' disease is a retinal vasculopathy of unknown origin. Ischaemic step associated retinal perivasculitis. Neovascularisation step after the previous one, characterised by vitreous haemorrhage in relation with retinal neo vessels. This observation presents the first reported case of this pathology in Brazzaville. A man of 32 ...

  8. Creutzfeldt-Jakob disease in Venezuela a case report

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    Alejandro J. Caraballo H.

    1991-06-01

    Full Text Available A case of Creutzfeldt-Jakob disease (CJD in a 32 year old man is presented. The clinical picture included a rapid progressive dementia associated with ataxia, global aphasia, myoclonus and pyramidal signs, death ocurred after 13 months. The diagnosis of CJD was confirmed by CT and neuropathological studies. This is the first report of CJD occurring in Venezuela.

  9. Congenital tricuspid valve disease and testicular agenesis: a case ...

    African Journals Online (AJOL)

    This is a report of a case of congenital tricuspid valve disease presenting with heart failure and pulmonary hypertension. Cardinal clinical features include breathlessness, easy fatigability since childhood, stunted growth, cyanosis, finger clubbing, a pansystolic murmur loudest at the left sternal edge in the fourth intercostal ...

  10. CASE REPORT : GRAVE'S DISEASE PRESENTING AS PARANOID SCHIZOPHRENIA

    Science.gov (United States)

    Singh, S.K.; Hatwal, A.; Agarwal, J.K.; Bajpai, H.S.; Sharma, I.

    1989-01-01

    SUMMARY The case of a 37 year old male is described who initially presented as paranoid schizophrenia unresponsive to anti-psychotic drug treatment and subsequently developed features of Grave's disease. Treatment with carbimazole alone improved his psychiatric symptoms. PMID:21927380

  11. Alexander's disease in a neurologically normal child: a case report

    International Nuclear Information System (INIS)

    Guthrie, Scott O.; Knowles, Paul; Marshall, Robert; Burton, Edward M.

    2003-01-01

    We report the clinical and MRI findings of symmetric hyperintensity involving the deep and subcortical white matter of the frontal lobes in a neurologically normal child with macrocephaly. In this patient, a serum test for mutations in glial fibrillary acidic protein, used to diagnose Alexander's disease (AD), was positive. This case indicates an extraordinarily mild or early form of juvenile-onset AD. (orig.)

  12. Gastroparesis in patients with inactive Crohn's disease: a case series

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    Oyen Wim JG

    2007-03-01

    Full Text Available Abstract Background Few studies have described patients with foregut dysmotility in inflammatory bowel disease. The aim of this case series was to evaluate clinical characteristics of 5 patients with inflammatory bowel disease and symptoms and signs of upper gut dysmotility. Case presentations We describe a series of four patients with Crohn's disease and one with indeterminate colitis who presented with severe symptoms and signs of gastroparesis. We reviewed medical records of all cases. Gastric emptying of a solid meal was assessed by scintigraphy. Small bowel enteroclysis, gastroduodenoscopy and colonoscopy with biopsies were performed to estimate the activity of the disease and to exclude organic obstruction. None of the patients had any signs of active inflammation or stricture. All of the patients had markedly delayed gastric emptying with a mean t 1/2 of 234 minutes (range 110–380 minutes; normal values 54–94 minutes. Conclusion Clinicians should consider impaired gastric emptying when evaluating patients with Crohn's disease and severe symptoms of upper gut dysmotility, which cannot be attributed to active inflammation or organic obstruction of the digestive tract. Symptoms in these patients are refractory to various therapeutic interventions including tube feeding and gastric surgery.

  13. Periodontal disease in a patient receiving Bevacizumab: a case report

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    Gujral Dorothy M

    2008-02-01

    Full Text Available Abstract Introduction Bevacizumab is a monoclonal antibody that inhibits the action of vascular endothelial growth factor (VEGF thereby acting as an angiogenesis inhibitor. As a result, supply of oxygen and nutrients to tissues is impaired and tumour cell growth is reduced. Reported side effects due to bevacizumab are hypertension and increased risk of bleeding. Bowel perforation has also been reported. Periodontal disease in patients on bevacizumab therapy has not been reported before. Case Presentation We report a case of a forty-three year old woman who developed periodontitis whilst receiving bevacizumab for lung cancer. The periodontal disease remained stable on discontinuation of the drug. Conclusion Further investigations are needed to determine the mechanism for bevacizumab-induced periodontal disease.

  14. A case of severe autoimmune hepatitis associated with Graves' disease

    Directory of Open Access Journals (Sweden)

    Samia Abdulla Bokhari

    2016-01-01

    Full Text Available Graves' disease is a common condition and is known to have a wide range of effects on a variety of organs. Hepatic dysfunction ranging from mild to severe due to direct effect of high circulating thyroid hormones as well as a deleterious effect of antithyroid medications (methimazole and propylthiouracil has been well - documented in literature. However, severe autoimmune hepatitis (AIH associated with Graves' disease is rare and limited to few case reports only. A 38-year-old woman presented with abdominal pain and yellowish discolouration of conjunctivae. On investigation, she was found to have Graves' disease and AIH. The liver histopathology showed typical features of AIH. She responded excellently to glucocorticoid therapy with normalisation of thyroid function and liver histology. The case is discussed with relevant literature review.

  15. Acute tubulointerstitial nephritis complicating Legionnaires' disease: a case report

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    Daumas Aurélie

    2012-04-01

    Full Text Available Abstract Introduction Legionnaires' disease is recognized as a multi-systemic illness. Afflicted patients may have pulmonary, renal, gastrointestinal tract and central nervous system complications. However, renal insufficiency is uncommon. The spectrum of renal involvement may range from a mild and transient elevation of serum creatinine levels to anuric renal failure requiring dialysis and may be linked to several causes. In our present case report, we would like to draw attention to the importance of the pathological documentation of acute renal failure by reporting a case of a patient with acute tubulointerstitial nephritis complicating Legionnaires' disease. Case presentation A 55-year-old Caucasian man was admitted to our hospital for community-acquired pneumonia complicated by acute renal failure. Legionella pneumophila serogroup type 1 was diagnosed. Although the patient's respiratory illness responded to intravenous erythromycin and ofloxacin therapy, his renal failure worsened, he became anuric, and hemodialysis was started. A renal biopsy was performed, which revealed severe tubulointerstitial nephritis. After initiation of steroid therapy, his renal function improved dramatically. Conclusions This case highlights the importance of kidney biopsies in cases where acute renal failure is a complicating factor in Legionnaires' disease. If the presence of acute tubulointerstitial nephritis can be confirmed, it will likely respond favorably to steroidal treatment and thus irreversible renal damage and chronic renal failure will be avoided.

  16. Gaucher disease with jawbone involvement: a case report.

    Science.gov (United States)

    Ahmadieh, Azadeh; Farnad, Fariborz; Sedghizadeh, Parish P

    2014-11-05

    Gaucher disease is an autosomal recessive systemic condition, and the most common of the lysosomal storage disorders. It is characterized by lipid accumulation in certain cells and organs, particularly macrophages, which appear on light microscopy as 'Gaucher cells' or vacuolated lipid-laden reticuloendothelial cells. Long bone involvement is common in Gaucher disease, whereas craniofacial bone involvement is extremely rare. Reports confirming the diagnoses of Gaucher disease involving craniofacial bones by histopathologic evidence are even rarer. A 46-year-old Caucasian Ashkenazi Jewish woman with Gaucher disease presented with jawbone pain and lytic radiographic lesions of her mandible. Surgical biopsy of a mandibular lesion revealed Gaucher cells infiltrating the mandible, which correlated with radiographic and clinical findings, supporting a diagnosis of Gaucher disease with jawbone involvement. Lysosomal storage diseases can have head and neck manifestations, and bone involvement in Gaucher disease is common. Therefore, careful consideration of signs and symptoms and medical history, with a thorough review of systems, is important when evaluating patients with lysosomal storage disorders to rule out head and neck involvement of disease. Biopsy may be warranted in some cases for more definitive diagnosis of painful jawbone lesions and to rule out other odontogenic and non-odontogenic conditions in the differential diagnosis.

  17. Castleman’s disease – a case report

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    Maciej Wilczak

    2011-03-01

    Full Text Available Angiofollicular lymphoid hyperplasia (Castleman’s disease is a very rare disease of lymphatic tissue, manifested by overgrowth of B lymphocytes and plasma cells in lymph nodes. The aetiology of this lymphocyte hyperplasia has not been conclusively explained and its pathogenesis is still not known. There are three types of the disease based on histological criteria: plasma-cell, hyaline-vascular and transitional. Clinically it is classified into two forms, localized and multicentric (disseminated/generalized. Castleman’s disease may develop not only in lymph nodes, but also in other organs and tissues, e.g. larynx, pancreas, meninges, etc. In patients with localized Castleman’s disease the most important therapy is radical surgery of the tumour, which creates conditions leading to complete recovery. In the case of multicentric diagnosis the prognosis is unsure.The paper describes an atypical case of Castleman’s disease, diagnosed as localized form in the patient’s pelvis minor with accompanying cystic changes in the mesocolon.

  18. A Pediatric Case of Cowden Syndrome with Graves’ Disease

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    Cláudia Patraquim

    2017-01-01

    Full Text Available Cowden syndrome (CS is a rare dominantly inherited multisystem disorder, characterized by an extraordinary malignant potential. In 80% of cases, the human tumor suppressor gene phosphatase and tensin homolog (PTEN is mutated. We present a case of a 17-year-old boy with genetically confirmed CS and Graves’ disease (GD. At the age of 15, he presented with intention tremor, palpitations, and marked anxiety. On examination, he had macrocephaly, coarse facies, slight prognathism, facial trichilemmomas, abdominal keratoses, leg hemangioma, and a diffusely enlarged thyroid gland. He started antithyroid drug (ATD therapy with methimazole and, after a 2-year treatment period without achieving a remission status, a total thyroidectomy was performed. Diagnosis and management of CS should be multidisciplinary. Thyroid disease is frequent, but its management has yet to be fully defined. The authors present a case report of a pediatric patient with CS and GD and discuss treatment options.

  19. A case of megacolon in Rio Grande Valley as a possible case of Chagas disease

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    Karl Reinhard

    2003-01-01

    Full Text Available We have been searching for evidence of Chagas disease in mummified human remains. Specifically, we have looked for evidence of alteration of intestinal or fecal morphology consistent with megacolon, a condition associated with Chagas disease. One prehistoric individual recovered from the Chihuahuan Desert near the Rio Grande exhibits such pathology. We present documentation of this case. We are certain that this individual presents a profoundly altered large intestinal tract and we suggest that further research should focus on confirmation of a diagnosis of Chagas disease. We propose that the prehistoric activity and dietary patterns in Chihuahua Desert hunter/gatherers promoted the pathoecology of Chagas disease.

  20. Grover's Disease after Heart Transplantation: A Case Report

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    Giovanbattista Ippoliti

    2012-01-01

    Full Text Available Grover's disease is a transient acantholytic dermatosis of unknown cause, manifesting clinically as a papular skin eruption that is usually located on the anterior chest and abdomen. Histologically characterized by an acantholytic pattern, it has been associated with numerous disorders, including hematologic malignancies, chronic renal failure, and HIV infection, as well as with chemotherapy and bone marrow and/or kidney transplant. Evaluation of followup and treatment is often complicated by spontaneous remission and the occasionally fluctuant course of the disease. Here we report the case of a patient with sudden onset of Grover's disease after heart transplantation. To the best of our knowledge, this is the first observation of Grover's disease as diagnosed after heart transplantation.

  1. Case report of Graves’ disease manifesting with odynophagia and heartburn

    Science.gov (United States)

    Evsyutina, Yulia; Trukhmanov, Alexander; Ivashkin, Vladimir; Storonova, Olga; Godjello, Elina

    2015-01-01

    Graves’ disease is an autoimmune disease, which can manifest with a variety of extrathyroidal clinical syndromes like ophthalmopathy, pretibial myxedema (dermopathy), acropathy, cardiomyopathy, and encephalopathy. Though quite rare, this disease can also manifest with gastrointestinal symptoms such as dysphagia, heartburn, nausea, vomiting and diarrhea. We report a clinical case of Graves’ disease manifesting with dysfunction of the esophagus and heartburn in a 61-year-old man. In the muscular layer of the esophagus we found dystrophic changes led to its atony, which was documented by endoscopy and high-resolution manometry. The pathology features of esophageal symptoms were: focal proliferation of the basal cells, vascular distension, and dystrophy of the epithelial cells. Antithyroid treatment led to decrease of all clinical symptoms after 5 d of Thiamazole administration. Complete restoration of peristalsis in the esophagus, according to manometry, was observed in 1 mo after initiation of treatment. PMID:26730171

  2. Swallowing endoscopy findings in Huntington's disease: a case report.

    Science.gov (United States)

    Alves, Thaís Coelho; Cola, Paula Cristina; Santos, Rarissa Rúbia Dallaqua Dos; Motonaga, Suely Mayumi; Silva, Roberta Gonçalves da

    2016-01-01

    Huntington's disease (HD) is a degenerative genetic disorder with autosomal-dominant transmission. The triad of symptoms of this disease consists of psychiatric disorders, jerky movements, and dementia. Oropharyngeal dysphagia, which is more evident with disease progression, is also present. Few studies have addressed the swallowing characteristics using objective analysis in this population. The purpose of this research was to describe the swallowing endoscopic findings of the pharyngeal phase in HD. This is a cross-sectional study addressing a clinical case which included two individuals of the same family, male, 32 and 63 years old, designated as individual A and individual B, with progression of the disease for five and 13 years, respectively. Consistent liquid, nectar, and puree were offered during the evaluation. There was presence of posterior oral spillage in liquid and nectar, small amount of pharyngeal residues, and no laryngeal penetration or aspiration in the individuals with HD in this study.

  3. Case report of Graves' disease manifesting with odynophagia and heartburn.

    Science.gov (United States)

    Evsyutina, Yulia; Trukhmanov, Alexander; Ivashkin, Vladimir; Storonova, Olga; Godjello, Elina

    2015-12-28

    Graves' disease is an autoimmune disease, which can manifest with a variety of extrathyroidal clinical syndromes like ophthalmopathy, pretibial myxedema (dermopathy), acropathy, cardiomyopathy, and encephalopathy. Though quite rare, this disease can also manifest with gastrointestinal symptoms such as dysphagia, heartburn, nausea, vomiting and diarrhea. We report a clinical case of Graves' disease manifesting with dysfunction of the esophagus and heartburn in a 61-year-old man. In the muscular layer of the esophagus we found dystrophic changes led to its atony, which was documented by endoscopy and high-resolution manometry. The pathology features of esophageal symptoms were: focal proliferation of the basal cells, vascular distension, and dystrophy of the epithelial cells. Antithyroid treatment led to decrease of all clinical symptoms after 5 d of Thiamazole administration. Complete restoration of peristalsis in the esophagus, according to manometry, was observed in 1 mo after initiation of treatment.

  4. A rare case of hidebound disease with dental implications

    Directory of Open Access Journals (Sweden)

    Vikram Bali

    2013-01-01

    Full Text Available Systemic sclerosis (also called as Scleroderma or hidebound disease is a chronic sclerotic disease of unknown etiology which causes diffuse, increased deposition of extra cellular matrix in connective tissue with vascular abnormalities, resulting in tissue hypoxia. The disease is characterized by diffuse fibrosis; degenerative changes; and vascular abnormalities in the skin (scleroderma, articular structures, and internal organs. Aesthetic and facial dysfunctions are followed by important oral and facial manifestations. Most oral manifestations begin with tongue rigidity and facial skin changes. Bone resorption of mandibular angle and widening of periodontal ligament space on periapical radiographs are important radiological findings. Other systemic changes include the involvement of internal organs, which lead to serious complications as well as disorders in the cardiac muscle and Raynaud΄s phenomenon. This is a case report of 30-year-old female patient with the classical features of this disease. This case is reported for its rarity and variable expressivity. The main aim of this article is to describe thorough presentation of the case report, various forms of scleroderma, pathogenesis, oral, extraoral, periodontal manifestations of scleroderma, and its treatment options. A brief review of the literature, focusing on dental alterations is also presented.

  5. A rare case of hidebound disease with dental implications.

    Science.gov (United States)

    Bali, Vikram; Dabra, Sarita; Behl, Ashima Bali; Bali, Rajiv

    2013-07-01

    Systemic sclerosis (also called as Scleroderma or hidebound disease) is a chronic sclerotic disease of unknown etiology which causes diffuse, increased deposition of extra cellular matrix in connective tissue with vascular abnormalities, resulting in tissue hypoxia. The disease is characterized by diffuse fibrosis; degenerative changes; and vascular abnormalities in the skin (scleroderma), articular structures, and internal organs. Aesthetic and facial dysfunctions are followed by important oral and facial manifestations. Most oral manifestations begin with tongue rigidity and facial skin changes. Bone resorption of mandibular angle and widening of periodontal ligament space on periapical radiographs are important radiological findings. Other systemic changes include the involvement of internal organs, which lead to serious complications as well as disorders in the cardiac muscle and Raynaud΄s phenomenon. This is a case report of 30-year-old female patient with the classical features of this disease. This case is reported for its rarity and variable expressivity. The main aim of this article is to describe thorough presentation of the case report, various forms of scleroderma, pathogenesis, oral, extraoral, periodontal manifestations of scleroderma, and its treatment options. A brief review of the literature, focusing on dental alterations is also presented.

  6. Celiac Disease and Concomitant Conditions: A Case-based Review.

    Science.gov (United States)

    Lodhi, Muhammad Uzair; Stammann, Tracy; Kuzel, Aaron R; Syed, Intekhab Askari; Ishtiaq, Rizwan; Rahim, Mustafa

    2018-02-02

    Celiac disease is a chronic autoimmune disease with genetic predisposition, triggered by the ingestion of gluten. It has a wide range of clinical manifestations ranging from asymptomatic forms to classic presentation of malabsorption with diarrhea and abdominal cramps. Celiac disease can also present with several other concomitant disorders (at the time of diagnosis or during the course of celiac disease) such as: type 1 diabetes, inflammatory bowel disease, rheumatoid arthritis, thyroid disorders, nutritional deficiencies, and gram-negative sepsis. We present a 57-year-old female with past medical history of rheumatoid arthritis, who presented to the emergency department with a complaint of chronic diarrhea, complicated by gram-negative sepsis. The family history of the patient was significant for celiac disease, type 1 diabetes, and rheumatoid arthritis. The patient was closely monitored and treated appropriately. In this case-based review, we explore different associated conditions of celiac disease in the literature, as well as the patient's risk of developing malignancy.

  7. Pediatric Gastrointestinal Diseases in Nigeria: Histopathologic Analysis of 74 Cases

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    Abudu

    2013-10-01

    Full Text Available BACKGROUND: Children are vulnerable to a vast number of diseases including gastrointestinal disorders, which may be associated with life threatening complications that sometimes result in mortality especially if left untreated. OBJECTIVE: To establish the age and sex distribution of children in the study population as well as the histopathological characteristics of gastrointestinal diseases that occurred in those children who were aged 14years and below in Sagamu, Southwestern Nigeria. MATERIALS AND METHODS: Demographic data such as age, sex, and clinical summary of children in the study population were extracted from the medical records of Olabisi Onabanjo University Teaching Hospital, Sagamu, Ogun State from January 2003 to December 2009. Based on this information, a review of paraffin embedded blocks and slides as well as histopathological reports of gastrointestinal diseases that occurred in those children aged 14years and below was undertaken at the Morbid Anatomy Department of the hospital. RESULTS: Seventy–four cases of gastrointestinal diseases were seen in children aged 14years and below. The majority (39.2% of gastrointestinal diseases were accounted for by appendiceal lesions. Hirschsprung’s disease, intussusceptions, enterocolitis and jejunal atresia accounted for 29.7%, 10.8%, 6.8% and 4.1% of cases respectively. Adenocarcinoma of the intestine was the predominant gastrointestinal tumour, occurring in 5 out of 7 children. Two cases of non-Hodgkin lymphoma were also seen. The ages of the children ranged from 2 to 14 years, with a mean age of 8.6years and a peak age incidence of gastrointestinal disease in the 10-14year age group. Male children were more commonly affected with the exception of appendiceal lesions, which occurred more in females (M:F ratio= 1.6:1.0. Acute suppurative appendicitis was the most prevalent lesion of the appendix, occurring in 13 out of 29 appendiceal lesions. Moderately differentiated to poorly

  8. Hydatid Disease of the Kidney: Report of 12 Cases

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    Y. FAZLALIZADEH

    1972-07-01

    Full Text Available The radiology diagnosis of primary hydatid disease of the kidney is possible when a renal mass is noted on the radiograph. part icula rly in the countries where this disease is prevalent. Cha racteristic radiological signs, hydatiduria, eosinophili and specific examination such as Casoni and Weinberg tests are a ll helpful to assist the diagnosis. Twelve cases of primary hydatid disease of the kidney have been seen in our 10 yea rs study. The diagno,sis in the majority of these cases was primarily radiological. In three cases hydatiduria was observed. In one case in which resection of the cyst and partial nephrectomy was done recurrence was observed after nine years. Etude radiologique de J2 cas de kyst hydatique primitive du rein, • La diagnostique radiologique de kyst hydatiquc primitive renale est possible. quand on peut noter une masse rena le en radiographic, particulie rement dans les contrces ou ceue maladie est preva lcnte. Les signes radiologiques cha ractcristiqucs, hyda tidurie, eosinophilie, et exarnen spccifiques, comme teste de Casoni , ct Weimberg assistent au diagnostique. Auteur a prcsentc 12 cas de kyst hydatique primitive renal, diagnostique radiologiquement pendan t ces dix derniers annees. Dans trois cas hydatidurie eta it observe et dans un cas, 9 ans apres la ncphrectomie partiellc et resection de kyst , on a rernarque la rechutte de la maladie.

  9. Amino acid-responsive Crohn's disease: a case study

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    Alvin Stein

    2010-12-01

    Full Text Available Alvin Stein1, Marty Hinz2, Thomas Uncini31Stein Orthopedic Associates, Plantation, FL, USA; 2Clinical Research, NeuroResearch Clinics Inc., Cape Coral, FL, USA; 3Laboratory, Fairview Regional Medical Center-Mesabi, Hibbing, MN, USAPurpose: This paper reviews the clinical course of a case of severe Crohn's disease and discusses the scientific ramifications of a novel treatment approach.Patients and methods: A case study of a 37-year-old male with a 22-year history of Crohn's disease whose clinical course had experienced no sustained remissions. The patient was treated with a protocol that utilized serotonin and dopamine amino acid precursors administered under the guidance of organic cation transporter assay interpretation.Results: Within 5 days of achieving the necessary balance of serotonin and dopamine, the patient experienced remission of symptoms. This remission has been sustained without the use of any Crohn's disease medications.Conclusion: In Crohn's disease, it is known that there is an increase of both synthesis and tissue levels of serotonin in specific locations. It is asserted that this is prima facie evidence of a significant imbalance in the serotonin–dopamine system, leading to serotonin toxicity. The hypothesis formulated is that improperly balanced serotonin and dopamine transport, synthesis, and metabolism is a primary defect contributing to the pathogenesis of Crohn's disease.Keywords: serotonin, dopamine, organic cation transporters, OCT

  10. Cochlear Implants and Psychiatric Assessments: a Norrie Disease Case Report.

    Science.gov (United States)

    Jacques, Denis; Dubois, Thomas; Zdanowicz, Nicolas; Gilain, Chantal; Garin, Pierre

    2017-09-01

    It is important to perform psychiatric assessments of adult patients who are candidates for cochlear implants both to screen them for psychiatric disorders and to assess their understanding and compliance with the procedure. Deafness is a factor of difficulty for conducting in-depth psychiatric interviews, but concomitant blindness may make it impossible. After a description of Norrie disease, a rare disease in which blindness and deafness may occur together, we propose a case report of a patient suffering from the disease and who consulted in view of a cochlear implant. Early information on cochlear implants appears to be necessary before total deafness occurs in patients suffering from Norrie disease. An inventory of digital communication tools that can be used by the patient is also highly valuable. Research should be supported for a more systematic use of psychiatric assessments prior to cochlear implants. In the special case of Norrie disease, we recommend early screening for mental retardation and related psychotic disorders and, depending on the patient's level of understanding, preventive information on the benefits and limits of cochlear implants before total deafness occurs.

  11. An Unusual Presentation of Addison's Disease-A Case Report.

    Science.gov (United States)

    Choudhary, Sandeep; Alam, Anwer; Dewan, Vivek; Yadav, Dinesh; Dubey, N K

    2011-07-01

    Addison's disease is most commonly due to autoimmune adrenalitis and tuberculosis and refers to primary hypoadrenalism caused by a total or near total destruction or dysfunction of both adrenal cortices. Usual manifestations involve chronic fatigue, muscle weakness, loss of appetite, nausea, vomiting, diarrhea, hypotension and hyperpigmentation of skin. We herein report a case of primary adrenal insufficiency presenting with fever and seizures in an 11-yr-old boy. His symptoms resolved after starting specific therapy. This kind of presentation of Addison's disease is rather unusual.

  12. A Case Of Ollier′s Disease With Intracranial Enchondroma

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    Sidharthan Neeraj

    2005-01-01

    Full Text Available The syndrome of multiple enchondromas is known as Ollier′s disease. Enchondromas are benign tumours of hyaline cartilage arising within the medullary cavity of tubular bones. We are reporting the case of a 16 year old girl with Ollier′s disease who presented with seizures and brainstem compression. A MRI scan of brain showed an intracranial space-occupying lesion in the region of clivus. The intracranial tumour was surgically removed and the histopathology confirmed the diagnosis of enchondroma. Intracranial enchondroma is an extremely rare situation and reported for the first time from south India.

  13. Linear Darier's disease: A case with bilateral presentation

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    Anal Jyoti Bordoloi

    2015-01-01

    Full Text Available Darier's disease is an autosomal dominant genodermatosis characterized by a persistent eruption of hyperkeratotic greasy papules mainly over the seborrheic sites of the body, usually associated with nail abnormalities and sometimes with mucous membrane lesions. The lesions typically occur in the younger age group and are associated with pruritus. We report here an atypical case of Linear Darier's disease with bilateral presentation in a middle-aged adult that could be confused with conditions such as lichen planus, inflammatory linear verrucous epidermal nevus, and wart.

  14. Clinico-pathological features of kidney disease in diabetic cases.

    Science.gov (United States)

    Furuichi, Kengo; Shimizu, Miho; Okada, Hirokazu; Narita, Ichiei; Wada, Takashi

    2018-03-21

    Diabetic kidney disease is the major cause of end-stage kidney disease in developed countries. However, the onset of kidney disorder and the progression pattern of kidney dysfunction and proteinuria greatly vary cases by cases. Therefore, risk classification with clinical data and pathological findings is important. Recent clinico-pathological study with kidney biopsy samples from diabetic patients revealed that pathological changes of diabetic nephropathy are characteristic and have special impacts on prognosis in each clinical stage. Moreover, comparison of the clinico-pathological findings of diabetic nephropathy with hypertensive nephrosclerosis revealed that there are few differences in their pathological findings in cases with low albuminuria and preserved estimated glomerular filtration rate (eGFR). Because it is so difficult to clearly distinguish pure kidney lesions caused by diabetes and kidney lesions due to effects other than diabetes, it is vital that these overlapped pathological findings be confirmed on kidney biopsy in cases of early stage diabetes. Further research is warranted regarding the pathogenesis of diabetic nephropathy and indication of kidney biopsy in diabetic cases.

  15. Two cases of bovine hypertrophic osteopathy (Marie-Bamberger's disease)

    International Nuclear Information System (INIS)

    Ravary, B.; Fecteau, G.

    2002-01-01

    Two cattle were presented with persistent lameness and Marie-Bamberger's disease was suspected because of the generalised deformity of the distal limbs. The diagnosis was confirmed after further tests (radiography, post-mortem and anatomopathological examinations). The first case was probably caused by a pulmonary infection. The cause of the second case could not be fully determined. The bony lesions characteristic of this condition result from stimulation of the osteogenic layer of the periosteum, and results from neoplasic lesions, pulmonary abscesses, bronchopneumonia, etc [it

  16. Atypical Primary Cutaneous Rosai Dorfman Disease: A Case Report.

    Science.gov (United States)

    Kinio, Anna E; Sawchuk, Michael A; Pratt, Melanie

    Rosai Dorfman disease (RDD) is a rare disorder that typically presents with bilateral cervical lymphadenopathy and follows a benign course. We present a case of late-onset atypical primary cutaneous RDD that is resistant to treatment modalities described in the literature. Case report. An 84-year-old woman presented with a 7-year history of cutaneous lesions histologically consistent with RDD. She later failed initial treatments of acitretin and thalidomide. Physicians must be aware of unusual presentations of RDD. Also, further treatment options must be explored for patients resistant to classical management of RDD.

  17. PSYCHIATRIC ASPECTS OF HUNTINGTON DISEASECASE REPORTS

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    Mirela Batta

    2004-04-01

    Full Text Available Background. Huntington disease occurrs rarely, it can be encountered not only by neurologists and psychiatrists but also by other medical practitioners. Its characteristic features are involuntary movements, cognitive disorders and gradual development of dementia. Diagnosis is given on the basis of these clinical features, positive familial anamnesis, with the laboratory exclusion of other neuropsychiatric diseases and with the help of neuroimaging methods (in particular NMR. The disease can be only confirmed by means of genetic analysis.Patients and methods. In this article, four cases of patients with Huntington disease and diverse psychiatric disorders that were hospitalised at the psychiatric department of the Maribor General Hospital between October 2002 and March 2003 are described. All the patients fulfilled the valid criteria for the diagnosis of Huntington disease. However, they differed according to their accompanying psychiatric psychopathology, age and social problems.Conclusions. The purpose of this article is to draw attention to different psychiatric symptoms and clinical manifestations of Huntington disease that are often misleading in the diagnostic process. In addition, exigency of early diagnostics, guidelines for referrals to genetic testing and psychiatric monitoring of these patients are emphasised.

  18. A Case of Multiple Sclerosis and Celiac Disease

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    H. Z. Batur-Caglayan

    2013-01-01

    Full Text Available Objectives. Multiple sclerosis (MS is an inflammatory autoimmune disorder of the central nervous system (CNS. Since a correlation between gluten intake and incidence of MS had been reported, the relationship of antigliadin antibodies and MS was debated. Case Report. We report the case of a 45-year-old female MS patient who is under interferon treatment. After seven years of monitoring, during her routine gastroenterological assessment, she was diagnosed with celiac disease. Conclusion. Beside the neurological manifestations that have been demonstrated in about 10% of celiac disease (CD patients, white-matter abnormalities in brain MRI are uncommon and controversial. But in the literature, MS seems to be associated with CD as in our patient. We suggest that MS patients with gastroenterological complaints should undergo an assessment for CD.

  19. IMPULSIVE-COMPULSIVE DISORDERS IN PARKINSON'S DISEASE. CLINICAL CASES

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    N. V. Fedorova

    2015-01-01

    Full Text Available Objective – a description of clinical cases of impulsive­compulsive disorders in Parkinson,s disease. The first clinical case. Patient N., 75 years old, suffering for 15 years from Parkinson,s disease, akinetic­rigid form of the disease, stage 4 by Hyun–Yar. Since 2009, he received levodopa/carbidopa 250/25 mg 5 times/day (daily dose of 1250 mg of levodopa; pramipexole 3.5 mg per day (daily dose 3.5 mg, amantadine sulfate 100 mg 5 times/day (daily dose 500 mg. While taking antiparkinsonian drugs the patient developed behavioral disorders such as dopamine disregulatory syndrome combined with punding, hypersexuality and compulsive shopping accompanied by visual hallucinations. The total equivalent dose of levodopa was 1600 mg per day. The second clinical case. Patient R., 52 years old, suffers from Parkinson,s disease about 5 years, a mixed form. She complained of slowness of movement, tremor in her left hand, sleep disturbances, poor mood. The clinic was appointed piribedil 50 mg 3 times per day. Despite the fact that the patient took only one of dopaminergic drugs in a therapeutic daily dose, she developed impulsive­compulsive disorder as hyper­ sexuality, compulsive shopping and binge eating. Results. In the first clinical case for correction of behavioral disorders in patients with Parkinson,s disease levodopa/carbidopa dose was reduced to 750 mg per day (3/4 Tab. 4 times a day; added to levodopa/benserazide dispersible 100 mg morning and levodopa/benserazide 100 mg before sleep (total dose of levodopa of 950 mg per day. Amantadine sulfate and pramipexole were canceled. It was added to the therapy of atypical neuroleptic clozapine dose 6,25 mg overnight. After 3 months marked improvement, regressed visual hallucina­ tions, improved family relationships, background mood became more stable. The patient continue to sing karaoke, but this hobby has be­ come less intrusive. In the second clinical event correction impulsive

  20. Histiocytosis versus (Hand-Schuller-Christian disease) - a case report

    International Nuclear Information System (INIS)

    Carneiro Filho, Jose Olimar; Leite, Marta Santos; Andrade Neto, Jose Moacyr

    2002-01-01

    The authors report a case of a 4-year-old girl with headache, irritability, othorrea and unilateral exophtalmus. Computed tomography and plain films showed large lytic areas in the vault and base of the skull. No other bones were involved. The diagnosis of histiocytosis X (Hand-Schuller-Christian disease) was confirmed by bone marrow biopsy. This paper presents the cytological and radiological findings of this patient as well as a review of the literature. (author)

  1. Laryngeal lipoma associated with Madelung's disease: a case report.

    Science.gov (United States)

    Landínez-Cepeda, Guillermo Arturo; Alarcos-Tamayo, Emilio V; Millás-Gómez, Teresa; Morais-Pérez, Darío

    2012-01-01

    Multiple symmetric lipomatosis is an alteration in the neck, upper trunk and upper extremities fat deposits. It produces an aesthetic problem and sometimes upper airway obstruction when the larynx is infiltrated by the mass. We report the case of a male with Madelung's disease, which began with acute dyspnea caused by laryngeal fat deposits and obstructive lipoma. Copyright © 2010 Elsevier España, S.L. All rights reserved.

  2. Madelung's disease: a case report and literature review

    International Nuclear Information System (INIS)

    Vidal, Maria da Graca Caminha; Londero, Thiza Massaia; Haygert, Carlos Jesus Pereira; Zagoury, Andre Rivas; Carrion, Rodrigo Previdello

    2010-01-01

    The authors report the case of a patient with Madelung's disease, which is clinically described as the presence of multiple and symmetric non-encapsulated masses of fatty tissue, usually involving the neck and the upper region of the trunk. Computed tomography was selected as the imaging method for diagnosis confirmation, considering its lower cost and higher availability as compared with magnetic resonance imaging. (author)

  3. Choroidal Coloboma in a Case of Tay-Sachs Disease

    OpenAIRE

    Ahmed, Nasreen Raees; Tripathy, Koushik; Kumar, Vivek; Gogia, Varun

    2014-01-01

    Coloboma as an ocular finding has been documented in various syndromes. Here we have a case of infantile Tay-Sachs disease associated with unilateral choroidal coloboma. To the best of our knowledge, such an association has not been documented in the literature. Whether such an association is a matter of chance or signifies the involvement of ganglioside metabolism in ocular embryogenesis remains to be elucidated.

  4. Choroidal coloboma in a case of tay-sachs disease.

    Science.gov (United States)

    Ahmed, Nasreen Raees; Tripathy, Koushik; Kumar, Vivek; Gogia, Varun

    2014-01-01

    Coloboma as an ocular finding has been documented in various syndromes. Here we have a case of infantile Tay-Sachs disease associated with unilateral choroidal coloboma. To the best of our knowledge, such an association has not been documented in the literature. Whether such an association is a matter of chance or signifies the involvement of ganglioside metabolism in ocular embryogenesis remains to be elucidated.

  5. Choroidal Coloboma in a Case of Tay-Sachs Disease

    Directory of Open Access Journals (Sweden)

    Nasreen Raees Ahmed

    2014-01-01

    Full Text Available Coloboma as an ocular finding has been documented in various syndromes. Here we have a case of infantile Tay-Sachs disease associated with unilateral choroidal coloboma. To the best of our knowledge, such an association has not been documented in the literature. Whether such an association is a matter of chance or signifies the involvement of ganglioside metabolism in ocular embryogenesis remains to be elucidated.

  6. Monilethrix – Case report of a rare disease

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    Tasleem Arif

    2015-01-01

    Full Text Available Monilethrix is a rare genetic disorder of hair characterized by beaded appearance of the hair shaft leading to hair fragility and patchy dystrophic alopecia. In this disorder, the hair shaft has alternate widenings (nodes and constrictions (internodes that lead to fracture of hair shaft and varying degree of alopecia. We report an eight year old Kashmiri boy who presented with diffuse hair loss since infancy. As monilethrix is a rare disease entity which prompted us to report this case.

  7. Rapidly aggravated Creutzfeldt-Jacob disease: autopsy-proven case

    Energy Technology Data Exchange (ETDEWEB)

    Park, Seung Hyun; Kang, Hyun Koo; Yu, Hyeon; Lee, Sang Chun [Seoul Veterans Hospital, Seoul (Korea, Republic of)

    2005-11-15

    Creutzfeldt-Jakob disease (DJD) is one of the transmissible spongiform encephalopathies, which is mediated by what has been known as 'prion'. It is a rare and fatal progressive neurodegenerative disease that affects the middle and old aged. There are a number of subtypes of CJD, one of which is the sporadic type characterized by rapidly progressing clinical symptoms, including progressive dementia, myoclonic jerk, and pyramidal or extrapyramidal syndrome. Patients usually end up dying within 1 to 2 years of contacting the disease. We report an autopsy-proven case of sporadic CJD with clinical symptoms that progressed within several days, along with dramatic changes on diffusion weighted magnetic resonance images.

  8. Hydatid disease in childhood: revisited report of an interesting case.

    Science.gov (United States)

    Jairajpuri, Zeeba Shamim; Jetley, Sujata; Hassan, Md Jaseem; Hussain, Musharraf

    2012-10-01

    Hydatid disease is a zoonosis caused by the tapeworm of Echinococcus spp. The disease is widely endemic in many sheep and cattle rearing locales. However, hydatidosis does not remain restricted to endemic geographical locales anymore but rather is a global health concern. It is a major public health burden causing significant morbidity and mortality. Echinococcus granulosus involvement in children has a different pattern than adults. Children of all age groups are susceptible and localization of the disease in the lungs is more commonly seen. Multiple liver cysts in the paediatric age group is relatively uncommon. We report an interesting case of multiple liver cysts in a 5-year old boy which was diagnosed as hydatid cysts on histopathological examination.

  9. ORAL MANIFESTATIONS OF CROHN'S DISEASE: A CASE REPORT

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    Muhvić-Urek Miranda

    2015-12-01

    Full Text Available Crohn´s disease is a chronic inflammatory bowel disease still with unknown etiology. In 0.5-20 % of patients, extraintestinal lesions in the oral cavity can be presented in forms of orofacial granulomatosis, cobblestone and corrugated oral mucosa, mucosal tags, deep linear ulcerations with hyperplastic folds, pyostomatits vegetans, aphthous ulcers, angular cheilitis, labial/facial edema and gingival erythema/edema. We describe a case of a 28-year-old male who was presented with oral lesions of Crohn´s disease and treatment procedure. The patient was candidate for biologic treatment so dental procedures and preparation of the patient for treatment are described. Good communication and cooperation between the patient's doctor and dentist are important for successful treatment.

  10. Whipple's disease. Report of five cases with different clinical features.

    Science.gov (United States)

    Ferrari, M de L; Vilela, E G; Faria, L C; Couto, C A; Salgado, C J; Leite, V R; Brasileiro Filho, G; Bambirra, E A; Mendes, C M; Carvalho, S de C; de Oliveira, C A; da Cunha, A S

    2001-01-01

    Whipple's disease (WD) is a rare systemic disease of infectious etiology which involves the small intestine but can virtually affect any organ. We present here five cases (four males and one female) ranging in age from 20 to 59 years. All patients had intestinal involvement associated or not with clinical manifestations linked to this organ. Vegetation in the tricuspid valve was observed in one patient, suggesting endocarditis caused by Tropheryma whippelii, with disappearance of the echocardiographic alterations after treatment. In one of the male patients the initial clinical manifestation was serologically negative spondylitis, with no diarrhea occurring at any time during follow-up. Ocular involvement associated with intestinal malabsorption and significant weight loss were observed in one case. In the other two cases, diarrhea was the major clinical manifestation. All patients were diagnosed by histological examination of the jejunal mucosa and, when indicated, of extraintestinal tissues by light and electron microscopy. After antibiotic treatment, full remission of symptoms occurred in all cases. A control examination of the intestinal mucosa performed after twelve months of treatment with sulfamethoxazole-trimethoprim revealed the disappearance of T. whippelii in four patients. The remaining patient was lost to follow-up.

  11. Whipple's disease. Report of five cases with different clinical features

    Directory of Open Access Journals (Sweden)

    FERRARI Maria de Lourdes de Abreu

    2001-01-01

    Full Text Available Whipple's disease (WD is a rare systemic disease of infectious etiology which involves the small intestine but can virtually affect any organ. We present here five cases (four males and one female ranging in age from 20 to 59 years. All patients had intestinal involvement associated or not with clinical manifestations linked to this organ. Vegetation in the tricuspid valve was observed in one patient, suggesting endocarditis caused by Tropheryma whippelii, with disappearance of the echocardiographic alterations after treatment. In one of the male patients the initial clinical manifestation was serologically negative spondylitis, with no diarrhea occurring at any time during follow-up. Ocular involvement associated with intestinal malabsorption and significant weight loss were observed in one case. In the other two cases, diarrhea was the major clinical manifestation. All patients were diagnosed by histological examination of the jejunal mucosa and, when indicated, of extraintestinal tissues by light and electron microscopy. After antibiotic treatment, full remission of symptoms occurred in all cases. A control examination of the intestinal mucosa performed after twelve months of treatment with sulfamethoxazole-trimethoprim revealed the disappearance of T. whippelii in four patients. The remaining patient was lost to follow-up.

  12. Coronary arterial disease after electroconvulsive therapy: a case report

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    Nataly Pimentel Rodrigues

    2015-06-01

    Full Text Available Objectives Unipolar depression (UPD is a leading cause of global burden of diseases, particularly among the elderly, whose treatment may be challenging. In such cases, ECT is often recommended due to its safety and efficacy. This report presents a case of a 67-year-old male inpatient that developed a rare cardiac complication during ECT. Methods Clinical case report with patient’s consent and bibliographic review. Results A 67-year-old male inpatient with recurrent severe psychotic depression was hospitalized and ECT was indicated after failure of the pharmacological treatment. A comprehensive clinical pre-evaluation revealed only nonspecific ST-segment changes in electrocardiogram. During the 7th ECT session, it was observed transitory ST-segment depression followed by a discrete increase of plasma troponin I. Severe tri-vessel coronary artery stenosis was found and a percutaneous coronary angioplasty was performed, with satisfactory psychiatric and cardiac outcomes. Conclusions Unipolar depression (UPD and cardiovascular disease are often coexistent conditions, especially among the elderly. In the current case, myocardial ischemia was detected lately during ECT therapy and its treatment allowed the UPD treatment to be completed adequately.

  13. Periodontal disease and spontaneous preterm birth: a case control study

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    Eley Barry

    2006-07-01

    Full Text Available Abstract Background Several studies have suggested an association between periodontal disease and prematurity but this finding has not been consistently observed. Methods Case control study. Cases (n = 50 were women who had delivered after spontaneous preterm labor at Results There was no difference in the proportion of sites with significant attachment loss (≥3 mm: Cases-3.2%, Controls-2.2% p = 0.21. The gingival crevicular fluid concentrations of elastase and gingipain were elevated in cases vs. controls 238.8 uU/ul vs. 159.6 uU/ul p = .007 and 2.70 uU/ul vs. 1.56 uU/ul p = .001. On multivariate analysis, the mean log concentration of elastase, but not of gingipain, remained a significant predictor of preterm labor p = .0.015. Conclusion We found no evidence that clinical periodontal disease is associated with spontaneous preterm birth. Elevated gingival crevicular fluid levels of elastase were associated with preterm birth but further research is needed before this can be assumed to be a causal relationship.

  14. The first case of imported Borrelia miyamotoi disease concurrent with Lyme disease.

    Science.gov (United States)

    Oda, Rentaro; Kutsuna, Satoshi; Sekikawa, Yoshiyuki; Hongo, Igen; Sato, Kozue; Ohnishi, Makoto; Kawabata, Hiroki

    2017-05-01

    Borrelia miyamotoi disease (BMD) is an emerging infectious disease caused by B. miyamotoi. Although BMD has been reported in the United States, Europe, and Japan, no case of imported BMD has been described in the world. Here, we report a 63-year-old American man living in Japan who presented with malaise, headache, myalgia, and arthralgia. We suspected Lyme disease because of his travel history to Minnesota and presence of erythema migrans. Serologic analysis supported our diagnosis, and doxycycline was administered for 14 days. However, we also suspected coinfection with BMD because of his fever, elevated liver function test results and his travel history. The patient was seropositive for the immunoglobulin M antibody to recombinant glycerophosphodiester phosphodiesterase, and was diagnosed with coinfection with BMD. This case suggests that BMD should be considered in febrile travelers returning from the Northeastern and Midwestern regions of the United States, and that BMD and Lyme disease coinfection should be considered to detect cases of imported BMD. Copyright © 2017 Japanese Society of Chemotherapy and The Japanese Association for Infectious Diseases. Published by Elsevier Ltd. All rights reserved.

  15. Clinical-histopathological correlation in a case of Coats' disease

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    Zajdenweber Moyses E

    2006-08-01

    Full Text Available Abstract Background Coats' disease is a non-hereditary ocular disease, with no systemic manifestation, first described by Coats in 1908. It occurs more commonly in children and has a clear male predominance. Most patients present clinically with unilateral decreased vision, strabismus or leukocoria. The most important differential diagnosis is unilateral retinoblastoma, which occurs in the same age group and has some overlapping clinical manifestations. Case presentation A 4 year-old girl presented with a blind and painful right eye. Ocular examination revealed neovascular glaucoma, cataract and posterior synechiae. Although viewing of the fundus was impossible, computed tomography disclosed total exsudative retinal detachment in the affected eye. The eye was enucleated and subsequent histopathological evaluation confirmed the diagnosis of Coats' disease. Conclusion General pathologists usually do not have the opportunity to receive and study specimens from patients with Coats' disease. Coats' disease is one of the most important differential diagnoses of retinoblastoma. Therefore, It is crucial for the pathologist to be familiar with the histopathological features of the former, and distinguish it from the latter.

  16. Climate change and animal diseases: making the case for adaptation.

    Science.gov (United States)

    Cáceres, Sigfrido Burgos

    2012-12-01

    The exponential expansion of the human population has led to overexploitation of resources and overproduction of items that have caused a series of potentially devastating effects, including ocean acidification, ozone depletion, biodiversity loss, the spread of invasive flora and fauna and climatic changes - along with the emergence of new diseases in animals and humans. Climate change occurs as a result of imbalances between incoming and outgoing radiation in the atmosphere. This process generates heat. As concentrations of atmospheric gases reach record levels, global temperatures are expected to increase significantly. The hydrologic cycle will be altered, since warmer air can retain more moisture than cooler air. This means that some geographic areas will have more rainfall, whereas others have more drought and severe weather. The potential consequences of significant and permanent climatic changes are altered patterns of diseases in animal and human populations, including the emergence of new disease syndromes and changes in the prevalence of existing diseases. A wider geographic distribution of known vectors and the recruitment of new strains to the vector pool could result in infections spreading to more and potentially new species of hosts. If these predictions turn out to be accurate, there will be a need for policymakers to consider alternatives, such as adaptation. This review explores the linkages between climate change and animal diseases, and examines interrelated issues that arise from altered biological dynamics. Its aim is to consider various risks and vulnerabilities and to make the case for policies favoring adaptation.

  17. Boxing training for patients with Parkinson disease: a case series.

    Science.gov (United States)

    Combs, Stephanie A; Diehl, M Dyer; Staples, William H; Conn, Lindsay; Davis, Kendra; Lewis, Nicole; Schaneman, Katie

    2011-01-01

    A nontraditional form of exercise recently applied for patients with Parkinson disease (PD) is boxing training. The primary purpose of this case series is to describe the effects of disease severity and duration of boxing training (short term and long term) on changes in balance, mobility, and quality of life for patients with mild or moderate to severe PD. The feasibility and safety of the boxing training program also were assessed. Six patients with idiopathic PD attended 24 to 36 boxing training sessions for 12 weeks, with the option of continuing the training for an additional 24 weeks (a seventh patient attended sessions for only 4 weeks). The 90-minute sessions included boxing drills and traditional stretching, strengthening, and endurance exercises. Outcomes were tested at the baseline and after 12, 24, and 36 weeks of boxing sessions (12-, 24-, and 36-week tests). The outcome measures were the Functional Reach Test, Berg Balance Scale, Activities-specific Balance Confidence Scale, Timed "Up & Go" Test, Six-Minute Walk Test, gait speed, cadence, stride length, step width, activities of daily living and motor examination subscales of the Unified Parkinson Disease Rating Scale, and Parkinson Disease Quality of Life Scale. Six patients completed all phases of the case series, showed improvements on at least 5 of the 12 outcome measures over the baseline at the 12-week test, and showed continued improvements at the 24- and 36-week tests. Patients with mild PD typically showed improvements earlier than those with moderate to severe PD. Despite the progressive nature of PD, the patients in this case series showed short-term and long-term improvements in balance, gait, activities of daily living, and quality of life after the boxing training program. A longer duration of training was necessary for patients with moderate to severe PD to show maximal training outcomes. The boxing training program was feasible and safe for these patients with PD.

  18. Lyme disease: case report of persistent Lyme disease from Pulaski County, Virginia

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    Palmieri JR

    2013-12-01

    Full Text Available James R Palmieri,1 Scott King,1 Matthew Case,1 Arben Santo21Department of Microbiology, Infectious and Emerging Diseases, 2Department of Pathology, Edward Via College of Osteopathic Medicine, Blacksburg, VA, USAAbstract: A 50-year-old woman from Pulaski, Virginia, presented to a local clinic with headaches, fever, generalized joint pain, excessive thirst and fluid intake, and a progressing rash on her back. On physical examination, she had a large circular red rash on her back with a bull's-eye appearance, 16 × 18 cm in diameter. Serologic tests confirmed a diagnosis of Lyme disease. The patient could recall a walk through the woods 3 weeks prior, although she never noticed a tick on her body. Following a prolonged course of antibiotics, this case report presents a patient with ongoing symptoms consistent with post-treatment Lyme disease.Keywords: arthritis, chronic Lyme disease (CLD, ELISA, erythema migrans, ixodid ticks, Lyme disease, post-treatment Lyme disease syndrome (PTLDS, Western blotting

  19. A Rare Case Of Graves’ Disease With Splenomegaly And Pancytopenia

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    Elayne Christinne Marcelino e Silva

    2017-07-01

    Full Text Available Introduction: Splenomegaly and pancytopenia are rare complications of Graves' disease with few reports in the literature about this association. The pathogenesis is unknown and immunological mechanisms seem to be involved. The possibility of hyperthyroidsm should always considered in patients with pancytopenia. Objective: Describe  clinical case of association between Grave's disease, splenomegaly and pancytopenia. Method: This is a case report, obtained through data from medical records of a reference hospital located in the city of Juazeiro do Norte, Ceara, Brazil. Case report: Patient, 46 years old, female, sought treatment at a reference hospital with abdominal pain that started two days earlier, prevalent in mesogastric region and left hypochondrium very intense and recurrent, associated with significant consuptive syndrome ( loss of 10 Kg in 4 months, asthenia, dyspnea on minimum exertion, irritability and fine tremor in extremitie. SHe denied fever, palpitations, heat intolerance, skin ou eye changes. A diffuse thyroid enlargement with the presence of thrill and murmur, digital clubbing, fixed and bright look, light exophthalmos and splenomegaly about 6 cm below the left costal margin were abserved after physical examination. Ultrasound examination (USG of the abdomen and CT scan showed moderate splenomegaly. Laboratory tests showed normocytic and normochromic anemia, leukocytosis and mild thrombocytopenia. Thyroid USG showed characteristic features of Graves' disease, a bone marrow biopsy revealed maturation preserved in all strains and lack of fibrosis and megakaryocytes present in normal number without atypia. treatment was set with propylthiouracil 300 mg a day and after the first revaluation after hospital discharge three weeks later a regression of splenomegaly has been observed. Conclusion: This case ilustrates the rare association between hyperthyroidism and splenomegaly with pancytopenia.

  20. Crossed cerebellar atrophy in cases with cerebrovascular disease

    International Nuclear Information System (INIS)

    Yagishita, Toshiyuki; Kojima, Shigeyuki; Hirayama, Keizo; Iwabuchi, Sadamu.

    1989-01-01

    Crossed cerebellar atrophy (CCA) was investigated by X-ray CT to establish the incidence, mechanism, and the relation to cerebral lesions in 130 cases of unilateral supratentorial cerebrovascular diseases. The 130 cases consisted of 83 males and 47 females with cerebral infarction (65 cases) and cerebral hemorrhage (65 cases). The patients' average age was 57.6 years. Crossed cerebellar atrophy was demonstrated in 8 cases (6.2%), 6 of whom had massive cerebral infarction in the middle cerebral artery area (9.2% of the 65 cases of cerebral infarction. The six cases of CCA caused by cerebral infarction had lesions in the frontal and temporal lobes. Two had a cerebral hemorrhage in the putamen and in the thalamus, respectively, accounting for 3.1% of the 65 cases of cerebral hemorrhage. Of the 2 cases, one had putaminal hemorrhage, and the other had thalamic hemorrhage. Cerebrovascular stroke had occured in these patients with CCA more than 2 months previously. In 5 of the 8 cases of CCA, atrophy was present in the basis pedunculi and the basis pontis on the side of the cerebral lesion. However, neither dilation nor deformity of the fourth ventricle was present in any of the patients, suggesting that none of the CCA patients had atrophy of the dentate nucleus. The CCA patients had massive cerebral lesion in the frontal and temporal lobes or atrophy of the basis pedunculi and basis pontis, suggesting the presence of the transsynaptic degeneration of the cortico-ponto-cerebellar pathway. In the case of the thalamic hemorrhage, who had not hemorrhagic lesion in the frontal and temporal lobes, atrophy of the basis peduncli and basis pontis was not observed. Though dilation or deformity of the fourth ventricle is not observed in this case, presence of the degeneration of the dentate-rubro-thalamic pathway cannot be denied. CCA seems to be caused by both the transsynaptic degeneration of the cortico-ponto-cerebellar pathway and the dentate-rubro-thalamic pathway. (J.P.N.)

  1. A Case Report of Cushing's Disease Presenting as Hair Loss.

    Science.gov (United States)

    Lefkowitz, Emily G; Cossman, Jack P; Fournier, John B

    2017-01-01

    Cushing's syndrome is a rare endocrine disorder that comprises a large group of signs and symptoms resulting from chronic exposure to excess corticosteroids. Most cases of Cushing's syndrome are due to increased adrenocorticotropic hormone production from a pituitary adenoma, which is referred to as Cushing's disease. Most of the signs and symptoms are nonspecific and common in the general population, making a diagnosis often challenging. However, several dermatological manifestations, such as fragile skin, easy bruising, and reddish purple striae, are more discriminatory. Because uncontrolled Cushing's syndrome of any etiology is associated with substantial morbidity, including increased cardiovascular disease and mortality, it is important to make an early diagnosis. Unfortunately, median delays of 2 years to diagnosis have been reported. We report a case of a woman who had multiple dermatological findings, including facial plethora, easy bruising, violaceous striae, hirsutism, and acne, the latter 2 signs reflecting androgen excess. Of interest, our patient presented with a chief complaint of hair loss, a common complaint in the general population that occurs with a greater frequency in patients with Cushing's disease and is attributed to androgenetic alopecia, but it is rarely the presenting symptom.

  2. Retroperitoneal unicentric Castleman's disease (giant lymph node hyperplasia: case report

    Directory of Open Access Journals (Sweden)

    Jaques Waisberg

    Full Text Available CONTEXT AND OBJECTIVE: Castleman's disease, or giant lymph node hyperplasia, is a rare disorder of the lymphoid tissue that causes lymph node enlargement. It is considered benign in its localized form, but aggressive in the multicentric type. The definitive diagnosis is based on postoperative pathological findings. The aim here was to describe a case of retroperitoneal unicentric Castleman's disease in the retroperitoneum. CASE REPORT: A 61-year old white male with weight loss and listlessness presented with moderate arterial hypertension and leukopenia. Abdominal tomography revealed a 5 x 4 x 5 cm oval mass of low attenuation, with inner calcification and intense enhancement on intravenous contrast, located in the retroperitoneal region, between the left kidney and the aorta, at the renal hilus. Exploratory laparotomy revealed a non-pulsatile solid oval mass situated in the retroperitoneum, adjacent to the left renal hilus. The retroperitoneal lesion was removed in its entirety. Examination of frozen samples revealed benign lymph node tissue and histopathological examination of the surgical sample revealed hyaline-vascular giant lymph node hyperplasia (Castleman's disease. The patient was discharged on the 12th day without significant events. Two months after the operation, the patient was readmitted with severe cardiac insufficiency, acute renal failure and bronchopneumonia, which progressed to acute respiratory insufficiency, sepsis and death.

  3. CASE REPORT CASE CASE R The many faces of hydatid disease

    African Journals Online (AJOL)

    CASE R. Case report. A 35-year-old man presented with a right chest wall mass. Chest radio- graphs revealed pleural thickening underlying a soft tissue mass which obscured the 5th to 7th ribs, and an expansile osteolytic lesion of the 7th rib. Ultrasound examination demonstrated a fluid-filled cystic lesion of the chest wall ...

  4. Primary immunodeficiency disease: a model for case management of chronic diseases.

    Science.gov (United States)

    Burton, Janet; Murphy, Elyse; Riley, Patty

    2010-01-01

    Patient-centered chronic care management is a new model for the management of rare chronic diseases such as primary immunodeficiency disease (PIDD). This approach emphasizes helping patients become experts on the management of their disease as informed, involved, and interactive partners in healthcare decisions with providers. Because only a few patients are affected by rare illnesses, these patients are forced to become knowledgeable about their disease and therapies and to seek treatment from a healthcare team, which includes physicians and nurse specialists who are equipped to manage the complexity of the disease and its comorbidities. Importantly, therapy for PIDD can be self-administered at home, which has encouraged the transition toward a proactive stance that is at the heart of patient-centered chronic care management. We discuss the evolution of therapy, the issues with the disease, and challenges with its management within the framework of other chronic disease management programs. Suggestions and rationale to move case management of PIDD forward are presented with the intent that sharing our experiences will improve process and better manage outcomes in this patient population. The patient-centered model for the management of PIDD is applicable to the primary care settings, where nurse case managers assist patients through education, support them and their families, and facilitate access to community resources in an approach, which has been described as "guided care." The model also applies specifically to immunology centers where patients receive treatment or instruction on its self-administration at home. Patient-centered management of PIDD, with its emphasis on full involvement of patients in their treatment, has the potential to improve compliance with treatment, and thus patient outcomes, as well as patients' quality of life. The patient-centered model expands the traditional model of chronic disease management, which relies on evidence

  5. Two Legionnaires' disease cases associated with industrial waste water treatment plants: a case report

    Directory of Open Access Journals (Sweden)

    Putus Tuula

    2010-12-01

    Full Text Available Abstract Background Finnish and Swedish waste water systems used by the forest industry were found to be exceptionally heavily contaminated with legionellae in 2005. Case presentation We report two cases of severe pneumonia in employees working at two separate mills in Finland in 2006. Legionella serological and urinary antigen tests were used to diagnose Legionnaires' disease in the symptomatic employees, who had worked at, or close to, waste water treatment plants. Since the findings indicated a Legionella infection, the waste water and home water systems were studied in more detail. The antibody response and Legionella urinary antigen finding of Case A indicated that the infection had been caused by Legionella pneumophila serogroup 1. Case A had been exposed to legionellae while installing a pump into a post-clarification basin at the waste water treatment plant of mill A. Both the water and sludge in the basin contained high concentrations of Legionella pneumophila serogroup 1, in addition to serogroups 3 and 13. Case B was working 200 meters downwind from a waste water treatment plant, which had an active sludge basin and cooling towers. The antibody response indicated that his disease was due to Legionella pneumophila serogroup 2. The cooling tower was the only site at the waste water treatment plant yielding that serogroup, though water in the active sludge basin yielded abundant growth of Legionella pneumophila serogroup 5 and Legionella rubrilucens. Both workers recovered from the disease. Conclusion These are the first reported cases of Legionnaires' disease in Finland associated with industrial waste water systems.

  6. Novel Methods in Disease Biogeography: A Case Study with Heterosporosis

    Directory of Open Access Journals (Sweden)

    Luis E. Escobar

    2017-07-01

    Full Text Available Disease biogeography is currently a promising field to complement epidemiology, and ecological niche modeling theory and methods are a key component. Therefore, applying the concepts and tools from ecological niche modeling to disease biogeography and epidemiology will provide biologically sound and analytically robust descriptive and predictive analyses of disease distributions. As a case study, we explored the ecologically important fish disease Heterosporosis, a relatively poorly understood disease caused by the intracellular microsporidian parasite Heterosporis sutherlandae. We explored two novel ecological niche modeling methods, the minimum-volume ellipsoid (MVE and the Marble algorithm, which were used to reconstruct the fundamental and the realized ecological niche of H. sutherlandae, respectively. Additionally, we assessed how the management of occurrence reports can impact the output of the models. Ecological niche models were able to reconstruct a proxy of the fundamental and realized niche for this aquatic parasite, identifying specific areas suitable for Heterosporosis. We found that the conceptual and methodological advances in ecological niche modeling provide accessible tools to update the current practices of spatial epidemiology. However, careful data curation and a detailed understanding of the algorithm employed are critical for a clear definition of the assumptions implicit in the modeling process and to ensure biologically sound forecasts. In this paper, we show how sensitive MVE is to the input data, while Marble algorithm may provide detailed forecasts with a minimum of parameters. We showed that exploring algorithms of different natures such as environmental clusters, climatic envelopes, and logistic regressions (e.g., Marble, MVE, and Maxent provide different scenarios of potential distribution. Thus, no single algorithm should be used for disease mapping. Instead, different algorithms should be employed for a more

  7. Comorbidities in rotator cuff disease: a case-control study.

    Science.gov (United States)

    Titchener, Andrew G; White, Jonathan J E; Hinchliffe, Sally R; Tambe, Amol A; Hubbard, Richard B; Clark, David I

    2014-09-01

    Rotator cuff disease is a common condition in the general population, but relatively little is known about its associated risk factors. We have undertaken a large case-control study using The Health Improvement Network database to assess and to quantify the relative contributions of some constitutional and environmental risk factors for rotator cuff disease in the community. Our data set included 5000 patients with rotator cuff disease who were individually matched with a single control by age, sex, and general practice (primary care practice). The median age at diagnosis was 55 years (interquartile range, 44-65 years). Multivariate analysis showed that the risk factors associated with rotator cuff disease were Achilles tendinitis (odds ratio [OR] = 1.78), trigger finger (OR = 1.99), lateral epicondylitis (OR = 1.71), and carpal tunnel syndrome (OR = 1.55). Oral corticosteroid therapy (OR = 2.03), oral antidiabetic use (OR = 1.66), insulin use (OR = 1.77), and "overweight" body mass index of 25.1 to 30 (OR = 1.15) were also significantly associated. Current or previous smoking history, body mass index of greater than 30, any alcohol intake, medial epicondylitis, de Quervain syndrome, cubital tunnel syndrome, and rheumatoid arthritis were not found to be associated with rotator cuff disease. We have identified a number of comorbidities and risk factors for rotator cuff disease. These include lateral epicondylitis, carpal tunnel syndrome, trigger finger, Achilles tendinitis, oral corticosteroid use, and diabetes mellitus. The findings should alert the clinician to comorbid pathologic processes and guide future research into the etiology of this condition. Copyright © 2014 Journal of Shoulder and Elbow Surgery Board of Trustees. Published by Mosby, Inc. All rights reserved.

  8. Danon disease: A case report and literature overview

    Directory of Open Access Journals (Sweden)

    Ćatović Suad

    2007-01-01

    Full Text Available Danon disease, a rare glycogen storage disease, is a dominant X-linked disorder. It is due to mutation in gene for lysosome-associated membrane protein 2 (LAMP 2. The LAMP 2 gene is located on Xq24, and its mutation causes primary deficiency of LAMP 2 and myocyte hypertrophy by accumulations of vacuoles containing glycogen. Danon disease is clinically characterized by the triad of hypertrophic cardiomyopathy (HCM, proximal myopathy and mental retardation. Myopathy and mental retardation can be absent, and cardiomyopathy is usually hypertrophic. This is a case report of the patient with genetically confirmed Danon disease and mixed cardiomyopathy, but without myopathy and mental retardation. ECG showed typical Wolff-Parkinson-White (WPW pattern while echocardiography demonstrated hypertrophy and dilatation of all cardiac chambers with impaired systolic and diastolic function. Male sex, early onset of symptoms, massive hypertrophy of the myocardium and ventricular preexcitation indicate a genetic basis for HCM. Therapeutic measures, except heart transplantation, do not improve prognosis substantially. Only an accurate diagnosis in patients with unexplained HCM helps in establishing of the appropriate treatment strategies and adequate genetic consultation. .

  9. [Wilson disease. A case report and review of the literature].

    Science.gov (United States)

    Alva-Moncayo, Edith; Castro-Tarín, María; González-Serrano, Adolfo

    2011-01-01

    Wilson disease is a problem of cuprum metabolism, with recesive autosomic hereditary transmission and a prevalence of one in 30,000 habitants. The cuprum is deposit in a progressive and irreversible way in the liver and encephalus and it is not liberated with quelant treatment. Neurological manifestations are tremor, disartria, extrapiramidal manifestations or distonia. Ophthalmic exploration shows corneal limb with sign of Kayser-Fleischer. a 15-year-old masculine patient with previous hepatitis outbreak in two times. During the last year he presented distonia, bradicinecious, stiffness and indifference with ictericia. Ophthalmological examination reported Kayser-Fleisher rings. Magnetic resonance of brain showed high dense images in lenticular, pallidus globe and caudate nucleus suggestive of Wilson disease. Ceruloplasmin concentration, cuprum in the liver biopsy confirmed the diagnosis. the importance of the case was the hepatic initial manifestations and two years after presented with inexpressive face, and it was considered a psychiatric disease, but the neurological evaluation and the liver biopsy confirmed the diagnosis of Wilson disease.

  10. Leflunomide-Induced Interstitial Lung Disease: A Case Report

    Directory of Open Access Journals (Sweden)

    Aygül Güzel

    2015-04-01

    Full Text Available Leflunomide (LEF induced interstitial pneumonitis is a very rare condition but potentially fatal. We report a case of LEF induced interstitial pneumonitis. A 63-year-old woman followed-up for 37 years with the diagnosis of rheumatoid arthritis treated with LEF (20 mg/day since 5 months were admitted to our hospital with cough, dyspnea, fever, and dark sputum.Chest radiography represented bilateral alveolar consolidation. High-resolution computed tomography demonstrated diffuse ground-glass appearance and interlobular septal thickening. Since the patient’s clinics and radiologic findings improved dramatically after the cessation of LEF and recieving oral steriod therapy, she was diagnosed as drug-induced interstitial lung disease. In conclusion, when nonspecific clinical signs such as respiratory distress, cough and fever seen during the use of LEF, drug-induced interstitial lung disease should be kept in mind for the differantial diagnosis.

  11. [Horseshoe kidney, stone disease and prostate cancer: a case presentation].

    Science.gov (United States)

    Hermida Pérez, J A; Bermejo Hernández, A; Hernández Guerra, J S; Sobenes Gutierrez, R J

    2013-01-01

    The horseshoe kidney is the most common congenital renal fusion anomalies. It occurs in 0.25% of the population, or 1 in every 400 people. It is more frequent in males (ratio 2:1). The most observed complication of horseshoe kidney is stone disease, although there may be others such as, abdominal pain, urinary infections, haematuria, hydronephrosis, trauma and tumours (most commonly associated with hypernephroma and Wilms tumour). We describe a case of a male patient with horseshoe kidney, stone disease and adenocarcinoma of the prostate. One carrier of this condition who suffered a transitional cell carcinoma of the prostate was found in a review of the literature. Copyright © 2012 Sociedad Española de Médicos de Atención Primaria (SEMERGEN). Publicado por Elsevier España. All rights reserved.

  12. Cranial Paget's disease - clinical case of symptomatic secondary basilar impression

    International Nuclear Information System (INIS)

    Gagov, E.; Gabrovsky, N.; Gabrovsky, S.

    2010-01-01

    A clinical case of 52 years old woman with history of periodic headaches for many years. The headache became more intensive and constant during the last 4-6 months. Instability by walking and stagger occurred as well as weakness in all 4 extremities, difficult swallowing and speech changes. Bulbar, quadripyramidal and archicerebellar symptoms were in hand. Pagets disease was ascertained engaging the skull with secondary basilar impression and compression of the cerebellum and the brain-stem leading to the above described clinical signs. Decompressive median suboccipital craniectomy was performed with laminectomy of C1. Occipital squama was thickened and highly vascularized.. Secondary basilar impression could occur in cranial Pagets disease with clinical symptoms resulting from the compression of the cerebellum and the brain-stem

  13. Computed tomography findings in cases of Minamata disease

    International Nuclear Information System (INIS)

    Shirakawa, Kenichi; Hirota, Koichi; Tsubaki, Tadao; Tanimura, Kenichi; Yamamoto, Tsuneo.

    1979-01-01

    Computed tomography (CT) was used to evaluate 57 patients with Minamata disease (methylmercury poisoning); it was proved to be an objective and practical screening procedure. A typical patient who presented an acute onset of concentric constriction of visual fields and cerebellar ataxia showed a low-density area in both occipital regions; cerebellar atrophy and enlarged cerebral cortical sulci. On the other hand, the majority of patients who presented a chronic onset, with only a mild concentric constriction of visual fields, dysdiadochokinesis, and sensory disturbance showed only a questionable or a mild cerebellar atrophy. An enlarged paracerebellar space in mild cerebellar atrophy was easily identified at the pontine angle cistern, the paravermian cistern, and the triangular space of the rectal sinus in the coronal section on an enhanced CT scan. These findings were compatible with pathological findings of mild cases of Minamata disease. The correlation of dysdiadochokinesis with the degree of cerebellar atrophy was demonstrated. (author)

  14. Septated pericarditis associated with Kawasaki disease: a brief case report.

    Science.gov (United States)

    Sonçaği, Arzu; Devrim, Ilker; Karagöz, Tevfik; Dilber, Embiya; Celiker, Alpay; Ozen, Seza; Seçmeer, Gülten

    2007-01-01

    Kawasaki disease (KD) is primarily the systemic vasculitis of childhood that affects mainly the medium-sized arteries, such as the coronary arteries. KD is the leading cause of acquired heart disease, whereas the incidence of rheumatic fever has declined. The most serious complication is coronary artery involvement. Among the children with KD who developed cardiac complications, pericarditis is a rare complication, with an incidence of 0.07%. We report our experience in a 5.5-year-old child with KD complicated with aneurysm of the left anterior descendant coronary artery and septated pericardial effusion, which has not been reported in the literature. The pericardial effusion disappeared very dramatically with intravenous immunoglobulin (IVIG) therapy. We would like to point out that septated pericardial effusion in cases of KD do not need any further therapy other than IVIG and high-dose acetylsalicylic acid.

  15. [A Forensic Autopsy Case Applied for Asbestos-Related Disease].

    Science.gov (United States)

    Makihara, Kosuke; Hamada, Tetsuo; Kasai, Kentaro; Tanaka, Toshiko; Sato, Hiroaki

    2016-03-01

    We had a forensic autopsy case that required additive pathological examination for the asbestos-related lung disease compensatory application afterwards. A man in his sixties with a history of occupational asbestos inhalation who had neither visited a hospital nor received a physical examination received forensic autopsy because of his death from unknown cause. An inmate said, "He developed cough and dyspnea, and died in the progression of the symptoms." The autopsy revealed widespread pleural plaques on both sides of the parietal pleura and multiple tumors in both sides of the lungs. The cause of death was diagnosed as lung cancer. Additional pathological examination was asked by his family to certify that he had suffered from asbestos-related lung disease in order to apply to the Asbestos-related Damage Relief Law. The Japanese criteria of the compensation law of asbestos-related lung cancer is the detection of more than 5,000 asbestos bodies per gram of dry lung tissue, while his number of asbestos bodies was 4,860. Asbestos bodies were reported to be accumulated in the distal lung parenchyma with no pathological changes. The present lung samples were collected from proximal section around the tumor, which might have made the number of asbestos bodies less than the criteria. Both the number of patients suffering from asbestos-related lung disease and the number of forensic autopsy cases have increased in Japan. Collecting lung samples from the appropriate lung section is essential and should be noted when the lung cancer is suspected at forensic autopsy in order to apply for asbestos-related lung disease compensation.

  16. Case report 387: Gaucher disease affecting the skeleton (left femur)

    International Nuclear Information System (INIS)

    Tabas, J.H.; Daffner, R.H.; Hartsock, R.J.; Blakley, J.B.

    1986-01-01

    A case is described of a non-Jewish (Italian) 49-year-old man who presented to the hospital with pain in the left hip. Radionuclide studies showed decreased tracer activity with 99m Tc MDP over a lytic area in the subtrochanteric region of the left femur. Increased activity, however, was present in the right temporal bone, low anterior rib cage and right tenth posterior rib. The presence of subendosteal sclerosis with some cortical thickening adjacent to the femoral lesion, suggested the possibility of malignant neoplasm, (e.g. chondrosarcoma). Biopsy of the bone marrow showed the presence of Gaucher disease. (orig./SHA)

  17. Case Report: Giant Right Atrium in Rheumatic Mitral Disease

    Directory of Open Access Journals (Sweden)

    Deniz Demir

    2014-06-01

    Full Text Available Dilation and hypertrophy of the atria occur in patients with valvular heart disease especially in mitral regurgitation, mitral stenosis or tricuspid abnormalities. Dilatation of the atriums which occurs slowly in time, becomes evident with ritim disturbances and embolic events. We report a case of an unusual giant right atrium in context of rheumatic mitral stenosis, mitral regurgitation, pulmonar hypertansion and severe tricuspid regurgitation in a 40-year-old man who underwent succesfull operations as mitral valve replacement, Maze-IV radiofrequency ablation, right atrium atrioplasty and De Vega anuloplasty. [J Contemp Med 2014; 4(2.000: 98-102

  18. Case report 387: Gaucher disease affecting the skeleton (left femur)

    Energy Technology Data Exchange (ETDEWEB)

    Tabas, J.H.; Daffner, R.H.; Hartsock, R.J.; Blakley, J.B.

    1986-08-01

    A case is described of a non-Jewish (Italian) 49-year-old man who presented to the hospital with pain in the left hip. Radionuclide studies showed decreased tracer activity with /sup 99m/Tc MDP over a lytic area in the subtrochanteric region of the left femur. Increased activity, however, was present in the right temporal bone, low anterior rib cage and right tenth posterior rib. The presence of subendosteal sclerosis with some cortical thickening adjacent to the femoral lesion, suggested the possibility of malignant neoplasm, (e.g. chondrosarcoma). Biopsy of the bone marrow showed the presence of Gaucher disease. (orig./SHA).

  19. Sweet's Syndrome associated with Hodgkin's disease: case report

    OpenAIRE

    Miranda, Carolina Villela da Rocha; Filgueiras, Fernanda de Marca; Obadia, Daniel Lago; Gripp, Alexandre Carlos; Alves, Maria de Fátima Scotelaro Guimarães

    2011-01-01

    A síndrome de Sweet é enfermidade cutânea rara e de etiologia pouco esclarecida. Cerca de 20% dos casos são associados a neoplasias hematológicas, sendo raros os casos relacionados à doença de Hodgkin. Relata-se caso de paciente masculino de 57 anos que desenvolveu a síndrome concomitantemente à neoplasia. As doenças foram controladas com o tratamento específico.Sweet's syndrome is a rare cutaneous disease of unknown etiology. About 20% of the cases are associated with hematological neoplasms...

  20. Treatment of alzheimer disease with CT scans - a case report

    Energy Technology Data Exchange (ETDEWEB)

    Cuttler, J.M. [Cuttler & Associates Inc., Vaughan, Ontario (Canada); Moore, E.R. [Dow Chemical Co., Midland, MI (United States); Hosfeld, V.D. [MidMichigan Health, Midland, MI (United States); Nadolski, D.L. [Midland Internal Medicine Associates PC, Midland, MI (United States)

    2016-03-15

    Alzheimer disease (AD) primarily affects older adults. This neurodegenerative disorder is the most common cause of dementia and is a leading source of their morbidity and mortality. U.S. patient care costs are about 200 billion dollars and will more than double by 2040. This case report describes the remarkable improvement of an advanced AD patient in hospice, who received five CT scans of the brain, about 40 mGy each, over a period of three months. The mechanism appears to be radiation-induced up-regulation of the patient's adaptive protection systems against AD, which partially restored cognition, memory, speech, movement, and appetite. (author)

  1. Treatment of alzheimer disease with CT scans - a case report

    International Nuclear Information System (INIS)

    Cuttler, J.M.; Moore, E.R.; Hosfeld, V.D.; Nadolski, D.L.

    2016-01-01

    Alzheimer disease (AD) primarily affects older adults. This neurodegenerative disorder is the most common cause of dementia and is a leading source of their morbidity and mortality. U.S. patient care costs are about 200 billion dollars and will more than double by 2040. This case report describes the remarkable improvement of an advanced AD patient in hospice, who received five CT scans of the brain, about 40 mGy each, over a period of three months. The mechanism appears to be radiation-induced up-regulation of the patient's adaptive protection systems against AD, which partially restored cognition, memory, speech, movement, and appetite. (author)

  2. Hidradenitis Suppurativa and Crohn’s Disease: A Case Series

    LENUS (Irish Health Repository)

    Kirthi, S

    2017-09-01

    Hidradenitis Suppurativa (HS) is characterized by chronic recurrent abscesses, nodules and draining sinus tracts with scar formation. Cutaneous Crohn’s Disease (CD) may also present similarly. We wished to identify and describe an Irish cohort with combined HS and CD, with a view to a better recognition of clinical manifestations and understanding of the pathophysiology underlying these two overlapping conditions. Cases were identified using the HIPE Code at Tallaght Hospital from 1990-2014 and retrospective review was performed. Seven patients with both HS and CD were identified, 5(71%) female. The median age of diagnosis with both conditions was 37 years. In all cases, CD had preceded the diagnosis of HS. All patients smoked. Six had an increased BMI and 43% had additional autoimmune conditions. All patients required treatment with a TNF-alpha inhibitor for HS with 5 of 6 subjects having reduced frequency of flare ups and clinically less active HS on follow up

  3. Clinicopathological features in 102 cases of Hirschsprung disease

    International Nuclear Information System (INIS)

    Ziad, Fouzia; Katchy, Kenneth; Alexander, Susan; Al-Ramadan, Saleema; Kumar, S.

    2006-01-01

    Hirschsprung disease (HD) is a predominantly childhood disorder of intestinal motility with a multifactorial and polygenic etiology. The objective of this study was to document the clinical and pathological features of HD in Kuwait, which has an estimated consanguinity rate of 54%. We analyzed all rectal and colonic biopsies (n=268) for suspected HD identified from the records of Pathology Department of Al Sabah Hospital for the period between 1994 and 2004. One hundred and two patients (87 males and15 females) had histologically confirmed HD identified from the records. Fifty-eight (57%) were neonates (< 1 month of age), while 21% were more than 4 months old. The diagnosis was based on open biopsy in 11 cases and rectal biopsies in 91 cases. Nine patients with open biopsies presented as intestinal obstruction, necrotizing enterocolitis, or perforation. The extent of disease was unknown in 13 patients. There were 67 males and 3 females with short segment HD. Nine had long segment, two ultra-short segment, and eight total colonic aganglionosis (TCA). Five TCA cases involved the small intestine. A skip area was observed in two cases. Six patients had other anomalies. A positive family history for HD was established in three patients. Two of these were male siblings from consanguineous marriage and had Waardenburg syndrome. The study has highlighted an exceptionally strong male predominance of short segment and a relatively high frequency at (5.6%) of small intestinal involvement in HD in Kuwait. These data call for a more detailed epidemiological study with special emphasis on genetics. (author)

  4. Hip Joint Trevor Disease: Literature Review and a Case Report

    Directory of Open Access Journals (Sweden)

    Mohammad Hallaj Moghadam

    2018-01-01

    Full Text Available Trevor disease or dysplasia epiphysealis hemimelica (DEH is an extremely rare condition with incidence of about 1:1,000,000. Male to female ratio of reporting case is 3:1, and usually diagnosed between two and eight years old. It usually affects the medial portion of the joint, but lateral involvement is not uncommon. Hip-joint was affected in less than 4% of existing cases in the literature. It would be very important to precisely mange the hip involvement to prevent from further articular cartilage destruction in this very young age. We report an infant boy with isolated DEH of hip. We found a total of 271 cases of DEH that reported between 1926 and 2017.The most sites of involvement are ankle joint and around the knee. Our search reaches out to ten cases of hip involvement. Hip involvement needs a patient specified decision. We observed our patient for three years with a desirable hip joint function.

  5. Paget's disease of the vulva: A review of 89 cases

    Directory of Open Access Journals (Sweden)

    Cherry O. Onaiwu

    2017-02-01

    Full Text Available The purpose of this study was to retrospectively review the clinical characteristics and outcomes of a series of women with Paget's disease of the vulva. A retrospective review was performed of 89 women with Paget's disease of the vulva evaluated at a single institution between 1966 and 2010. Medical records were reviewed for demographic information, clinical data, pathologic findings, treatment modalities and outcomes. We found that the primary treatment was surgery for 74 (83.1% patients, with positive margins noted in 70.1% of cases. Five patients (5.6% underwent topical treatment with imiquimod and/or 5-fluorouracil, one patient (1.1% underwent laser ablation and treatment was unknown in 9 patients (10.1%. The majority of patients had multiple recurrences, with 18% having four or more recurrences. There were no significant differences in recurrence rates between patients who underwent surgery and those who did not. Furthermore, there was no association between positive margins following primary surgery and recurrence. Forty-one patients (46.1% were diagnosed with 53 synchronous or metachronous cancers. Seven patients (7.9% were found to have invasive vulvar cancer with 1 mm or more depth of invasion, but none of the patients died of Paget's disease or associated vulvar/vaginal cancer. Our findings suggest that the majority of patients with Paget's disease of the vulva develop multiple recurrences regardless of treatment modality or margin status. Alternatives to surgery are needed to better care for women with this disease.

  6. An Unexpected Case of Lyme Disease in a Soldier Serving in Northern Iraq

    Science.gov (United States)

    2010-05-01

    MILITARY MEDICINE, 175,5:367,2010 An Unexpected Case of Lyme Disease in a Soldier Serving in Northern Iraq CPT Jeremy B. Fisher, SP USA *; CPT...Christopher E. Curtis, MC USAt 188143 ABSTRACT Lyme disease is a tick-transmitted disease caused by the spirochete Borrelia burgdorferi. Cases have been...Turkey.3-S We report an unexpected case of Lyme disease from Iraq. CASE REPORT A 28-year-old active duty Army male, on a deployment to northern Iraq

  7. Magnetic resonance imaging in prostate disease. Review of 58 cases

    Energy Technology Data Exchange (ETDEWEB)

    Gevenois, P.A.; Van Regemorter, G.; Van Gansbeke, D.; Delcour, C.; Corbusier, A.; Struyven, J.

    1987-03-01

    Forty-eight patients with prostatic disease (benign prostatic hyperplasia (B.P.H.), carcinoma, cysts, myoma and prostatitis) and 10 normal volunteers underwent magnetic resonance imaging (M.R.I) of the prostate. The prostatic parenchyma was best evaluated by a T2-weighted spin-echo pulse sequence. The prostate in patients with B.P.H. often had an homogeneous or more rarely a nodular appearance on T2-weighted images. In most cases, a peripheral dark rim is observed. All prostate in patients with carcinoma had an heterogeneous appearance on T2-weighted images. While most of the prostatic carcinomas appeared hypointense relative to adjacent prostatic parenchyma, some of the neoplasms had a high or mixed-high and low signal. The myoma showed a low-signal nodule like carcinoma. The cyst appears as a liquid tumor. The prostatitis had an homogeneous bright signal. With the used methodology, MRI can differentiate prostatic diseases in many cases. Nevertheless the technique has to be optimalized to improve its accuracy.

  8. Magnetic resonance imaging in prostate disease. Review of 58 cases

    International Nuclear Information System (INIS)

    Gevenois, P.A.; Van Regemorter, G.; Van Gansbeke, D.; Delcour, C.; Corbusier, A.; Struyven, J.

    1987-01-01

    Forty-eight patients with prostatic disease (benign prostatic hyperplasia (B.P.H.), carcinoma, cysts, myoma and prostatitis) and 10 normal volunteers underwent magnetic resonance imaging (M.R.I) of the prostate. The prostatic parenchyma was best evaluated by a T2-weighted spin-echo pulse sequence. The prostate in patients with B.P.H. often had an homogeneous or more rarely a nodular appearance on T2-weighted images. In most cases, a peripheral dark rim is observed. All prostate in patients with carcinoma had an heterogeneous appearance on T2-weighted images. While most of the prostatic carcinomas appeared hypointense relative to adjacent prostatic parenchyma, some of the neoplasms had a high or mixed-high and low signal. The myoma showed a low-signal nodule like carcinoma. The cyst appears as a liquid tumor. The prostatitis had an homogeneous bright signal. With the used methodology, MRI can differentiate prostatic diseases in many cases. Nevertheless the technique has to be optimalized to improve its accuracy [fr

  9. Familial polycystic kidney disease in Nigeria: A report of two cases ...

    African Journals Online (AJOL)

    A case of familial polycystic kidney disease is reported. Although isolated cases of adult polycystic kidney disease have been reported in our environment, no case to our knowledge has been reported with a familial link. Polycystic kidney disease is said to be rare in Africans. Although it commonly terminates in chronic renal ...

  10. Treatment of Graves' disease with I131: Case Report

    International Nuclear Information System (INIS)

    Sanchez Povis, J.E.; Sandoval Okuma, J.C.; Contreras Carreno, S.E.; Cabello Morales, E.

    2006-01-01

    Objective: To describe the response and complications in children and teenagers suffering Graves' disease who received radioactive iodine therapy. Material and Methods: The clinical records of the patients attended at Paediatric Endocrinology Unit of Hospital Nacional Cayetano Heredia who received therapy with I 131 were reviewed. Demographic characteristics, thyroid weight, radioactive iodine uptake, I 131 dose and clinical and thyroid function evolution were registered. Results: We include thirteen patients: 1 male and 12 females. The mean thyroid weight obtained was 47.56 ± 10.70 gr. Initial calculated dose was 3.92 ± 0.95 mCi, with a total dose of 4.47 ± 1.66 mCi, and mean following period of 2.76 years. Ten patients received only one dose, 2 patients received two doses and one patient received three doses; all cases remitted. Patients who received only 1 dose showed disease remission at 13.13 weeks and mean remission period of the population was 24.62 weeks. Hypothyroidism prevalence up to six months from initiating treatment was 66.66%, and up to 257 weeks 83.33%. Conclusions: We conclude that I 131 was 100% effective in the treatment of children and adolescents suffering Graves' disease in this small series. (author)

  11. Orbital lymphoma associated with Graves’ disease: A case report

    Directory of Open Access Journals (Sweden)

    Hajduković Zoran

    2014-01-01

    Full Text Available Introduction. The presence of bilateral exophthalmos and palpebral, periorbital edema associated with hyperthyroidism is most often considered as an initial sign of Graves’ ophthalmopathy. However, in up to 20% of cases, Graves’ ophthalmopathy might precede the occurrence of hyperthyroidism, which is very important to be considered in the differential diagnosis, especially if it is stated as unilateral. Among other less common causes of non-thyroid-related orbitopathy, orbital lymphoma represents rare conditions. We presented of a patient with Graves’ disease, initially manifested as bilateral orbitopathy and progressive unilateral exophthalmos caused by the marginal zone B-cell non-Hodgkin lymphoma of the orbit. Case report. A 64-yearold man with the 3-year history of bilateral Graves’ orbitopathy and hyperthyroidism underwent the left orbital decompression surgery due to the predominantly left, unilateral worsening of exophthalmos resistant to the previously applied glucocorticoid therapy. A year after the surgical treatment, a substantial exophthalmos of the left eye was again observed, signifying that other non-thyroid pathology could be involved. Orbital ultrasound was suggestive of primary orbital lymphoma, what was confirmed by orbital CT scan and the biopsy of the tumor tissue. Detailed examinations indicated that the marginal zone B-cell non-Hodgkin lymphoma extended to IV - B-b CS, IPI 3 (bone marrow infiltration: m+ orbit+. Upon the completion of the polychemiotherapy and the radiation treatment, a complete remission of the disease was achieved. Conclusion. Even when elements clearly indicate the presence of thyroid-related ophthalmopathy, disease deteriorating should raise a suspicion and always lead to imaging procedures to exclude malignancy.

  12. Creutzfeldt-Jakob Disease: Analysis of Four Cases

    Directory of Open Access Journals (Sweden)

    Ali Al Balushi

    2016-08-01

    Full Text Available Background: Creutzfeldt-Jakob disease (CJD is a rare, rapidly progressive neurodegenerative disease that almost always results in death in under a year from onset of symptoms. Here, we report four cases of CJD with different clinical presentations diagnosed at our institution over two-year period. Cases: The first patient is an 82-year-old woman who presented with depression, cognitive decline and word-finding difficulty over 4 weeks. The patient deteriorated neurologically to akinetic mutism and death within 6 weeks of presentation. The second patient is a 54-year-old woman with liver cirrhosis who presented with confusion, ataxia and multiple falls over 4 weeks. She was treated initially for hepatic encephalopathy, but continued to progress to mutism, startle myoclonus and obtundation. Death occurred within 4 weeks of presentation. The third patient is a 58-year-old woman who presented with an 8-week history of confusion, urinary incontinence, Parkinsonism, ataxia and myoclonus. Death occurred within 2 months from presentation. The fourth patient is a 67-year-old man who presented with a 6-week history of headache, blurred vision, ataxia and personality change and progressed to confusion, myoclonus, akinetic mutism and obtundation. Death occurred within 3 weeks from presentation. Conclusion: These 4 cases highlight the varied possible clinical presentations of CJD and demonstrate the importance of considering CJD in patients with atypical presentations of rapidly progressive cognitive decline. To diagnose CJD, brain biopsy remains the gold standard. However, the presence of CSF protein 14-3-3, typical MRI findings and suggestive EEG abnormalities all support the diagnosis.

  13. Anton's syndrome due to cerebrovascular disease: a case report

    Directory of Open Access Journals (Sweden)

    Maddula Mohana

    2009-09-01

    Full Text Available Abstract Introduction Anton's syndrome describes the condition in which patients deny their blindness despite objective evidence of visual loss, and moreover confabulate to support their stance. It is a rare extension of cortical blindness in which, in addition to the injury to the occipital cortex, other cortical centres are also affected, with patients typically behaving as if they were sighted. Case presentation We present a case report of an 83-year-old white woman with cortical blindness as a result of bilateral occipital lobe infarcts. Despite her obvious blindness, illustrated by her walking into objects, the patient expressed denial of visual loss and demonstrated confabulation in her accounts of her surroundings, consistent with a diagnosis of Anton's syndrome. Conclusions A suspicion of cortical blindness and Anton's syndrome should be considered in patients with atypical visual loss and evidence of occipital lobe injury. Cerebrovascular disease is the most common cause of Anton's syndrome, as in our patient. However, any condition that may result in cortical blindness can potentially lead to Anton's syndrome. Recovery of visual function will depend on the underlying aetiology, with cases due to occipital lobe infarction after cerebrovascular events being less likely to result in complete recovery. Management in these circumstances should accordingly focus on secondary prevention and rehabilitation.

  14. A CASE OF RENAL DISEASE IN HIV INFECTED PATIENT

    Directory of Open Access Journals (Sweden)

    Ni Made Vina Septiani

    2013-11-01

    Full Text Available Normal 0 false false false EN-US X-NONE X-NONE MicrosoftInternetExplorer4 Kidney diseases in human immunodeficiency virus (HIV infected patients has been been fourth leading cause of death after sepsis, pneumonia, and liver disease. HIV-associated nephropathy (HIVAN is the most common. We report a case, a male patient, 48 years, who experienced shortness of breath, cough and intermittent fever and has been reported as HIV positive, without previous antiretroviral treatment and last CD4+ count is 89 cells/mm3. There are elevated BUN and SC from day to day during treatment and proteinuria +2 as a sign of kidney disease with normal blood pressure and there was no edema. Patients given an antibiotic and ACE inhibitors as antiproteinuria. Patients with suspicion of HIVAN in this case can progress very rapidly and causes progressive decline in renal function. Prognosis of patients with HIVAN if not handled properly will develop end stage renal disease (ESRD in 1-4 months and had a mortality rate 4.7 times higher than HIV patients without renal impairment. /* Style Definitions */ table.MsoNormalTable {mso-style-name:"Table Normal"; mso-tstyle-rowband-size:0; mso-tstyle-colband-size:0; mso-style-noshow:yes; mso-style-priority:99; mso-style-qformat:yes; mso-style-parent:""; mso-padding-alt:0in 5.4pt 0in 5.4pt; mso-para-margin:0in; mso-para-margin-bottom:.0001pt; mso-pagination:widow-orphan; font-size:11.0pt; font-family:"Calibri","sans-serif"; mso-ascii-font-family:Calibri; mso-ascii-theme-font:minor-latin; mso-fareast-font-family:"Times New Roman"; mso-fareast-theme-font:minor-fareast; mso-hansi-font-family:Calibri; mso-hansi-theme-font:minor-latin; mso-bidi-font-family:"Times New Roman"; mso-bidi-theme-font:minor-bidi;}

  15. Combined Creutzfeldt-Jakob/ Alzheimer's Disease Cases are Important in Search for Microbes in Alzheimer's Disease.

    Science.gov (United States)

    Bastian, Frank O

    2017-01-01

    The question whether Alzheimer's disease is infectious as brought up in the recent editorial published in the Journal of Alzheimer's Disease is complicated by the controversy whether the causal agent is a microbe or a misfolded host protein (amyloid). The replicating amyloid (prion) theory, based upon data from studies of Creutzfeldt-Jakob disease (CJD) and other transmissible spongiform encephalopathies (TSEs), has been challenged since the prion can be separated from TSE infectivity, and spiroplasma, a wall-less bacterium, has been shown to be involved in the pathogenesis of CJD. Further support for a microbial cause for AD comes from occurrence of mixed CJD/AD cases involving up to 15% of AD brains submitted to brain banks. The association of CJD with AD suggests a common etiology rather than simply being a medical curiosity. A co-infection with the transmissible agent of CJD, which we propose to be a Spiroplasma sp., would explain the diversity of bacteria shown to be associated with cases of AD.

  16. Endometrial carcinoma occuring from polycystic ovary disease : A case report

    International Nuclear Information System (INIS)

    Seong, Su Ok; Jeon, Woo Ki

    1996-01-01

    Endometrial carcinoma usually occurs in postmenopausal women ; less than 5% occurs in women under the age of 40. Up to one quarter of endometrial carcinoma patients below this age have PCO(polycystic ovary disease, Stein-Leventhal syndrome). The increased incidence of endometrial carcinoma in patients with PCO is related to chronic estrogenic stimulation. We report MR imaging in one case of endometrial carcinoma occuring in a 23 year old woman with PCO and had complained of hypermenorrhea for about three years. On T2-weighted MR image the endometrial cavity was seen to be distended with protruded endometrial masses of intermediate signal intensity, and the junctional zone was disrupted beneath the masses. Both ovaries were best seen on T2-weighted MR imaging and showed multiple small peripheral cysts and low signal-intensity central stroma

  17. Metachronous Paget's disease of the breast: case report.

    Science.gov (United States)

    Gubitosi, A; Moccia, G; Malinconico, F A; Iside, G; Gilio, F; Cognetti, C; Foroni, F; Docimo, G; Ruggiero, R; Docimo, L; Agresti, M

    2009-04-01

    Paget breast disease is a kind of intraductal carcinoma that through an intracanalicular diffusion invades the basal epidermal layer, reaching the areola and nipple, producing a typical erythematous desquamative eczematous-like lesion. This neoplasia can remain undetected for a long time and inadequately treated as a dermatological affection. Synchronous or metachronous lesions are very uncommon. Surgical choice is conditioned by the presence of a tumor below the epidermal lesion, by its dimensions, and by the possible lymph node involvement. Surgical therapy can be radical or conservative. From our experience we think that lesion biopsy is always necessary to formulate a correct diagnosis and to schedule an appropriate therapeutic approach. In our case, a biopsy was performed first, then on the basis of the frozen section analysis a radical mastectomy with axillary third level lymph nodes dissection, because of the large dimensions of the lesion and the previous history of a methachronous lesion.

  18. Endometrial carcinoma occuring from polycystic ovary disease : A case report

    Energy Technology Data Exchange (ETDEWEB)

    Seong, Su Ok; Jeon, Woo Ki [Inje Univ. College of Medicine, Seoul (Korea, Republic of)

    1996-12-01

    Endometrial carcinoma usually occurs in postmenopausal women ; less than 5% occurs in women under the age of 40. Up to one quarter of endometrial carcinoma patients below this age have PCO(polycystic ovary disease, Stein-Leventhal syndrome). The increased incidence of endometrial carcinoma in patients with PCO is related to chronic estrogenic stimulation. We report MR imaging in one case of endometrial carcinoma occuring in a 23 year old woman with PCO and had complained of hypermenorrhea for about three years. On T2-weighted MR image the endometrial cavity was seen to be distended with protruded endometrial masses of intermediate signal intensity, and the junctional zone was disrupted beneath the masses. Both ovaries were best seen on T2-weighted MR imaging and showed multiple small peripheral cysts and low signal-intensity central stroma.

  19. Dysprosody nonassociated with neurological diseases--a case report.

    Science.gov (United States)

    Pinto, José Antonio; Corso, Renato José; Guilherme, Ana Cláudia Rocha; Pinho, Sílvia Rebelo; Nóbrega, Monica de Oliveira

    2004-03-01

    Dysprosody also known as pseudo-foreign dialect, is the rarest neurological speech disorder. It is characterized by alterations in intensity, in the timing of utterance segments, and in rhythm, cadency, and intonation of words. The terms refers to changes as to duration, fundamental frequency, and intensity of tonic and atonic syllables of the sentences spoken, which deprive an individual's particular speech of its characteristics. The cause of this disease is usually associated with neurological pathologies such as brain vascular accidents, cranioencephalic traumatisms, and brain tumors. The authors report a case of dysprosody attended to at the Núcleo de Otorrinolaringologia e Cirurgia de Cabeça e Pescoço de São Paulo (NOSP). It is about a female patient with bilateral III degree Reinke's edema and normal neurological examinations that started presenting characteristics of the German dialect following a larynx microsurgery.

  20. Addison's Disease in Evolution: An Illustrative Case and Literature Review.

    Science.gov (United States)

    Hinz, Laura E; Kline, Gregory A; Dias, Valerian C

    2014-09-01

    To present a case of symptomatic autoimmune adrenal insufficiency with initially normal serum cortisol and to caution about limitations of the current diagnostic algorithm for adrenal insufficiency, which does not reflect the pathophysiology of early disease. We describe the clinical presentation and relevant investigations of a patient ultimately found to have Addison's disease, which is followed by a focused review of the literature. A 41-year-old Caucasian woman with autoimmune hypothyroidism, premature ovarian failure, and microscopic colitis presented with nausea, salt craving, increased skin pigmentation, and postural hypotension. Initial bloodwork revealed a normal morning cortisol of level of 19.2 μg/dL (normal, 7.2 to 25 μg/dL) but an adrenocorticotropic hormone (ACTH) level 10 times normal, at 513.6 pg/mL (normal, <52.5 pg/mL). Her potassium was normal, but her aldosterone level was 4.12 ng/dL (normal, 12.3 to 62.5 ng/dL) and her renin activity was increased (23.0 mg/dL/hour; normal, <6.0 mg/dL/hour). Six weeks after initial presentation, she was found to have anti-adrenal antibodies. It was not until 10 weeks after her initial symptomatic presentation that her morning cortisol level was found to be subnormal and a formal diagnosis of adrenal insufficiency was made. The present case and literature review reveal that common diagnostic approaches will miss patients with (possibly symptomatic) early adrenal insufficiency. We suggest that serum ACTH level testing or tests of mineralocorticoid function be included in the initial step of investigation for suspected primary adrenal insufficiency.

  1. Complications in autopsy cases of Hashimoto's disease with special reference to A-bomb exposure

    International Nuclear Information System (INIS)

    Asano, Masahide; Kato, Hiroo

    1978-01-01

    To clarify a relationship between A-bomb exposure and Hashimoto's disease and that between Hashimoto's disease and carcinoma of the thyroid gland, autopsy cases of Hashimoto's disease (112 cases in Hiroshima and 43 cases in Nagasaki) were examined. Incidence of Hashimoto's disease was not related to exposure doses and ages at the time of exposure. Incidence of carcinoma of the thyroid gland from Hashimoto's disease was 1.3% (2 cases), and there was no relationship between them. Incidence of ovarian cancer as cancer accompanied with Hashimoto's disease was significantly high, but that of stomach cancer was significantly low. Incidence of total cancer from Hashimoto's disease was also significantly low. Incidences of rheumatic fever and rheumatoid arthritis which were collagen diseases and diseases similar to them complicated by Hashimoto's disease was significantly high. (Tsunoda, M.)

  2. Genetic Engineering and Sustainable Crop Disease Management: Opportunities for Case-by-Case Decision-Making

    Directory of Open Access Journals (Sweden)

    Paul Vincelli

    2016-05-01

    Full Text Available Genetic engineering (GE offers an expanding array of strategies for enhancing disease resistance of crop plants in sustainable ways, including the potential for reduced pesticide usage. Certain GE applications involve transgenesis, in some cases creating a metabolic pathway novel to the GE crop. In other cases, only cisgenessis is employed. In yet other cases, engineered genetic changes can be so minimal as to be indistinguishable from natural mutations. Thus, GE crops vary substantially and should be evaluated for risks, benefits, and social considerations on a case-by-case basis. Deployment of GE traits should be with an eye towards long-term sustainability; several options are discussed. Selected risks and concerns of GE are also considered, along with genome editing, a technology that greatly expands the capacity of molecular biologists to make more precise and targeted genetic edits. While GE is merely a suite of tools to supplement other breeding techniques, if wisely used, certain GE tools and applications can contribute to sustainability goals.

  3. Hydatid disease of the spine: A rare case

    Directory of Open Access Journals (Sweden)

    Mona Agnihotri

    2017-01-01

    Full Text Available Hydatid disease or hydatidosis is the most widespread zoonosis caused by Echinococcus granulosus. Liver and lungs are the most common sites. Bone involvement is rare and reported in 0.5%–4% with spinal involvement reported in 50% of these cases. We present a case of spinal hydatidosis in a 35-year-old male presenting with lower extremity weakness and numbness. Magnetic resonance imaging (MRI of the spine showed multiple cystic lesions at the T9–T11 level with involvement of the paraspinal muscles. The lesion was seen intraspinal, intradural, intramedullary, and epidural. Radiological impression was aneurysmal bone cyst. The patient underwent laminectomy, and the excised cysts showed characteristic features of hydatid cyst (HC on histopathology. The patient was started on antihelminthic therapy postoperatively. MRI is a diagnostic modality for HC, but the unusual location and absence of characteristic features can cause diagnostic difficulty. A high index of suspicion should be kept in patients residing in endemic areas and presenting with unusual cystic lesion of spine.

  4. Morgellons disease, illuminating an undefined illness: a case series

    Directory of Open Access Journals (Sweden)

    Harvey William T

    2009-07-01

    Full Text Available Abstract Introduction This review of 25 consecutive patients with Morgellons disease (MD was undertaken for two primary and extremely fundamental reasons. For semantic accuracy, there is only one "proven" MD patient: the child first given that label. The remainder of inclusive individuals adopted the label based on related descriptions from 1544 through 1884, an internet description quoted from Sir Thomas Browne (1674, or was given the label by practitioners using similar sources. Until now, there has been no formal characterization of MD from detailed examination of all body systems. Our second purpose was to differentiate MD from Delusions of Parasitosis (DP, another "informal" label that fit most of our MD patients. How we defined and how we treated these patients depended literally on factual data that would determine outcome. How they were labeled in one sense was irrelevant, except for the confusing conflict rampant in the medical community, possibly significantly skewing treatment outcomes. Case presentation Clinical information was collected from 25 of 30 consecutive self-defined patients with Morgellons disease consisting of laboratory data, medical history and physical examination findings. Abnormalities were quantified and grouped by system, then compared and summarized, but the numbers were too small for more complex mathematical analysis. The quantification of physical and laboratory abnormalities allowed at least the creation of a practical clinical boundary, separating probable Morgellons from non-Morgellons patients. All the 25 patients studied meet the most commonly used DP definitions. Conclusions These data suggest Morgellons disease can be characterized as a physical human illness with an often-related delusional component in adults. All medical histories support that behavioral aberrancies onset only after physical symptoms. The identified abnormalities include both immune deficiency and chronic inflammatory markers that

  5. The association between periodontal disease and chronic obstructive pulmonary disease: a case control study.

    Science.gov (United States)

    Öztekin, Görkem; Baser, Ulku; Kucukcoskun, Meric; Tanrikulu-Kucuk, Sevda; Ademoglu, Evin; Isik, Gulden; Ozkan, Gulcihan; Yalcin, Funda; Kiyan, Esen

    2014-08-01

    Although there are studies evaluating the effects of periodontal health on chronic obstructive pulmonary disease (COPD), the effects of COPD - a systemic disease, on periodontal tissue is unknown. The aim of this study is to evaluate the effects of COPD on periodontal tissues by comparing COPD patients and controls. Fifty-two COPD patients and 38 non-COPD controls were included in this case-control study. Number of teeth, plaque index (PI), gingival index (GI), bleeding on probing, clinical attachment level and probing depth were included in the periodontal examination. In addition to clinical evaluations, gingival crevicular fluid (GCF) levels of high-sensitive C-reactive protein (hs-CRP), interleukin-1 beta (IL-lb) and prostaglandin-E2 (PGE2), and serum hs-CRP levels were measured in COPD patients and the controls. The number of teeth was significantly lower while PI and GI were significantly higher in COPD patients when compared to the controls. As well as serum hs-CRP levels, the GCF levels of hs-CRP, IL-1b and PGE2 were significantly higher in COPD patients than the controls. Our results demonstrated that COPD may be associated with periodontal disease as manifested by lower number of teeth and higher levels of inflammatory mediators especially CRP in GCF. This finding may be a reflection of systemic effects of COPD on periodontal tissues. Poor oral health behavior of COPD patients have to be considered in larger size group studies in the future.

  6. Von Willebrand's disease: case report and review of literature.

    Science.gov (United States)

    Echahdi, Hanae; El Hasbaoui, Brahim; El Khorassani, Mohamed; Agadr, Aomar; Khattab, Mohamed

    2017-01-01

    Von Willebrand Disease (VWD) is the most common human inherited bleeding disorder due to a defect of Von Willebrand Factor (VWF), which a glycoprotein crucial for platelet adhesion to the subendothelium after vascular injury. VWD include quantitative defects of VWF, either partial (type 1 with VWF levels < 50 IU/dL) or virtually total (type 3 with undetectable VWF levels) and also qualitative defects of VWF (type 2 variants with discrepant antigenic and functional VWF levels). The most bleeding forms of VWD usually do not concern type 1 patients with the mildest VWF defects (VWF levels between 30 and 50IU/dL). Von willebrand factor is a complex multimeric protein with two functions: it forms a bridge between the platelets and areas of vascular damage and it binds to and stabilizes factor VIII, which is necessary for the clotting cascade. By taking a clinical history of bleeding (mucocutaneous bleeding symptoms suggestive of a primary haemostatic disorder, a quantitative or qualitative abnormality of VWF is possible) it is important to think about VWD and to make the appropriate diagnosis. If the VWD is suspected diagnostic tests should include an activated partial thromboplastin time, bleeding time, factor VIII: C Ristocetin cofactor and vWF antigen. Additional testing of ristocetin induced plattlet adhesion (RIPA) the multimeric structure and collagen binding test and genanalysis allow diagnosing the different types of von. Willebrand Disease. The treatment of choice in mild forms is the synthetic agent desmopressin. In patients with severe type 1, type 2B, 2N and type 3 or in people who do not response to desmopressin, the appropriate treatment is a factor VIII concentrate that is rich of VWF. We report a case of infant in 27-month-old boy who had been referred due to haemorrhagic shock. His birth histories, his familie's social history and developmental milestones were unremarkable. He was born at full term with no antenatal or perinatal complications. Prior to

  7. Pre-admission antibiotics for suspected cases of meningococcal disease.

    Science.gov (United States)

    Sudarsanam, Thambu D; Rupali, Priscilla; Tharyan, Prathap; Abraham, Ooriapadickal Cherian; Thomas, Kurien

    2017-06-14

    2.15; N = 477; 18 clinical failures; moderate-quality evidence), or neurological sequelae (RR 1.29, 95% CI 0.63 to 2.62; N = 477; 29 with sequelae; low-quality evidence). No adverse effects of treatment were reported. Estimated treatment costs were similar. No data were available on disease burden due to sequelae. We found no reliable evidence to support the use pre-admission antibiotics for suspected cases of non-severe meningococcal disease. Moderate-quality evidence from one RCT indicated that single intramuscular injections of ceftriaxone and long-acting chloramphenicol were equally effective, safe, and economical in reducing serious outcomes. The choice between these antibiotics should be based on affordability, availability, and patterns of antibiotic resistance.Further RCTs comparing different pre-admission antibiotics, accompanied by intensive supportive measures, are ethically justified in people with less severe illness, and are needed to provide reliable evidence in different clinical settings.

  8. Pott's disease: a case of Mycobacterium xenopi infection of the spine.

    Science.gov (United States)

    Alfreijat, Majd; Ononiwu, Chiagozie; Sexton, Carlton

    2012-01-01

    Pott's disease is an infection of the spine with Mycobacterium tuberculosis that causes destruction of the spine elements resulting in progressive kyphosis. We are describing a rare case of Pott's disease where Mycobacterium xenopi was the inculpated organism.

  9. Addison’s Disease Mimicking as Acute Pancreatitis: A Case Report

    Science.gov (United States)

    Chaudhuri, Sayani; Rao, Karthik N; Ommurugan, Balaji; Varghese, George

    2017-01-01

    Over past two decades there has been significant improvement in medical field in elucidating the underlying pathophysiology and genetics of Addison’s disease. Adrenal insufficiency (Addison’s disease) is a rare disease with an incidence of 0.8/100,000 cases. The diagnosis may be delayed if the clinical presentation mimics a gastrointestinal disorder or psychiatric illness. We report a case of Addison’s disease presenting as acute pain in abdomen mimicking clinical presentation of acute pancreatitis. PMID:28571196

  10. First case of Mycobacterium heckeshornense cavitary lung disease in the Latin America and Caribbean region

    NARCIS (Netherlands)

    Coitinho, C.; Greif, G.; Ingen, J. van; Laserra, P.; Robello, C.; Rivas, C.

    2016-01-01

    A case of cavitary pulmonary disease caused by Mycobacterium heckeshornense in Uruguay is described. This is the first case reported in the Latin America and Caribbean region, showing that this species is a worldwide opportunistic human pathogen.

  11. Coats' disease in Tanzania: first case report and literature review ...

    African Journals Online (AJOL)

    Background: Coats' disease is an exudative retinal detachment with vascular telangiectasis occurring mostly in male children, the age group most affected by retinoblastoma. Objectives: To compare the differential diagnoses of Coats' disease. To establish recommendation to early disease detection. Materials and Methods:

  12. Gaucher's disease: Report of 11 cases with review of literature ...

    African Journals Online (AJOL)

    Gaucher's disease (GD) is a lysosomal storage disorder due to glucocerebrosidase deficiency; it's one of the rare genetic diseases for which therapy is now available. The purpose of this work is to study the epidemiological features of the disease and to highlight the diagnostic difficulties. We performed an 11-year ...

  13. A suspected case of Addison's disease in cattle.

    Science.gov (United States)

    Lambacher, Bianca; Wittek, Thomas

    2015-09-01

    A 4.75-year old Simmental cow was presented with symptoms of colic and ileus. The clinical signs and blood analysis resulted in the diagnosis of suspected primary hypoadrenocorticism (Addison's disease). Although Addison's disease has been frequently described in other domestic mammals, to our knowledge, this disease has not previously been reported in cattle.

  14. [Etiological analysis of 264 cases with chronic kidney disease stage 2 to 5 in children].

    Science.gov (United States)

    Miao, Qianfan; Shen, Qian; Xu, Hong; Sun, Li; Tang, Xiaoshan; Fang, Xiaoyan; Liu, Haimei; Zhai, Yihui; Bi, Yunli; Wang, Xiang; Chen, Hong

    2015-09-01

    To study and summarize the etiology of children patients with chronic kidney disease (CKD) stage 2 to 5 seen in Children's Hospital of Fudan University from Jan. 2004 to Dec. 2013. By complying with the NKF-K/DOQI guidelines, we collected data of 264 cases of children patients with CKD stage 2-5 from Jan. 2004 to Dec. 2013 in the medical record system of Children's Hospital of Fudan University. And we retrospectively analyzed their age and CKD stage at first diagnosis, primary diseases, complications, etc. In the collected 264 cases, 52 cases (19.7%) were diagnosed at stage 2, 67 (25.4%) at stage 3, 52 (19.7%) at stage 4 and 93 (35.2%) at stage 5. For disease causes, 116 cases (43.9%) had congenital anomalies of the kidney and urinary tract (CAKUT), 61 cases (23.1%) had glomerular disease, 15 (5.7%) had hereditary kidney disease, 14 (5.3%) had other diseases and in 58 cases (22.0%) the causes of disease were unknown. In the group with age between 0 and 3.0 and 3.1 and 6.0 years, 57.1% (24 cases) and 60.0% (30 cases) had primary disease with CAKUT. In the group with age older than 10 years, 49.2% (30 cases) had primary disease with glomerular disease and 32.0% (32 cases) with unknown causes. The major cause of CKD stage 2-5 in children in our hospital during the last ten years was CAKUT (43.9%), followed by glomerular disease (23.1%). The primary diseases of CKD were significantly different between the 2 age groups. CAKUT was more common in infants and preschool children while for adolescents, glomerular disease was the major cause.

  15. Progressive diaphyseal dysplasia (Engelmann's disease) - Report of a case -

    International Nuclear Information System (INIS)

    Soh, M. H.; Rhee, S. J.; Won, J. J.

    1981-01-01

    Progressive diaphyseal dysplasia is a rare condition and radiographic finding provides conclusive proof. We have experienced a sporadic case of progressive disphyseal dysplasia (Engelmann's disease) of 8 year-old-Korean girl, confirmed by radiographic skeletal survey and biopsy. This patient was admitted to the Jeonbug National University Hospital because of painful swelling of the distal part of the right femur after trauma and intermittent pain in her lower legs with a peculiar wadding gait for 2 years. On a physical examination, the patient appeared thin and slender. The skeletal musculature was poorly developed and the upper and lower extremities were weak. She walked with a peculiar wadding gait. The height was normal. No joint abnormality was noted and the mental state was alert. The child was the product of a normal gestation and delivery. Radiographic studies of the skeleton showed a generalized and symmetrical distribution of the bone characterized by cortical thickening, fusiform enlargement, and a narrowed medullary cavity in the diaphyseal of long bones while the epiphyses and metaphyses was not involved. Abrupt demarcation of the lesion with loss of normal trabecular pattern was note. Elongation of the extremities relative to the size of the child was present. The above radiographic findings showed involvement of all the long tubular bones such as the ulna, radius, tibia, fibula, femur and humerus. A sight sclerosis of the base of her skull was present, but the calvarium was not involved. The hands, feet, pelvis, spine, clavicle, rib, scapula and mandible were not affected. There was no specific laboratory finding except for the slightly elevated ESR. Histological examination of the bone biopsies from the femur revealed thickening of periosteum and proliferation of the walls of the small arterioles with reduction in the size of the lumen. The bony cortex showed essentially normal bone with the increased osteoblastic and osteoclastic activity

  16. An advanced case of indium lung disease with progressive emphysema.

    Science.gov (United States)

    Nakano, Makiko; Tanaka, Akiyo; Hirata, Miyuki; Kumazoe, Hiroyuki; Wakamatsu, Kentaro; Kamada, Dan; Omae, Kazuyuki

    2016-09-30

    To report the occurrence of an advanced case of indium lung disease with severely progressive emphysema in an indium-exposed worker. A healthy 42-year-old male smoker was employed to primarily grind indium-tin oxide (ITO) target plates, exposing him to indium for 9 years (1998-2008). In 2004, an epidemiological study was conducted on indium-exposed workers at the factory in which he worked. The subject's serum indium concentration (In-S) was 99.7 μg/l, while his serum Krebs von den Lungen-6 level was 2,350 U/ml. Pulmonary function tests showed forced vital capacity (FVC) of 4.17 l (91.5% of the JRS predicted value), forced expiratory volume in 1 s (FEV 1 ) of 3.19 l (80.8% of predicted), and an FEV 1 -to-FVC ratio of 76.5%. A high-resolution chest computed tomography (HRCT) scan showed mild interlobular septal thickening and mild emphysematous changes. In 2008, he was transferred from the ITO grinding workplace to an inspection work section, where indium concentrations in total dusts had a range of 0.001-0.002 mg/m 3 . In 2009, the subject's In-S had increased to 132.1 μg/l, and pulmonary function tests revealed obstructive changes. In addition, HRCT scan showed clear evidence of progressive lung destruction with accompanying severe centrilobular emphysema and interlobular septal thickening in both lung fields. The subject's condition gradually worsened, and in 2015, he was registered with the Japan Organ Transplant Network for lung transplantation (LTx). Heavy indium exposure is a risk factor for emphysema, which can lead to a severity level that requires LTx as the final therapeutic option.

  17. PRIMARY GIANT HYDATID DISEASE OF THE SPLEEN: A RARE CASE REPORT WITH REVIEW OF LITERATURE

    Directory of Open Access Journals (Sweden)

    Subramanyam

    2015-02-01

    Full Text Available The most common organ involved in hydatid disease is the liver, followed by the lungs. Hydatid disease of spleen is a rare clinical condition, as even in the endemic region the frequency is reported to be 0.5 – 4% of abdominal hydatid diseases. Most commonly splenic involvement is secondary i.e., along with other organs. Primary hydatid diseases in s pleen is rare, here we are reporting a rare case of primary splenic hydatid disease

  18. Spinal cord disease in children with malignancies: Clinical cases ...

    African Journals Online (AJOL)

    Four cases of children with malignancies and spinal cord pathology are presented. Current knowledge of this ... literature review. Case presentations .... The CD4 percentage of lymphocytes was only 6.9%, raising questions about antiretroviral ...

  19. Case report: a case of intractable Meniere's disease treated with autogenic training

    Directory of Open Access Journals (Sweden)

    Nakai Kimiko

    2008-01-01

    Full Text Available Abstract Background Psychological stress plays an important role in the onset and course of Meniere's disease. Surgical therapy and intratympanic gentamicin treatment are options for cases that are intractable to conventional medical therapy. Psychotherapy, however, including autogenic training (AT, which can be used for general relaxation, is not widely accepted. This paper describes the successful administration of AT in a subject suffering from intractable Meniere's disease. Case presentation A 51-year-old male patient has suffered from fluctuating right sensorineural hearing loss with vertigo since 1994. In May 2002, he was first admitted to our hospital due to a severe vertigo attack accompanied by right sensorineural hearing loss. Spontaneous nystagmus toward the right side was observed. Since April 2004, he has experienced vertigo spells with right-sided tinnitus a few times per month that are intractable to conventional medical therapy. After four months, tympanic tube insertion was preformed in the right tympanic membrane. Intratympanic injection of dexamethasone was ineffective. He refused Meniett therapy and intratympanic gentamicin injection. In addition to his vertigo spells, he suffered from insomnia, tinnitus, and anxiety. Tranquilizers such as benzodiazepines and antidepressants such as serotonin selective re-uptake inhibitors (SSRIs failed to stop the vertigo and only slightly improved his insomnia. In December 2006, the patient began psychological counseling with a psychotherapist. After brief psychological counseling along with cognitive behavior therapy (CBT, he began AT. He diligently and regularly continued his AT training in his home according to a written timetable. His insomnia, tinnitus, and vertigo spells disappeared within a few weeks after only four psychotherapy sessions. In order to master the six standard formulas of AT, he underwent two more sessions. Thereafter, he underwent follow-up for 9 months with no

  20. Is there an association between Fahr′s disease and cardiac conduction system disease?: A case report

    Directory of Open Access Journals (Sweden)

    Prashanth Panduranga

    2012-01-01

    Full Text Available Background: Fahr′s disease is a rare neurodegenerative disorder of unknown cause characterized by idiopathic basal ganglia calcification that is associated with neuropsychiatric and cognitive impairment. No case of Fahr′s disease with associated cardiac conduction disease has been described in the literature to date. The objective of this case report was to describe a young female with various cardiac conduction system abnormalities and bilateral basal ganglia calcifica-tion suggestive of Fahr′s disease. Case Report: A 19-year-old female was transferred to our hospital for a pacemaker insertion. Her past medical history included cognitive impairment and asymptomatic congenital complete heart block since birth. Her manifestations in-cluded cognitive impairment, tremors, rigidity, ataxia, bilateral basal ganglia calcification without clinical manifesta-tions of mitochondrial cytopathy. She also had right bundle branch block, left anterior fascicular block, intermittent complete heart block, atrial arrhythmias with advanced atrioventricular blocks and ventricular asystole manifested by Stokes-Adams seizures, which was diagnosed as epilepsy. Conclusions: According to our knowledge, this was the first case report of a su spected association between Fahr′s disease and isolated cardiac conduction system disease. In addition, this case illustrated that in patients with heart blocks and seizures, a diagnosis of epilepsy needs to be made with caution and such patients need further evaluations by a cardiologist or electrophysiologist to consider pacing and prevent future catastrophic events.

  1. Constructing disease-centric knowledge graphs : A case study for depression (short version)

    NARCIS (Netherlands)

    Huang, Zhisheng; Yang, Jie; van Harmelen, Frank; Hu, Qing

    2017-01-01

    In this paper we show how we used multiple large knowledge sources to construct a much smaller knowledge graph that is focussed on single disease (in our case major depression disorder). Such a disease-centric knowledge-graph makes it more convenient for doctors (in our case psychiatric doctors) to

  2. Menetrier's Disease: Case Report | Edino | Nigerian Journal of ...

    African Journals Online (AJOL)

    Cytomegalovirus and Helicobacter pylori infections, and transforming growth factor alpha , have been implicated in the aetiopathogenesis of the disease. Conservative treatment by aggressive eradication of H.pylori improves patients' symptoms, and abrogates the protein losing enteropathy characteristic of the disease.

  3. Case Report: CT and MRI findings in Lhermitte- Duclos disease ...

    African Journals Online (AJOL)

    ... lesion of uncertain origin. It is linked to an autosomal- dominant phakomatosis known as Cowden's disease in 40% of patients. The MRI features of LDD are almost unique and can be considered diagnostic. We report on a patient who presented with the typical MRI features of the above disease, and discuss the imaging ...

  4. Cortical involvement of marchiafava-bignami disease: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Jang, Han Won [Yeungnam University College of Medicine, Daegu (Korea, Republic of)

    2007-03-15

    Marchiafava-Bignami disease is a rare complication of chronic alcoholism and this malady typically manifests as callosal lesion. I report here on one patient with Marchiafava-bignami disease (MBD) who has symmetric restricted diffusion in both lateral-frontal cortices, in addition to the callosal lesion.

  5. [Trichoma (Plica polonica) - a contemporary case with a historical disease].

    Science.gov (United States)

    Wolf, Florian; Scherr, Martin; Scherthöffer, Dirk; Bäuml, Josef; Förstl, Hans

    2008-01-01

    We describe a 62-year-old patient with a chronic delusional disorder who presented with severely matted hair ("plica polonica"). Until the late 19th century such dreadlocks were considered as cause, consequence and treatment of mental disease. The historical development of "plica polonica" is briefly reviewed as an example of early and once popular psychiatric disease concepts.

  6. Smoking associated with malignancy, hypertension, chronic obstructive pulmonary disease and concurrent coronary artery disease: report of nine cases.

    Science.gov (United States)

    Dwivedi, S; Srivastava, S; Dwivedi, G

    2006-01-01

    Tobacco smoking in any form is a major risk factor for coronary artery disease (CAD), hypertension (HTN), chronic obstructive pulmonary disease (COPD), oral, nasopharyngeal, bronchial and other visceral malignancies. Cessation of smoking exerts considerable beneficial effect on development, recurrence and prognosis of these diseases. Present communication is based on the study of nine cases who had concurrent CAD, HTN, COPD and mitotic and/or pre malignant lesions due to unabated smoking. The youngest patient was a 35-year-old male having a smoking index of 300, presenting with acute coronary syndrome, COPD, HTN and buccal leukoplakia. Associated genitourinary malignancies in two cases and osteosarcoma in one case was an unusual presentation. These cases highlight the prognosis and public health implications of continuous smoking.

  7. Ultrasonographic findings of Weber-Christian disease of the breast: a case report

    International Nuclear Information System (INIS)

    Jang, Ji Youn; Chang, Yun Woo; Lee, Dong Wha

    2005-01-01

    Weber-Christian disease is a rare inflammatory disease of subcutaneous fat necrosis that may or may not involve systemic disease. We report the results of a breast sonograph of a case diagnosed as a form of an acute lobular panniculitis affecting the breast and upper limbs

  8. Disseminated hydatid disease presenting as fever of unknown origin: A case report and review of literature

    Directory of Open Access Journals (Sweden)

    Nikhil Gupta

    2015-01-01

    Full Text Available Human hydatid disease occurs due to infection with larval form of Echinococcus granulosus. The disseminated hydatid disease is a very rare finding. Disseminated hydatid disease presenting as a cause of fever of unknown origin is a rare phenomenon. We present to you such a rare case.

  9. 20 CFR 10.116 - What additional evidence is needed in cases based on occupational disease?

    Science.gov (United States)

    2010-04-01

    ... based on occupational disease? 10.116 Section 10.116 Employees' Benefits OFFICE OF WORKERS' COMPENSATION... of Proof § 10.116 What additional evidence is needed in cases based on occupational disease? (a) The... occupational diseases. The medical report should also include the information specified on the checklist for...

  10. Appendiceal immunoglobulin G4-related disease mimicking appendiceal tumor or appendicitis: A case report

    International Nuclear Information System (INIS)

    Kim, Hyun Soo; Kang, Won Kyung; Chung, Dong Jin

    2016-01-01

    Immunoglobulin G4 (IgG4)-related disease is an autoimmune disease that forms tumorous lesions. Several cases involving various organs are reported, however, IgG4-related disease involving appendix has not been reported yet. In this report, we presented a case of IgG4-related disease of appendix, which raised a suspicion of appendiceal tumor or usual appendicitis and, therefore, led to unnecessary surgical resection. IgG4-related disease should be considered in the differential diagnosis for a mass-like swelling of the appendix, in order to avoid unnecessary surgery

  11. Appendiceal immunoglobulin G4-related disease mimicking appendiceal tumor or appendicitis: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Hyun Soo; Kang, Won Kyung; Chung, Dong Jin [Yeouido St. Mary' s Hospital, The Catholic University of Korea, Seoul (Korea, Republic of)

    2016-02-15

    Immunoglobulin G4 (IgG4)-related disease is an autoimmune disease that forms tumorous lesions. Several cases involving various organs are reported, however, IgG4-related disease involving appendix has not been reported yet. In this report, we presented a case of IgG4-related disease of appendix, which raised a suspicion of appendiceal tumor or usual appendicitis and, therefore, led to unnecessary surgical resection. IgG4-related disease should be considered in the differential diagnosis for a mass-like swelling of the appendix, in order to avoid unnecessary surgery.

  12. Neuro-Behçet disease mimicking brain tumor: A case report

    Science.gov (United States)

    Tramontini, Pedro L.; Finkelsztejn, Alessandro; Duarte, Juliana Á.; Santos, Guilherme T.; Roesler, Rafael; Isolan, Gustavo R.

    2017-01-01

    Background: Behçet's disease (BD) is an inflammatory multisystem disease with unknown etiology, and consists of a TRIAD comprising recurrent oral ulcers, genital ulcers, and uveitis. In some cases, the disease affects the central nervous system, called Neuro-Behçet Disease (NBD). Few cases of NBD simulating a brain tumor have been previously reported. Case Description: Here, we describe the case of a 46-year-old male patient with a previous diagnosis of brain tumor who was later diagnosed for BD. Conclusion: This case highlights the importance of differential diagnosis of lesions with tumoral features. Checking for the possibility of NBD may help avoiding biopsy in these types of cases. PMID:28695044

  13. Isolated Primary Hydatid Disease of Omentum; Report of a Case and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Ali Ghafouri

    2010-09-01

    Full Text Available Hydatid disease, most commonly caused by the larval stage ofEchinococcus granulosus, affects mainly human liver andlung, and rarely other parts of the body. It is prevalent in mostsheep-raising Mediterranean Countries including Iran. Peritonealhydatid cyst, either primary or secondary, represents anuncommon but significant manifestation of the disease. Thepresent case report describes a case of primary isolated hydatiddisease of omentum, which to our knowledge constitutesthe first case of this kind in Iran.

  14. Localised prostate cancer and hemophilia A (AHA: Case report and management of the disease

    Directory of Open Access Journals (Sweden)

    Francesco Celestino

    2014-09-01

    Full Text Available Acquired Hemophilia A (AHA is a rare bleeding diathesis characterized by the development of autoantibodies against factor VIII (FVIII. About half of the cases are idiopathic and the other half are associated with autoimmune diseases, postpartum problems, infections, inflammatory bowel disease, drugs, lymphoproliferative disorders or solid tumors . AHA is associated with malignancies in 7-15% of cases. We report a case of AHA in a 65 year old patient with prostatic carcinoma, who underwent retropubic radical prostatectomy (RP.

  15. Our experience in eight cases with urinary hydatid disease: A series of 372 cases held in nine different clinics

    International Nuclear Information System (INIS)

    Yilmaz, Y.; Koesem, M; Ceylan, K.; Koeseoglu, B.; Yalcinkaya, I.; Arslan, H.; Guenes, M.; Soeylemez, Oe

    2006-01-01

    Hydatid disease, a parasitic infestation caused by the larval stage of the cestode Echinococcus granulosus, is diagnosed commonly in the east and south-east regions of Turkey. The aim of this study is to emphasize the relatively frequent occurrences of echinococcosis in our region, and to discuss therapeutic options and treatment results according to current literature. A retrospective 10-year review of nine different clinics' records of the Research Hospital of the Medical School of Yuezuencue Y 1 l University revealed 372 hydatid disease cases that were localized in various organs and treated surgically (271 cases) or drained percutaneously (99 cases). Hydatid disease was diagnosed by ultrasonography (US) and computed tomography scans (CT) and confirmed histopathologically. The involved organ was lung in 203 cases (131 adults, 72 children), liver in 150, spleen in 9, brain in 2, kidneys in 7 cases and the retrovesical area in 1 case. The urogenital system is involved at a rate of 2.15%. Two hundred and seventy-one cases were treated surgically and 99 percutaneously. Two cases with renal hydatid cyst refused the surgical procedure (one had a solitary kidney with hydatid cyst). Albendazole was administered to 192 patients; 93 patients had open surgical procedure and 99 patients underwent percutaneous procedure. Cysts were excised totally in the open surgical procedure; however, involved kidneys were removed totally (four cases) except one. Cystectomy and omentoplasty was performed in one case. Complications were as follows: in six cases, cystic material was spilled into the bronchial cavity during the dissection and a renal hydatid cyst ruptured and spilled retroperitoneally. Hydatid disease is a serious health problem in Turkey. The mainly affected organs are liver and lung. It can be treated surgical or by percutaneous aspiration. (author)

  16. Seasonal ataxia: a case report of a disappearing disease | Moyo ...

    African Journals Online (AJOL)

    Case report: We present the case of a 35 year old woman from western Nigeria who was admitted in October 2012 with acute onset of gait instability and bilateral hand tremors, preceded by several episodes of vomiting. She had ingested a meal containing roasted larvae of the African silkworm, 2 hours before the onset of ...

  17. Hodgkin's disease of the prostate: a detailed case report

    International Nuclear Information System (INIS)

    Klotz, L.H.; Herr, H.W.

    1986-01-01

    Malignant lymphoma of the prostate is an unusual entity, and nonHodgkin's lymphoma constitutes most reported cases. We report a well documented case of Hodgkin's lymphoma, initially involving the gastrointestinal tract and spleen, which recurred in the prostate following initial remission with chemotherapy. Treatment with external beam radiotherapy resulted in a rapid complete response that has been sustained for 18 months

  18. Case report: a case of intractable Meniere's disease treated with autogenic training.

    Science.gov (United States)

    Goto, Fumiyuki; Nakai, Kimiko; Kunihiro, Takanobu; Ogawa, Kaoru

    2008-01-25

    Psychological stress plays an important role in the onset and course of Meniere's disease. Surgical therapy and intratympanic gentamicin treatment are options for cases that are intractable to conventional medical therapy. Psychotherapy, however, including autogenic training (AT), which can be used for general relaxation, is not widely accepted. This paper describes the successful administration of AT in a subject suffering from intractable Meniere's disease. A 51-year-old male patient has suffered from fluctuating right sensorineural hearing loss with vertigo since 1994. In May 2002, he was first admitted to our hospital due to a severe vertigo attack accompanied by right sensorineural hearing loss. Spontaneous nystagmus toward the right side was observed. Since April 2004, he has experienced vertigo spells with right-sided tinnitus a few times per month that are intractable to conventional medical therapy. After four months, tympanic tube insertion was preformed in the right tympanic membrane. Intratympanic injection of dexamethasone was ineffective. He refused Meniett therapy and intratympanic gentamicin injection. In addition to his vertigo spells, he suffered from insomnia, tinnitus, and anxiety. Tranquilizers such as benzodiazepines and antidepressants such as serotonin selective re-uptake inhibitors (SSRIs) failed to stop the vertigo and only slightly improved his insomnia. In December 2006, the patient began psychological counseling with a psychotherapist. After brief psychological counseling along with cognitive behavior therapy (CBT), he began AT. He diligently and regularly continued his AT training in his home according to a written timetable. His insomnia, tinnitus, and vertigo spells disappeared within a few weeks after only four psychotherapy sessions. In order to master the six standard formulas of AT, he underwent two more sessions. Thereafter, he underwent follow-up for 9 months with no additional treatment. He is now free from drugs, including

  19. Diverticular disease and the risk of colon cancer - a population-based case-control study.

    Science.gov (United States)

    Granlund, J; Svensson, T; Granath, F; Hjern, F; Ekbom, A; Blomqvist, P; Schmidt, P T

    2011-09-01

    Colon cancer and diverticular disease are most common in the Western world and their incidences tend to increase with advancing age. The association between the diseases remains unclear. To analyse the risk of colon cancer after hospitalisation for diverticular disease. Nationwide case-control study. A total of 41,037 patients with colon cancer during 1992-2006, identified from the Swedish Cancer Register were included. Each case was matched with two control subjects. From the Swedish Inpatient Register, cases and control subjects hospitalised for diverticular disease were identified. Odds ratios (OR) and confidence intervals for receiving a diagnosis of colon cancer after hospital discharge for diverticular disease were calculated. Colon cancer mortality was compared between patients with or without diverticular disease. Within 6months after an admission due to diverticular disease, OR of having a colon cancer diagnosis were up to 31.49 (19.00-52.21). After 12 months, there was no increased risk. The number of discharges for diverticular disease did not affect the risk. Colon cancer mortality did not differ between patients with and without diverticular disease. Diverticular disease does not increase the risk of colon cancer in the long term, and a history of diverticular disease does not affect colon cancer mortality. The increased risk of colon cancer within the first 12months after diagnosing diverticular disease is most likely due to surveillance and misclassification. Examination of the colon should be recommended after a primary episode of symptomatic diverticular disease. © 2011 Blackwell Publishing Ltd.

  20. Pediatric Dupuytren's disease: case report and review of the literature

    African Journals Online (AJOL)

    children, delayed diagnosis of DD can result in debilitating disease and ... 1). The boy was subsequently taken to the operating room, where a Bruner incision was made on the palmar aspect of ... history, diabetes mellitus, and sex. However ...

  1. A CURIOUS CASE OF FEVER AND INTERSTITIAL LUNG DISEASE

    OpenAIRE

    Dr. Shahid Mahdi; Dr. Darpanarayan Hazra; Dr. Zainab Mahdi

    2017-01-01

    Antisynthetase syndrome is a rare chronic autoimmune inflammatory myopathy with fever, interstitial lung disease, Raynaud’s phenomenon and polyarthritis. The exact underlying cause of antisynthetase syndrome is not yet known. Diagnosis is made with presence of Jo-1 (Histydyl t RNA synthase) antigen in a patient with underlying interstitial lung disease, myositis, arthritis, Raynaud’s phenomenon and mechanic’s hand. Some of the other antisynthetase anti bodies are PL-7 (antigen – threonyl-tRNA...

  2. Creutzfeldt-Jakob disease: a case report and differential diagnoses.

    Science.gov (United States)

    Kojima, Gotaro; Tatsuno, Brent K; Inaba, Michiko; Velligas, Stephanie; Masaki, Kamal; Liow, Kore K

    2013-04-01

    Sporadic Creutzfeldt-Jakob disease is a rare neurodegenerative disorder of unknown etiology that causes rapidly progressive dementia. This disease is uniformly fatal and most patients die within 12 months. Clinical findings include myoclonus, visual disturbances, and cerebellar and pyramidal/extrapyramidal signs in addition to rapidly progressive cognitive and functional impairment. These findings are all non-specific and it is often difficult and challenging to diagnose premortem because of low awareness and clinical suspicion. We present a 66-year-old woman with a 5-month history of rapidly progressive dementia. After a series of extensive diagnostic examinations and continuous follow-up, she was diagnosed with probable sporadic Creutzfeldt-Jakob disease based on Centers for Disease Control and Prevention (CDC) criteria, with key findings of rapidly progressive dementia, blurry vision, extrapyramidal signs (cogwheel rigidity), and abnormal hyperintensity signals on diffusion-weighted MRI. Her symptoms progressively worsened and she died 7 months after the onset. The postmortem brain autopsy demonstrated the presence of abnormal protease-resistant prion protein by Western Blot analysis. A literature review was performed on differential diagnoses that present with rapidly progressive dementia and thereby mimic sporadic Creutzfeldt-Jakob disease. These include Alzheimer's disease, dementia with Lewy Bodies, frontotemporal dementia, meningoencephalitis, corticobasal degeneration, progressive supranuclear palsy, CADASIL, and paraneoplastic encephalomyelitis.

  3. Autoimmune disease and risk for Parkinson disease A population-based case-control study

    DEFF Research Database (Denmark)

    Rugbjerg, K.; Friis, S.; Ritz, B.

    2009-01-01

    Objective: Inflammatory mediators are increased in autoimmune diseases and may activate microglia and might cause an inflammatory state and degeneration of dopaminergic neurons in the brain. Thus, we evaluated whether having an autoimmune disease increases the risk for developing Parkinson disease...... do not support the hypothesis that autoimmune diseases increase the risk for Parkinson disease. The decreased risk observed among patients with rheumatoid arthritis might be explained by underdiagnosis of movement disorders such as Parkinson disease in this patient group or by a protective effect...

  4. A Case of New Familiar Genetic Variant of Autosomal Dominant Polycystic Kidney Disease-2: A Case Study.

    Science.gov (United States)

    Litvinchuk, Tetiana; Tao, Yunxia; Singh, Ruchi; Vasylyeva, Tetyana L

    2015-01-01

    Autosomal dominant polycystic kidney disease (ADPKD) is characterized by renal cyst formation due to mutations in genes coding for polycystin-1 [PKD1 (85-90% of cases), on ch 16p13.3] and polycystin-2 [PKD2 (10-15% of cases), on ch 4q13-23] and PKD3 gene (gene unmapped). It is also associated with TSC2/PKD1 contiguous gene syndrome. ADPKD is usually inherited, but new mutations without a family history occur in approximately 10% of the cases. A 17-year-old boy was followed up for bilateral cystic kidney disease, hypertension, and obesity since he was 13 years old. The diagnosis was an accidental finding during abdominal CT at age 13 to rule out appendicitis. A renal ultrasonogram also demonstrated a multiple bilateral cysts. Because of parental history of bilateral renal cysts, PKD1 and PKD2, genetic testing was ordered. Results showed, PKD2 variant 1:3 bp deletion of TGT; nucleotide position: 1602-1604; codon position: 512-513; mRNA reading frame maintained. The same mutation was later identified in his father. A smaller number of patients have a defect in the PKD2 locus on chromosome 4 (resulting in PKD2 disease). There are no known published cases on this familiar genetic variant of ADPKD-2 cystic kidney disease. In this case, the disease is present unusually early in life.

  5. The Infrapopliteal Arterial Occlusions Similar to Buerger Disease: Report of Two Cases

    Directory of Open Access Journals (Sweden)

    Kimihiro Igari

    2014-01-01

    Full Text Available We herein present two cases that required the differential diagnosis of Buerger disease. Case 1 involved a 55-year-old male with a smoking habit who was admitted with ulcers and coldness in his fingers and toes. Angiography showed blockage in both the radial and posterior tibial arteries, which led to an initial diagnosis of Buerger disease. However, a biopsy of the right posterior tibial artery showed pathological findings of fibromuscular dysplasia (FMD. Case 2 involved a 28-year-old male with intermittent claudication who was examined at another hospital. Angiography showed occlusion of both popliteal and crural arteries, and the patient was suspected to have Buerger disease. However, computed tomography disclosed an abnormal slip on both sides of the popliteal fossa, and we diagnosed him with bilateral popliteal artery entrapment syndrome (PAES. These cases illustrate that other occlusive diseases, such as FMD and PAES, may sometimes be misdiagnosed as Buerger disease.

  6. An Adult Form of Gaucher Disease Associated with Portal Hypertension: A Case

    Directory of Open Access Journals (Sweden)

    Ahmet Dulger

    2013-04-01

    Full Text Available Gaucher disease (GD is an inborn error of metabolism that affects the recycling of cellular glycolipids. Glucosylceramide (also called glucocerebroside accumulate within the lysosomes of cells. Gaucher%u2019s disease is most common lysosomal storage disease and its incidence is 1/75.000. Three types of this disease have been defined. During the course of disease, it was reported that hepatosplenomegaly, portal hypertension, hyperferritinemia, splenic infarcts and splenic nodules might develop. Therefore, as in our case; Gaucher%u2019s disease must be remembered in the setting of hepatosplenomegaly, portal hypertension, hyperferritinemia, splenic infarcts and splenic nodules of unknown etiology.

  7. Rethinking the Poverty-disease Nexus: the Case of HIV/AIDS in South Africa.

    Science.gov (United States)

    Pienaar, Kiran

    2017-09-01

    While it is well-established that poverty and disease are intimately connected, the nature of this connection and the role of poverty in disease causation remains contested in scientific and social studies of disease. Using the case of HIV/AIDS in South Africa and drawing on a theoretically grounded analysis, this paper reconceptualises disease and poverty as ontologically entangled. In the context of the South African HIV epidemic, this rethinking of the poverty-disease dynamic enables an account of how social forces such as poverty become embodied in the very substance of disease to produce ontologies of HIV/AIDS unique to South Africa.

  8. Radiologic study of 42 cases of Wilson disease

    International Nuclear Information System (INIS)

    Xie, Y.; Zhang, X.; Xu, X.; Zhang, Z.; Feng, Y.

    1985-01-01

    This paper reports 42 cases of bone and joint radiographic changes in hepatolenticular degeneration. The cases were proven clinically by opthalmologic and laboratory study, and their radiographic and joint changes were divided into five groups: no abnormal findings, osteoporosis, osteomalacia (rickets), distinct changes, and miscellaneous. Distinct changes include marginal bone fragments, angulation of carpal bones, squaring of metacarpal heads, and calcification of the joint capsule or tendon insertion. The mechanism causing the bone changes in heptolenticular degeneration is also discussed. (orig.)

  9. Radiologic study of 42 cases of Wilson disease

    Energy Technology Data Exchange (ETDEWEB)

    Xie, Y.; Zhang, X.; Xu, X.; Zhang, Z.; Feng, Y.

    1985-02-01

    This paper reports 42 cases of bone and joint radiographic changes in hepatolenticular degeneration. The cases were proven clinically by opthalmologic and laboratory study, and their radiographic and joint changes were divided into five groups: no abnormal findings, osteoporosis, osteomalacia (rickets), distinct changes, and miscellaneous. Distinct changes include marginal bone fragments, angulation of carpal bones, squaring of metacarpal heads, and calcification of the joint capsule or tendon insertion. The mechanism causing the bone changes in heptolenticular degeneration is also discussed.

  10. Calf enlargement associated with neurologic disease: two uncommon cases.

    Science.gov (United States)

    Harwood, S C; Honet, J C

    1988-01-01

    Muscle enlargement and hypertrophy are rare findings in neurogenic lesions. The two in combination have been reported in cases of peripheral nerve lesions, polyneuropathy, and poliomyelitis. True and pseudo muscle hypertrophy are the two possible etiologies, whereas infiltration, stretch, or exercise of the muscle are the causative factors. We report two cases of unilateral calf enlargement, one occurring after surgery for S1 radiculopathy with associated cramping, and the other after poliomyelitis.

  11. Chagas disease study using satellite image processing: A Bolivian case

    Science.gov (United States)

    Vargas-Cuentas, Natalia I.; Roman-Gonzalez, Avid; Mantari, Alicia Alva; Muñoz, Luis AnthonyAucapuma

    2018-03-01

    Remote sensing is the technology that has enabled us to obtain information about the Earth's surface without directly contacting it. For this reason, currently, the Bolivian state has considered a list of interesting applications of remote sensing in the country, including the following: biodiversity and environment monitoring, mining and geology, epidemiology, agriculture, water resources and land use planning. The use of satellite images has become a great tool for epidemiology because with this technological advance we can determine the environment in which transmission occurs, the distribution of the disease and its evolution over time. In that context, one of the important diseases related to public health in Bolivia is Chagas disease, also known as South American Trypanosomiasis. Chagas is caused by a blood-sucking bug or Vinchuca, which causes serious intestinal and heart long term problems and affects 33.4% of the Bolivian population. This disease affects mostly humble people, so the Bolivian state invests millions of dollars to acquire medicine and distribute it for free. Due to the above reasons, the present research aims to analyze some areas of Bolivia using satellite images for developing an epidemiology study. The primary objective is to understand the environment in which the transmission of the disease happens, and the climatic conditions under which occurs, observe the behavior of the blood-sucking bug, identify in which months occur higher outbreaks, in which months the bug leaves its eggs, and under which weather conditions this happens. All this information would be contrasted with information extracted from the satellite images and data from the Ministry of Health, and the Institute of Meteorology in Bolivia. All this data will allow us to have a more integrated understanding of this disease and promote new possibilities to prevent and control it.

  12. A case of Bowen's disease on chronic radiodermatitis

    International Nuclear Information System (INIS)

    Ohmori, Masaki; Suenaga, Yoshinori; Fuchi, Kougi.

    1992-01-01

    A 62-year-old female radiologist with chronic radiodermatitis on the dorsal side of her left fingers is reported. She had an occupational history of practicing fluoroscopy for about thirty years. Histopathologically Bowen's disease on the 2nd, 3rd and 4th fingers and squamous cell carcinoma on the 5th finger were found in the radiodermatitis lesion. All the lesions were surgically removed and repaired with a full thickness skin graft. The literature on Bowen's disease caused by radiotherapy and occupational irradiation was also reviewed. (author)

  13. Hallervorden-Spatz disease: 2 cases of siblings

    International Nuclear Information System (INIS)

    Song, Jong Gi; Kim, In One; Kim, Woo Sun; Yeon, Kyung Mo

    1994-01-01

    We report two patients with Hallervorden-Spatz disease, who were diagnosed by same MR findings of marked low signal intensity in the globus and substantia nigra. They presented with ataxic and spastic gait, intention tremor, delayed mental development, and dysarthria. They were 7 year-old male and 8 year-old female siblings, who were healthy until 3 years of age when they suffered from progressive symptoms. T2-weighted images showed marked low signal intensity in the globus pallidus and substantia nigra indicating an increased iron deposition, and it might suggest Hallervorden-Spatz disease

  14. Case report 379: 'Ulcer osteoma' associated with sickle cell disease

    International Nuclear Information System (INIS)

    Wiggins, T.; Bohrer, S.P.

    1986-01-01

    In summary, a 32-year-old black man with homozygous sickle cell anemia has been presented. The patient developed bilateral ulcers of the leg which never completely healed. In one leg he demonstrated a focal, fusiform, periosteal reaction which probably in time would become incorporated into the cortex, resulting in the formation of an ulcer osteoma of the tibia associated with sickle cell disease. The ulcer osteoma has the same radiological appearance as the ulcer osteoma in individuals in Africa without sickle cell disease. (orig./SHA)

  15. Late onset Pompe disease- new genetic variant: Case report ...

    African Journals Online (AJOL)

    The patient was not given enzyme replacement therapy due to cost but received high protein therapy and Oxygen supplementation using Oxygen extractor machine. She is worsening due to respiratory failure. Conclusion: This is a new genetic variant isolated of late-onset Pompe disease which presents with almost pure ...

  16. Norrie disease. Diagnosis of a simplex case by DNA analysis.

    Science.gov (United States)

    Chynn, E W; Walton, D S; Hahn, L B; Dryja, T P

    1996-09-01

    Norrie disease is a rare, X-linked recessive disorder characterized by congenital blindness due to malformed retinas. We describe a simplex patient who had leukokoria and whose clinical diagnosis was confirmed only after molecular genetics analysis. DNA analysis was also used to determine the carrier status of relatives of the proband.

  17. Sequential MRI in a case of Creutzfeldt-Jakob disease

    International Nuclear Information System (INIS)

    Tribl, G.G.; Zeitlhofer, J.; Asenbaum, S.; Wessely, P.; Strasser, G.; Prayer, D.; Jarius, C.

    2002-01-01

    A 48-year-old man suddenly developed clinically and electroencephalographically nonspecific dementia. On MRI sequences, only diffusion-weighted images (DWI) of the cortex were unequivocally pathological. Obvious atrophy and basal ganglia signal changes appeared only 9 months after the onset. Brain biopsy confirmed Creutzfeldt-Jakob disease (CJD). In rapidly progressive dementia, we recommend DWI for early diagnosis of CJD. (orig.)

  18. Preventing the Dutch Disease: The case of Indonesia

    International Nuclear Information System (INIS)

    Ramli, R.

    1992-01-01

    Indonesia has been more successful than other oil exporters in mitigating the effects of the Dutch Disease, the decline in non-oil production associated with an oil boom. Typically sharp increases in oil revenues promote the accumulation of international reserves, increasing monetization, and raising prices of non-traded relative to traded goods. The latter causes contraction of output and employment in the traded sector. This study analyzes the magnitude of the boom and its fiscal, monetary, and production effects. Proxies of the real exchange rate, defined as the relative price of traded to non-traded goods, were constructed. The effect of the exchange rate on the Dutch Disease problems were examined. The movement of the real exchange rate and the remarkable growth of production of traded goods indicate that the effects of the Dutch Disease on the Indonesian economy were remarkably limited. Structural and policy-induced factors, such as exchange rate protection and micro interventions, that mitigated the anticipated effects of the Dutch Disease are examined

  19. Paediatric cardiac anaesthesia in sickle cell disease: a case series

    African Journals Online (AJOL)

    Paediatric patients with SCD and congenital heart defects may require ... Patients with sickle cell disease (SCD) presenting for cardiac ... fluid, calculated according to body weight, was initiated. ... oxygen mixture and intravenous fentanyl (5–10 mcg/kg) and .... erythropoiesis, and in this way reduces HbS production.

  20. Fabry's Disease: Case Series and Review of Literature | Wani ...

    African Journals Online (AJOL)

    Fabry's disease is an X‑linked lysosomal storage disorder caused by a deficiency of alpha‑galactosidase A enzyme with the progressive accumulation of globotriaosylceramide in vascular endothelial cells leading to cardiovascular, renal, gastrointestinal, neuropathic, lenticular, and dermatological manifestations. It is a rare ...

  1. Familial cases of Norrie disease detected by copy number analysis.

    Science.gov (United States)

    Arai, Eisuke; Fujimaki, Takuro; Yanagawa, Ai; Fujiki, Keiko; Yokoyama, Toshiyuki; Okumura, Akihisa; Shimizu, Toshiaki; Murakami, Akira

    2014-09-01

    Norrie disease (ND, MIM#310600) is an X-linked disorder characterized by severe vitreoretinal dysplasia at birth. We report the results of causative NDP gene analysis in three male siblings with Norrie disease and describe the associated phenotypes. Three brothers with suspected Norrie disease and their mother presented for clinical examination. After obtaining informed consent, DNA was extracted from the peripheral blood of the proband, one of his brothers and his unaffected mother. Exons 1-3 of the NDP gene were amplified by polymerase chain reaction (PCR), and direct sequencing was performed. Multiplex ligation-dependent probe amplification (MLPA) was also performed to search for copy number variants in the NDP gene. The clinical findings of the three brothers included no light perception, corneal opacity, shallow anterior chamber, leukocoria, total retinal detachment and mental retardation. Exon 2 of the NDP gene was not amplified in the proband and one brother, even when the PCR primers for exon 2 were changed, whereas the other two exons showed no mutations by direct sequencing. MLPA analysis showed deletion of exon 2 of the NDP gene in the proband and one brother, while there was only one copy of exon 2 in the mother. Norrie disease was diagnosed in three patients from a Japanese family by clinical examination and was confirmed by genetic analysis. To localize the defect, confirmation of copy number variation by the MLPA method was useful in the present study.

  2. Sequential MRI in a case of Creutzfeldt-Jakob disease

    Energy Technology Data Exchange (ETDEWEB)

    Tribl, G.G.; Zeitlhofer, J.; Asenbaum, S.; Wessely, P. [Universitaetsklinik fuer Neurologie, Allgemeines Krankenhaus Wien (Austria); Strasser, G.; Prayer, D. [Universitaetsklinik fuer Radiodiagnostik, Allgemeines Krankenhaus Wien (Austria); Jarius, C. [Klinisches Institut fuer Neurologie, Universitaet Wien (Austria)

    2002-03-01

    A 48-year-old man suddenly developed clinically and electroencephalographically nonspecific dementia. On MRI sequences, only diffusion-weighted images (DWI) of the cortex were unequivocally pathological. Obvious atrophy and basal ganglia signal changes appeared only 9 months after the onset. Brain biopsy confirmed Creutzfeldt-Jakob disease (CJD). In rapidly progressive dementia, we recommend DWI for early diagnosis of CJD. (orig.)

  3. Anemia: monosymptomatic celiac disease. A report of 3 cases

    NARCIS (Netherlands)

    Depla, A. C.; Bartelsman, J. F.; Mulder, C. J.; Tytgat, G. N.

    1990-01-01

    Patients with monosymptomatic celiac disease (CD) can escape diagnosis for a long period. Anemia is a common finding in CD, although anemia as the sole symptom is relatively unknown. We report on three patients who presented with iron deficiency anemia and no other symptom, in whom CD was considered

  4. What is the probability of successive cases of Legionnaires' disease occurring in European hotels?

    Science.gov (United States)

    Ricketts, K D; Slaymaker, E; Verlander, N Q; Joseph, C A

    2006-04-01

    Public health officials will normally take action at accommodation sites following an association with a cluster of cases of Legionnaires' disease. This paper seeks to determine the likelihood of such a cluster occurring at a site once it has been associated with a single case of the disease, and therefore whether more should be done at sites following individual cases. Information for UK residents reported to the EWGLINET system between 1993 and 2000 was included in a dataset. The size and country of hotel visited by the cases were divided into six country groups (France, Italy, Spain, Turkey, other Europe and other World), and eight size groups (30%; the probability of a subsequent case occurring within 2 years of the first reached over 50% in some instances. There may be support for early intervention at some accommodation sites following a first case of Legionnaires' disease, in specific country and size groups.

  5. HYPNOSIS IN THE TREATMENT OF MORGELLONS DISEASE: A Case Study1

    Science.gov (United States)

    Gartner, Ashley M.; Dolan, Sara L.; Stanford, Matthew S.; Elkins, Gary R.

    2014-01-01

    Morgellons Disease is a condition involving painful skin lesions, fibrous growths protruding from the skin, and subcutaneous stinging and burning sensations, along with symptoms of anxiety, depression, fatigue, and memory and attention deficits. The etiological and physiological bases of these symptoms are unclear, making the diagnosis controversial and challenging to treat. There are currently no established treatments for Morgellons Disease. The following case example depicts treatment of a woman with Morgellons Disease using hypnotherapy. Data from this case example suggest that hypnotherapy is a promising intervention for the physical and psychological symptoms associated with Morgellons Disease. PMID:21390982

  6. Neuro-Behçet's disease in Peru: a case report and literature review.

    Science.gov (United States)

    Molina, Roberto A; Huerta-Rosario, Andrely; Alva Díaz, Carlos Alexander; Mejía Rojas, Koni Katerin; Mori, Nicanor; Romero Sánchez, Roberto

    2017-06-20

    Behçet's disease is a vasculitis that can cause inflammatory lesions in multiple organs or systems including the nervous system. Most cases worldwide have been reported along what is called the "Silk Route" from the Mediterranean region to Japan, so it is considered a rare disease in Latin American countries. The frequency of neurological involvement ranges from 5 to 13%. We present the case of a young adult woman with diagnostic criteria for Behçet's disease and manifestations of neurological disease, as well as a review of the literature.

  7. Traumatic funicular phlebitis of the thoracic wall resembling Mondor's disease: a case report

    Directory of Open Access Journals (Sweden)

    Kondo Takeshi

    2011-03-01

    Full Text Available Abstract Introduction Mondor's disease is a peculiar form of thrombophlebitis, involving a superficial vein in the subcutaneous fat of the breast or anterior chest wall. Case presentation The author presents a case of a 35-year-old male Japanese patient with cord-like induration in the right lateral thoracic wall. This lesion was diagnosed as traumatic funicular phlebitis, resembling Mondor's disease. Conclusion Traumatic funicular phlebitis, resembling Mondor's disease, is a clinical entity which may give suggestive insight to the etiology of Mondor's disease itself.

  8. Case report of Mikulicz`s disease: A modern concept of an old entity

    Directory of Open Access Journals (Sweden)

    Božić Ksenija

    2016-01-01

    Full Text Available Introduction. Modern knowlegde defines Mikulicz´s disease as a part of immunoglobulin G4-related disease. The main feature is the presence of lymphoplasmacytic infiltrates, immunoglobulin G4 plasma cells positivity, distinctive storiform fibrosis and moderate eosinophilia. Case report. A 59-years old male presented with a mild keratoconjuctivitis sicca and enlarged lacrimal and salivary glands during the last two years. Althought clinical presentation of the patient was typical, earlier testing did not pinpoint Mikulicz ´s disease. By typical clinical presentation, elevated serum immunoglobulin G4 level and histopathological finding of lacrimal glands tissue we diagnosed Mikulicz´s disease successfully treated with corticosteroid therapy. Conclusion. We reported the first case of IgG4-related Mikulicz´s disease in Serbia. Our report highlights IgG4-related Mikulicz` s disease as an important differential diagnosis with Sjögren`s syndrome and lymphoproliferative disease in rheumatological practice.

  9. Chronic disease and independence in old age: A case study

    International Nuclear Information System (INIS)

    Reeves, Pauline J.

    2006-01-01

    This report uses case study methodology to examine the issue of long-term care of the elderly in the United Kingdom, including where that care should take place. The report will examine the difficulties inherent in maintaining independent living for the elderly (in particular the danger and cost of falls). The case study presented is that of an elderly female patient who had suffered from chronic rheumatoid arthritis for over 10 years. She was admitted to hospital several times from December 2003 to January 2004. The discussion of her case is set in the context of the sociology of ageing; long-term care of the elderly and the UK National Service Frameworks, of which standard six relates to falls in the elderly. The report will also consider the problems in deciding whether it is necessary to terminate independent living for an individual

  10. Flow cytometry of duodenal intraepithelial lymphocytes improves diagnosis of celiac disease in difficult cases.

    Science.gov (United States)

    Valle, Julio; Morgado, José Mario T; Ruiz-Martín, Juan; Guardiola, Antonio; Lopes-Nogueras, Miriam; García-Vela, Almudena; Martín-Sacristán, Beatriz; Sánchez-Muñoz, Laura

    2017-10-01

    Diagnosis of celiac disease is difficult when the combined results of serology and histology are inconclusive. Studies using flow cytometry of intraepithelial lymphocytes (IELs) have found that celiac patients have increased numbers of γδ IELs, along with a decrease in CD3-CD103 + IELs. The objective of this article is to assess the role of flow cytometric analysis of IELs in the diagnosis of celiac disease in difficult cases. A total of 312 patients with suspicion of celiac disease were included in the study. Duodenal biopsy samples were used for histological assessment and for flow cytometric analysis of IELs. In 46 out of 312 cases (14.7%) the combination of serology and histology did not allow the confirmation or exclusion of celiac disease. HLA typing had been performed in 42 of these difficult cases. Taking into account HLA typing and the response to a gluten-free diet, celiac disease was excluded in 30 of these cases and confirmed in the remaining 12. Flow cytometric analysis of IELs allowed a correct diagnosis in 39 out of 42 difficult cases (92.8%) and had a sensitivity of 91.7% (95% CI: 61.5% to 99.8%) and a specificity of 93.3% (95% CI: 77.9% to 99.2%) for the diagnosis of celiac disease in this setting. Flow cytometric analysis of IELs is useful for the diagnosis of celiac disease in difficult cases.

  11. Subhyaloid Hemorrhage in a Case of Devic's Disease | Chakraborti ...

    African Journals Online (AJOL)

    We diagnosed the case as neuromyelitis optica (NMO) based on the examination and investigation findings. NMO or Devic's syndrome is an uncommon clinical syndrome associating unilateral or bilateral optic neuritis and transverse myelitis. Subhyaloid hemorrhage, as an ophthalmic feature of NMO, has not been reported ...

  12. Waterborne Disease Case Investigation: Public Health Nursing Simulation.

    Science.gov (United States)

    Alexander, Gina K; Canclini, Sharon B; Fripp, Jon; Fripp, William

    2017-01-01

    The lack of safe drinking water is a significant public health threat worldwide. Registered nurses assess the physical environment, including the quality of the water supply, and apply environmental health knowledge to reduce environmental exposures. The purpose of this research brief is to describe a waterborne disease simulation for students enrolled in a public health nursing (PHN) course. A total of 157 undergraduate students completed the simulation in teams, using the SBAR (Situation-Background-Assessment-Recommendation) reporting tool. Simulation evaluation consisted of content analysis of the SBAR tools and debriefing notes. Student teams completed the simulation and articulated the implications for PHN practice. Student teams discussed assessment findings and primarily recommended four nursing interventions: health teaching focused on water, sanitation, and hygiene; community organizing; collaboration; and advocacy to ensure a safe water supply. With advanced planning and collaboration with partners, waterborne disease simulation may enhance PHN education. [J Nurs Educ. 2017;56(1):39-42.]. Copyright 2017, SLACK Incorporated.

  13. Morgellons disease, illuminating an undefined illness: a case series

    OpenAIRE

    Harvey, William T; Bransfield, Robert C; Mercer, Dana E; Wright, Andrew J; Ricchi, Rebecca M; Leitao, Mary M

    2009-01-01

    Abstract Introduction This review of 25 consecutive patients with Morgellons disease (MD) was undertaken for two primary and extremely fundamental reasons. For semantic accuracy, there is only one "proven" MD patient: the child first given that label. The remainder of inclusive individuals adopted the label based on related descriptions from 1544 through 1884, an internet description quoted from Sir Thomas Browne (1674), or was given the label by practitioners using similar sources. Until now...

  14. Darier disease with oral and esophageal involvement: A case report

    Directory of Open Access Journals (Sweden)

    Magesh Karuppur Thiagarajan

    2011-01-01

    Full Text Available A 58-year-old man presented with itchy papular eruptions all over the body since 15 years. Intraoral examination revealed raised papular lesions on the labial mucosa, hard palate, and tongue. The histopathology of the oral and skin lesions was confirmative of Darier disease (DD. This patient also showed esophageal involvement, which was confirmed histopathologically. Such a presentation of DD, with oral and esophageal involvement, is rare.

  15. [IgG4-related disease - a case report].

    Science.gov (United States)

    Milczarek-Banach, Justyna; Brodzińska, Kinga; Jankowska, Anna; Ambroziak, Urszula; Szczepankiewicz, Benedykt; Nałęcz-Janik, Jolanta; Miśkiewicz, Piotr

    2017-09-29

    Immunoglobulin G4-related disease (IgG4-RD) is a comparatively new condition that may involve more than one organ. The lack of characteristic, pathognomonic clinical symptoms may delay the diagnosis of this disease. The diagnosis is based upon clinical manifestation, elevated serum levels of IgG4 and histopathologic examination with immunohistochemical staining to reveal infiltration of IgG4-positive plasma cells. The first line treatment is oral glucocorticoids. 38-year-old woman with Hashimoto disease, chronic sinusitis and chronic hepatitis of unknown etiology was admitted to the Department of Endocrinology because of moderate eyelids swelling accompanied by redness for 3 years. Graves' orbitopathy and systemic vasculitis were suspected, however both were excluded (negative antibodies results: anty-TSHR, ANCA, ANA). Serologic investigation of Sjögren's syndrome was also negative. In Magnetic Resonance Imaging (MRI) of orbits there were described bilateral mild extension of lateral rectus muscles, normal signal of adipose tissue and bilateral lacrimal glands enlargement. Moreover, increased IgG4 serum levels were detected. The material derived from perinasal sinuses surgery was analyzed in histopathology examination with immunohistochemical staining, which revealed characteristic features of chronic inflammatory process and increased numbers of IgG4 - positive plasma cells (>50 in a large field of view). The diagnosis of IgG4-RD was established. Because of non-effective oral methylprednisolone therapy in the past, the patient was referred to Clinic of Rheumatology for further treatment. After the therapy with methylprednisolone and azathioprine there were observed the significant reduction of symptoms. Because of lack of characteristic symptoms of IgG4- RD, it should be always considered in differential diagnosis of chronic inflammatory diseases of various organs.

  16. Yellow fever vaccine-associated neurological disease, a suspicious case.

    Science.gov (United States)

    Beirão, Pedro; Pereira, Patrícia; Nunes, Andreia; Antunes, Pedro

    2017-03-02

    A 70-year-old man with known cardiovascular risk factors, presented with acute onset expression aphasia, agraphia, dyscalculia, right-left disorientation and finger agnosia, without fever or meningeal signs. Stroke was thought to be the cause, but cerebrovascular disease investigation was negative. Interviewing the family revealed he had undergone yellow fever vaccination 18 days before. Lumbar puncture revealed mild protein elevation. Cultural examinations, Coxiella burnetti, and neurotropic virus serologies were negative. Regarding the yellow fever virus, IgG was identified in serum and cerebrospinal fluid (CSF), with negative IgM and virus PCR in CSF. EEG showed an encephalopathic pattern. The patient improved gradually and a week after discharge was his usual self. Only criteria for suspect neurotropic disease were met, but it's possible the time spent between symptom onset and lumbar puncture prevented a definite diagnosis of yellow fever vaccine-associated neurological disease. This gap would have been smaller if the vaccination history had been collected earlier. 2017 BMJ Publishing Group Ltd.

  17. Dental erosion caused by gastroesophageal reflux disease: a case report.

    Science.gov (United States)

    Cengiz, Seda; Cengiz, M Inanç; Saraç, Y Sinasi

    2009-07-22

    Chronic regurgitation of gastric acids in patients with gastroesophageal reflux disease may cause dental erosion, which can lead in combination with attrition or bruxism to extensive loss of coronal tooth tissue. This clinical report describes treatment of severe tooth wear of a gastroesophageal reflux disease patient who is 54-year-old Turkish male patient. After his medical treatment, severe tooth wear, bruxism and decreased vertical dimensions were determined. The vertical dimension was re-established and maxillary and mandibular anterior and posterior teeth were prepared for metal-ceramic restorations. Metal-ceramic fixed partial dentures were fabricated as full mouth restorations for both maxillary and mandibular arches because of splinting all teeth. And then maxillary stabilization splint was fabricated for his bruxism history. Significant loss of coronal tooth structure must taken into consideration. Gastroesophageal reflux disease by itself or in combination with attrition, abrasion or bruxism may be responsible for the loss. An extensive diagnostic evaluation is essential for the medical and dental effects of the problem.

  18. Mucinous adenocarcinoma arising at the anastomotic site after operation for hirschsprung's disease: case report

    International Nuclear Information System (INIS)

    Kim, Hyung Soo; Yoon, Kwon Ha

    2004-01-01

    To our knowledge, rectal cancer arising at the anastomotic site after surgery for Hirschsprung's disease has not been reported. We report a case of mucinous adenocarcinoma arising at the anastomotic site after Soave operation 26 years ago

  19. Familial benign chronic neutropenia associated with periodontal disease. A case report.

    Science.gov (United States)

    Deasy, M J; Vogel, R I; Macedo-Sobrinho, B; Gertzman, G; Simon, B

    1980-04-01

    A rare case report of periodontal disease associated with familial benign chronic neutropenia is presented. The medical, dental and family histories as well as clinical and histologic observations are described and discussed.

  20. Pylephlebitis and Crohn’s disease: A rare case of septic shock

    Directory of Open Access Journals (Sweden)

    Stefano Scaringi

    2017-01-01

    Conclusion: This case highlights the importance of promptly considerate and treat mesenteric pylephlebitis in presence of a septic shock in a Crohn’s disease patient who is not showing clinical signs of peritonitis.

  1. MR findings in acute Lyme disease affecting the knee. A case report

    International Nuclear Information System (INIS)

    Cavallaro, A.; Harrer, T.; Richter, H.; Bautz, W.; Fellner, F.A.

    2002-01-01

    In this paper we report a case with primarily unspecific arthralgia after surgical therapy of hallux valgus deformity and consecutive reflex sympathetic dystrophy in which MR led to the diagnosis of Lyme disease. (orig.)

  2. MR findings in acute Lyme disease affecting the knee. A case report

    Energy Technology Data Exchange (ETDEWEB)

    Cavallaro, A.; Harrer, T.; Richter, H.; Bautz, W.; Fellner, F.A. [Friedrich-Alexander-Univ., Erlangen-Nuernberg (Germany)

    2002-07-01

    In this paper we report a case with primarily unspecific arthralgia after surgical therapy of hallux valgus deformity and consecutive reflex sympathetic dystrophy in which MR led to the diagnosis of Lyme disease. (orig.)

  3. Association of congenit anomalia anus rectal with Hirschsprung's disease: a case report

    International Nuclear Information System (INIS)

    Abbud, E.A.; Sales, C.R.V. de; Goncalves, E.G.

    1989-01-01

    The recognition of Hirschsprung's disease is often delayed in children with imperforated anus. Because of this rare association, it is reported one case in which its occurred in conjunction with Down's syndrome. (author) [pt

  4. A report of three cases of untreated Graves' disease associated with pancytopenia in Malaysia.

    Science.gov (United States)

    Rafhati, Abdullah Noor; See, Chee Keong; Hoo, Fan Kee; Badrulnizam, Long Bidin Mohamed

    2014-01-01

    Generally, clinical presentations of Graves' disease range from asymptomatic disease to overt symptomatic hyperthyroidism with heat intolerance, tremor, palpitation, weight loss, and increased appetite. However, atypical presentation of Graves' disease with hematological system involvement, notably pancytopenia, is distinctly uncommon. Hereby, we present and discuss a series of three untreated cases of Graves' disease clinically presented with pancytopenia and the hematological abnormalities that responded well to anti-thyroid treatment. With resolution of the thyrotoxic state, the hematological parameters improved simultaneously. Thus, it is crucial that anti-thyroid treatment be considered in patients with Graves' disease and pancytopenia after a thorough hematological evaluation.

  5. Huntington disease: a case study of early onset presenting as depression.

    Science.gov (United States)

    Duesterhus, Pia; Schimmelmann, Benno Graf; Wittkugel, Oliver; Schulte-Markwort, Michael

    2004-10-01

    Huntington disease is a dominantly inherited, neurodegenerative disease characterized by choreiform movement disturbances and dementia, usually with adult onset. The rare juvenile-onset Huntington disease differs from the adult phenotype. A case presenting twice, at age 10 with all the signs of a major depression and age 14 with mutism and rigidity, is reported. Meanwhile, the father developed the adult variant of Huntington disease. The boy's diagnosis was confirmed by molecular genetic analysis and magnetic resonance imaging. It is important to be aware of hereditary conditions such as Huntington disease and to provide family counseling before genetic testing and after the diagnosis is confirmed.

  6. Castleman s Disease with an Unusual Radiological Presentation: A Case Report

    Directory of Open Access Journals (Sweden)

    Nesrin Ocal

    2014-02-01

    Full Text Available Castleman%u2019s disease is a rare lymphoproliferative pathology which has two clinicoradiological forms; localized disease and disseminated disease. Histopathologically, Castleman%u2019s disease is evaluated in three groups; hyaline-vascular type, plasma cell type and mixed type. Patients are often asymptomatic and are diagnosed by radiological findings. The most common radiological presentation is huge lymph nodes in mediastinal area. Lymphadenopathy is most frequently observed in paratracheal lymph nodes. We wanted to emphasize this very rare entity and remind you this disease by the mean of a case with different radiological appearance.

  7. Psychiatric Symptomatology in Early-Onset Binswanger’s Disease: Two Case Reports

    Directory of Open Access Journals (Sweden)

    R. M. Lawrence

    1995-01-01

    Full Text Available We describe two cases of Binswanger's disease of pre-senile onset which presented with affective and psychotic symptoms well before the appearance of cognitive deterioration and neurological signs, initially evading an accurate diagnosis. Psychiatrists should be aware of white matter disease and its role in the pathogenesis of psychiatric illness. Particular attention should be given to a history of hypertension as a risk factor in the early identification of these cases.

  8. Tay-Sach disease with "cherry-red spot"--first reported case in Malaysia.

    Science.gov (United States)

    Chan, L Y; Balasubramaniam, S; Sunder, R; Jamalia, R; Karunakar, T V N; Alagaratnam, J

    2011-12-01

    We present a rare case of Tay-Sachs disease with retinal 'cherry-red spots' in a 19-month-old Malay child. Molecular genetic studies confirmed the diagnosis. The case highlights that 'cherry-red spot' is a useful clinical clue in Tay-Sachs disease and several other lysosomal storage disorders. It serves as an ideal illustration of the eye as a window to inborn error of metabolism.

  9. Morgellons disease, illuminating an undefined illness: a case series.

    Science.gov (United States)

    Harvey, William T; Bransfield, Robert C; Mercer, Dana E; Wright, Andrew J; Ricchi, Rebecca M; Leitao, Mary M

    2009-07-01

    This review of 25 consecutive patients with Morgellons disease (MD) was undertaken for two primary and extremely fundamental reasons. For semantic accuracy, there is only one "proven" MD patient: the child first given that label. The remainder of inclusive individuals adopted the label based on related descriptions from 1544 through 1884, an internet description quoted from Sir Thomas Browne (1674), or was given the label by practitioners using similar sources. Until now, there has been no formal characterization of MD from detailed examination of all body systems. Our second purpose was to differentiate MD from Delusions of Parasitosis (DP), another "informal" label that fit most of our MD patients. How we defined and how we treated these patients depended literally on factual data that would determine outcome. How they were labeled in one sense was irrelevant, except for the confusing conflict rampant in the medical community, possibly significantly skewing treatment outcomes. Clinical information was collected from 25 of 30 consecutive self-defined patients with Morgellons disease consisting of laboratory data, medical history and physical examination findings. Abnormalities were quantified and grouped by system, then compared and summarized, but the numbers were too small for more complex mathematical analysis. The quantification of physical and laboratory abnormalities allowed at least the creation of a practical clinical boundary, separating probable Morgellons from non-Morgellons patients. All the 25 patients studied meet the most commonly used DP definitions. These data suggest Morgellons disease can be characterized as a physical human illness with an often-related delusional component in adults. All medical histories support that behavioral aberrancies onset only after physical symptoms. The identified abnormalities include both immune deficiency and chronic inflammatory markers that correlate strongly with immune cytokine excess. The review of 251

  10. Quantitative Systems Pharmacology: A Case for Disease Models.

    Science.gov (United States)

    Musante, C J; Ramanujan, S; Schmidt, B J; Ghobrial, O G; Lu, J; Heatherington, A C

    2017-01-01

    Quantitative systems pharmacology (QSP) has emerged as an innovative approach in model-informed drug discovery and development, supporting program decisions from exploratory research through late-stage clinical trials. In this commentary, we discuss the unique value of disease-scale "platform" QSP models that are amenable to reuse and repurposing to support diverse clinical decisions in ways distinct from other pharmacometrics strategies. © 2016 The Authors Clinical Pharmacology & Therapeutics published by Wiley Periodicals, Inc. on behalf of The American Society for Clinical Pharmacology and Therapeutics.

  11. Monolobar Caroli's Disease in Left Lobe of the Liver: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Cho, Han Il; Lee, Young Hwan; Jeon, Se Jeong; Roh, Byung Suk; Juhng, Seon Kwan [Wonkwang University Hospital, Iksan (Korea, Republic of)

    2010-06-15

    Caroli's disease is a rare congenital hepatobiliary disease characterized by multifocal segmental dilatation of the intrahepatic bile ducts and hepatic fibrosis that can cause bile duct stones, cholangitis, and cholangiocarcinoma. The disease may diffusely affect the liver or be localized to one lobe or segment. Less than 20% of all reported cases of Caroli's disease are the monolobar type. We report a case of Caroli's disease of the monolobar type, which was confined to segment 4a of the liver in a 30-year-old man. The disease was diagnosed by CT and Gd-EOB-DTPA enhanced MRI, and confirmed histopathologically after a hepatic lobectomy

  12. Disease awareness may increase risk of suicide in young onset dementia: A case report

    Directory of Open Access Journals (Sweden)

    Maria Alice Tourinho Baptista

    Full Text Available ABSTRACT Studies report that people with young onset Alzheimer's disease (YOAD have higher levels of disease awareness compared to those with late onset AD. We report a case of a man with YOAD who had preserved awareness of disease, depression and risk of suicide associated with the development of the dementia. Cognitive functioning, disease severity, depressive symptoms and awareness of disease were assessed using validated measures. The person with YOAD showed a moderate level of disease severity and high degree of dependence for activities of daily living. There was recognition of memory problems and routine changes with presence of intense pessimism, low self-esteem and suicidal ideation. This case points to the existence of specific issues related to young onset dementia and the clinical importance of identifying and treating patients who might be aware of their condition.

  13. Spontaneous closure of patent ductus arteriosus after an episode of Kawasaki disease: a case report

    Directory of Open Access Journals (Sweden)

    Lin Ming-Chih

    2012-01-01

    Full Text Available Abstract Introduction Kawasaki disease is regarded as systemic vasculitis. Many experts believe that not only coronary arteries but also other small arteries are involved during the period of systemic inflammation. However, the evidence to support this point view is limited. Case presentation We report the case of a one-year-four-month-old Taiwanese girl whose patent ductus arteriosus was incidentally found during an episode of Kawasaki disease. The ductus closed spontaneously after the acute phase of Kawasaki disease. Conclusions In this patient, the patent ductus arteriosus may have closed spontaneously after Kawasaki disease due to its involvement in the generalized vasculitis that this disease incurs. This would support the theory that the vasculitis of Kawasaki disease is limited not only to coronary arteries but also to all medium- sized arteries.

  14. Active Surveillance of Hansen's Disease (Leprosy): Importance for Case Finding among Extra-domiciliary Contacts

    Science.gov (United States)

    Moura, Maria L. N.; Dupnik, Kathryn M.; Sampaio, Gabriel A. A.; Nóbrega, Priscilla F. C.; Jeronimo, Ana K.; do Nascimento-Filho, Jose M.; Miranda Dantas, Roberta L.; Queiroz, Jose W.; Barbosa, James D.; Dias, Gutemberg; Jeronimo, Selma M. B.; Souza, Marcia C. F.; Nobre, Maurício L.

    2013-01-01

    Hansen's disease (leprosy) remains an important health problem in Brazil, where 34,894 new cases were diagnosed in 2010, corresponding to 15.3% of the world's new cases detected in that year. The purpose of this study was to use home visits as a tool for surveillance of Hansen's disease in a hyperendemic area in Brazil. A total of 258 residences were visited with 719 individuals examined. Of these, 82 individuals had had a previous history of Hansen's disease, 209 were their household contacts and 428 lived in neighboring residences. Fifteen new Hansen's disease cases were confirmed, yielding a detection rate of 2.0% of people examined. There was no difference in the detection rate between household and neighbor contacts (p = 0.615). The two groups had the same background in relation to education (p = 0.510), household income (p = 0.582), and the number of people living in the residence (p = 0.188). Spatial analysis showed clustering of newly diagnosed cases and association with residential coordinates of previously diagnosed multibacillary cases. Active case finding is an important tool for Hansen's disease control in hyperendemic areas, enabling earlier diagnosis, treatment, decrease in disability from Hansen's disease and potentially less spread of Mycobacterium leprae. PMID:23516645

  15. An interesting case of peripheral vascular disease, vascular reperfusion, and subsequent development of pain due to Paget's disease of bone.

    Science.gov (United States)

    Kwun, Sunna; Tucci, Joseph R

    2013-01-01

    To present a case of Paget's disease of bone that was unmasked after vascular reperfusion. In this case study, we review the presentation, evaluation, diagnosis, and management of a patient with Paget's disease and peripheral vascular disease. A 79-year-old-woman with a history of coronary artery heart disease and recent finding of a T5 compression fracture was hospitalized for evaluation of right lower extremity claudication. Angiography demonstrated a focal complete occlusion of the distal right femoral and popliteal arteries. A self-expanding stent was placed in the distal femoral and popliteal arteries. Approximately 48 hours after the procedure, the patient developed severe, right lower leg pain. On endocrine evaluation, the patient was found to have clinical signs suggesting Paget's disease of bone, which was subsequently confirmed by imaging. This patient's development of severe pain following reperfusion of distal femoral and popliteal arteries is in keeping with the known and aforementioned hypervascularity of pagetic bone. The finding of increased warmth over an area of skeletal deformation should always raise the possibility of Paget's disease of bone.

  16. Osteomesopyknosis associated to renal lithiasis. Case report. Differential diagnosis of the axial osteoesclerosant diseases

    International Nuclear Information System (INIS)

    Quintana, Gerardo; Fernandez, Andres; Restrepo, Jose Felix; Rojas, Adriana; Calvo, Enrique; Rondon, Federico; Sanchez, Alvaro; Forero, Elias; Iglesias, Antonio

    2004-01-01

    In this article we present a brief description of the bone diseases characterized by osteosclerosis. We present our experience with their morpho-radiological changes, we describe a case of osteomesopyknosis associated to renal lithiasis and we propose a classification for osteosclerosant diseases of the axial skeleton with practical differential diagnosis of these conditions

  17. Addison's Disease and Dilated Cardiomyopathy: A Case Report and Review of the Literature.

    Science.gov (United States)

    Mozolevska, Viktoriya; Schwartz, Anna; Cheung, David; Shaikh, Bilal; Bhagirath, Kapil M; Jassal, Davinder S

    2016-01-01

    Addison's disease is often accompanied by a number of cardiovascular manifestations. We report the case of a 30-year-old man who presented with a new onset dilated cardiomyopathy due to Addison's disease. The clinical presentation, treatment, and outcomes of this rare hormone mediated cardiac disorder are reviewed.

  18. Pott's disease: a case of Mycobacterium xenopi infection of the spine

    Directory of Open Access Journals (Sweden)

    Majd Alfreijat

    2013-01-01

    Full Text Available Pott's disease is an infection of the spine with Mycobacterium tuberculosis that causes destruction of the spine elements resulting in progressive kyphosis. We are describing a rare case of Pott's disease where Mycobacterium xenopi was the inculpated organism.

  19. Hydatid cyst of testis: An unusual presentation of hydatid disease - case report and review of literature

    Directory of Open Access Journals (Sweden)

    Baldev Singh

    2001-01-01

    Full Text Available Hvdatid disease is a parasitic disease usually caused by Echinococcus granulosus. It usually presents with liver involvement and uncommonly lungs and rarely other organs are involved. A rare case of hydatid testis is reported that was provisionally diagnosed as testicular tumor.

  20. Possibility of environmentally-safe casing soil disinfection for control of cobweb disease of button mushroom

    OpenAIRE

    Potočnik Ivana; Rekanović Emil; Stepnović Miloš; Milijašević-Marčić Svetlana; Todorović Biljana; Nikolić-Bujanović Ljiljana; Čekerevac Milan

    2014-01-01

    The soil-borne pathogen Cladobotryum dendroides causes cobweb disease of button mushroom (Agaricus bisporus) and its significant yield losses. Casing soil disinfection by toxic formaldehyde is a widespread practice. The aim of this study was to investigate the potential of two environmentally friendly substances, colloidal silver and peracetic acid, against C. dendroides. Their biological efficacy (impact on mushroom yield), effectiveness (disease control) ...

  1. Dermatitis herpetiformis: celiac disease of the skin. Report of two cases

    Directory of Open Access Journals (Sweden)

    Beatriz Di Martino Ortiz

    2018-02-01

    Full Text Available Dermatitis herpetiformis or Duhring-Brocq disease is a chronic, autoimmune, pleomorphic disease, characterized by lesions on extension surfaces, accompanied by intense pruritus, and is usually associated with celiac disease, gluten sensitivity, gluten sensitivity ataxia and some forms of IgA neuropathy. Two cases of dermatitis herpetiformis are presented in female patients and we make a brief review of the literature on the treatment of this pathology.

  2. How air pollution influences clinical management of respiratory diseases. A case-crossover study in Milan

    OpenAIRE

    Santus Pierachille; Russo Antonio; Madonini Enzo; Allegra Luigi; Blasi Francesco; Centanni Stefano; Miadonna Antonio; Schiraldi Gianfranco; Amaducci Sandro

    2012-01-01

    Abstract Background Environmental pollution is a known risk factor for multiple diseases and furthermore increases rate of hospitalisations. We investigated the correlation between emergency room admissions (ERAs) of the general population for respiratory diseases and the environmental pollutant levels in Milan, a metropolis in northern Italy. Methods We collected data from 45770 ERAs for respiratory diseases. A time-stratified case-crossover design was used to investigate the association bet...

  3. Constitutional hepatic dysfunction (Gilbert's disease), about eleven cases studied in the Hospital Obrero de Lima

    OpenAIRE

    León Navarro, Oswaldo

    2014-01-01

    We report eleven cases of Constitutional Hepatic dysfunction (Gilbert's disease), studied at the Department of Gastroenterology of the Hospital Obrero de Lima. We place this disease in the group of non Chronicles Hemolytic jaundice due to congenital defects in bilirubin metabolism. It is noted, according to the new concepts of bilirubin metabolism, the pathogenic mechanism of this disease is related to deficient activity of glucuronyltransferase, the enzyme responsible for bilirubin conjugati...

  4. Cushing Disease Presenting as Primary Psychiatric Illness: A Case Report and Literature Review.

    Science.gov (United States)

    Rasmussen, Sean A; Rosebush, Patricia I; Smyth, Harley S; Mazurek, Michael F

    2015-11-01

    We report the case of a woman with long-standing refractory depression and psychotic features who was eventually diagnosed with Cushing disease. After surgical treatment of a pituitary adenoma, she experienced gradual psychiatric recovery and was eventually able to discontinue all psychotropic medication. We review the psychiatric components of Cushing disease, implications of psychiatric illnesses for the treatment and prognosis of Cushing disease, and potential pathophysiological mechanisms linking glucocorticoid excess to psychiatric illness.

  5. Paradoxical euthyroid hormone profile in a case of Graves' disease with cardiac failure

    Directory of Open Access Journals (Sweden)

    Kapoor Aditya

    2011-07-01

    Full Text Available Abstract Cardiac failure is an uncommon complication of juvenile hyperthyroidism. We describe an adolescent boy with Graves' disease who developed manifestations of heart failure while on antithyroid medications. There was no evidence of any underlying cardiac disease. He had paradoxical euthyroid hormone profile which rose to hyperthyroid range when the manifestations of the cardiac failure subsided. The case highlights several unusual features of Graves' disease.

  6. A case with post-irradiation cerebral vascular disease accompanied by Cushing's disease

    International Nuclear Information System (INIS)

    Shinoda, Keiichi; Kuriyama, Takanobu; Kimura, Fumiharu; Kawamura, Hiroshi; Takamatsu, Junta; Mozai, Toshiji

    1986-01-01

    A 37-year-old man with Cushing's disease developed cerebral infarction 13 years after Co-60 irradiation of a total dose of 60 Gy for pituitary adenoma. Cerebral angiography showed stenosis or occlusion of the bilateral carotid arteries and basilar artery which were in the radiation fields. A basophilic adenoma was resected, and symptoms of Cushing's disease have resolved. The vascular lesions are likely to be not only radiation-induced, but also stimulated by hyperlipemia and hypertension associated with Cushing's disease. It is recommended to eliminate factors, such as hyperlipemia and hypertension, stimulatig vascular damage in the management of patients treated with radiation therapy. The relevant literature is reviewed. (Namekawa, K.)

  7. [Whipple's disease endocarditis: report of 5 cases and review of the literature].

    Science.gov (United States)

    Aïouaz, H; Célard, M; Puget, M; Vandenesch, F; Mercusot, A; Fenollar, F; Delahaye, F; Obadia, J F; Tebib, J; Rousset, H

    2005-10-01

    Endocarditic lesions (infectious endocarditis) associated with Whipple's disease are exceptional. We report five cases from the cardiovascular and pneumologic hospital Louis Pradel in Lyon. We have collected all cases of Tropheryma whipplei endocarditis diagnosed between 1995 and 2004. Five men with a mean age of 53 years at time of diagnosis. The symptoms were essentially cardiovascular: murmur, embolism in 3 cases, and heart failure secondary to valvular insufficiency in 2 cases. The valvular involvement, double in 3 cases, was more often aortic. Vegetations were present in all patients and valvular destruction sometimes very important. A low grade fever was present in 4 cases, associated with weight loss in 2 cases. The only extra-cardiac symptoms were arthralgias or arthritis in all cases, considered in 3 patients as seronegative rheumatoid arthritis, B27+ spondylarthritis, and psoriasic arthritis. Their was no other clinical manifestations of Whipple's disease, particularly digestive, ocular, neurologic or adenopathy, and duodenal biopsies secondarily performed in 4 cases were non contributive. This differs from literature as an extra-cardiac location was identified in 11 out of 17 cases. The diagnosis was obtained by histology and PCR on the cardiac valves, as all the patients underwent surgery. The evolution was favourable with a prolonged antibiotic therapy. These report confirms the existence of endocarditic forms of the Whipple's disease, in which the single extra-cardiac manifestation is rheumatologic, and reminds us the usefulness of histology and PCR on the cardiac valves at the time of valvular surgery.

  8. Bilateral pleural effusion and interstitial lung disease as unusual manifestations of kikuchi-fujimoto disease: case report and literature review

    Directory of Open Access Journals (Sweden)

    Magdalena Fernandez-Martinez

    2010-11-01

    Full Text Available Abstract Background Kikuchi-Fujimoto's disease (KFD, also called histiocytic necrotizing lymphadenitis, is a rare, idiopathic and self-limited condition usually characterized by cervical lymphadenopathy and fever, most often affecting young patients. Aetiology is unknown. Differential diagnosis includes mainly malignant lymphoma, tuberculous lymphadenitis and systemic lupus erythematosus (SLE, so early diagnosis is crucial. Pleuropulmonary involvement due to isolated KFD has been seldom reported. Case Presentation a 32-year-old man, on treatment for iatrogenic hypothyroidism, was admitted due to high grade fever and painful cervical lymphadenopathies. KFD was diagnosed by lymph node biopsy. Some days after admission the patient got worse, he developed generalized lymphadenopathy, bilateral pleural effusion and interstitial lung disease. All of them resolved with prednisone and after two years of following up he remains asymptomatic and without evidence of any other associated disease. Conclusion Pleural effusion and interstitial lung disease are very uncommon manifestations of KFD. In our experience, treatment with oral prednisone was effective.

  9. Chronic meningococcemia: a rare presentation of meningococcal disease: case report

    Directory of Open Access Journals (Sweden)

    Antonio Adolfo Guerra Soares Brandão

    2012-03-01

    Full Text Available Chronic meningococcemia is a rare clinical presentation within the spectrumof infections due to Neisseria meningitidis, which was first described in 1902.It is defined as a chronic and benign meningococcal bacteremia withoutmeningeal signs or symptoms with at least one week’s duration, characterizedby intermittent or continuous fever, polymorphic cutaneous rash, and migratoryarthropathy. The incidence is believed to be around 1:200,000 inhabitants. Itaffects predominantly young people and adults, and it is equally distributedbetween genders. Diagnosis may be challenging in the early stages of thedisease because of the difficulty in isolating Neisseria meningitidis (it reaches74% of positivity in advanced stages. Recently, the use of PCR for detectingNeisseria sp antigen in skin biopsies specimens has been considered for thoseculture-negative cases. The authors report a case of a 54-year-old femalepatient who sought medical attention for a five-day fever followed by arthralgiaand skin lesions predominantly in the lower limbs. The patient progressed toa toxemic clinical status that improved after the administration of antibiotictherapy, which consisted of oxacillin and ceftriaxone. The diagnosis of chronicmeningococcemia was performed after the isolation of Neisseria meningitidisin two different blood sample cultures. This is, to our knowledge, the firstcase of chronic meningococcemia described in Brazil (up to the writing of thisreport.

  10. Resource Requirements Planning for Hospitals Treating Serious Infectious Disease Cases

    Energy Technology Data Exchange (ETDEWEB)

    Vugrin, Eric D. [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Verzi, Stephen Joseph [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Finley, Patrick D. [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Turnquist, Mark A. [Cornell Univ., Ithaca, NY (United States); Wyte-Lake, Tamar [Veterans Emergency Management Evaluation Center; Griffin, Ann R. [Veterans Emergency Management Evaluation Center; Ricci, Karen J. [Veterans Emergency Management Evaluation Center; Plotinsky, Rachel [Providence Health and Services, Renton, WA (United States)

    2015-02-01

    This report presents a mathematical model of the way in which a hospital uses a variety of resources, utilities and consumables to provide care to a set of in-patients, and how that hospital might adapt to provide treatment to a few patients with a serious infectious disease, like the Ebola virus. The intended purpose of the model is to support requirements planning studies, so that hospitals may be better prepared for situations that are likely to strain their available resources. The current model is a prototype designed to present the basic structural elements of a requirements planning analysis. Some simple illustrati ve experiments establish the mo del's general capabilities. With additional inve stment in model enhancement a nd calibration, this prototype could be developed into a useful planning tool for ho spital administrators and health care policy makers.

  11. Depressive symptoms associated with hereditary Alzheimer's disease: a case description.

    Science.gov (United States)

    Contreras, Mónica Yicette Sánchez; Vargas, Paula Alejandra Osorio; Ramos, Lucero Rengifo; Velandia, Rafael Alarcón

    The authors describe a family group studied by the Centro de Biología Molecular y Biotecnología, and the Clínica de la Memoria, las Demencias y el Envejecimiento (Universidad Tecnológica de Pereira, Colombia), and evaluate the association of depressive symptoms with Alzheimer's disease (AD). This family presented a hereditary pattern for AD characterized by an early onset of dementia symptoms, a long preclinical depressive course, and, once the first symptoms of dementia appeared, a rapid progression to severe cognitive function impairment. The authors found a high prevalence of depressive symptoms in this family and propose that the symptoms could be an important risk factor for developing AD in the presence of other risk factors such as the APOE E4 allele.

  12. Occupational characteristics of cases with asbestos-related diseases in The Netherlands

    NARCIS (Netherlands)

    A. Burdorf (Alex); M. Dahhan; P. Swuste (Paul)

    2003-01-01

    textabstractOBJECTIVE: To describe the occupational background of cases with an asbestos-related disease and to present overall mesothelioma risks across industries with historical exposure to asbestos. METHODS: For the period 1990-2000, cases were collected from records held by

  13. Investigation of a cluster of ten cases of Hodgkin's disease in an occupational setting

    NARCIS (Netherlands)

    Swaen, G.M.H.; Slangen, J.J.M.; Ott, M.G.; Kusters, E.; van den Langenbergh, G.; Arends, J.W.; Zober, A.

    1996-01-01

    The objective of this study was to identify occupational exposures that might be etiologically linked to an unusual cluster of ten cases of Hodgkin's disease. The cases were identified within the active workforce of a large chemical manufacturing firm over a 23-year period by the medical director of

  14. Suicide and chronic kidney disease: a case-control study.

    Science.gov (United States)

    Liu, Chao-Han; Yeh, Ming-Kung; Weng, Shu-Chuan; Bai, Meng-Yi; Chang, Jung-Chen

    2017-09-01

    The association of chronic kidney disease (CKD) and dialysis with suicide is not well established. The objectives of this study were to assess the association of suicide with CKD and dialysis and investigate whether differences exist between dialysis modalities or the durations of dialysis. Data were obtained from the Taiwan National Health Insurance Research Database. A total of 51 642 patients who died from suicide between 2000 and 2012 and 206 568 living control patients matched by age, gender and residency area were examined. Known risk factors included sociodemographic characteristics, physical comorbidities and psychiatric disorders, which were controlled for as covariates in the analysis. The crude odds ratios (ORs) and adjusted ORs (aORs) for various risk factors were obtained using conditional logistic regression. After potential confounders were controlled for, CKD was significantly associated with an increased risk of suicide [aOR = 1.25, 95% confidence interval (CI) = 1.17-1.34]. End-stage renal disease patients on haemodialysis (HD) had an increased risk of suicide compared with controls (aOR = 3.35, 95% CI = 3.02-3.72). Moreover, patients who initially underwent dialysis within 0-3 months had a significantly increased risk of suicide (aOR = 20.26, 95% CI = 15.99-25.67). CKD and HD are positively associated with suicide. Suicide is preventable; therefore, assessing mental and physical disorders is essential and recommended to all physicians, particularly those treating patients in the early phase of HD. © The Author 2016. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved.

  15. Angiofollicular Lymph Node Hyperplasia(=Castleman's Disease)-Report of A Case-

    Energy Technology Data Exchange (ETDEWEB)

    Gil, Hak Jun; Oh, Yoon Kyung; Yoon, Sei Chul; Shinn, Kyung Sub; Bahk, Yong Whee [Catholic University College of Medicine, Seoul (Korea, Republic of)

    1987-06-15

    Angiofollicular lymph node hyperplasia (AFLNH) with well marginated lymphoid masses, is a rare benign disease of unknown etiology. The majority of the disease develop intrathoracically. Histologically this disease can be divided into the hyaline-vascular and the plasma cell types with the hyaline-vascular type prevailing. The plasma cell variant has been associated with nephritic syndrome, anemia, growth failure, fever, hyperglobulinemia, peripheral neuropathy, and hypoalbuminemia. Surgical resection is known to be treatment of choice in most cases, and radiotherapy is reserved for advanced, unresectable lesions. We report a complete remission of AFLNH in a case treated by surgical excision followed by irradiation.

  16. Conn syndrome and Crohn disease in a pediatric case: an interesting parallel.

    Science.gov (United States)

    Rodriguez, Manuel A; Alkhoury, Fuad; Malvezzi, Leopoldo; Diaz, Alejandro

    2013-01-01

    Conn syndrome is characterized by surreptitious secretion of aldosterone in which patients are found to have hypertension, hypokalemia, and metabolic alkalosis. Although rare, the most common presentation in the pediatric population is bilateral hyperplasia of the adrenal glands as opposed to an adenoma. Crohn disease is part of the spectrum of inflammatory bowel disease, which manifests in children as flare-ups of bloody diarrhea and abdominal pain. The association of concurrent Conn syndrome and Crohn disease has been previously presented in two cases in adults. This is the first pediatric case to be reported in the literature.

  17. Case report of Lewy body disease mimicking Creutzfeldt-Jakob disease in a 44-year-old man.

    Science.gov (United States)

    Saint-Aubert, Laure; Pariente, Jérémie; Dumas, Herve; Payoux, Pierre; Brandel, Jean-Philippe; Puel, Michèle; Vital, Anne; Guedj, Eric; Lesage, Suzanne; Peoc'h, Katell; Brefel Courbon, Christine; Ory Magne, Fabienne

    2016-07-30

    Few patients are reported with dementia with Lewy bodies before fifty years-old, which may partly reflect the difficulty of accurate diagnosis in young population. We report the case of a 44-year-old male with pathologically confirmed sporadic dementia with Lewy bodies, who did not fulfil the revised clinical criteria for this disease. We document this atypical case with clinical and cognitive evaluation, imaging, biochemistry, genetics and pathology investigations. Creutzfeldt-Jakob disease was first suspected in this patient with no previous medical history, who developed acute and rapid cognitive impairment, L-dopa-non-responsive parkinsonism, and delusion. Positive 14-3-3 protein was initially detected in cerebrospinal fluid and until the late stages of the disease. Severe atrophy with no diffusion hypersignal was found on structural MRI as well as an extensive hypometabolism on (18)F-FDG-PET, in comparison to age-matched healthy volunteers. Genetic investigation found no alpha-synuclein gene mutation. The patient died within 5 years, and post-mortem examination found numerous Lewy bodies and Lewy neurites consistent with pure Lewy body disease. This comprehensively described case illustrates that dementia with Lewy bodies can occur in young patients with atypical clinical presentation. Biochemistry and neuroimaging investigations can sometimes be insufficient to allow accurate diagnostic. More specific markers to support such diagnosis are needed.

  18. IgG4-related lung disease presenting as interstitial lung disease with bronchiolitis: A case report.

    Science.gov (United States)

    Chen, Chiu-Fan; Chu, Kuo-An; Tseng, Yen-Chiang; Wu, Chang-Che; Lai, Ruay-Sheng

    2017-12-01

    IgG4-related disease is a rare and novel disease entity that tends to involve multiple organs. The pulmonary manifestation of this disease is highly variable and may mimic lung cancer, pneumonia, interstitial lung disease (ILD), sarcoidosis, and so forth. Small airway disease is rarely reported in IgG4-related lung disease (IgG4-RLD). In the current study, we describe a rare case of IgG4-RLD with patterns of ILD and bronchiolitis. A 43-year-old man had chronic cough and dyspnea on exertion for 4 years. Initial chest radiography showed diffuse interstitial infiltration. Follow-up chest computed tomography 4 years later revealed bilateral diffuse centrilobular nodules with tree-in-bud pattern, bronchial wall thickening, and mediastinal lymph nodes. Bilateral diffuse multifocal ground-glass opacities and mosaic attenuation were also observed. Pulmonary function test revealed mixed restrictive and obstructive ventilatory impairment. Video-assisted thoracoscopic surgery (VATS) lung biopsy showed interstitial fibrosis with lymphoplasmacytic infiltration rich in IgG4-positive plasma cells. Serum IgG4 level also showed remarkable elevation. Therefore, IgG4-RLD is confirmed. VATS wedge resection of right upper lobe and mediastinal lymph node. The patient responded well to steroid and immunosuppression therapy, and was regular followed-up in outpatient clinic. IgG4-RLD should be considered not only in ILD, but also in small airway disease. Serum IgG4 level may be a useful tool for screening.

  19. Creutzfeldt-Jakob disease and lyophilised dura mater grafts: report of two cases.

    Science.gov (United States)

    Esmonde, T; Lueck, C J; Symon, L; Duchen, L W; Will, R G

    1993-01-01

    Two further cases of Creutzfeldt-Jakob disease (CJD) in association with cadaveric dura mater grafts are described. The clinical features of all such reported cases resemble more closely those of sporadic CJD, in contrast with kuru and the cases of CJD which have arisen after therapy with human pituitary-derived growth hormone. This observation may reflect the route of inoculation of the agent. PMID:8410042

  20. Sudden unexpected death from natural diseases: Fifteen years' experience with 484 cases in Seychelles.

    Science.gov (United States)

    Zhao, Peng; Wang, Ji-Gang; Gao, Peng; Li, Xia; Brewer, Rubell

    2016-01-01

    The aim of this study is to identify and subclassify sudden natural death (sudden death from natural diseases) cases in Seychelles. A total of 484 sudden natural death cases with autopsy at the Clinical Pathology Laboratory, Victoria Hospital, Seychelles between 1997 through 2012 were retrospectively reviewed. Among them, 363 cases (75%) were male and 121 (25%) were female. The most frequent sudden deaths were attributed to cardiovascular diseases (78.5%), and then followed by infectious diseases (9.9%), and gastrointestinal diseases (9.1%). This is the largest population-based study on sudden natural deaths in Seychelles. Copyright © 2015 Elsevier Ltd and Faculty of Forensic and Legal Medicine. All rights reserved.

  1. First Identified Case in Literature: Association of Achalasia and Celiac Diseases

    Directory of Open Access Journals (Sweden)

    Ibrahim Bucak

    2014-12-01

    Full Text Available Celiac disease has been shown to cause problems related to gastrointestinal system motility such as reduction of the esophageal sphincter pressure and prolongation of gastric emptying time. Achalasia disease is a motor disorder that is charac¬terized by the absence of esophageal peristalsis and by incomplete relaxation of the lower esophageal sphincter. Association of achalasia and celiac diseases has not been reported yet. Our patient with growth and developmental retardation had vomiting effects which lasted for 3 years. Celiac disease was diagnosed serologically and histopathologically in our patient; we determined achalasia disease with esophagoscopic examination, upper gastrointestinal system contrast study, and esophageal manometer. Esophageal balloon dilatation was applied. This case is presented because of the interesting association between celiac disease and achalasia disease.

  2. Clinical Review of 20 Cases of Addison’s Disease in Korea

    Science.gov (United States)

    Sung, Sang Kyu; Kwon, Yong Joon; Lee, Bum Woo; Kim, Doo Man; Yoo, Hyung Joon

    1988-01-01

    Addison’s disease is a rare disorder resulting from a chronic deficiency of adrenol cortical hormone. The clinical manifestations and data of 20 patients with Addison’s disease were reviewed. They include 14 previously reported cases, total cases in korea upto 1987, and 6 new cases at National Medical Center. 13 patients were male and 7 female. The mean age was 39 years and ranged from 16 to 61 years.Common symptoms included mucocutaneous pigmentation (95 %), general weakness (90%), nasuea and/or vomiting (80%), and weight loss (60%).Initial mean blood pressure was 96±21/63±14 mmHg and adrenal crises were found in 5 cases (25%).Laboratory data indicated hyponatremia and hyperkalemia, resulting in a decreased Na/K ratio. Serum basal cortisol and ACTH levels were 2.86±1.93 ug/dl and 482.5/269.3 pg/ml, respectively.Extra-adrenal tuberculosis was present in 14 cases (70%) as a very high incidence.These 14 cases (70%) were presumed to be due to adrenal tuberculosis, although only two cases were confirmed as such by histopathology. The other cases were likely to be non-tuberculous. Thus, tuberculosis may be-considered as a predominant cause of Addision’s disease in Korea. Other features were not unusual. But further detailed and extensive studies are necessary including more pathologically confirmed cases. PMID:3153796

  3. A CASE STUDY ON POINT PROCESS MODELLING IN DISEASE MAPPING

    Directory of Open Access Journals (Sweden)

    Viktor Beneš

    2011-05-01

    Full Text Available We consider a data set of locations where people in Central Bohemia have been infected by tick-borne encephalitis (TBE, and where population census data and covariates concerning vegetation and altitude are available. The aims are to estimate the risk map of the disease and to study the dependence of the risk on the covariates. Instead of using the common area level approaches we base the analysis on a Bayesian approach for a log Gaussian Cox point process with covariates. Posterior characteristics for a discretized version of the log Gaussian Cox process are computed using Markov chain Monte Carlo methods. A particular problem which is thoroughly discussed is to determine a model for the background population density. The risk map shows a clear dependency with the population intensity models and the basic model which is adopted for the population intensity determines what covariates influence the risk of TBE. Model validation is based on the posterior predictive distribution of various summary statistics.

  4. Using prediction markets of market scoring rule to forecast infectious diseases: a case study in Taiwan.

    Science.gov (United States)

    Tung, Chen-yuan; Chou, Tzu-chuan; Lin, Jih-wen

    2015-08-11

    The Taiwan CDC relied on the historical average number of disease cases or rate (AVG) to depict the trend of epidemic diseases in Taiwan. By comparing the historical average data with prediction markets, we show that the latter have a better prediction capability than the former. Given the volatility of the infectious diseases in Taiwan, historical average is unlikely to be an effective prediction mechanism. We designed and built the Epidemic Prediction Markets (EPM) system based upon the trading mechanism of market scoring rule. By using this system, we aggregated dispersed information from various medical professionals to predict influenza, enterovirus, and dengue fever in Taiwan. EPM was more accurate in 701 out of 1,085 prediction events than the traditional baseline of historical average and the winning ratio of EPM versus AVG was 64.6 % for the target week. For the absolute prediction error of five diseases indicators of three infectious diseases, EPM was more accurate for the target week than AVG except for dengue fever confirmed cases. The winning ratios of EPM versus AVG for the confirmed cases of severe complicated influenza case, the rate of enterovirus infection, and the rate of influenza-like illness in the target week were 69.6 %, 83.9 and 76.0 %, respectively; instead, for the prediction of the confirmed cases of dengue fever and the confirmed cases of severe complicated enterovirus infection, the winning ratios of EPM were all below 50 %. Except confirmed cases of dengue fever, EPM provided accurate, continuous and real-time predictions of four indicators of three infectious diseases for the target week in Taiwan and outperformed the historical average data of infectious diseases.

  5. The motor and cognitive features of Parkinson's disease in patients with concurrent Gaucher disease over 2 years: a case series.

    Science.gov (United States)

    Collins, Lucy M; Williams-Gray, Caroline H; Morris, Elizabeth; Deegan, Patrick; Cox, Timothy M; Barker, Roger A

    2018-05-29

    We report the cognitive features and progression of Parkinson's disease (PD) in five patients with concurrent Gaucher disease. The patients presented at an earlier age than patients with sporadic PD, as previously noted by others; but in contrast to many previous reports, our patients followed a variable clinical course. While two patients developed early cognitive deficits and dementia, three others remained cognitively intact over the follow-up period. Thus, in this small case series, PD in the context of GD more closely resembles idiopathic PD in terms of its clinical heterogeneity in contrast to PD associated with GBA heterozygote mutations.

  6. Adult onset glycogen storage disease type II (adult onset Pompe disease): report and magnetic resonance images of two cases

    International Nuclear Information System (INIS)

    Del Gaizo, Andrew; Banerjee, Sima; Terk, Michael

    2009-01-01

    Glycogen storage disease type II (GSDII), also referred to as Pompe disease or acid maltase deficiency, is a rare inherited condition caused by a deficiency in acid alpha-glucosidase (GAA) enzyme activity. The condition is often classified by age of presentation, with infantile and late onset variants (Laforet et al. J Neurology 55:1122-8, 2000). Late onset tends to present with progressive proximal muscle weakness and respiratory insufficiency (Winkel et al. J Neurology 252:875-84, 2005). We report two cases of biopsy confirmed adult onset GSDII, along with key Magnetic Resonance (MR) images. (orig.)

  7. Intrapartum electrocardiogram alteration in fetuses with congenital heart disease: a case-control study.

    Science.gov (United States)

    Gay, Estelle; Bornallet, Géraldine; Gaucherand, Pascal; Doret, Muriel

    2015-11-01

    To assess if the fetal electrocardiogram especially ST segment is modified by congenital heart diseases: modifications in frequencies of the different ST events and modifications in signal quality. A retrospective case-control study, comparing frequencies of the different ST events and the quality of the signal between fetuses with congenital heart diseases and fetuses without congenital heart disease. From 2000 to 2011, fifty-eight fetuses with congenital heart disease had their heart rate recording using a STAN device during labor. Control group was fetuses who were born just before a case and had a STAN as a second line for intrapartum surveillance. Cases and controls were matched on parity, gestational age at birth, presence of growth restriction and umbilical artery pH. Frequencies of the different ST event and quality of the signal were first analyzed for the global labor recording, and then separately for the first and the second phase of labor. No statistically significant difference in ST event frequencies between fetuses with congenital heart disease and the control group was found. Regarding the quality of the signal, 11.49% (±18.82) of recording time is a signal loss for fetus with congenital heart disease whereas only 5.18% (±10.67) for the control group (p=0.028). This is the first study investigating for intrapartum electrocardiogram modification in fetus with congenital heart disease. Congenital heart diseases do not modify frequencies of ST events. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

  8. Pulmonary veno-occlusive disease Report of case and revision of the literature

    International Nuclear Information System (INIS)

    Jimenez Q, Andres; Palacios, Diana; Camacho D, Fidel

    2008-01-01

    The real incidence of veno-occlusive pulmonary disease (VOPD) is unknown because it is largely under diagnosed or is not classified as primary pulmonary hypertension, being in fact a variant of the primary disease, primarily affecting post-capillary pulmonary vasculature. It is also known as isolated pulmonary venous sclerosis, obstructive disease of the pulmonary veins or primary pulmonary hypertension of the venous type. VOPD is the result of multiple aggressors associated to a great variety of risk factors. The diagnosis of the disease requires clinical, radiological and pathological features. Vasodilators, immunosuppressants, anticoagulation and oxygen have been proposed as treatments with a poor prognosis, and with reported survival limited by the scarce reporting of cases. We report a case of veno-occlusive pulmonary disease which was diagnosed in this institution.

  9. A retrospective study of diseases in Ambystoma mexicanum: a report of 97 cases.

    Science.gov (United States)

    Takami, Yoshinori; Une, Yumi

    2017-06-16

    Ambystoma mexicanum kept as pets are affected by a variety of diseases. However, no reports regarding the incidence of specific diseases are available. This study aimed to identify the diseases that occur frequently in this species by surveying the incidence of conditions in pet A. mexicanum specimens brought to a veterinary hospital. The sample comprised 97 pet A. mexicanum individuals brought to the authors' hospital during the 82-month period, i.e., from January 2008 to October 2014. In total, 116 diseases were identified. The most common disease was hydrocoelom (32 cases; 27.5% of all cases). Elucidating the pathogenesis of hydrocoelom, which has a high prevalence rate, is vital to maintaining the long-term health of A. mexicanum pets.

  10. Lyme Disease Manifestations in the Foot and Ankle: A Retrospective Case Series.

    Science.gov (United States)

    Miller, Jason R; Dunn, Karl W; Braccia, Domenick; Ciliberti, Louis J; Becker, Dina K; Hollinger, Joshua K; Brand, Shelley M

    Lyme disease is the result of Borrelia burgdorferi bacterial infection after exposure from a tick bite. A pathognomonic finding in early-stage Lyme disease is an expanding, red macular ring known as erythema migrans. Lyme arthritis is a late-stage manifestation of this disease, affecting the large, weightbearing joints with intermittent pain and swelling. The existing data on Lyme disease and subsequent arthritis have reported manifestations in the lower extremity, primarily in the knee and ankle and less commonly the small joints of the foot. We present a retrospective case series of 11 cases of painful arthritis in the foot and ankle with confirmatory Lyme disease testing. Copyright © 2015 American College of Foot and Ankle Surgeons. Published by Elsevier Inc. All rights reserved.

  11. Prenatal Diagnosis of a Case of Norrie Disease with Late Development of Bilateral Ocular Malformation.

    Science.gov (United States)

    Wu, Li Hong; Chen, Li-Hong; Xie, Hongning; Xie, Ying-Jun

    2017-06-01

    We report a case of Norrie disease, diagnosed by prenatal ultrasound, confirmed by Sanger sequencing of the DNP gene from the aborted fetal cord blood and histologically. Prenatal ultrasound revealed no abnormality in either eye at 22 +1 and 31 +4 gestational weeks, but at 36 +5 gestational weeks both eyes had massive vitreous cavity opacities with complete retinal detachment. Norrie disease was initially suspected because of an older male sibling with the disease. To our knowledge, prenatal ultrasound diagnosis of Norrie disease has been previously described only one case in 1993 in a 34-week-old fetus. The normal eye development until after 31 + 4 gestational weeks provides insight into the first manifestation and then the rapid progression of the eye disease.

  12. Multiple endocrine diseases in dogs: 35 cases (1996-2009).

    Science.gov (United States)

    Blois, Shauna L; Dickie, Erica; Kruth, Stephen A; Allen, Dana G

    2011-06-15

    To characterize a population of dogs from a tertiary care center with 2 or more endocrine disorders, including the specific disorders and time intervals between diagnosis of each disorder. Retrospective case series. 35 dogs with 2 or more endocrine disorders. Medical records were reviewed, and the following was recorded: clinical signs, physical examination findings, and the results of CBC, serum biochemical analysis, urinalysis, aerobic bacterial culture of urine samples, endocrine testing, diagnostic imaging, and necropsy. 35 dogs with more than 1 endocrine disorder were identified. Seventy-seven percent (27/35) of the dogs were male, and the mean age at the time of diagnosis of the first endocrinopathy was 7.9 years. Miniature Schnauzer was the most common breed. Twenty-eight of 35 (80%) dogs had 2 disorders; 7 (20%) had 3 disorders. The most common combinations of disorders included diabetes mellitus and hyperadrenocorticism in 57.1 % (20/35) of dogs; hypoadrenocorticism and hypothyroidism in 22.9% (8/35) of dogs; and diabetes mellitus and hypothyroidism in 28.6% (10/35) of dogs. A mean of 14.5 months elapsed between diagnosis of the first and second endocrine disorders, whereas there was a mean of 31.1 months between diagnosis of the first and third endocrine disorders. Results suggested that the occurrence of multiple endocrine disorders was uncommon in dogs. The most common combinations of endocrine disorders in this population of dogs were diabetes mellitus and hyperadrenocorticism, followed by hypoadrenocorticism and hypothyroidism.

  13. Two cases of joint disease in post-medieval church cemetery of St. Ilija.

    Science.gov (United States)

    Durić, Marija; Rakocević, Zoran; Bumbasirević, Marko; Lesić, Aleksandar; Kelecević, Julija

    2004-01-01

    Evidence of disease was analyzed from the skeletal remains of 11 individuals dating to the post-Medieval period from church cemetery of St. Ilija in Serbia. Two individuals showed pathological condition affecting joints. It was supposed that first individual had been suffering from Legg-Calvé-Perthes disease. It seems that this condition remained untreated, with extensive bone remodeling, and that the deformity of femoral head and acetabulum caused secondary degenerative joint disease at a relatively early age of this individual. Second case was related to the bony akylosis of the hand finger, probably caused by Dupuytren's disease. In addition, we discussed development of differential diagnosis in both pathological conditions.

  14. 'Cherry red spot' in a patient with Tay-Sachs disease: case report.

    Science.gov (United States)

    Aragão, Ricardo Evangelista Marrocos de; Ramos, Régia Maria Gondim; Pereira, Felipe Bezerra Alves; Bezerra, Andreya Ferreira Rodrigues; Fernandes, Daniel Nogueira

    2009-01-01

    Tay-Sachs disease is an autosomal recessive disorder of sphingolipid metabolism, caused by enzyme hexosaminidase A deficiency that leads to an accumulation of GM2 in neurocytes which results in progressive loss of neurological function. The accumulation of lipid in retinal ganglion cells that leads to a chalk-white appearance of the fundus called 'cherry red spot' is the hallmark of Tay-Sachs disease. It is also seen in others neurometabolic diseases as well as in central retinal artery occlusion. This case reports a child with Tay-Sachs disease in a family with four previous similar deaths without diagnostic.

  15. [Sandhoff's and Tay-Sachs disease--based on our own cases].

    Science.gov (United States)

    Kokot, Witold; Raczyńska, Krystyna; Krajka-Lauer, Jarosława; Iwaszkiewicz-Bilikiewicz, Barbara; Wierzba, Jolanta

    2004-01-01

    The authors described two infant with Sandhoff's and Tay-Sachs disease. Tay-Sachs disease is well-known inherited disease leading to an accumulation of gangliosides in the brain and retina. Sandhoffs disease (GM2 gangliosidosis type 0) was diagnosed in an infant, in whom a progressive neurological disorder and cherry-red foveal spots were developed. In addition, to the general clinical examination, indirect ophthalnoscopy, blood white cells enzymatic examination are used to make definitive diagnosis. In this cases, the early eye fundus examination allowed us to make the proper diagnosis. The fundus change is characterized by the "cherry-red spot" in the central area.

  16. A case of immunoglobulin G-4 related sclerosing disease mimicking lung cancer

    International Nuclear Information System (INIS)

    Kwon, Soo Hee; Lee, Young Kyung; Shim, Mi Suk; Lee, Hyang Im

    2013-01-01

    Immunoglobulin (Ig) G4-related sclerosing disease is a recently described systemic fibro-inflammatory disease associated with an elevated circulating level of IgG4 and extensive IgG4-positive lymphoplasmacytic infiltration, resulting in sclerosing inflammation involving various body organs. We experienced one case where surgery confirmed IgG4-related sclerosing disease as a solitary lung mass mimicking lung cancer. We report radiologic findings including chest computed tomography and positron emission tomography computed tomography, with clinical manifestations of IgG4-related sclerosing disease.

  17. Politics, culture, and the legitimacy of disease: the case of Buerger's disease.

    Science.gov (United States)

    Lockwood, Stephen J; Bresler, Scott C; Granter, Scott R

    2016-09-01

    Thromboangiitis obliterans (TAO) or Buerger's disease is a rare form of vasculitis with distinctive clinical and pathological features that carries significant morbidity, often leading to amputation, and is strongly associated with tobacco smoking. Despite its distinctive clinicopathological characteristics, the existence of TAO as an entity sui generis was challenged for many years as it languished in relative obscurity. Then, as societal attitudes towards smoking changed, TAO not only became accepted as a disease entity, it quite literally became a poster child to illustrate the ills of smoking. Herein, we examine the history of TAO to illustrate the power of societal attitudes and politics in shaping medicine.

  18. Depression in Parkinson's disease: A case-control study.

    Directory of Open Access Journals (Sweden)

    Yu-Hsuan Wu

    Full Text Available To evaluate the association between Parkinson's disease (PD prognosis and the patient's onset of depression.A total of 353 patients with newly-diagnosed PD and a history of depression were enrolled. On the basis of the onset of depression before or after PD diagnosis, we divided participants into PD patients with pre- or post-diagnostic depression. Cox's regression analysis was used to detect risks between the onset of depression and outcomes (including death, accidental injury, dementia, and aspiration pneumonia. The association between the onset of depression and levodopa equivalent dosage (LED and cumulative equivalent dosage of antidepressants were assessed.PD patients with post-diagnostic depression were associated with significantly higher risks of dementia (adjusted HR = 2·01, p = 0·015, and were older (58·5 ± 17·7 vs. 53·7 ± 18·6, p = 0·020 at the time of PD diagnosis than PD patients with pre-diagnostic depression. The higher incident rate of accidental injury was also noted in PD patients with post-diagnostic depression (48·1 vs. 31·3/1000 person-years, HR = 1·60, p = 0·041, but no statistical significance was observed in the adjusted hazard ratio (HR (HR = 1·52, p = 0·069. Otherwise, mortality, motor condition and severity of depression revealed no significant difference between PD patients with pre-diagnostic and post-diagnostic depression.PD patients with post-diagnostic depression had higher incidence of dementia, implying different onset time of depression could be associated with different subtypes and spreading routes which should be examined in follow-up studies.

  19. A Case-Control study of the prevalence of neurological diseases in inflammatory bowel disease (IBD

    Directory of Open Access Journals (Sweden)

    Francisco de Assis Aquino Gondim

    2015-02-01

    Full Text Available Neurological diseases are common in inflammatory bowel disease (IBD patients, but their exact prevalence is unknown. Method We prospectively evaluated the presence of neurological disorders in 121 patients with IBD [51 with Crohn's disease (CD and 70 with ulcerative colitis (UC] and 50 controls (gastritis and dyspepsia over 3 years. Results Our standard neurological evaluation (that included electrodiagnostic testing revealed that CD patients were 7.4 times more likely to develop large-fiber neuropathy than controls (p = 0.045, 7.1 times more likely to develop any type of neuromuscular condition (p = 0.001 and 5.1 times more likely to develop autonomic complaints (p = 0.027. UC patients were 5 times more likely to develop large-fiber neuropathy (p = 0.027 and 3.1 times more likely to develop any type of neuromuscular condition (p = 0.015. Conclusion In summary, this is the first study to prospectively establish that both CD and UC patients are more prone to neuromuscular diseases than patients with gastritis and dyspepsia.

  20. General gastroscopy of gastroesophageal reflux disease: Analysis of 4086 cases

    Directory of Open Access Journals (Sweden)

    Zhi-wei HU

    2018-04-01

    Full Text Available Objective To analyze the characteristics of gastroesophageal reflux disease (GERD under general gastroscope. Methods The detection rates of GERD related abnormalities such as esophagitis, Barrett esophagus and hiatal hernia under the first gastroscopy of the adult GERD patients from January 2013 to January 2017 in our center and the statistical relationship between the abnormal findings were analyzed retrospectively. Results A total of 4086 GERD patients, 2004 males and 2082 females, were included in this study, and the age was 18-89(50.4±13.3 years old. The detection rate of non erosive GERD was 78.7%, esophagitis 21.3%; non Barrett esophagus 87.7%, suspected Barrett esophagus 8.3%, Barrett esophagus 3.9%; generally normal cardia 61.4%, short segment hiatus hernia 20.4%, and long segment hiatal hernia 18.2%. The detection rates of esophagitis showed statistically significant differences (P0.013. Comparing the three age groups of 18-39, 40-59 and ≥60 years old, the detection rate of hiatal hernia was significantly higher in the group of ≥60 years old than in the 18-39 and 40-59 years old groups (P=0.007, while there was no significant difference (P>0.013 between the 18-39 and 40-59 years old groups. The detection rate of esophagitis was significantly higher in ≥60 years old group than in 18-39 and 40-59 years old groups (P=0.004, P=0.008, while no significant statistically difference (P>0.013 was found between the later two groups. Conclusions Gastroscopy can be used as a basic examination means for GERD; short segment hiatal hernia can be regarded as an early form of hiatal hernia, and is of importantreference value for the diagnosis and treatment of GERD; more serious hiatal hernia and esophagitis could be found in elderly GERD patients. DOI: 10.11855/j.issn.0577-7402.2018.01.08

  1. A case of immunoglobulin G4-related respiratory disease with multiple lung cysts: A case report

    Directory of Open Access Journals (Sweden)

    Hironori Mikumo

    2017-01-01

    Full Text Available A 48-year-old man was admitted for evaluation of abnormal shadows on chest radiograph. Chest computed tomography (CT showed cysts, nodules, and cervical and axillary lymphadenopathies. Elevated serum levels of IgG4 and interleukin (IL-6 suggested IgG4-related disease (IgG4-RD or multicentric Castleman's disease (MCD. Histologic findings of the cervical lymph node and right lung S6 biopsies revealed numerous IgG4-positive plasma cells. Although CT findings of the lungs were atypical for IgG4-RD, consistent histologic findings, clinical symptoms, and laboratory data made us conclude IgG4-RD. Because histologic findings of IgG4-RD and MCD have similarities, differentiating between the two diseases should consider the clinical presentation.

  2. First reported case of unilateral Graves' disease in the left lobe of a bilobar thyroid gland.

    Science.gov (United States)

    Chen, Louis C; Green, Jennifer B

    2011-06-01

    Unilateral Graves' disease is a rare disease variant that can occur in a bilobar thyroid gland. We report the first documented case of unilateral Graves' disease in the left lobe of a bilobar thyroid gland and review the pertinent literature. A 48-year-old man presented in June 2010 with thyrotoxicosis. I-131 radioisotope uptake was elevated at 33.4%, and scintigraphy revealed that uptake of the radioisotope was uniformly increased in the left lobe of the thyroid gland. Ultrasonography of the thyroid gland revealed a non-nodular, enlarged, and heterogeneous left lobe; Doppler investigation of the lobe showed hypervascularity classically seen in Graves' disease. The right lobe of the thyroid, on the other hand, appeared homogeneous and hypovascular on ultrasonography. Thyroid-stimulating immunoglobulin was significantly elevated at 191% (reference range disease was the most likely diagnosis. As has occasionally been described in the literature, unilateral involvement of the thyroid gland is a rare presentation of Graves' disease. Pre-existing functional or structural differences (either congenital or acquired) between the two lobes may contribute to this rare presentation. To our knowledge, this is the first reported case of unilateral Graves' disease presenting in the left lobe of a bilobar thyroid gland. Although the pathophysiology of unilateral Graves's disease has not been clearly elucidated, clinicians should be aware that Graves' disease can present unilaterally in either lobe of the thyroid gland.

  3. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): a clinicoradiological profile of three cases including two with skeletal disease

    International Nuclear Information System (INIS)

    Gupta, Priya; Babyn, Paul

    2008-01-01

    Originally described as sinus histiocytosis with massive lymphadenopathy, Rosai-Dorfman disease (RDD) is a rare histiocytic proliferative disorder with a distinctive microscopic appearance. A rare entity, RDD is often under-diagnosed because of a low index of suspicion by both radiologist and pathologist. Through this article, we wish to apprise radiologists of the spectrum of disease that can be encountered in this disorder. RDD can mimic other common childhood skeletal diseases including benign Langerhans cell histiocytosis and lymphoma. The clinical and radiological manifestations of RDD vary depending upon organ involvement, and its imaging features are often confused with those of other disorders. RDD should be considered in the differential diagnosis of unifocal and multifocal skeletal involvement caused by granulomatous diseases, infections, pseudogranulomatous lesions and malignancy. As long-term outcome is usually good, a conservative approach is justified in most cases. Contrasted with its typical appearance, presenting with bilateral symmetrical cervical adenopathy (as shown in one patient), we also report extranodal involvement of bone in two patients. Extranodal disease occurs along with concomitant nodal disease in about 43% of patients. In 23% of patients, isolated extranodal RDD can be seen, most commonly in the head and neck. In two of our patients, we observed extranodal involvement with skeletal involvement away from the head and neck not associated with lymphadenopathy. Skeletal involvement in RDD without lymphadenopathy is rare, occurring only in 2% of all the patients reported to date. (orig.)

  4. A Case of Sporadic Creutzfeldt-Jakob Disease Presenting as Conversion Disorder.

    Science.gov (United States)

    Yegya-Raman, Nikhil; Aziz, Rehan; Schneider, Daniel; Tobia, Anthony; Leitch, Megan; Nwobi, Onyi

    2017-01-01

    Background . Creutzfeldt-Jakob disease is a rare disorder of the central nervous system. Its initial diagnosis may be obscured by its variable presentation. This case report illustrates the complexity of diagnosing this disease early in the clinical course, especially when the initial symptoms may be psychiatric. It offers a brief review of the literature and reinforces a role for consultation psychiatry services. Methods . PUBMED/MEDLINE was searched using the terms "Creutzfeldt-Jakob disease", "psychiatric symptoms", "conversion disorder", "somatic symptom disorder", "functional movement disorder", and "functional neurologic disorder". Case . The patient was a 64-year-old woman with no prior psychiatric history who was initially diagnosed with conversion disorder and unspecified anxiety disorder but soon thereafter was discovered to have Creutzfeldt-Jakob disease. Discussion . This case highlights the central role of psychiatric symptoms in early presentations of Creutzfeldt-Jakob disease. Still, few other cases in the literature report functional neurological symptoms as an initial sign. The consultation psychiatrist must remain alert to changing clinical symptoms, especially with uncharacteristic disease presentations.

  5. [Coexisting systemic lupus erythematosus and sickle cell disease: case report and literature review].

    Science.gov (United States)

    Robazzi, Teresa Cristina M V; Alves, Crésio; Abreu, Laís; Lemos, Gabriela

    2015-01-01

    To report a case of coexisting systemic lupus erythematosus (SLE) and sickle cell disease (SCD) with a review of the literature on the topic. Report of case and research of the association between SLE and SCD in literature through scientific articles in health sciences databases, such as LILACS, MEDLINE/Pubmed and Scielo, until May 2012. Descriptors used: 1. Sickle cell anemia; 2. Sickle cell disease; 3. Systemic lupus erythematosus; 4. Hemoglobinopathies. The authors describe an association between SLE and SS hemoglobinopathy in an eight-year-old female patient displaying articular, hematologic and neuropsychiatric manifestations during clinical evolution. Forty-five cases of association between SLE and SCD are described in literature, mostly adult (62.2%), women (78%) and with the SS phenotype in 78% of the cases, and different clinical manifestations. Compared with our patient, articular, hematologic and neuropsychiatric manifestations were present in 76%, 36% and 27% of the cases, respectively. SLE and SCD are chronic diseases that have several clinical and laboratory findings in common, meaning difficult diagnosis and difficulty in finding the correct treatment. Although the association between these diseases is not common, it is described in literature, so it is imperative that physicians who treat such diseases be alert to this possibility. Copyright © 2012 Elsevier Editora Ltda. All rights reserved.

  6. Positive 14-3-3 and tau proteins in a sporadic Creutzfeldt-Jakob disease case and a brief perspective of prion diseases in Colombia.

    Science.gov (United States)

    Escandón-Vargas, Kevin; Zorrilla-Vaca, Andrés; Corral-Prado, Raúl Heli

    2016-02-24

    Prion diseases are rare neurodegenerative disorders occurring worldwide and affecting both humans and animals. Herein, we present the case of a patient diagnosed with definite sporadic Creutzfeldt-Jakob disease in Cali, Colombia. Besides neurological examination, 14-3-3 and tau proteins were valuable tools supporting the diagnosis. We also present a brief perspective of the prion diseases reported in Colombia to date. Although the incidence of prion diseases is unknown in Colombia, our literature review revealed that one case of scrapie in 1981 and 29 human sporadic cases of Creutzfeldt-Jakob disease have been documented and published in our country.

  7. Reducing selection bias in case-control studies from rare disease registries.

    Science.gov (United States)

    Cole, J Alexander; Taylor, John S; Hangartner, Thomas N; Weinreb, Neal J; Mistry, Pramod K; Khan, Aneal

    2011-09-12

    In clinical research of rare diseases, where small patient numbers and disease heterogeneity limit study design options, registries are a valuable resource for demographic and outcome information. However, in contrast to prospective, randomized clinical trials, the observational design of registries is prone to introduce selection bias and negatively impact the validity of data analyses. The objective of the study was to demonstrate the utility of case-control matching and the risk-set method in order to control bias in data from a rare disease registry. Data from the International Collaborative Gaucher Group (ICGG) Gaucher Registry were used as an example. A case-control matching analysis using the risk-set method was conducted to identify two groups of patients with type 1 Gaucher disease in the ICGG Gaucher Registry: patients with avascular osteonecrosis (AVN) and those without AVN. The frequency distributions of gender, decade of birth, treatment status, and splenectomy status were presented for cases and controls before and after matching. Odds ratios (and 95% confidence intervals) were calculated for each variable before and after matching. The application of case-control matching methodology results in cohorts of cases (i.e., patients with AVN) and controls (i.e., patients without AVN) who have comparable distributions for four common parameters used in subject selection: gender, year of birth (age), treatment status, and splenectomy status. Matching resulted in odds ratios of approximately 1.00, indicating no bias. We demonstrated bias in case-control selection in subjects from a prototype rare disease registry and used case-control matching to minimize this bias. Therefore, this approach appears useful to study cohorts of heterogeneous patients in rare disease registries.

  8. Reducing selection bias in case-control studies from rare disease registries

    Directory of Open Access Journals (Sweden)

    Mistry Pramod K

    2011-09-01

    Full Text Available Abstract Background In clinical research of rare diseases, where small patient numbers and disease heterogeneity limit study design options, registries are a valuable resource for demographic and outcome information. However, in contrast to prospective, randomized clinical trials, the observational design of registries is prone to introduce selection bias and negatively impact the validity of data analyses. The objective of the study was to demonstrate the utility of case-control matching and the risk-set method in order to control bias in data from a rare disease registry. Data from the International Collaborative Gaucher Group (ICGG Gaucher Registry were used as an example. Methods A case-control matching analysis using the risk-set method was conducted to identify two groups of patients with type 1 Gaucher disease in the ICGG Gaucher Registry: patients with avascular osteonecrosis (AVN and those without AVN. The frequency distributions of gender, decade of birth, treatment status, and splenectomy status were presented for cases and controls before and after matching. Odds ratios (and 95% confidence intervals were calculated for each variable before and after matching. Results The application of case-control matching methodology results in cohorts of cases (i.e., patients with AVN and controls (i.e., patients without AVN who have comparable distributions for four common parameters used in subject selection: gender, year of birth (age, treatment status, and splenectomy status. Matching resulted in odds ratios of approximately 1.00, indicating no bias. Conclusions We demonstrated bias in case-control selection in subjects from a prototype rare disease registry and used case-control matching to minimize this bias. Therefore, this approach appears useful to study cohorts of heterogeneous patients in rare disease registries.

  9. Type 1 diabetes mellitus, coeliac disease, and lymphoma: a report of four cases.

    LENUS (Irish Health Repository)

    O'Connor, T M

    2012-02-03

    INTRODUCTION: Patients with Type 1 diabetes mellitus have a high prevalence of coeliac disease, symptoms of which are often mild, atypical, or absent. Untreated coeliac disease is associated with an increased risk of malignancy, particularly of lymphoma. We describe four patients with Type 1 diabetes mellitus and coeliac disease who developed lymphoma. CASE REPORTS: Two patients were male and two female. In three patients, coeliac disease and lymphoma were diagnosed simultaneously. Enteropathy-associated T cell lymphoma occurred in two patients, Hodgkin\\'s disease in one, and B cell lymphoma in one. Response to treatment was in general poor, and three patients died soon after the diagnosis of lymphoma was made. CONCLUSION: As the relative risk of lymphoma is reduced by a gluten-free diet, a high index of suspicion for coeliac disease should exist in all Type 1 diabetic patients with unexplained constitutional or gastrointestinal symptoms.

  10. Vocal symptoms in Parkinson disease treated with levodopa. A case report.

    Science.gov (United States)

    Schley, W S; Fenton, E; Niimi, S

    1982-01-01

    This is a report of a patient with unusually severe hoarseness in the absence of vocal fold pathology demonstrating Parkinson disease as one of the neurological diseases in which vocal symptoms occur. Although it is classifiably a severe, progressive, degenerative disorder, the popularity of pharmacotherapy for Parkinson disease during the past decade has resulted in improved functionality for an undetermined course of time in most patients. The classically described deterioration of speech ad voice may develop in a variant manner difficult to distinguish as disease-related, as this case report illustrates. An explanation of the hoarseness based on dyssynchronous vocal fold motion related to the disease is suggested by the acoustic methods (spectrography, waveform analysis) used in this study, and supported by strobe light laryngoscopy. This conclusion is important because of the extremely high incidences of varying degrees of hoarseness reported in recent studies of Parkinson disease.

  11. Case Report: Ursodeoxycholic acid treatment in Niemann-Pick disease type C; clinical experience in four cases

    Science.gov (United States)

    Movsesyan, Nina; Platt, Frances M.

    2017-01-01

    In this case series, we demonstrate that Ursodeoxycholic acid (UDCA) improves liver dysfunction in Niemann-Pick type C (NPC) and may restore a suppressed cytochrome p450 system. NPC disease is a progressive neurodegenerative lysosomal storage disease caused by mutations in either the NPC1 or NPC2 genes. Liver disease is a common feature presenting either acutely as cholestatic jaundice in the neonatal period, or in later life as elevated liver enzymes indicative of liver dysfunction. Recently, an imbalance in bile acid synthesis in a mouse model of NPC disease was linked to suppression of the P450 detoxification system and was corrected by UDCA treatment. UDCA (3α, 7β-dihydroxy-5β-cholanic acid), a hydrophilic bile acid, is used to treat various cholestatic disorders. In this report we summarise the findings from four independent cases of NPC, three with abnormal liver enzyme levels at baseline, that were subsequently treated with UDCA. The patients differed in age and clinical features, they all tolerated the drug well, and in those with abnormal liver function, there were significant improvements in their liver enzyme parameters. PMID:29119141

  12. Unexplained infertility as primary presentation of celiac disease, a case report and literature review

    Directory of Open Access Journals (Sweden)

    Mohammadreza Ghadir

    2011-01-01

    Full Text Available Background: Celiac sprue (gluten sensitive enteropathy is an autoimmune disease which is hereditary and its pathology mainly bases on immunologic intolerance to gluten. It has a vast variety of signs and symptoms and its clinical features range from a silent disease to a typical gastrointestinal disorder. In this study we reviewed and summarized some other related issues about this disease and its relation with infertility.Case: The case is a 26 years old lady who had referred to a gynecologist because of infertility for 2 years and later it revealed that she has celiac sprue.Conclusion: Screening for its silent or subtle types especially among suspicious cases such as unexplained infertility seems to be a cost effective action. Meanwhile, in time administration of a gluten-free diet can lead to an almost complete cure

  13. Perforated Duodenum – An Unusual Etiology of Fournier's Disease: A Case Report

    Directory of Open Access Journals (Sweden)

    Yung-Chin Lee

    2003-12-01

    Full Text Available Fournier's disease, a form of necrotizing fasciitis, is a rapidly progressing subcutaneous infection of the male genitalia. We report a case of Fournier's disease with the unusual etiology of a perforated duodenum. This patient suffered from progressive right scrotal swelling after percutaneous transhepatic cholangeal drainage. Scrotal exploration revealed a large abscess with muscle necrosis that had spread up to the right retroperitoneal space. Radiologic studies and second abdominal exploration documented the origin as a perforated duodenum. The pus distribution in this case suggested that the infection process differed from that in previous reports. In future cases of Fournier's disease involving previous abdominal events, we recommend that abdominal origins be carefully surveyed before scrotal exploration.

  14. Presence of voltage-gated potassium channel complex antibody in a case of genetic prion disease.

    Science.gov (United States)

    Jammoul, Adham; Lederman, Richard J; Tavee, Jinny; Li, Yuebing

    2014-06-05

    Voltage-gated potassium channel (VGKC) complex antibody-mediated encephalitis is a recently recognised entity which has been reported to mimic the clinical presentation of Creutzfeldt-Jakob disease (CJD). Testing for the presence of this neuronal surface autoantibody in patients presenting with subacute encephalopathy is therefore crucial as it may both revoke the bleak diagnosis of prion disease and allow institution of potentially life-saving immunotherapy. Tempering this optimistic view is the rare instance when a positive VGKC complex antibody titre occurs in a definite case of prion disease. We present a pathologically and genetically confirmed case of CJD with elevated serum VGKC complex antibody titres. This case highlights the importance of interpreting the result of a positive VGKC complex antibody with caution and in the context of the overall clinical manifestation. 2014 BMJ Publishing Group Ltd.

  15. Leptospira Exposure and Patients with Liver Diseases: A Case-Control Seroprevalence Study

    Science.gov (United States)

    Alvarado-Esquivel, Cosme; Sánchez-Anguiano, Luis Francisco; Hernández-Tinoco, Jesús; Ramos-Nevárez, Agar; Margarita Cerrillo-Soto, Sandra; Alberto Guido-Arreola, Carlos

    2016-01-01

    The seroepidemiology of Leptospira infection in patients suffering from liver disease has been poorly studied. Information about risk factors associated with infection in liver disease patients may help in the optimal planning of preventive measures. We sought to determine the association of Leptospira IgG seroprevalence and patients with liver diseases, and to determine the characteristics of the patients with Leptospira exposure. We performed a case-control study of 75 patients suffering from liver diseases and 150 age- and gender-matched control subjects. Diagnoses of liver disease included liver cirrhosis, steatosis, chronic hepatitis, acute hepatitis, and amoebic liver abscess. Sera of participants were analyzed for the presence of anti- Leptospira IgG antibodies using a commercially available enzyme immunoassay. Anti-Leptospira IgG antibodies were found in 17 (22.7%) of 75 patients and in 15 (10.0%) of 150 control subjects (OR = 2.32; 95% CI: 1.09-4.94; P=0.03). This is the first age- and gender-matched case control study about Leptospira seroprevalence in patients with liver diseases. Results indicate that Leptospira infection is associated with chronic and acute liver diseases. Results warrants for additional studies on the role of Leptospira exposure in chronic liver disease. PMID:27493589

  16. Two cases of possible neuro-Sweet disease with meningoencephalitis as the initial manifestation

    Directory of Open Access Journals (Sweden)

    Go Makimoto

    2012-02-01

    Full Text Available We report 2 cases that were considered to be neuro-Sweet disease. They initially manifested with meningoencephalitis and no skin lesions, and rapidly improved with corticosteroid therapy. In both cases, patients complained of meningitic symptoms such as fever and headache, and HLA-B54 and -Cw1 turned out to be positive over the clinical course. Cerebrospinal fluid analysis showed increased levels of lymphocytes and protein. In case #1, fluid-attenuated inversion recovery (FLAIR, magnetic resonance imaging (MRI and diffusion-weighted images (DWI showed high-intensity signals in the right dorsal medulla oblongata, bilateral dorsal midbrain, and left thalamus. In case #2, FLAIR and DWI showed high-intensity signals in the bilateral cerebellar cortex and left caudate nucleus. Symptoms and MRI images were markedly improved in both cases after corticosteroid pulse therapy. According to published diagnostic criteria, these 2 cases were considered possible neuro-Sweet disease. These cases suggest that the combination of meningoencephalitis and HLA specificity is important to consider the possibility of neuro-Sweet disease, even without skin lesions.

  17. Multifocal oral melanoacanthoma associated with Addison's disease and hyperthyroidism: a case report.

    Science.gov (United States)

    Dantas, Thinali Sousa; Nascimento, Isabelly Vidal do; Verde, Maria Elisa Quezado Lima; Alves, Ana Paula Negreiros Nunes; Sousa, Fabrício Bitu; Mota, Mário Rogério Lima

    2017-01-01

    Oral melanoacanthoma is a mucocutaneous, pigmented, rare, benign, and probably reactive lesion. This paper reports for the first time in the literature a case of multifocal oral melanoacanthoma in a patient diagnosed with Addison's disease and concomitant Graves' disease with hyperthyroidism. The patient presented with oral pigmented lesions, which were hypothesized to be mucosal pigmentation associated with Addison's disease. Due to their unusual clinical pattern, these oral lesions were biopsied and diagnosed as oral melanoacanthoma on histopathology and immunohistochemistry for HMB-45. At the moment of this report, the patient was being treated for her systemic conditions, but the lesions had not regressed. Reactive hyperpigmentation of the skin and mucous membranes may be found in Addison's disease and hyperthyroidism. This case reinforces the hypothesis of a reactive nature for oral melanoacanthoma and highlights the need for investigation of endocrine disorders in patients with multifocal oral melanoacanthoma.

  18. Visual art therapy in sporadic Creutzfeldt-Jakob disease: a case study.

    Science.gov (United States)

    Shrestha, Rajeet; Trauger-Querry, Barbara; Loughrin, Athena; Appleby, Brian S

    2016-01-01

    This paper describes the diagnostic and treatment utility of visual art therapy in a case of sporadic Creutzfeldt-Jakob disease. Visual art therapy was compared longitudinally with clinical and neuroimaging data over five-month period in an autopsy-confirmed case of sporadic Creutzfeldt-Jakob disease of MM2-cortical subtype. Art therapy sessions and content were useful in ascertaining neuropsychiatric symptoms during the course of her illness. Art therapy offered a unique emotional and cognitive outlet as illness progressed. Patients and families affected by sporadic Creutzfeldt-Jakob disease may benefit from art therapy despite the rapidly progressive nature of the illness. Art therapy can also be useful for assessment of patients with sporadic Creutzfeldt-Jakob disease by healthcare professionals.

  19. The effect of long working hours on cerebrovascular and cardiovascular disease; A case-crossover study.

    Science.gov (United States)

    Shin, Kyong-Sok; Chung, Yun Kyung; Kwon, Young-Jun; Son, Jun-Seok; Lee, Se-Hoon

    2017-09-01

    This study investigated the relationship between weekly working hours and the occurrence of cerebro-cardiovascular diseases using a case-crossover study design. We investigated average working hours during the 7 days before the onset of illness (hazard period) and average weekly working hours between 8 days and 3 months before the onset of cerebro-cardiovascular diseases (control period) for 1,042 cases from the workers' compensation database for 2009. Among all subjects, the odds ratio by conditional logistic regression for the risk of cerebro-cardiovascular diseases with a 10 hr increase in average weekly working hours was 1.45 (95% confidence interval [CI]: 1.22-1.72), a significant association. An increase in average weekly working hours may trigger the onset of cerebro-cardiovascular disease. Am. J. Ind. Med. 60:753-761, 2017. © 2017. Wiley Periodicals, Inc. © 2017 Wiley Periodicals, Inc.

  20. Pneumocystis jirovecii pneumonia in systemic autoimmune rheumatic disease: A case-control study.

    Science.gov (United States)

    Tadros, Susan; Teichtahl, Andrew J; Ciciriello, Sabina; Wicks, Ian P

    2017-06-01

    Pneumocystis jirovecii pneumonia (PJP) is an opportunistic fungal infection that affects the immunocompromised. Patients with systemic autoimmune rheumatic disease are increasingly recognised as an at-risk clinical population with a high mortality. This case-control study examined differences in the characteristics and peripheral blood parameters between patients with systemic autoimmune rheumatic disease who developed PJP and gender, age and disease-matched controls. Historical data collected between 2002 and 2013 at the Royal Melbourne Hospital, Australia were reviewed. Cases were defined by having a systemic autoimmune rheumatic disease and a diagnosis of PJP (either a positive toluidine blue O stain or P. jirovecii PCR, with a concurrent respiratory illness that was clinically consistent with PJP). Controls were matched for age, gender and disease in a 4:1 ratio. Peripheral blood results were retrieved from an in-house pathology database. Clinical information including glucocorticoid exposure, PJP prophylaxis, comorbidities and month of admission were retrieved from medical notes. After adjustment for corticosteroid exposure and C-reactive protein, lymphocyte count on admission (0.4 vs. 1.3; p = 0.04) and at nadir (0.2 vs. 0.8 × 10 9 /L; p = 0.05) was significantly lower in cases than in controls. Cases (n = 11) were more frequently Caucasian rather than non-Caucasian (81.8% vs. 65.9%; p = 0.04). In addition, cases more commonly presented in autumn (March to May) than in other seasons (OR = 7.3; 95% CI: 1.4-38.7; p = 0.02). These data demonstrate that patients with systemic autoimmune rheumatic disease who develop PJP have significantly greater lymphopenia than age, gender and disease-matched controls, independent of corticosteroid exposure, as well as a potential ethnicity and seasonal predilection to PJP. This may help to inform prophylactic guidelines for PJP in these patients. Copyright © 2017 Elsevier Inc. All rights reserved.

  1. Celiac disease symptoms in a female collegiate tennis player: a case report.

    Science.gov (United States)

    Leone, James E; Gray, Kimberly A; Massie, John E; Rossi, Jennifer M

    2005-01-01

    To present the case of a collegiate tennis player with celiac disease symptoms. Celiac disease is a common intestinal disorder that is often confused with other conditions. It causes severe intestinal damage manifested by several uncomfortable signs and symptoms. Failure by the sports medicine staff to recognize symptoms consistent with celiac disease and treat them appropriately can have deleterious consequences for the athlete. Irritable bowel syndrome, Crohn disease, Addison disease, lupus erythematosus, juvenile rheumatoid arthritis, lactose intolerance, herpes zoster, psychogenic disorder (depression), fibromyalgia, complex regional pain syndrome, hyperthyroidism, anemia, type I diabetes. The athlete underwent a series of blood and allergen tests to confirm or refute a diagnosis of celiac disease. When celiac disease was suspected, dietary modifications were made to eliminate all wheat-based and gluten-based products from the athlete's diet. The athlete was able to fully compete in a competitive National Collegiate Athletic Association Division I tennis program while experiencing the debilitating effects associated with celiac disease. The immediacy of symptom onset was notable because the athlete had no history of similar complaints. Celiac disease is a potentially life-threatening condition that affects more people than reported. A properly educated sports medicine staff can help to identify symptoms consistent with celiac disease early, so damage to the intestine is minimized. Prompt recognition and appropriate management allow the athlete to adjust the diet accordingly, compete at a high-caliber level, and enjoy a healthier quality of life.

  2. Case Reports of Cat Scratch Disease with Typical and Atypical Clinical Manifestations: A Literature Review

    Directory of Open Access Journals (Sweden)

    Gulshan Umbreen

    2017-04-01

    Full Text Available Cat scratch disease (CSD is the most well-known zoonotic disease spread by domestic animals like cats. Cats are the source of Bartonella henselae. Most patients more than ninety percent 3-12 days after a scratch from a cat, undoubtedly a little cat with insects present with one or more erythematous injuries at the site of inoculation, the sore is typically a crusted papule or, once in a while, a pustule. More than half of cases in one study show that the systemic indications went with the lymphadenopathy. These may incorporate fever, discomfort, migraine and anorexia and frequently happen in immunocompromised patients. Atypically clinical manifestations happen are altered mental status, perplexity, prolonged fever, respiratory protestations (atypical pneumonitis, Joint pain, synovitis, Back agony is uncommon. The hypothesis of the study to find out that cat scratch disease cause typical and atypical clinical manifestation. Study was conducted July 2015 to September 2015. The methodology sections of a review article are listed all of the databases and citation indexes that were searched such as Web of Science and PubMed and any individual journals that were searched. Various case reports were mentioned in the study. Case reports of cat scratch diseases with typical and atypical clinical manifestation included in the study. The objective of review of these reporting cases is to make physicians aware about cat scratch diseases and also need to create awareness about cat scratch disease in pet owner. Although it is self-limiting needs to report to health authorities. There are few cases reported in which mostly cases reported in twain, japan, Brazil, Texas, United States, Dhaka, Spain with typical and atypical clinical manifestation

  3. Pulmonary arteriovenous fistulae associated with Rendu-Osler-Weber disease: report of two cases and review of the literature

    International Nuclear Information System (INIS)

    Souza, Ricardo Pires de; Setubal, Roger; Soares, Ademir Humberto; Gomes, Marcio Rogerio Alcala; Mayo, Suzete Varela

    1996-01-01

    Pulmonary arteriovenous fistula (PAVF) is an uncommon disease with approximately 500 cases report in the literature and its association with Rendu-Osler-Weber (ROW) disease is well established. This study relates two cases of PAVF associated with ROW disease and proceeds a literature review with particularly emphasis on imaging diagnosis modalities used nowadays. (author). 14 refs., 6 figs

  4. Hyaline vascular-type castleman disease presenting as an esophageal submucosal tumor: case report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Ki Nam; Lee, Ki Nam; Kang, Myong Jin; Roh, Mee Sook; Choi, Pil Jo; Yang, Doo Kyung [Donga University, Pusan (Korea, Republic of)

    2006-03-15

    Castleman disease is a relatively rare disorder of lymphoid tissue that involves the gastrointestinal tract in a variety of clinical and pathologic manifestation. A submucosal location has never been described in the medical literature. We report a case of esophageal Castleman disease involving the submucosal layer in a 62-year-old man, which was confirmed on pathology. Esophagography and CT demonstrated an intramural tumor, and a leiomyoma or leiomyosarcoma was suspected based on the known incidence of such tumors.

  5. Gaucher disease diagnosed after bone marrow trephine biopsy — a report of two cases

    Directory of Open Access Journals (Sweden)

    Anna Dmoszyńska

    2011-07-01

    Full Text Available The hematologist is at the forefront of specialists to whom patients with Gaucher disease present because of cytopenia and hepatosplenomegaly. Usually, patients with such symptoms have undergone trephine biopsy. We present the cases of two patients in whom Gaucher disease was suspected because of the discovery of Gaucher cells in trephine biopsy, and subsequently confirmed via enzymatic and molecular investigations. (Folia Histochemica et Cytobiologica 2011; Vol. 49, No. 2, pp. 352–356

  6. HYPNOSIS IN THE TREATMENT OF MORGELLONS DISEASE: A Case Study1

    OpenAIRE

    Gartner, Ashley M.; Dolan, Sara L.; Stanford, Matthew S.; Elkins, Gary R.

    2011-01-01

    Morgellons Disease is a condition involving painful skin lesions, fibrous growths protruding from the skin, and subcutaneous stinging and burning sensations, along with symptoms of anxiety, depression, fatigue, and memory and attention deficits. The etiological and physiological bases of these symptoms are unclear, making the diagnosis controversial and challenging to treat. There are currently no established treatments for Morgellons Disease. The following case example depicts treatment of a...

  7. Surgery or radiotherapy for the treatment of bone hydatid disease: a retrospective case series

    Directory of Open Access Journals (Sweden)

    Zengru Xie

    2015-04-01

    Conclusion: This retrospective case series describes, for the first time, the clinical outcomes in a series of patients treated with radiotherapy for bone hydatid disease. Although no direct comparison between the treatment groups could be made due to methodological limitations of the study design, this study indicates that well-designed prospective randomized controlled clinical trials assessing radiotherapy may be warranted in patients with inoperable hydatid disease of the bones.

  8. Capgras delusion for animals and inanimate objects in Parkinson?s Disease: a case report

    OpenAIRE

    Islam, Lucrezia; Piacentini, Sylvie; Soliveri, Paola; Scarone, Silvio; Gambini, Orsola

    2015-01-01

    Background Capgras delusion is a delusional misidentification syndrome, in which the patient is convinced that someone that is well known to them, usually a close relative, has been replaced by an impostor or double. Although it has been frequently described in psychotic syndromes, including paranoid schizophrenia, over a third of the documented cases of Capgras delusion are observed in patients with organic brain lesions or neurodegenerative disease, including Parkinson?s Disease. Variants o...

  9. Computed tomographic follow-up in a case of Addison's disease

    International Nuclear Information System (INIS)

    Pozzi Mucelli, R.S.; Pozzi Mucelli, F.; Muner, G.

    1985-01-01

    Addison's disease of the adrenal glands presents with different pathological findings depending on the stage. In the acute phase the adrenal glands are bilaterally enlarged while in the chronic phase the glands are small and calcified. A case of Addison's disease with follow-up over a period of a year by CT is reported. CT showed the evolution of the adrenal glands from bilateral masses to small calcified glands. The differential diagnostic problems in the acute phase are also discussed. (orig.)

  10. A Case of Advanced Unicentric Retroperitoneal Castleman's Disease, Associated With Psoriasis

    Directory of Open Access Journals (Sweden)

    Mohammad Ali Mohagheghi

    2017-05-01

    Full Text Available We present here a 32-year-old male with advanced lately diagnosed, right sided retroperitoneal mass, which had been already treated due to progressive muco-cutaneous lesions clinically consistent with psoriasis, during recent four years. The advanced retroperitoneal mass resected surgically and reported as hyaline-vascular castleman disease with a dense focus of coarse calcification, on histopathology. Association of psoriasis and castlman disease is discussed in this case report. 

  11. IgG4-related disease with sinonasal involvement: A case series

    International Nuclear Information System (INIS)

    Prabhu, Shailesh M; Yadav, Vikas; Irodi, Aparna; Mani, Sunithi; Varghese, Ajoy Mathew

    2014-01-01

    We present the imaging findings in two cases of IgG4-related disease involving the sinonasal region in the pediatric age group. Imaging findings in IgG4-related disease affecting the nasal cavity and paranasal sinuses have been rarely reported in literature. The diagnosis is made by a combination of clinical, imaging, and histopathologic findings. Radiologists should be aware of the imaging findings of this condition to ensure early diagnosis and treatment

  12. Case series of Creutzfeldt-Jakob disease in a third-level hospital in Quito.

    Science.gov (United States)

    Torres Herrán, Germaine Eleanor; Ortega Herrera, Andrés Damián; Burbano, Braulio Martinez; Serrano-Dueñas, Marcos; Ortiz Yepez, María Angélica; Barrera Madera, Raúl Alberto; Masabanda Campaña, Luis Alfredo; Baño Jiménez, Guillermo David; Santos Saltos, Denny Maritza; Correa Díaz, Edgar Patricio

    2018-04-27

    Creutzfeldt-Jakob disease is a rare and fatal neurodegenerative disorder that affects mammals and humans. The prevalence of this disease in the United States is 0.5 to 1 per million inhabitants. So far in Ecuador, we do not know what the prevalence or incidence is, and only one case report has been written. We present a case series of Creutzfeldt-Jakob disease in a third-level hospital in Quito. The average age of symptom onset in our patients was 58.8 years. The male to female ratio was 1:1. Two patients began with cognitive/behavioral symptoms, while 4 patients began with focal neurological signs; 1 case with ataxia, 2 with gait disorders and 1 with vertigo and headache. All of the patients had the clinical features established by the World Health Organization. In addition, the entire cohort was positive for the 14-3-3 protein in cerebrospinal fluid, and had high signal abnormalities in caudate and putamen nucleus in DWI and FLAIR IRM. Only in one case, did we reach a definitive diagnosis through a pathological study. All other cases had a probable diagnosis. In this series of cases, 6 out of 6 patients died. The average time from the onset of the symptoms to death in this cohort was 13 months. This is the first report of a series of cases of Creutzfeldt-Jakob disease in Quito. Although definitive diagnosis must be histopathological, there are ancillary tests currently available that have allowed us to obtain a diagnosis of the disease.

  13. Pulmonary talc granulomatosis mimicking malignant disease 30 years after last exposure: a case report

    Directory of Open Access Journals (Sweden)

    Krimsky William S

    2008-07-01

    Full Text Available Abstract Introduction Pulmonary talc granulomatosis is a rare disorder characterized by the development of foreign body granuloma secondary to talc exposure. Previous case reports have documented the illness in current intravenous drug users who inject medications intended for oral use. We present a rare case of the disease in a patient with a distant history of heroin abuse who presented initially with history and imaging findings highly suggestive of malignancy. Case presentation A 53-year-old man reported a 4-month history of increasing dyspnea and weight loss. He had a long history of smoking and admission chest X-ray revealed a density in the right hemithorax. Computed tomography confirmed a probable mass with further speculated opacities in both lung fields suspicious for malignant spread. Biopsies obtained using endobronchial ultrasound-guided aspiration returned negative for malignancy and showed bronchial epithelial cells with foreign body giant cell reaction and polarizable birefringent talc crystals. Conclusion This case demonstrates a rare presentation of talc granulomatosis three decades after the last likely exposure. The history and imaging findings in a chronic smoker were initially strongly suggestive of malignant disease, and we recommend that talc-induced lung disease is considered in any patient with multiple scattered pulmonary lesions and a history of intravenous drug use. Confirmation of the disease by biopsy is essential, but unfortunately there are few successful proven management options for patients with worsening disease.

  14. A Kikuchi-Fujimoto Disease Case Mimicking T Cell Lymphoma with Prolonged Fever

    Directory of Open Access Journals (Sweden)

    Serap Yadigar

    2014-01-01

    Full Text Available Kikuchi-Fujimoto disease (KFD is a self-limited disease characterized by necrotizing lymphadenitis. Although cervical lymphadenitis in young women is the most familiar clinical presentation, it may take place in the etiology of fever in cases presenting with fever of unknown origin. A 33-year-old male case admitted with fever, nausea, vomiting, weight loss, and leukopenia for one month, subsequently developing axillary lymphadenopathy during followup, diagnosed as KFD with typical histopathological findings, and showing full recovery after the excision of lymph node was presented in this report.

  15. Use of Sugammadex in Strumpell-Lorrain Disease: a Report of Two Cases

    Directory of Open Access Journals (Sweden)

    José Antonio Franco-Hernández

    2013-01-01

    Full Text Available Content: : Strumpell-Lorrain disease - or familial spastic paraplegia (FSP - is a rare hereditary neurological disorder, mainly characterized by variable degrees of stiffness and weakening of the muscles, with cognitive impairment, deafness, and ataxia in the more severe cases. We describe two female siblings with FSP programmed for cholecystectomy and subtotal colectomy, respectively, and also how we dealt with the anesthetic management in both cases and review the literature on this disease in relation to anesthesia. Keywords: Spastic Paraplegia, Hereditary, Anesthesia, Neuromuscular blockade/sugammadex.

  16. Eosinophilic Gastrointestinal Disorder in Coeliac Disease: A Case Report and Review

    Directory of Open Access Journals (Sweden)

    Dennis N. F. Lim

    2012-01-01

    Full Text Available Eosinophilic gastrointestinal disorder is a rare disorder characterised by eosinophilic infiltration of the gastrointestinal tract. There are various gastrointestinal manifestations with eosinophilic ascites being the most unusual and rare presentation. Diagnosis requires high index of suspicion and exclusion of various disorders associated with peripheral eosinophilia. There are no previous case reports to suggest an association between eosinophilic gastrointestinal disorder and coeliac disease in adults. We report a case of eosinophilic ascites and gastroenteritis in a 30-year-old woman with a known history of coeliac disease who responded dramatically to a course of steroids.

  17. Completed suicide in an autopsy-confirmed case of early onset Alzheimer's disease.

    Science.gov (United States)

    Hartzell, Jennifer Wiener; Geary, Richard; Gyure, Kymberly; Chivukula, Venkata Ravi; Haut, Marc W

    2018-04-01

    We report a case of a 57-year-old male with clinically diagnosed and autopsy-confirmed early onset Alzheimer's disease who completed suicide by gunshot wound to the chest. This case has several unique aspects that have not been discussed in previous case reports of completed suicide in Alzheimer's disease. In particular, our patient's death was highly planned with successful compensation for his cognitive deficits. After all firearms had been removed from the home as a safety precaution, he obtained a new weapon, hid it and left himself cues to find and use it. The case is discussed in the context of literature differentiating the neural circuitry propagating impulsive versus planned suicidal acts.

  18. Association between bullous pemphigoid and neurologic diseases: a case-control study.

    Science.gov (United States)

    Casas-de-la-Asunción, E; Ruano-Ruiz, J; Rodríguez-Martín, A M; Vélez García-Nieto, A; Moreno-Giménez, J C

    2014-11-01

    In the past 10 years, bullous pemphigoid has been associated with other comorbidities and neurologic and psychiatric conditions in particular. Case series, small case-control studies, and large population-based studies in different Asian populations, mainland Europe, and the United Kingdom have confirmed this association. However, no data are available for the Spanish population. This was an observational, retrospective, case-control study with 1:2 matching. Fifty-four patients with bullous pemphigoid were selected. We compared the percentage of patients in each group with concurrent neurologic conditions, ischemic heart disease, diabetes, chronic obstructive pulmonary disease, and solid tumors using univariate logistic regression. An association model was constructed with conditional multiple logistic regression. The case group had a significantly higher percentage of patients with cerebrovascular accident and/or transient ischemic attack (odds ratio [OR], 3.06; 95% CI, 1.19-7.87], dementia (OR, 5.52; 95% CI, 2.19-13.93), and Parkinson disease (OR, 5; 95% CI, 1.57-15.94). A significantly higher percentage of cases had neurologic conditions (OR, 6.34; 95% CI, 2.89-13.91). Dementia and Parkinson disease were independently associated with bullous pemphigoid in the multivariate analysis. Patients with bullous pemphigoid have a higher frequency of neurologic conditions. Copyright © 2013 Elsevier España, S.L.U. and AEDV. All rights reserved.

  19. A case developing minimal change disease during the course of IgG4-related disease.

    Science.gov (United States)

    Yamada, Kazunori; Zoshima, Takeshi; Ito, Kiyoaki; Mizushima, Ichiro; Hara, Satoshi; Horita, Shigeto; Nuka, Hiromi; Hamano, Ryoko; Fujii, Hiroshi; Yamagishi, Masakazu; Kawano, Mitsuhiro

    2017-07-01

    We describe a 66-year-old male with immunoglobulin G4-related disease (IgG4-RD) presenting with minimal change disease (MCD). Three years prior to this admission, the patient had been diagnosed with IgG4-RD. The development of sudden massive proteinuria (4+; 16.7 g/gCr) with a weight gain of 8 kg within a two-week period was noted, and nephrotic syndrome was suspected. The patient's serum IgG4 level did not increase and hypocomplementemia was not found. A renal biopsy showed no cellular infiltration in the renal interstitium, and no spiking or bubbling was found on periodic acid methenamine silver staining. On electron microscopy, foot process effacement was seen, but no subepithelial electron-dense deposits were found. The patient was diagnosed with MCD. Ten days after starting prednisolone (60 mg/day), proteinuria was negative. Since IgG4-RD and MCD share a T-helper 2-dominant immunoreaction, the development of MCD in IgG4-RD patients may reflect more than a mere coincidence.

  20. Lhermitte-Duclos disease associated with Cowden's syndrome: Case report and review of the literature

    International Nuclear Information System (INIS)

    Hernandez Restrepo, Michel; Neira Escobar, Fabian; Borbon Garzon, Alejandra and others

    2011-01-01

    Cerebellar gangliocytoma or Lhermitte-duclos disease is an unusual pathology, with few reports in the medical literature. it is a tumoral lesion of hamartomatous origin located on the cerebellar cortex, with clinical manifestations related to mass effect at the posterior fossa. In some cases, it is associated with multiple hamartomatous neoplasms or Cowden's syndrome. This report describes a case assessed at Hospital Universitario de la Samaritana and its histolopathogical confirmation, and includes a review of its more relevant semiological and clinical features.

  1. A Rare Case of Parkinson's Disease with Severe Neck Pain Owing to Crowned Dens Syndrome

    OpenAIRE

    Teruyuki Takahashi; Masato Tamura; Keiichi Osabe; Takashi Tamiya; Kenji Miki; Mai Yamaguchi; Kanno Akira; Satoshi Kamei; Toshiaki Takasu

    2014-01-01

    Background: Pain is regarded as one of the most common nonmotor symptoms in Parkinson's disease (PD). In particular, musculoskeletal pain has been reported as the most common type of PD-associated pain. Crowned dens syndrome (CDS), related to microcrystalline deposition in the periodontoid process, is the main cause of acute or chronic cervical pain. Case Presentation: This report describes the case of an 87-year-old woman who had severe bradykinesia, muscle rigidity, gait disturbance and nec...

  2. Occupational characteristics of cases with asbestos-related diseases in The Netherlands

    OpenAIRE

    Burdorf, Alex; Dahhan, M.; Swuste, Paul

    2003-01-01

    textabstractOBJECTIVE: To describe the occupational background of cases with an asbestos-related disease and to present overall mesothelioma risks across industries with historical exposure to asbestos. METHODS: For the period 1990-2000, cases were collected from records held by two law firms. Information on jobs held, previous employers, activities performed and specific products used were obtained from patients themselves or next of kin. Branches of industry and occupations were coded and t...

  3. Ethical Dilemmas in Protecting Individual Rights versus Public Protection in the Case of Infectious Diseases

    Directory of Open Access Journals (Sweden)

    Kai-Lit Phua

    2013-01-01

    Full Text Available Infectious diseases—including emerging and re-emerging diseases such as Ebola and tuberculosis—continue to be important causes of morbidity and mortality in the globalizing, contemporary world. This article discusses the ethical issues associated with protecting the rights of individuals versus the protection of the health of populations in the case of infectious diseases. The discussion uses the traditional medical ethics approach together with the public health approach presented by Faden and Shebaya. 3 Infectious diseases such as Ebola hemorrhagic fever, Nipah virus and HIV/AIDS (together with tuberculosis will be used to illustrate particular points in the discussion.

  4. Small Bowel Adenocarcinoma Complicating Coeliac Disease: A Report of Three Cases and the Literature Review

    Directory of Open Access Journals (Sweden)

    Hafida Benhammane

    2012-01-01

    Full Text Available Coeliac disease is associated with an increased risk of malignancy, not only of intestinal lymphoma but also of small intestinal adenocarcinoma which is 82 times more common in patients with celiac disease than in the normal population. We report three additional cases of a small bowel adenocarcinoma in the setting of coeliac disease in order to underline the epidemiological features, clinicopathological findings, and therapeutic approaches of this entity based on a review of the literature. The three patients underwent a surgical treatment followed by adjuvant chemotherapy based on capecitabine/oxaliplatin regimen, and they have well recovered.

  5. CELIAC DISEASE IN CHILDREN. A HISTORY CASE WITH ONSET AT THE AGE OF 17 YEARS

    Directory of Open Access Journals (Sweden)

    M. O. Revnova

    2013-01-01

    Full Text Available Being one of the most common representatives of malabsorbtion syndrome celiac disease has been diagnosed more and more often in Russia. Celiac disease is a hereditary condition with high prevalence and different symptoms called «Great Mimic». The article deals with diagnostics based on testing the antibodies to tTG, DPG, biopsies of the duodenum and gluten free diet. There is given an example of severe case of celiac disease in a 17-years-old boy with weight loss, delayed sexual development and severe gastrointestinal symptoms. Gluten free diet and proper treatment led to permanent remission.

  6. Histological Changes in Autoimmune Hepatitis with Graves' Disease: A Child Case Report.

    Science.gov (United States)

    Yamada, Mamiko; Shibata, Hironori; Masugi, Yohei; Ishi, Tomohiro; Kameyama, Kaori; Ebinuma, Hirotoshi; Hasegawa, Tomonobu

    2017-08-15

    We herein report a child case of autoimmune hepatitis (AIH) accompanied with Graves' disease. Elevated aminotransferase levels were found in a 12-year-old Japanese girl with Graves' disease. In her first liver biopsy, necrosis and inflammation was limited to the centrilobular area, while the second biopsy showed different findings. Namely, portal injury newly appeared, including interface hepatitis, which represents the histological characteristics of AIH. As the histological findings at the onset of AIH do not always show typical findings, a re-biopsy is considered to be important in individuals suspected to have AIH. AIH should be included in the differential diagnosis of liver dysfunction in Graves' disease, even in children.

  7. Fibrocystic disease of vulvar ectopic breast tissue. Case report and review of the literature.

    Science.gov (United States)

    Baykal, C; Tulunay, G; Usubutun, A; Küçükali, T; Ozer, S; Demir, O F

    2004-01-01

    Mammary glands located in the vulvar region have been named as ectopic breast tissue or anogenital mammary glands by different authors. Literature on pathologies of ectopic breast tissue located in the vulvar region is rare. Most of the reports are about the malignancies arising from this ectopic tissue. We report a case of fibrocystic disease of the mammary glands in the vulva in a 25-year-old pregnant woman. Her disease was exaggerated during pregnancy. Ectopic breast tissue in the vulva is a rare entity and fibrocystic disease of this tissue has rarely been reported in the English literature. Copyright (c) 2004 S. Karger AG, Basel.

  8. Subglotic Stenosis as manifestation in Wegener’s Disease. A case report

    Directory of Open Access Journals (Sweden)

    Sofía VALLE-OLSEN

    2017-03-01

    Full Text Available Introduction and objective: Subglottic stenosis in Wegener’s disease (WD represents a diagnostic challenge because it’s ability to be the unique manifestation. Description: A case of subglottic GW described in a woman 14 years. Discussion: Subglottic stenosis secondary to Wegener’s disease use to be associated with ENT symptoms, so these can give us the etiological suspicion. In the absence of these, we should include it in the differential diagnosis and try a histopathologic confirmation. Conclusions: In an isolated subglottic stenosis, rule Wegener's disease performing a biopsy of nasal mucosa and two serology tests.

  9. First documented case of snake fungal disease in a free-ranging wild snake in Louisiana

    Science.gov (United States)

    Glorioso, Brad M.; Waddle, J. Hardin; Green, David E.; Lorch, Jeffrey M.

    2016-01-01

    Snake fungal disease (SFD) is a recently documented mycotic disease characterized by scabs or crusty scales, subcutaneous nodules, abnormal molting, cloudiness of the eyes (not associated with molting), and localized thickening or crusting of the skin. SFD has been documented in many species in the Eastern and Midwestern United States within the last decade. SFD has proven lethal in many snakes, and the disease is recognized as an emerging threat to wild snake populations. Here, we describe the first documented case of SFD in Louisiana in a free-ranging wild snake.

  10. Tumefactive immunoglobulin G4-related disease involving the dura mater: A case report and literature review

    International Nuclear Information System (INIS)

    Lee, Jeong Hoon; Lee, Ji Hoon; Ko, Yong; Paik, Seoung Sam; Lee, Young Jun; Park, Dong Woo

    2015-01-01

    Immunoglobulin G4 (IgG4)-related disease is a well-known disorder characterized by an inflammatory reaction with an increase in the number of IgG4-positive plasma cells associated with sclerosis. IgG4-related disease often affects the dura mater with a pattern of diffuse thickening when the central nervous system is involved. However, some nodular dural thickening requires discrimination from tumors because of obviously different treatment options. We report of a case of IgG4-related disease with tumefactive dural involvement

  11. Crohn’s disease and Trabecular Metal implants: a report of two cases and literature review

    Directory of Open Access Journals (Sweden)

    C. Peron

    2015-10-01

    Full Text Available Aim The aim of the present study was to report two cases with Crohn’s disease in whom dental implants successfully osseointegrated and remained functionally stable up to 13 and 12 months of follow-up, respectively. Cases presentation In cases 1 (age 35 years and 2 (age 36 years, tooth 24 and 14, respectively, were atraumatically extracted and a particulated bone grafting material (buccal and palatal aspect of the defect and a Trabecular Metal implant (11.5 mm length, 4.7 mm diameter were inserted in each extraction socket. After implant placement and abutment connection with the final torque (25 Ncm, the provisional restoration was adapted in the oral cavity creating the emergence profile. The provisional crown was screw-retained and had slight occlusal contacts in the centric occlusion (intercuspation position. A periapical radiograph was taken as a control radiograph at the baseline. Postoperatively, antibiotics were prescribed as well as analgesics and an oral rinse was recommended. In both cases, the provisional restoration was removed after 2 weeks and replaced with a full ceramic restoration. Case-1 and case-2 were followed up after 13 months and 12 months respectively. In both cases postoperative healing was uneventful and radiographs taken at follow-up showed no evidence of crestal bone loss. Implants in both cases demonstrated an excellent clinical condition at follow-up. Conclusion Trabecular Metal implants can osseointegrate and remain functionally stable in patients with Crohn’s disease.

  12. Intrathecal antibody production in two cases of yellow fever vaccine associated neurotropic disease in Argentina.

    Science.gov (United States)

    Pires-Marczeski, Fanny Clara; Martinez, Valeria Paula; Nemirovsky, Corina; Padula, Paula Julieta

    2011-12-01

    During the period 2007-2008 several epizootics of Yellow fever with dead of monkeys occurred in southeastern Brasil, Paraguay, and northeastern Argentina. In 2008 after a Yellow fever outbreak an exhaustive prevention campaign took place in Argentina using 17D live attenuated Yellow fever vaccine. This vaccine is considered one of the safest live virus vaccines, although serious adverse reactions may occur after vaccination, and vaccine-associated neurotropic disease are reported rarely. The aim of this study was to confirm two serious adverse events associated to Yellow fever vaccine in Argentina, and to describe the analysis performed to assess the origin of specific IgM against Yellow fever virus (YFV) in cerebrospinal fluid (CSF). Both cases coincided with the Yellow fever vaccine-associated neurotropic disease case definition, being clinical diagnosis longitudinal myelitis (case 1) and meningoencephalitis (case 2). Specific YFV antibodies were detected in CSF and serum samples in both cases by IgM antibody-capture ELISA. No other cause of neurological disease was identified. In order to obtain a conclusive diagnosis of central nervous system (CNS) infection the IgM antibody index (AI(IgM) ) was calculated. High AI(IgM) values were found in both cases indicating intrathecal production of antibodies and, therefore, CNS post-vaccinal YFV infection could be definitively associated to YFV vaccination. Copyright © 2011 Wiley Periodicals, Inc.

  13. Creutzfeldt-Jakob disease: report of four cases and review of the literature.

    Science.gov (United States)

    Atalay, Fatma Öz; Tolunay, Şahsine; Özgün, Gonca; Bekar, Ahmet; Zarifoğlu, Mehmet

    2015-01-01

    Creutzfeldt-Jakob disease is a very rare, progressive neurodegenerative disorder that is incurable and always fatal. It is one of the transmissible spongiform encephalopathies caused by prions. Multiple vacuoles in neuropil and neuronal loss in the gray matter gives the classical sponge-like appearance of brain and are responsible for the typical clinical symptoms. In this report, we present 4 cases referred to the neurology department of Uludağ University with neurological symptoms. Patients were evaluated with electroencephalogram and magnetic resonance imaging, and performed brain biopsies for further investigation. For definitive diagnosis of Creutzfeldt-Jakob disease, accumulation of prion protein in brain was detected immunohistochemically. Patients died within weeks in consequence of rapid progression of the disease. Although Creutzfeldt-Jakob disease is an infrequent disorder, when a patient presents with characteristic clinical symptoms such as rapidly progressive dementia with myoclonus, the diagnosis of Creutzfeldt-Jakob disease should be taken into consideration.

  14. Thyrotropin-producing pituitary adenoma simultaneously existing with Graves' disease: a case report.

    Science.gov (United States)

    Arai, Nobuhiko; Inaba, Makoto; Ichijyo, Takamasa; Kagami, Hiroshi; Mine, Yutaka

    2017-01-06

    Thyrotropin-producing pituitary tumor is relatively rare. In particular, concurrent cases associated with Graves' disease are extremely rare and only nine cases have been reported so far. We describe a case of a thyrotropin-producing pituitary adenoma concomitant with Graves' disease, which was successfully treated. A 40-year-old Japanese woman presented with mild signs of hyperthyroidism. She had positive anti-thyroid-stimulating hormone receptor antibody, anti-thyroglobulin antibody, and anti-thyroid peroxidase antibody. Her levels of serum thyroid-stimulating hormone, which ranged from low to normal in the presence of high levels of serum free thyroid hormones, were considered to be close to a state of syndrome of inappropriate secretion of thyroid-stimulating hormone. Magnetic resonance imaging showed a macropituitary tumor. The coexistence of thyrotropin-producing pituitary adenoma and Graves' disease was suspected. Initial therapy included anti-thyroid medication, which was immediately discontinued due to worsening symptoms. Subsequently, surgical therapy for the pituitary tumor was conducted, and her levels of free thyroid hormones, including the thyroid-stimulating hormone, became normal. On postoperative examination, her anti-thyroid-stimulating hormone receptor antibody levels decreased, and the anti-thyroglobulin antibody became negative. The coexistence of thyrotropin-producing pituitary adenoma and Graves' disease is rarely reported. The diagnosis of this condition is complicated, and the appropriate treatment strategy has not been clearly established. This case suggests that physicians should consider the coexistence of thyrotropin-producing pituitary adenoma with Graves' disease in cases in which thyroid-stimulating hormone values range from low to normal in the presence of thyrotoxicosis, and the surgical treatment of thyrotropin-producing pituitary adenoma could be the first-line therapy in patients with both thyrotropin-producing pituitary adenoma

  15. Phenotype and natural history of elderly onset inflammatory bowel disease: a multicentre, case-control study.

    Science.gov (United States)

    Mañosa, M; Calafat, M; de Francisco, R; García, C; Casanova, M J; Huelín, P; Calvo, M; Tosca, J; Fernández-Salazar, L; Arajol, C; Zabana, Y; Bastida, G; Hinojosa, J; Márquez, L; Barreiro-de-Acosta, M; Calvet, X; Monfort, D; Gómez-Garcia, M R; Rodríguez, E; Huguet, J M; Rojas-Feria, M; Hervias, D; Atienza, R; Busquets, D; Zapata, E; Dueñas, C; Charro, M; Martínez-Cerezo, F J; Plaza, R; Vázquez, J M; Gisbert, J P; Cañete, F; Cabré, E; Domènech, E

    2018-03-01

    Onset during old age has been reported in upto 10% of total cases of inflammatory bowel disease (IBD). To evaluate phenotypic characteristics and the use of therapeutic resources in patients with elderly onset IBD. Case-control study including all those patients diagnosed with IBD over the age of 60 years since 2000 who were followed-up for >12 months, identified from the IBD databases. Elderly onset cases were compared with IBD patients aged 18 to 40 years at diagnosis, matched by year of diagnosis, gender and type of IBD (adult-onset). One thousand three hundred and seventy-four elderly onset and 1374 adult-onset cases were included (62% ulcerative colitis (UC), 38% Crohn's disease (CD)). Among UC patients, elderly onset cases had a lower proportion of extensive disease (33% vs 39%; P < 0.0001). In CD, elderly onset cases showed an increased rate of stenosing pattern (24% vs 13%; P < 0.0001) and exclusive colonic location (28% vs 16%; P < 0.0001), whereas penetrating pattern (12% vs 19%; P < 0.0001) was significantly less frequent. Regarding the use of therapeutic resources, there was a significantly lower use of corticosteroids (P < 0.0001), immunosuppressants (P < 0.0001) and anti-TNFs agents (P < 0.0001) in elderly onset cases. Regarding surgery, we found a significantly higher surgery rate among elderly onset UC cases (8.3% vs 5.1%; P < 0.009). Finally, elderly onset cases were characterised by a higher rate of hospitalisations (66% vs 49%; P < 0.0001) and neoplasms (14% vs 0.5%; P < 0.0001). Elderly onset IBD shows specific characteristics and they are managed differently, with a lower use of immunosuppressants and a higher rate of surgery in UC. © 2018 John Wiley & Sons Ltd.

  16. Familial aggregation of Alzheimer's disease and related disorders: A collaborative re-analysis of case-control studies

    NARCIS (Netherlands)

    C.M. van Duijn (Cornelia); D.G. Clayton (David); V. Chandra; L. Fratiglioni (Laura); A.B. Graves; A. Heyman; A.F. Jorm; E. Kokmen (Emre); K. Kondo; J.A. Mortimer; W.A. Rocca; S.L. Shalat; H. Soininen; A. Hofman (Albert)

    1991-01-01

    textabstractCase-control studies of Alzheimer's disease were re-analysed to examine the association of Alzheimer's disease with family history in first degree relatives of dementia, Down's syndrome and Parkinson's disease. Overall, the relative risk of Alzheimer's disease for those with at least one

  17. Syngeneic graft-versus-host disease: a report of two cases and literature review.

    Science.gov (United States)

    Latif, T; Pohlman, B; Kalaycio, M; Sobecks, R; Hsi, E D; Andresen, S; Bolwell, B J

    2003-09-01

    Rappeport et al first reported the clinical syndrome of graft-versus-host disease (GVHD) in syngeneic bone marrow transplant patients. Recently, there have been more reports of a GVHD-like syndrome in syngeneic bone marrow transplant patients (SGVHD) that may result in significant clinical morbidity. A total of 17 cases of SGVHD in syngeneic bone marrow transplant patients have been reported to date in the medical literature. The current report reviews these cases and presents two additional cases of severe SGVHD that have occurred at our institution.

  18. Malaysia's First Transplanted Case of Chronic Granulomatous Disease: The Journey of Overcoming Obstacles.

    Science.gov (United States)

    Ismail, Intan Hakimah; Jamli, Faizah Mohamed; Othman, Ida Shahnaz; Noh, Lokman Mohd; Abdul Latiff, Amir Hamzah

    2016-05-17

    The awareness of primary immunodeficiency (PID) in Malaysia is still not forthcoming. Certain practical issues such as lack of clinical immunologists and specialized laboratory diagnostic facilities remain to be addressed. However, great efforts taken by passionate clinicians and scientists in the immunology networking have ascertained some prevalence. Despite the limitation, all suspected cases of PID are being properly investigated and competently managed. In this case report we highlighted the obstacles we faced in managing PID patients, particularly preparing for bone marrow transplant. This is the first transplanted case of chronic granulomatous disease in Malaysia, which emphasizes the importance of collaborative work to ensure further morbidities or mortalities are prevented.

  19. Malaysia’s First Transplanted Case of Chronic Granulomatous Disease: The Journey of Overcoming Obstacles

    Science.gov (United States)

    Ismail, Intan Hakimah; Jamli, Faizah Mohamed; Othman, Ida Shahnaz; Noh, Lokman Mohd; Abdul Latiff, Amir Hamzah

    2016-01-01

    The awareness of primary immunodeficiency (PID) in Malaysia is still not forthcoming. Certain practical issues such as lack of clinical immunologists and specialized laboratory diagnostic facilities remain to be addressed. However, great efforts taken by passionate clinicians and scientists in the immunology networking have ascertained some prevalence. Despite the limitation, all suspected cases of PID are being properly investigated and competently managed. In this case report we highlighted the obstacles we faced in managing PID patients, particularly preparing for bone marrow transplant. This is the first transplanted case of chronic granulomatous disease in Malaysia, which emphasizes the importance of collaborative work to ensure further morbidities or mortalities are prevented. PMID:27417247

  20. The case-fatality rate of meningococcal disease in Catalonia, 1990-1997.

    Science.gov (United States)

    Domínguez, Angela; Cardeñosa, Neus; Pañella, Helena; Orcau, Angels; Companys, Maria; Alseda, Miquel; Oviedo, Manuel; Carmona, Glòria; Minguell, Sofia; Salleras, Lluis

    2004-01-01

    The objective was to analyse the case-fatality rate (CFR) of meningococcal disease (MD) in Catalonia, Spain. A retrospective study was carried out. Clinical histories of cases of MD reported for the period 1990-1997 in Catalonia were reviewed. For all cases, the variables gender, age, clinical type, y of presentation, province, phenotype and death by meningococcal disease were collected. The association between death and the other variables was studied by bivariate and unconditional logistic regression analysis. In the 2343 cases studied there were 146 deaths (6.2%) due to meningococcal disease. The CFR was higher in females (OR: 1.5, 95%CI: 1.1-2.1), in the 20 to 49 y (OR: 2.4, 95%CI: 1.2-4.9) and > or = 50 y (OR: 5.3, 95%CI: 2.8-10.1) age groups, in cases with septicaemia (OR: 2.4, 95%CI: 1.6-3.5), in the cases produced by serogroup A (OR: 4.7, 95%CI: 1.0-23.4) and in cases occurring during 1993 (OR: 2.1, 95%CI: 1.1-4.1) or in the province of Lleida (OR: 2.9, 95%CI: 1.2-7.2). In the multivariate analysis, death was associated with the 20-49 y age group (OR: 3.9, 95%CI: 1.8-8.4), the > or = 50 y age group (OR: 7.3, 95%CI: 3.6-14.7), septicaemia (OR: 3.1; 95%CI: 2.0-4.7) and residing in the province of Lleida (OR: 3.2; 95%CI: 1.2-8.5). The CFR of meningococcal disease in Catalonia was not associated with the emergent phenotype C:2b:P1.2,5 strain, which caused an outbreak in other regions of Spain.

  1. [IgG4-related lung disease: analysis of 8 cases and literature review].

    Science.gov (United States)

    Han, G J; Hu, H; Mao, D; Bai, X; She, D Y; Zhao, S F; Wen, Z L; Gao, J

    2017-03-12

    Objective: To improve the understanding and treatment of IgG4-related lung disease (IgG4-RLD). Methods: The clinical characteristics, serum IgG4 levels, pathological features, chest CT, therapy and prognosis of 8 patients with IgG4-RLD were retrospectively analyzed. These patients were admitted to the People's Liberation Army General Hospital and the pathological diagnosis was made between December 2005 and March 2016. Relevant literatures were reviewed. Results: The 8 patients with IgG4-RLD included 4 men and 4 women, with an average age of (59±4) years (range, 37-74). The respiratory symptoms included shortness of breath, cough, and expectoration. Extra-pulmonary symptoms included abdominal pain, facial edema, and fever. Extrapulmonary organs were involved in 7 cases. Serum IgG4 levels were elevated in 8 cases, with an average concentration of(17±6)g/L. Chest CT showed solid lung nodules in 6, alveolar-interstitial infiltration in 5, bronchovascular lesions in 3 and ground glass shadows in 2 cases. PET/CT was performed in 2 cases and it showed multiple organ involvement with higher radioactivity uptake(SUVmax2.9-4.2). The pathological examination found lymphocyte and plasma cell infiltration in 7, fibrous tissue hyperplasia in 5, and occlusive vasculitis in 2 cases. On immunohistochemical staining, the ratio of IgG4-positive plasma cells to IgG-positive plasma cells was higher than 40%in 3 cases. The number of IgG4-positive plasma cells was 10-50/HP in 8 cases. The misdiagnosis rate was 100% before the final diagnosis was made. Three cases received glucocorticoids with immunosuppressant therapy, 2 received surgery combined with glucocorticoid therapy, 2 received glucocorticoid therapy alone, and 1 only received surgery. The follow-up time was 4-132 months, with remission in 7 cases, and disease progression in 1 case, but no death. A total of 195 cases of IgG4-RLD were reviewed from the literature, among whom 111 cases were admitted with respiratory symptoms

  2. Bowen's disease: report of a case in a Nigerian man | Yahya | West ...

    African Journals Online (AJOL)

    Bowen's disease (cutaneous squamous cell carcinoma in situ), like other cancers of the skin, is rare in black people – to our knowledge, only about 43 cases have been published in the medical literature. We report a 59-year old Nigerian man who presented with a five-year history of a mildly pruritic, slowly enlarging ...

  3. Extramammary Paget ’s disease Of Glans Penis: A Rare Case Report

    LENUS (Irish Health Repository)

    Inder, M S

    2018-06-01

    We present the case of an 83-year-old man with Extramammary Paget’s disease (EMPD) of the penis. He underwent a total penectomy and histopathology confirms the association of underlying invasive high grade urothelial carcinoma. Penile EMPD is rare and can be misinterpreted for benign skin conditions. A high index of suspicion is required for correct diagnosis and appropriate treatment.

  4. Risk factors for Creutzfeldt-Jakob disease: a reanalysis of case-control studies.

    NARCIS (Netherlands)

    D.P.W.M. Wientjens (Dorothee); Z. Davanipour; K. Kondo; W.B. Matthews; R.G. Will (Robert); C.M. van Duijn (Cornelia); A. Hofman (Albert)

    1996-01-01

    textabstractTo review the evidence for risk factors of Creutzfeldt-Jakob disease (CJD), we pooled and reanalyzed the raw data of three case-control studies. The pooled data set comprised 178 patients and 333 control subjects. The strength of association between CJD and putative risk factors was

  5. [Familial astrocytoma associated with von Recklinghausen's disease: report of two cases].

    Science.gov (United States)

    Ito, Y; Oki, S; Mikami, T; Ogasawara, H; Kawamoto, Y; Sato, H; Yamaguchi, S; Hayashi, Y; Shindo, H

    1997-03-01

    Two cases of astrocytoma associated with von Recklinghausen's disease (neurofibromatosis type; NF-1) were reported. The first case wes a 60-year-old man who had been diagnosed as von Recklinghausen's disease on the basis of skin findings. Magnetic resonance imaging (MRI) showed a tumor in the left temporal lobe. Partial removal was performed with neuronavigator, and because of the existence of Rosenthal fiber the histological diagnosis was pilocytic astrocytoma. Radiation therapy was performed. The second case was a 6-year-old boy suffering from headache and left hemiparesis including his face. MRI showed a tumor with a cyst in the right thalamus and obstructive hydrocephalus. Initially CT-guided stereotactic biopsy was performed, and the histological diagnosis, on the basis of increased cellularity, pleomorphism and nuclear atypia without necrosis or vascular proliferation, was anaplastic astrocytoma. Radiation and chemo-immuno therapy were carried out after V-P shunt. It is well known that von Recklinghausen's disease (NF-1) is often associated with optic glioma (5-36%). In the literature, the glioma seldom occurs in other parts of the brain, supratentorial glioma especially is rare. Only two familial cases of supratentorial glioma associated with von Recklinghausen's disease have been reported. The prognosis of supratentorial glioma associated with NF-1 was poor in these reports. In this paper, the diagnostic and therapeutic problems are discussed.

  6. Neisseria meningitidis Infecting a Prosthetic Knee Joint: A New Case of an Unusual Disease

    Directory of Open Access Journals (Sweden)

    Berta Becerril Carral

    2017-01-01

    Full Text Available Primary meningococcal meningitis is an infrequent but known disease. However, the infection of a prosthetic joint with Neisseria meningitidis is rare. We hereby describe the second case of an arthroplasty infected with Neisseria meningitidis that responded favourably to prosthesis retention with surgical debridement, in combination with antibiotics treatment.

  7. Hydatid cyst disease of the thyroid gland: report of two cases.

    Science.gov (United States)

    Akbulut, Sami; Demircan, Firat; Sogutcu, Nilgun

    2015-04-01

    Hydatid cyst disease may develop in any organ of the body, most frequently in the liver and lung, but occasionally can affect other organs such as the thyroid gland. Although the prevalence of thyroidal cyst disease varies by region, literature data suggest that it ranges between 0% and 3.4%. The aim of this report was to share 2 cases with thyroid hydatid cyst. Two female patients aged 26 and 57 years were admitted to our outpatient clinic with different complaints. While the first case presented with front of the neck swelling and pain, the second case presented with hoarseness, sore throat, and neck swelling. Both patients were living in a rural area in the southeastern region of Turkey and had had a long history of animal contact. Both patients had undergone previous surgeries for hydatid cyst disease. Both patients presented with a clinical picture consistent with typical multinodular goiter, and both underwent total thyroidectomy after detailed examinations and tests. The exact diagnosis was made after histopathologic examination in both patients. They both had a negative indirect hemagglutination test studied from blood samples. They both have had no recurrences during a 4-year follow-up. In conclusion, although thyroid gland is rarely affected, hydatid cyst disease should not be overlooked in differential diagnosis of cystic lesions of thyroid gland in patients who live in regions where hydatid cyst disease is endemic and who had hydatid cysts in other regions of their body.

  8. [Forensic Analysis of 6 Cases of Sudden Death due to Hyperthyroid Heart Disease].

    Science.gov (United States)

    Zhang, M Z; Li, B X; Zhao, R; Guan, D W; Zhang, G H; Wu, X; Zhu, B L; Li, R B

    2017-10-01

    To analyse the cases of sudden death due to hyperthyroid heart disease, and explore the general information of deaths and the forensic pathological characteristics to provide reference evidence for forensic identification of such cases. Six cases of sudden death due to hyperthyroid heart disease between 2001 and 2016 were selected from School of Forensic Medicine, China Medical University. The general information (gender and age), clinical manifestations, medical history, anatomical and histopathological findings, biochemical parameters and cause of death were analysed retrospectively. Most of the 6 patients had definite history of hyperthyroidism, and they all showed certain degrees of symptoms of cardiovascular disease; had obvious incentive factors of death; histopathological examination of thyroid conformed to the performances of diffuse toxic goiter; with increase of cardiac weight, dilatation of cardiac chambers, myocardial hypertrophy and focal necrosis; postmortem biochemical analyses of pericardial fluid could be used as an additional method for diagnostic of sudden death due to hyperthyroid heart disease. The identification of death due to hyperthyroid heart disease should be based on the clinical history and the results of autopsy, histopathological examination, postmortem toxicology tests. The postmortem biochemical detection of thyroid and cardiac function should be performed if necessary. Copyright© by the Editorial Department of Journal of Forensic Medicine

  9. A case of disseminated hydatid disease by surgery involving multiple organs

    Directory of Open Access Journals (Sweden)

    Asli Tanrivermis Sayit

    2014-09-01

    Full Text Available Hydatid disease is the most common parasitic infection in the world, and is caused by the parasite Echinococcus granulosus. The most common site of this disease is the liver (75%, followed by the lungs, kidney, bones, and brain. Multiple abdominal organ and peritoneal involvement can also be seen in some cases. The dissemination of hydatid cyst disease can develop spontaneously or secondary to trauma or surgery. Here, we present the case of a 69-year-old man with multiple cyst hydatidosis, who underwent surgery for acute appendicitis approximately 20 years previously. Computed tomography of the abdomen shows the multiple active and inactive cystic lesions in the liver, spleen, right kidney, and mesentery. This patient required surgery several times, as well as medical treatment, after the rupture of a mesenteric hydatid cyst during the appendectomy. Combined anthelmintic treatment was recommended to the patient who refused further surgical treatment.

  10. Perivascular fibrosis and IgG4-related disease: a case report

    Directory of Open Access Journals (Sweden)

    S. Monti

    2014-11-01

    Full Text Available Immunoglobulin G4-related disease (IgG4-RD is a newly recognized fibroinflammatory condition which can potentially involve any organ. Some characteristic histopathologic features with lymphoplasmacytic infiltrate, an increased number of IgG4+ cells, storiform fibrosis and obliterative phlebitis are the mainstay for diagnosis. Serum IgG4 levels often increase. We report the case of a patient with perivascular fibrotic lesions involving the aortic arch and the splenic hilum, with a surgical biopsy-proven diagnosis of IgG4-related disease. The patient is now undergoing a low-dose corticosteroid maintenance therapy without evidence of new localizations of the disease. This case highlights the need for increasing awareness and recognition of this new, emerging clinical condition.

  11. Hydatid disease of the soft tissues of the lower limb: findings in three cases

    International Nuclear Information System (INIS)

    Martin, J.; Marco, V.; Zidan, A.; Marco, C.

    1993-01-01

    Three cases of hydatid disease are reported, all presenting as soft tissue lesions in the lower extremities. All three cases were studied with ultrasound (US), two with computed tomography (CT), and two with magnetic resonance (MR) imaging techniques. Two patients presented with multivesicular lesions which were considered diagnostic of hydatid disease. The third patient showed a lesion with a predominantly solid pattern, closely mimicking a soft-tissue neoplasm. US was not diagnostic, but MR outlined vesicular structures and a fibrous pericyst. Hydatid disease presenting in the soft tissues can therefore be diagnosed with confidence when it shows multivesicular lesions but MR may be the most useful imaging technique when a complex or solid pattern is present. (orig.)

  12. Surgical protocol for confirmed or suspected cases of Ebola and other highly transmissible diseases.

    Science.gov (United States)

    Badia, Josep M; Rubio-Pérez, Inés; Arias Díaz, Javier; Guirao Garriga, Xavier; Serrablo, Alejandro; Jover Navalón, José M

    2016-01-01

    Outbreaks of viral haemorrhagic fevers such as Ebola can lead to imported cases in Europe and America. The eventuality of surgery in the setting of Ebola Virus Disease (EVD) is low, but the Spanish Association of Surgeons elaborated a surgical protocol for EVD. Elective surgical procedures are not indicated. Emergency cases can be considered in: persons under investigation, possible cases and early confirmed cases. In some conditions usually treated by surgery a medical treatment can be tested. All cases must be treated in high technology hospitals. These hospitals must be equipped with adequate means for healthcare provider's protection. All members of the healthcare team should practice thorough simulation prior to caring for a possible Ebola patient. This protocol is based on international guidelines on use of Personal Protective Equipment, protocols of other scientific societies, and specific recommendations for the operating room environment. Copyright © 2015 AEC. Publicado por Elsevier España, S.L.U. All rights reserved.

  13. Diagnostic challenges of early Lyme disease: Lessons from a community case series

    Directory of Open Access Journals (Sweden)

    Schwarzwalder Alison

    2009-06-01

    Full Text Available Abstract Background Lyme disease, the most common vector-borne infection in North America, is increasingly reported. When the characteristic rash, erythema migrans, is not recognized and treated, delayed manifestations of disseminated infection may occur. The accuracy of diagnosis and treatment of early Lyme disease in the community is unknown. Methods A retrospective, consecutive case series of 165 patients presenting for possible early Lyme disease between August 1, 2002 and August 1, 2007 to a community-based Lyme referral practice in Maryland. All patients had acute symptoms of less than or equal to 12 weeks duration. Patients were categorized according to the Centers for Disease Control and Prevention criteria and data were collected on presenting history, physical findings, laboratory serology, prior diagnoses and prior treatments. Results The majority (61% of patients in this case series were diagnosed with early Lyme disease. Of those diagnosed with early Lyme disease, 13% did not present with erythema migrans; of those not presenting with a rash, 54% had been previously misdiagnosed. Among those with a rash, the diagnosis of erythema migrans was initially missed in 23% of patients whose rash was subsequently confirmed. Of all patients previously misdiagnosed, 41% had received initial antibiotics likely to be ineffective against Lyme disease. Conclusion For community physicians practicing in high-risk geographic areas, the diagnosis of Lyme disease remains a challenge. Failure to recognize erythema migrans or alternatively, viral-like presentations without a rash, can lead to missed or delayed diagnosis of Lyme disease, ineffective antibiotic treatment, and the potential for late manifestations.

  14. [Histological features of celiac disease in south Tunisia: a study of 114 pediatric cases].

    Science.gov (United States)

    Kallel, Rim; Krichen-Makni, Saloua; Ellouze, Sameh; Châari, Chiraz; Charfi, Slim; Sellami, Ahmed; Tahri, Mohamed-Nabil; Hachicha, Mongia; Sellami-Boudawara, Tahya

    2009-04-01

    To report the histological features of celiac disease in a paediatric population originating from south Tunisia. A retrospective study of a series of duodenal biopsies from 114 children with celiac disease diagnosed over a period of 6 years (from January 1999 to December 2004). The diagnosis was confirmed by histological results, serological studies and clinical response to gluten free diet. The average age of patients was of 6.2 years (range 6 months-15 years). Sex ratio was 0.71. Symptoms were dominated by chronic diarrhea (48%), weight loss (50%) and anemia (20.1%). Histological findings showed an intraepithelial lymphocytosis (Marsh type 1) in 12.2% of cases, type 2 was present in 1.7% of cases and type 3 (villous atrophy) in 86% of cases. A treatment with a gluten-free diet was indicated for all patients, only the cases who haven't presented a clinical amelioration (11 cases) have beneficed a control biopsie; a villous atrophy was persistent in 80% of this patients. Histological features in duodenal biopsies for the diagnosis and the follow-up of patients with coeliac disease. This allows an appropriate treatment and prevents further complications.

  15. Addison's disease presenting with idiopathic intracranial hypertension in 24-year-old woman: a case report

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    Moore Peter

    2010-02-01

    Full Text Available Abstract Introduction Idiopathic intracranial hypertension can rarely be associated with an underlying endocrine disorder such as Cushing's syndrome, hyperthyroidism, or with administration of thyroxine or growth hormone. Though cases of idiopathic intracranial hypertension associated with Addison's disease in children have been reported, there is only one documented case report of this association in adults. We describe a case of an acute adrenal insufficiency precipitated by idiopathic intracranial hypertension in a Caucasian female. Case presentation A 24-year-old Caucasian woman was acutely unwell with a background of several months of generalised fatigue and intermittent headaches. She had unremarkable neurological and systemic examination with a normal computerised tomography and magnetic resonance imaging of the brain. Normal cerebrospinal fluid but increased opening pressure at lumbar puncture suggested intracranial hypertension. A flat short synacthen test and raised level of adrenocorticotrophic hormone were consistent with primary adrenal failure. Conclusion Addison's disease can remain unrecognised until precipitated by acute stress. This case suggests that idiopathic intracranial hypertension can rarely be associated with Addison's disease and present as an acute illness. Idiopathic intracranial hypertension is possibly related to an increase in the levels of arginine vasopressin peptide in serum and cerebrospinal fluid secondary to a glucocorticoid deficient state.

  16. Preventing a Mass Disease: The Case of Gallstones Disease: Role and Competence for Family Physicians.

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    Portincasa, Piero; Di Ciaula, Agostino; Grattagliano, Ignazio

    2016-07-01

    Gallstone formation is the result of a complex interaction between genetic and nongenetic factors. We searched and reviewed the available literature to define how the primary prevention of gallstones (cholesterol gallstones in particular) could be applied in general practice. Electronic bibliographical databases were searched. Prospective and retrospective cohort studies and case-controlled studies were analyzed and graded for evidence quality. The epidemiological data confirmed that genetic factors are estimated to account for only approximately 25% of the overall risk of gallstones, while metabolic/environmental factors are at least partially modifiable in stone-free risk groups, and are thus modifiable by primary prevention measures related to diet, lifestyle, and environmental factors (i.e., rapid weight loss, bariatric surgery, somatostatin or analogues therapy, transient gallbladder stasis, and hormone therapy). There is no specific recommendation for the secondary prevention of recurrent gallstones. Family physicians can contribute to preventing gallstones due to their capability to identify and effectively manage several risk factors discussed in this study. Although further studies are needed to better elucidate the involvement of epigenetic factors that may regulate the effect of environment and lifestyle on gene expression in the primary prevention of gallstone formation, preventive interventions are feasible and advisable in the general practice setting.

  17. Secondary Hypertension, Erythrocytosis, and Unilateral Renal Cystic Disease in a Submariner: A Case Report.

    Science.gov (United States)

    Forbes, Angela S; Yeo, Fred E

    Erythrocytosis, or increased red blood cell mass, may be primary as in the case of polycythemia vera (PV), or secondary due to a variety of causes related to erythropoietin (EPO) secretion and hypoxia. Chronic pulmonary disease and certain EPO-secreting tumors should be addressed and excluded early during the course of evaluation for a patient presenting with increased red blood cell mass. Inclusion of the JAK2 V617F gene mutation in the recent World Health Organization criteria for the diagnosis of PV allows for facilitated diagnosis and guides therapy. EPO levels can be helpful in diagnosis and guiding therapy, but in the case of cystic renal diseases, EPO levels are often not elevated, creating diagnostic uncertainty. This report describes a case of symptoms directly attributable to erythrocytosis in the setting of negative JAK2 mutation and normal EPO levels. The subsequent discovery of a large cystic renal kidney and PV were the leading diagnostic considerations. 2016.

  18. Use of bortezomib in heavy-chain deposition disease: a report of 3 cases.

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    Patel, Kinjal; Dillon, John J; Leung, Nelson; Bomback, Andrew S; Appel, Gerald B; D'Agati, Vivette; Canetta, Pietro A

    2014-07-01

    Heavy-chain deposition disease (HCDD) is a rare complication of plasma cell dyscrasia in which monoclonal heavy chains deposit in glomerular and tubular basement membranes of the kidney. Clinical and pathologic features of HCDD have been well described in case reports and series, but evidence supporting specific therapies is sparse. Historically, the disease has had a poor prognosis, intensifying the need to clarify optimal treatments. We describe 3 cases of HCDD with biopsy-proven glomerular involvement, severe nephrotic syndrome, and decline in kidney function that were treated successfully with bortezomib, a proteasome inhibitor. None of these patients had multiple myeloma. In all cases, bortezomib-based therapy resulted in sustained resolution of nephrotic syndrome and improvement in kidney function. All 3 patients developed peripheral neuropathy; otherwise, treatment was well tolerated. To our knowledge, this is the first description of the clinical effectiveness of bortezomib against HCDD. Copyright © 2014 National Kidney Foundation, Inc. Published by Elsevier Inc. All rights reserved.

  19. Intracardiac thrombus in Behçet's disease: Two case reports

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    Brahem Radhia

    2005-07-01

    Full Text Available Abstract Intracardiac thrombus in Behçet's disease is an extremely rare manifestation. We report two such cases. A 20-year-old man presented with dyspnoea, cough and haemoptysis. Right heart thrombus associated with pulmonary artery aneurysm and thromboembolism was identified by helical CT and transoesophageal echocardiography. The second case was a 29-year-old male admitted for fever and chest pain. A diagnosis of right atrial thrombosis associated with pulmonary embolism and hyperhomocysteinemia was made. Due to the absence of haemodynamic compromise, medical management consisting of immunosupressive and anticoagulation therapy was adopted which resulted in complete dissolution of the thrombus with dramatic clinical improvement in both cases of clinical status. Conclusion intracardiac thrombus is a rare complication of Behçet's disease. As shown in our patients, medical treatment should be considered as the first line.

  20. Recent US Case of Variant Creutzfeldt-Jakob Disease-Global Implications.

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    Maheshwari, Atul; Fischer, Michael; Gambetti, Pierluigi; Parker, Alicia; Ram, Aarthi; Soto, Claudio; Concha-Marambio, Luis; Cohen, Yvonne; Belay, Ermias D; Maddox, Ryan A; Mead, Simon; Goodman, Clay; Kass, Joseph S; Schonberger, Lawrence B; Hussein, Haitham M

    2015-05-01

    Variant Creutzfeldt-Jakob disease (vCJD) is a rare, fatal prion disease resulting from transmission to humans of the infectious agent of bovine spongiform encephalopathy. We describe the clinical presentation of a recent case of vCJD in the United States and provide an update on diagnostic testing. The location of this patient's exposure is less clear than those in the 3 previously reported US cases, but strong evidence indicates that exposure to contaminated beef occurred outside the United States more than a decade before illness onset. This case exemplifies the persistent risk for vCJD acquired in unsuspected geographic locations and highlights the need for continued global surveillance and awareness to prevent further dissemination of vCJD.

  1. [Complicated jejunoileal diverticular disease: a 12 cases' serie and literature review].

    Science.gov (United States)

    López Marcano, Aylhin Joana; Ramia, José Manuel; De la Plaza Llamas, Roberto; Alonso, Soledad; Gonzales Aguilar, Johnny David; Kühnhardt Barrantes, Andree Wolfgang

    2017-01-01

    To perform a retrospective analysis of a series of complicated JID (jejunoileal diverticulitis) cases surgically treated in our service during the period from 2002 to 2015. We treated 12 cases of jejunoileal complicated diverticulosis. 7 women and 5 men. The mean age was 76 years. The clinical presentation in all cases was acute abdominal pain, one with gastrointestinal bleeding. All cases had leukocytosis, neutrophilia and increased acute phase reactants. All patients underwent emergency abdominal CT. In 11 cases, there was consistency between imaging studies and surgical findings. Diverticula were located: jejunum (9) and ileum (3). Urgent exploratory laparotomy was always done and findings were: diverticular perforation with peritonitis (7 cases), diverticular perforation with abscess (4 cases) and in one case an ischemic area with diverticular perforation after embolization. Intestinal resection and anastomosis was performed in all cases. There were no patients, in which the diagnosis of diverticulosis jejunoileal was previously known. Complications were: Clavien I (2), Clavien IIIa (1), Clavien IVb (1), Clavien V (1). Jejunoileal diverticulitis is a rare entity, usually the first sign of onset of diverticular disease not previously known. Abdominal CT is of great diagnostic value. Resection of the affected segment is the treatment of choice.

  2. Coats' disease and congenital retinoschisis in a single eye: a case report and DNA analysis.

    Science.gov (United States)

    Berinstein, D M; Hiraoka, M; Trese, M T; Shastry, B S

    2001-01-01

    The clinical features of Coats' disease and congenital retinoschisis (RS) are distinctly different. Therefore, finding changes consistent with Coats' disease and congenital RS in a single eye is an unusual occurrence. The following report describes two cases with a Coats' telangiectatic lesion in one region of the retina separated by normal retina and the presence of central and peripheral congenital RS. Molecular genetic analysis of the Norrie disease and RS genes failed to identify disease-causing or polymorphic mutations in either of the genes, suggesting that the above condition is clinically and genetically a different disorder. Further studies are needed to identify the genes responsible for the above disorder and associated ocular manifestations. Copyright 2001 S. Karger AG, Basel.

  3. Multiple Sclerosis and autoimmune diseases: clinical cases and review of the literature

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    A. Protti

    2011-09-01

    Full Text Available Multiple sclerosis (MS, the most frequent demyelinating disease in adults, is thought to be an autoimmune disease. Symptoms and signs observed in MS reflect lesions present mainly in the white matter of the central nervous system (CNS. The diagnosis remains difficult, at least concerning presenting symptoms, because of their low specificity. Diagnosis criteria are usually based on dissemination of signs in time and space, evoked potentials, findings of magnetic resonance imaging, results of cerebrospinal fluid examination, and the exclusion of other diagnosis possibly explaining the clinical signs. However, no clinical and paraclinical investigation can distinguish with certainity MS from other conditions such as autoimmune or inflammatory diseases predominantly affecting the central nervous system. These other disorders include systemic lupus erythematosus, antiphospholipid syndrome, Behcet disease, Sjogren syndrome, sarcoidosis and vasculitides. We present four clinical cases showing the difficulty in reaching a proper diagnosis...

  4. Disease as a theoretical concept: the case of "HPV-itis".

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    Broadbent, Alex

    2014-12-01

    If there is any value in the idea that disease is something other than the mere absence of health then that value must lie in the way that diseases are classified. This paper offers further development of a view advanced previously, the Contrastive Model of Disease: it develops the account to handle asymptomatic disease (previously excluded); and in doing so it relates the model to a broadly biostatistical view of health (where before the model was neutral in the naturalism debate). The developments are prompted by considering cancers featuring viruses as prominent causes, since these appear to amount to cases where the prescriptions of the Contrastive Model could be followed, but aren't. The resulting Irrelevance Objection claims that the Contrastive Model is irrelevant to medical science and practice. The paper seeks to rebut the Irrelevance Objection. Copyright © 2014 Elsevier Ltd. All rights reserved.

  5. Changes in haemostasis and thrombosis associated with thyroid disease: Presentation of 2 cases.

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    Rodilla Fiz, A M; Garví López, M; Gómez Garrido, M; Girón la Casa, M

    2016-01-01

    There is a relationship between thyroid diseases and primary and secondary changes in haemostasis. The most frequent association between them are hypocoagulability states with clinical hypothyroidism and vascular thrombophilia (hypercoagulability and/or hypofibrinolysis) with hyperparathyroidism. However, there are recent studies that have detected changes in haemostasis -primary and secondary- associated with thyroid diseases with normal hormone levels, suggesting other pathogenic mechanisms not yet known. The cases are presented of 2 patients with thyroid disease that required surgery: one multinodular goitre and one papillary carcinoma of the thyroid, both with normal hormone levels. They were shown to have haemostasis disorders during the preoperative work up. These showed a Factor VII deficiency and a Factor XI deficiency along with a thrombotic disease of unknown origin, respectively. Copyright © 2015 Sociedad Española de Anestesiología, Reanimación y Terapéutica del Dolor. Publicado por Elsevier España, S.L.U. All rights reserved.

  6. Hypopituitarism Presenting as Adrenal Insufficiency and Hypothyroidism in a Patient with Wilson's Disease: a Case Report.

    Science.gov (United States)

    Lee, Hae Won; Kang, Jin Du; Yeo, Chang Woo; Yoon, Sung Woon; Lee, Kwang Jae; Choi, Mun Ki

    2016-08-01

    Wilson's disease typically presents symptoms associated with liver damage or neuropsychiatric disturbances, while endocrinologic abnormalities are rare. We report an unprecedented case of hypopituitarism in a patient with Wilson's disease. A 40-year-old woman presented with depression, general weakness and anorexia. Laboratory tests and imaging studies were compatible with liver cirrhosis due to Wilson's disease. Basal hormone levels and pituitary function tests indicated secondary hypothyroidism and adrenal insufficiency due to hypopituitarism. Brain MRI showed T2 hyperintense signals in both basal ganglia and midbrain but the pituitary imaging was normal. She is currently receiving chelation therapy along with thyroid hormone and steroid replacement. There may be a relationship between Wilson's disease and hypopituitarism. Copper deposition or secondary neuronal damage in the pituitary may be a possible explanation for this theory.

  7. [Gestational trophoblastic diseases in cesarean scar: an analysis of 20 cases].

    Science.gov (United States)

    Zhang, Ge'er; Pan, Zimin

    2017-05-25

    To analyze the clinical features, diagnosis and treatment of gestational trophoblastic diseases in cesarean scar. Clinical data of three cases of gestational trophoblastic diseases in cesarean scar diagnosed in Women's Hospital, Zhejiang University School of Medicine during December 2011 and December 2016 were collected. And literature search was performed in Wanfang data, VIP, CNKI, PubMed, ISI Web of Knowledge and EMbase database. A total of 20 cases of gestational trophoblastic diseases were included in the analysis. Clinical features were mainly abnormal vaginal bleeding after menopause, artificial abortion or medical abortion, which might be accompanied by abdominal pain. Serum β-human chorionic gonadotropin (β-hCG) levels were increased in 19 patients. The sonographic features were increase of uterine volume, honeycomb-like abnormal intrauterine echo (or described as multiple cystic dark area, multiple anechoic area and multiple liquid dark area) or heterogeneity echo conglomeration, and no clear bound with muscular layer in some cases. There were abundant blood flow signals inside or around the lesions. The ultrasonography indicated that the lesions were located in the anterior side of the uterine isthmus with the involvement of cesarean section scar. In 12 cases with lesions in cesarean scar shown by preliminary diagnosis, 9 underwent uterine artery embolization (UAE) for pretreatment; the blood loss greater than 1500 mL was observed in only one case without UAE; no patient received hysterectomy. In 8 patients whose lesions were not shown in cesarean scar, only one case received UAE pretreatment, and hysterectomy was performed in 3 cases due to blood loss greater than 1500 mL. Two cases were lost in follow-up and no death was reported in remaining 18 cases. The serum β-hCG levels returned to normal or satisfactory level during the follow-up in 17 cases with increased β-hCG levels before treatment and no recurrence was observed. The misdiagnosis rate and

  8. ROSAI-DORFMAN DISEASE WITH CERVICAL LYMPHADENOPATHY AND ORBITAL INVOLVEMENT: A CASE REPORT

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    Sameer Saleem, Sundas Younas, Kamran Qayyum

    2015-07-01

    Full Text Available Rosai-Dorfman disease (RDD, which is also called as sinus histiocytosis with massive lymphadenopathy (SHML, is a rare histiocytic disorder which occurs due to the over-production of non Langerhans sinus histiocytes. It is a nonmalignant disorder that most frequently affects children and young adults and typically presents with fever, night sweats, nonpainful cervical lymphadenopathy, leukocytosis and an elevated ESR. Extranodal involvement may also occur, thus a variety of organs in the body can be affected. Although some viral etiology has been implicated, the disease generally is considered to have an unknown cause. RDD can often be misdiagnosed as lymphoma, leukemia or tuberculosis, so it is imperative to distinguish it from these conditions as well as other forms of histiocytosis because of difference in the modes of treatment. Diagnosis of Rosai-Dorfman disease is based on biopsy of affected tissue. Biopsy showing the presence of emperipolesis, or the engulfment of lymphocytes and other immune cells by histiocytes that express S-100 antigen is diagnostic of Rosai-Dorfman disease. Once diagnosed, further workup including imaging studies are undertaken in order to determine the extent of the disease. In majority of cases, the disease resolves on its own however, treatments including corticosteroids, chemotherapy, surgical treatment or radiotherapy are carried out in severe or persistent disease or when organ function is at stake (e.g. breathing obstruction, kidney failure, visual problems. The case we report is that of a 16 year old girl who presented with a 6 month history of gradual onset drooping of left upper eyelid with mild proptosis of the left eye alongwith mild drooping of right upper eyelid, low grade fever, night sweats and cervical lymphadenopathy. Blood workup showed increased ESR, CT scan of orbits showed superior orbital masses and diagnostic biopsy revealed Rosai-Dorfman disease.

  9. Cerebral involvement in a patient with Goodpasture's disease due to shortened induction therapy: a case report

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    Preul Christoph

    2009-11-01

    Full Text Available Abstract Introduction Goodpasture's disease is a rare immunological disease with formation of pathognomonic antibodies against renal and pulmonary basement membranes. Cerebral involvement has been reported in several cases in the literature, yet the pathogenetic mechanism is not entirely clear. Case presentation A 21-year-old Caucasian man with Goodpasture's disease and end-stage renal disease presented with two generalized seizures after a period of mild cognitive disturbance. Blood pressure and routine laboratory tests did not exceed the patient's usual values, and examination of cerebrospinal fluid was unremarkable. Cerebral magnetic resonance imaging (MRI revealed multiple cortical and subcortical lesions on fluid-attenuated inversion recovery sequences. Since antiglomerular basement membrane antibodies were found to be positive with high titers, plasmapheresis was started. In addition, cyclophosphamide pulse therapy was given on day 13. Encephalopathy and MRI lesions disappeared during this therapy, and antiglomerular basement membrane antibodies were significantly reduced. Previous immunosuppressive therapy was performed without corticosteroids and terminated early after 3 months. The differential diagnostic considerations were cerebral vasculitis and posterior reversible encephalopathy syndrome. Vasculitis could be seen as an extrarenal manifestation of the underlying disease. Posterior reversible encephalopathy syndrome, on the other hand, can be triggered by immunosuppressive therapy and may appear without a hypertensive crisis. Conclusion A combination of central nervous system symptoms with a positive antiglomerular basement membrane test in a patient with Goodpasture's disease should immediately be treated as an acute exacerbation of the disease with likely cross-reactivity of antibodies with the choroid plexus. In our patient, a discontinuous strategy of immunosuppressive therapy may have favored recurrence of Goodpasture's disease.

  10. Adipokines as Possible New Predictors of Cardiovascular Diseases: A Case Control Study

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    Laura Pala

    2012-01-01

    Full Text Available Background and Aims. The secretion of several adipocytokines, such as adiponectin, retinol-binding protein 4 (RBP4, adipocyte fatty acid binding protein (aFABP, and visfatin, is altered in subjects with abdominal adiposity; these endocrine alterations could contribute to increased cardiovascular risk. The aim of the study was to assess the relationship among adiponectin, RBP4, aFABP, and visfatin, and incident cardiovascular disease. Methods and Results. A case-control study, nested within a prospective cohort, on 2945 subjects enrolled for a diabetes screening program was performed. We studied 18 patients with incident fatal or nonfatal IHD (Ischemic Heart Disease or CVD (Cerebrovascular Disease, compared with 18 matched control subjects. Circulating adiponectin levels were significantly lower in cases of IHD with respect to controls. Circulating RBP4 levels were significantly increased in CVD and decreased in IHD with respect to controls. Circulating aFABP4 levels were significantly increased in CVD, while no difference was associated with IHD. Circulating visfatin levels were significantly lower in cases of both CVD and IHD with respect to controls, while no difference was associated with CVD. Conclusions. The present study confirms that low adiponectin is associated with increased incidents of IHD, but not CVD, and suggests, for the first time, a major effect of visfatin, aFABP, and RBP4 in the development of cardiovascular disease.

  11. Visceral Fat and Novel Biomarkers of Cardiovascular Disease in Patients With Addison's Disease: A Case-Control Study.

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    Bergthorsdottir, Ragnhildur; Ragnarsson, Oskar; Skrtic, Stanko; Glad, Camilla A M; Nilsson, Staffan; Ross, Ian Louis; Leonsson-Zachrisson, Maria; Johannsson, Gudmundur

    2017-11-01

    Patients with Addison's disease (AD) have increased cardiovascular mortality. To study visceral fat and conventional and exploratory cardiovascular risk factors in patients with AD. A cross-sectional, single-center, case-control study. Patients (n = 76; n = 51 women) with AD and 76 healthy control subjects were matched for sex, age, body mass index (BMI), and smoking habits. The primary outcome variable was visceral abdominal adipose tissue (VAT) measured using computed tomography. Secondary outcome variables were prevalence of metabolic syndrome (MetS) and 92 biomarkers of cardiovascular disease. The mean ± standard deviation age of all subjects was 53 ± 14 years; mean BMI, 25 ± 4 kg/m2; and mean duration of AD, 17 ± 12 years. The median (range) daily hydrocortisone dose was 30 mg (10 to 50 mg). Median (interquartile range) 24-hour urinary free cortisol excretion was increased in patients vs controls [359 nmol (193 to 601 nmol) vs 175 nmol (140 to 244 nmol); P 1] and vasodilatory protective marker was decreased (FC, <1). Twenty-six patients (34%) vs 12 control subjects (16%) fulfilled the criteria for MetS (P = 0.01). Despite higher cortisol exposure, VAT was not increased in patients with AD. The prevalence of MetS was increased and several biomarkers of cardiovascular disease were adversely affected in patients with AD. Copyright © 2017 Endocrine Society

  12. A 17 year-old girl with a demyelinating disease requiring mechanical ventilation: a case report

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    Katsenos Chrysostomos

    2013-01-01

    Full Text Available Abstract Background Demyelinating diseases cause destruction of the myelin sheath, while axons are relatively spared. Pathologically, demyelination can be the result of an inflammatory process, viral infection, acquired metabolic derangement and ischemic insult. Three diseases that can cause inflammatory demyelination of the CNS are: Multiple sclerosis (MS, Acute disseminated encephalomyelitis (ADEM and Acute hemorrhagic leucoencephalitis. Differentiation is not always easy and there is considerable overlaping. Data about adults with acute demyelination requiring ICU admission is limited. Case presentation A 17 year old Greek female was hospitalised in the ICU because of acute respiratory failure requiring mechanical ventilation. She had a history of febrile disease one month before, acute onset of paraplegia, diplopia, progressive arm weakness and dyspnea. Her consciousness was not impaired. A demyelinating central nervous system (CNS disease, possibly post infectious encephalomyelitis (ADEM was the underlying condition. The MRI of the brain disclosed diffused expanded cerebral lesions involving the optic nerve, basal ganglia cerebellum, pons and medulla oblongata. There was also extended involvement of the cervical and thoracic part of the spinal cord. CSF leukocyte count was elevated with lymphocyte predominance. The patient required mechanical ventilation for two months. Then she was transferred to a rehabilitation centre. Three years later she remains paraplegic. Since then she has not suffered any other demyelination attack. Conclusions Demyelinating diseases can cause acute respiratory failure when the spinal cord is affected. Severe forms of these diseases, making necessary ICU admission, is less frequently reported. Intensivists should be aware of the features of these rare diseases.

  13. Human Embryonic Stem Cell Therapy in Crohn’s Disease: A Case Report

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    Shroff, Geeta

    2016-01-01

    Patient: Male, 21 Final Diagnosis: Crohn’s disease Symptoms: Intolerance to specific foods • abdominal pain and diarrhea Medication: Human embryonic stem cell therapy Clinical Procedure: Human embryonic stem cell transplantation Specialty: Gastroenterology Objective: Unusual or unexpected effect of treatment Background: Crohn’s disease is a chronic inflammatory disease of the intestines, mainly the colon and ileum, related with ulcers and fistulae. It is estimated to affect 565 000 people in the United States. Currently available therapies, such as antibiotics, thiopurines, and anti-tumor necrosis factor-alpha agents, are only observed to reduce the complications associated with Crohn’s disease and to improve quality of life, but cannot cure the disease. Stem cell therapy appears to have certain advantages over conventional therapies. Our study aimed to evaluate the efficacy of human embryonic stem cell therapy in a patient with Crohn’s disease. Case Report: A 21-year-old male with chief complaints of intolerance to specific foods, abdominal pain, and diarrhea underwent human embryonic stem cell therapy for two months. After undergoing human embryonic stem cell therapy, the patient showed symptomatic relief. He had no complaints of back pain, abdominal pain, or diarrhea and had improved digestion. The patient had no signs and symptoms of skin infection, and had improved limb stamina, strength, and endurance. The condition of patient was stable after the therapy. Conclusions: Human embryonic stem cell therapy might serve as a new optimistic treatment approach for Crohn’s disease. PMID:26923312

  14. An uncommon presentation of Kikuchi Fujimoto disease: a case report with literature review.

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    Ranabhat, Sabin; Tiwari, Mamta; Kshetri, Jiwan; Maharjan, Sushna; Osti, Bidur Prasad

    2015-09-26

    Kikuchi-Fujimoto disease is so named because Kikuchi and Fujimoto were the first scientists to describe it in Japan in 1972. Although the disease has been reported from all over the world and more so from Asia, it is rare. To date only eight cases have been reported from Nepal. Cervical lymphadenopathy, fever and raised Erythrocyte Sedimentation Rate are usual presenting features of this disease. We describe a case which presented with thrombocytopenia and axillary lymphadenopathy in addition to the usual features. Out of the total eight cases that have been reported from Nepal so far, no patients had thrombocytopenia and only one patient had axillary lymphadenopathy. A 24-year-old Nepali female presented with a 3-week history of low-grade fever, headache, and painful, discrete, unilateral left-sided cervical and axillary lymphadenopathy. Among the multitude of tests that were carried out, Erythrocyte Sedimentation Rate was raised and there was thrombocytopenia while other tests were normal. Painful lymphadenopathy pointed to bacterial lymphadenitis while chronic low-grade fever suggested tuberculosis. A cervical lymph node was excised for histopathological examination to reach an accurate diagnosis. On the basis of pathognomonic features viz., paracortical foci composed of various types of histiocytes including crescentic type in the background of abundant apoptotic karyorrhectic debris, a diagnosis of Kikuchi-Fujimoto disease was made. On follow-up evaluation after 6 weeks, the patient had no systemic symptoms, enlarged lymph nodes had regressed in size significantly, and Erythrocyte Sedimentation Rate and platelet count had become normal. Kikuchi-Fujimoto disease should be kept in the differential diagnosis of lymphadenopathy in young patients, female or male even in tuberculosis-endemic countries and even in patients who have unusual features; for example thrombocytopenia and involvement of axillary lymph nodes in addition to cervical lymph nodes as in this

  15. Life-threatening hyperkalemia following zoledronic acid infusion for Paget's disease: a case report

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    Naysmith Morag

    2011-08-01

    Full Text Available Abstract Introduction Zoledronic acid is a highly effective treatment in Paget's disease for persistent bone pain and prevention of further progression of the disease. The commonest electrolyte abnormality is hypocalcemia. To the best of our knowledge this is the first case of hyperkalemia secondary to zoledronic acid to be published in the world literature. The commonest arrhythmia related to zoledronic acid is atrial fibrillation. Case presentation We describe the case of an 80-year-old Caucasian man, with a history of ischemic heart disease, who had an in-hospital cardiac arrest related to hyperkalemia. Increasing potassium levels were noted following his first zoledronic acid infusion for symptomatic control of bone pain secondary to Paget's disease. Our patient suffered a cardiac arrest 10 days following the zoledronic acid infusion. Our patient's biochemistry and electrocardiogram output were monitored until his death 26 days after his cardiac arrest. Our patient developed paroxysmal atrial fibrillation in the post-resuscitation period and there was persistent hyperkalemia that required prolonged treatment with calcium resonium. All other possible causes of hyperkalemia were excluded. Conclusion In our patient's case persistent hyperkalemia and life-threatening arrhythmias were associated with use of zoledronic acid. These side effects have not been reported before and the causative mechanism is far from clear as there are no obvious systemic effects of zoledronic acid. The combination of zoledronic acid with predisposing factors such as structural heart disease might account for the clinical picture we witnessed. As a result, electrolyte monitoring should be adopted early in zoledronic acid use. Further studies are required to elucidate the underlying mechanism of hyperkalemia and identify the target group of patients where zoledronic acid can be safely administered. Great caution is advised in patients with underlying heart conditions.

  16. IgG4-Related Kidney Disease: Report of a Case Presenting as a Renal Mass

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    Daniele Bianchi

    2017-01-01

    Full Text Available IgG4-related disease (IgG4-RD is a nosological entity defined as a chronic immune-mediated fibro-inflammatory condition characterized by a tendency to form tumefactive, tissue-destructive lesions or by organ failure. Urologic involvement in IgG4-RD has been described in some short series of patients and in isolated case reports, most often involving the kidneys in so-called IgG4-related kidney disease (IgG4-RKD. The disease can occasionally mimic malignancies and is at risk of being misdiagnosed due to its rarity. We report the case of a 56-year-old man presenting with a right renal mass suspected of being malignant. Laboratory tests showed normal creatinine levels, a high erythrocyte sedimentation rate, and high levels of C-reactive protein and microalbuminuria. The patient underwent radical right nephroureterectomy and histopathologic examination revealed features proving IgG4-RKD. He was therefore referred to immunologists. Typical clinical presentation of IgG4-RKD includes altered renal function with inconstant or no radiologic findings. Conversely, in the case we presented, a single nodule was detected upon imaging evaluation, thus mimicking malignancy. This raises the issue of a proper differential diagnosis. A multidisciplinary approach can be useful, although in clinical practice the selection of patients suspected of having IgG4-RKD is critical in the cases presenting with a renal mass that mimics malignancy.

  17. Systemic mast cell disease (SMCD) and bone pain. A case treated with radiotherapy

    Energy Technology Data Exchange (ETDEWEB)

    Hesselmann, S.; Micke, O.; Schaefer, U.; Willich, N. [University Hospital Muenster (Germany). Dept. of Radiotherapy and Radiooncology

    2002-05-01

    Background: Systemic mast cell disease (SMCD) is a rare disease characterized by a multitopic proliferation of cytologically and/or functionally abnormal tissue mast cells. SMCD preferentially involves the skin, spleen, liver, lymph nodes and the bone marrow. The cause of SMCD is unknown. Bony pain, caused by mast cell infiltration of the marrow cavity, is present in up to 28% of cases and is frequently chronic and difficult to palliate with medical therapy. Case Report: We report one case of refractory bone pain in a 54-year-old female Caucasian patient with advanced SMCD and associated bony involvement, which was treated with radiotherapy for pain palliation. Between 1995 and 1998, the patient was irradiated at four different locations: 1) right shoulder and proximal right humerus, 2) both hands, 3) both knees, 4) left humerus with a total dose of 40 Gy in 2.0 or 2.5 Gy daily fractions. Results: Different results of pain palliation were achieved. In one location the pain was reduced for 55 months until her death due to disease progression, whereas in two other locations a pain control was maintained for 3 and 6 months after radiotherapy. In one location, no pain reduction was achieved. Severe side effects were not observed. Conclusion: Palliative radiotherapy has a role in the control of severe intractable bone pain in patients with advanced SMCD, though in some cases the effect may be short or incomplete. The observed palliation of pain can even differ in the same patient. (orig.)

  18. Systemic mast cell disease (SMCD) and bone pain. A case treated with radiotherapy

    International Nuclear Information System (INIS)

    Hesselmann, S.; Micke, O.; Schaefer, U.; Willich, N.

    2002-01-01

    Background: Systemic mast cell disease (SMCD) is a rare disease characterized by a multitopic proliferation of cytologically and/or functionally abnormal tissue mast cells. SMCD preferentially involves the skin, spleen, liver, lymph nodes and the bone marrow. The cause of SMCD is unknown. Bony pain, caused by mast cell infiltration of the marrow cavity, is present in up to 28% of cases and is frequently chronic and difficult to palliate with medical therapy. Case Report: We report one case of refractory bone pain in a 54-year-old female Caucasian patient with advanced SMCD and associated bony involvement, which was treated with radiotherapy for pain palliation. Between 1995 and 1998, the patient was irradiated at four different locations: 1) right shoulder and proximal right humerus, 2) both hands, 3) both knees, 4) left humerus with a total dose of 40 Gy in 2.0 or 2.5 Gy daily fractions. Results: Different results of pain palliation were achieved. In one location the pain was reduced for 55 months until her death due to disease progression, whereas in two other locations a pain control was maintained for 3 and 6 months after radiotherapy. In one location, no pain reduction was achieved. Severe side effects were not observed. Conclusion: Palliative radiotherapy has a role in the control of severe intractable bone pain in patients with advanced SMCD, though in some cases the effect may be short or incomplete. The observed palliation of pain can even differ in the same patient. (orig.)

  19. Statistical studies on heart disease of the pathological autopsy cases in the Atomic Bomb Hospital

    Energy Technology Data Exchange (ETDEWEB)

    Matushita, H [Hiroshima Red Cross Hospital (Japan); Hamada, T; Ishida, S

    1976-03-01

    Of 1230 autopsied cases in the Atomic Bomb Hospital and in the Red-Cross Hospital from 1956 to March, 1975, a statistical study was made on 118 cases in which primary or secondary heart disease had been found. The results are as follows. The incidence of myocardial infarction was 2.4 times higher in the group exposed to the atomic bomb within 2 km distance from the bombed area than that it was in the unexposed group. The incidence of acquired valvular disease was 4.1 times higher in the exposed group than in the unexposed group. From the standpoint of the incidence of myocardiosis, there was no difference between the groups. The incidence of pericarditis was 1.5 times higher in the exposed group than in the unexposed group. The incidence of cor pulmonale was 1.8 times higher in the exposed group than in the unexposed group. The incidence of other heart disease including congenital disease was, however, 1.6 times higher in the unexposed group than in the exposed group. The incidence of general heart disease was 1.7 times higher in the exposed group than in the unexposed group. The incidence of hypertrophy of the heart (more than 400 g) was 1.2 times higher in the exposed group than in the unexposed group.

  20. Statistical studies on heart disease of the pathological autopsy cases in the Atomic Bomb Hospital

    International Nuclear Information System (INIS)

    Matushita, Hiroshi; Hamada, Tadao; Ishida, Sadamu.

    1976-01-01

    Of 1230 autopsied cases in the Atomic Bomb Hospital and in the Red-Cross Hospital from 1956 to March, 1975, a statistical study was made on 118 cases in which primary or secondary heart disease had been found. The results are as follows. The incidence of myocardial infarction was 2.4 times higher in the group exposed to the atomic bomb within 2 km distance from the bombed area than that it was in the unexposed group. The incidence of acquired valvular disease was 4.1 times higher in the exposed group than in the unexposed group. From the standpoint of the incidence of myocardiosis, there was no difference between the groups. The incidence of pericarditis was 1.5 times higher in the exposed group than in the unexposed group. The incidence of cor pulmonale was 1.8 times higher in the exposed group than in the unexposed group. The incidence of other heart disease including congenital disease was, however, 1.6 times higher in the unexposed group than in the exposed group. The incidence of general heart disease was 1.7 times higher in the exposed group than in the unexposed group. The incidence of hypertrophy of the heart (more than 400 g) was 1.2 times higher in the exposed group than in the unexposed group. (Namekawa, K.)

  1. Local disease-ecosystem-livelihood dynamics: reflections from comparative case studies in Africa.

    Science.gov (United States)

    Leach, Melissa; Bett, Bernard; Said, M; Bukachi, Salome; Sang, Rosemary; Anderson, Neil; Machila, Noreen; Kuleszo, Joanna; Schaten, Kathryn; Dzingirai, Vupenyu; Mangwanya, Lindiwe; Ntiamoa-Baidu, Yaa; Lawson, Elaine; Amponsah-Mensah, Kofi; Moses, Lina M; Wilkinson, Annie; Grant, Donald S; Koninga, James

    2017-07-19

    This article explores the implications for human health of local interactions between disease, ecosystems and livelihoods. Five interdisciplinary case studies addressed zoonotic diseases in African settings: Rift Valley fever (RVF) in Kenya, human African trypanosomiasis in Zambia and Zimbabwe, Lassa fever in Sierra Leone and henipaviruses in Ghana. Each explored how ecological changes and human-ecosystem interactions affect pathogen dynamics and hence the likelihood of zoonotic spillover and transmission, and how socially differentiated peoples' interactions with ecosystems and animals affect their exposure to disease. Cross-case analysis highlights how these dynamics vary by ecosystem type, across a range from humid forest to semi-arid savannah; the significance of interacting temporal and spatial scales; and the importance of mosaic and patch dynamics. Ecosystem interactions and services central to different people's livelihoods and well-being include pastoralism and agro-pastoralism, commercial and subsistence crop farming, hunting, collecting food, fuelwood and medicines, and cultural practices. There are synergies, but also tensions and trade-offs, between ecosystem changes that benefit livelihoods and affect disease. Understanding these can inform 'One Health' approaches towards managing ecosystems in ways that reduce disease risks and burdens.This article is part of the themed issue 'One Health for a changing world: zoonoses, ecosystems and human well-being'. © 2017 The Authors.

  2. Multicentric Castleman's Disease in a Hepatitis C-Positive Intravenous Drug User: A Case Report

    Directory of Open Access Journals (Sweden)

    D. Y. Talukder

    2011-01-01

    Full Text Available Introduction. We report a rare presentation of Castleman's disease in a hepatitis C-positive patient and present a short review of treatments described in other similar case reports and studies. Case Presentation. A 46-year-old male with untreated hepatitis C and a 16-year history of intravenous drug use presented with pleuritic chest pain and bony pain in the knee, hip, and lower back, on a background of unexplained weight loss of 40 kilograms, fevers, night sweats, and repeated infections over the last two years. Examination discovered tender hepatomegaly, a warm right knee effusion, and painless lymphadenopathy. The patient was reactive to Epstein Barr virus and cytomegalovirus; however, HIV and HHV-8 viral testing was negative. Osteomyelitis of vertebrae T8–T11 and septic arthritis of the knee were found on investigation. A lymph node biopsy revealed histology suggestive of plasmacytic Castleman's disease. The patient is to commence rituximab treatment. Conclusion. Castleman's disease continues to present in novel ways, which may lead to difficulties in clinicopathologic diagnosis. A growing body of evidence suggests larger studies are required to determine the best treatment for multicentric Castleman's disease, particularly in patients with a concomitant disease, including hepatitis C.

  3. Early Disseminated Lyme Disease Masquerading as Mononucleosis: A Case Report.

    Science.gov (United States)

    Tumminello, Richard; Glaspey, Lindsey; Bhamidipati, Anita; Sheehan, Patrick; Patel, Sundip

    2017-12-01

    Disseminated Lyme disease can be difficult to diagnose, as it begins with nonspecific signs and symptoms, which, if not treated correctly, can lead to atrioventricular conduction blocks and meningitis. In addition, the diagnosis can be further complicated by potentially false-positive test results. We report a case of early-disseminated Lyme disease presenting with Borrelia meningitis and concomitant Lyme carditis, which was misdiagnosed as mononucleosis. A young, previously healthy patient had been hiking in the woods of upstate New York and 4 weeks later developed fever, night sweats, and myalgias. He was diagnosed with mononucleosis via a positive rapid heterophile agglutination antibody test to the Epstein-Barr virus at a walk-in clinic and was started on medications, but then subsequently developed left hip pain, a facial droop, and a very long first-degree atrioventricular conduction block. He went to the Emergency Department, where he had testing that confirmed disseminated Lyme disease. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: This case highlights the difficulty in early diagnosis of disseminated Lyme disease and how a potentially false-positive laboratory test can lead to the complications of Borrelia meningitis and Lyme carditis in untreated young healthy patients. Emergency physicians need to consider Lyme disease in patients with nonspecific signs and symptoms, especially if they have been outdoors for prolonged periods of time in Lyme-endemic areas. Copyright © 2017 Elsevier Inc. All rights reserved.

  4. [A case of Neuro-Behçet's disease with early onset of bipolar mood disorder].

    Science.gov (United States)

    Nakano, Yuko; Hatanaka, Yuki; Ikebuchi, Emi; Shimizu, Teruo; Nanko, Shinichiro; Utsumii, Takeshi

    2004-01-01

    In this report, we describe a case of Neuro-Behçet's disease with early onset of bipolar mood disorder. A 53-year-old man with neuropathy including dysphasia and dyslalia developed bipolar mood disorder with anxiety, agitation, depressive mood, talkativeness, hyperkinesias, and appetite rise, and soon exhibited severe personality deterioration. Oral aphthae, cell proliferation and elevated IL-6 levels in spinal fluid, and the patient's clinical downhill course with remission and relapse in spite of good reaction to steroid preparation indicated the possibility of Neuro-Behçet's disease. Brain MRI showed clear swelling of the brain stem area, especially in the pons, in the active phase with low signal in T1-weighted images contrasting with clear high signal in T2-weighted images and FLAIR. At the time of remission, atrophy of the brain stem was shown. These findings were consistent with the view reported in recent years concerning the brain image of Neuro-Behçet's disease, which seemed to be useful for the differential diagnosis. This case manifested two outstanding clinical features. First, it showed bipolar mood swing or mixed state distinguishable from disinhibition or euphoria in deteriorated personality, which is common in this condition. A clear bipolar mood disorder has not been described until now in Neuro-Behçet's disease. Second, subclinical dysthymia or hypomanic phase occurred before clear onset of the disease. In Neuro-Behçet's disease, it is currently considered that psychiatric symptoms may appear in the early stage, but there is controversy as to whether they can precede the other symptoms. Our case indicated very early onset of psychiatric symptoms in this condition.

  5. A rare complication of CMV infection in Crohn's disease - hemophagocytic syndrome: a case report.

    Science.gov (United States)

    Pop, Corina Silvia; Becheanu, Gabriel; Calagiu, Dorina; Jantea, Petruţa-Violeta; Rădulescu, Dragoş Mihai; Pariza, George; Mavrodin, Carmen-Iuliana; Bold, Adriana; Costache, Adrian; Nemeş, Roxana Maria

    2015-01-01

    We report a case of CMV (cytomegalovirus) infection in a Crohn's disease patient, resulting in severe hemophagocytic syndrome and death. A 63-year-old man with a 10-year history of ileal and colonic Crohn's disease presented with general malaise, loss of appetite and weight loss over the last month. He was in clinical remission for two years, with maintenance therapy 5-Aminosalicylic acid (5-ASA)-derived Mesalamine. The patient had no prior immunomodulators or suppressive treatment. A colonoscopy was performed and we found appearance suggestive of active Crohn's disease, confirmed by histopathological examination. A diagnosis of an exacerbation of Crohn's disease was established. Although the specific treatment was initiated, patient's general condition degraded progressively and diarrheal stools appeared, followed by an episode of massive gastrointestinal bleeding - hematochezia. We performed a new colonoscopy and the pathological examination revealed Crohn's ileocolitis with superimposed CMV infection. Despite the initiation of Ganciclovir alongside with other intensive care measures, he increasingly deteriorated and chest X-ray confirmed multilobar pneumonia. The occurrence of rapidly progressing pancytopenia and evidence for disseminated intravascular coagulopathy as well as hyperferritinemia, raised the suspicion of hemophagocytic syndrome confirmed by bone marrow aspiration. Hence, CMV-associated hemophagocytic syndrome in the context of recent corticotherapy for Crohn's disease was established. There is enough evidence that supports the gravity of the CMV infection in the case of inflammatory bowel disease (IBD) patients, especially the ones on immunomodulator treatment. The hemophagocytic syndrome reactively occurs in patients with infections in cases of immunodeficiency, displaying a hematological aspect of multiple organ dysfunction syndrome.

  6. A brain mass in a patient with Behcet's disease: a case report.

    Science.gov (United States)

    Alfedaghi, Ahmad S; Masters, Y; Mourou, M; Eshak, O

    2015-09-30

    This case report describes an uncommon presentation of Behcet's disease which manifested as neuro-Behcet's disease. Although it is not the first reported case in the medical literature, it is a possible differential in a patient presenting with a brain tumor. Since the diagnosis of neuro-Behcet's disease depends largely on the clinical picture and medical history, it should be considered prior to opting for invasive diagnostic methods. Our patient is a 36-year-old white man from Kuwait. He presented with acute onset of headache, vomiting, and right-sided weakness. Magnetic resonance imaging of his brain showed a mass in the brain stem. He then revealed that he had a history of recurrent painful oral and genital ulcers for the past 10 years, which suggested a diagnosis of Behcet's disease. A brain biopsy was recommended by a neurosurgeon at the time, but the patient refused the procedure. After initiating steroid therapy, the mass began to regress and, eventually, was undetectable on subsequent imaging of his brain. This case of neuro-Behcet's disease reflects the need to consider this diagnosis in a patient of less than 40 years of age presenting with a suspected brain tumor. This may delay the need for invasive diagnostic methods, especially if such methods are not desired by the patient. In the management of suspected neuro-Behcet's disease, initiating steroid therapy and measuring the response is a reasonable option before seeking a definitive diagnosis via brain biopsy. If the response to steroids is minimal then a brain biopsy should be performed.

  7. Alignment between chronic disease policy and practice: case study at a primary care facility.

    Science.gov (United States)

    Draper, Claire A; Draper, Catherine E; Bresick, Graham F

    2014-01-01

    Chronic disease is by far the leading cause of death worldwide and of increasing concern in low- and middle-income countries, including South Africa, where chronic diseases disproportionately affect the poor living in urban settings. The Provincial Government of the Western Cape (PGWC) has prioritized the management of chronic diseases and has developed a policy and framework (Adult Chronic Disease Management Policy 2009) to guide and improve the prevention and management of chronic diseases at a primary care level. The aim of this study is to assess the alignment of current primary care practices with the PGWC Adult Chronic Disease Management policy. One comprehensive primary care facility in a Cape Town health district was used as a case study. Data was collected via semi-structured interviews (n = 10), focus groups (n = 8) and document review. Participants in this study included clinical staff involved in chronic disease management at the facility and at a provincial level. Data previously collected using the Integrated Audit Tool for Chronic Disease Management (part of the PGWC Adult Chronic Disease Management policy) formed the basis of the guide questions used in focus groups and interviews. The results of this research indicate a significant gap between policy and its implementation to improve and support chronic disease management at this primary care facility. A major factor seems to be poor policy knowledge by clinicians, which contributes to an individual rather than a team approach in the management of chronic disease patients. Poor interaction between facility- and community-based services also emerged. A number of factors were identified that seemed to contribute to poor policy implementation, the majority of which were staff related and ultimately resulted in a decrease in the quality of patient care. Chronic disease policy implementation needs to be improved in order to support chronic disease management at this facility. It is possible that similar

  8. [Parathyroid disease: The full spectrum, from adenoma to carcinoma. Report of 3 cases].

    Science.gov (United States)

    Stoopen-Margain, Enrique; Valanci-Aroesty, Sofía; Castañeda-Martínez, Leopoldo; Baquera-Heredia, Javier; Sainz-Hernández, Juan Carlos

    Primary hyperparathyroidism is a disease characterised by the autonomous production of parathyroid hormone. The most common cause is an adenoma, followed by hyperplasia, and rarely carcinoma. Three cases are presented. The first case is associated with a brown tumour that was diagnosed as hyperplasia after study and surgery. The second case was related to pathological fractures, and a lower right adenoma 236 times bigger than a normal parathyroid was excised. The last case presented with abdominal pain and heartburn. Histopathology reported a carcinoma, which was removed using surgery en bloc. All patients have improved. Hyperparathyroidism symptoms are very difficult to identify and diagnose, thus a detailed and broad approach is needed when hyperparathyroidism is suspected. Copyright © 2016 Academia Mexicana de Cirugía A.C. Publicado por Masson Doyma México S.A. All rights reserved.

  9. Aortitis With Severe Aortic Regurgitation in Behcet's Disease: A Case Report

    Directory of Open Access Journals (Sweden)

    Hsin-Hui Chiu

    2010-01-01

    Full Text Available Behcet's aortitis is a rare, but one of the most severe complications of Behcet's disease. We report a 24-year-old woman who was noted initially to have aortitis and severe aortic regurgitation caused by Behcet's disease. After receiving aortic valve replacement, aortoplasty and immunosuppressant therapy, her condition became stationary. As far as we are aware, she is the youngest case that has undergone surgery. The early onset of hemodynamic decompensation is considered to be related to delay in diagnosis and lack of steroid treatment.

  10. Adrenal Castleman's disease mimicking other adrenal neoplasms: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Hong, Seung Baek; Lee, Nam Kyung; Kim, Suk; Han, Ga Jin; Ha, Hong Koo; Ku, Ja Yoon; Ahn, Sang Jeong; Lee, Chang Hun [Pusan National University Hospital, Pusan National University School of Medicine, Busan (Korea, Republic of)

    2017-01-15

    We present a rare case of adrenal Castleman's disease with hyaline vascular type mimicking other adrenal neoplasms in a 65-year-old woman. Although rare, the hyaline vascular type of adrenal Castleman's disease should be included in the differential diagnosis if an adrenal mass shows a well-defined, highly enhancing solid adrenal mass with peripheral rim enhancement, multiple satellite lymph nodes, and peritoneal thickening around the dominant mass on computed tomography as shown in this patient.

  11. Adult Niemann-Pick disease type B with myositis ossificans: a case report

    Directory of Open Access Journals (Sweden)

    Russka Shumnalieva

    2016-07-01

    Full Text Available Niemann-Pick Disease (NPD is a rare autosomal recessive lysosomal lipid storage disorder. The disease is caused by gene mutations that affect the metabolism of sphingolipids. The dysfunctions cause sphingomyelin to accumulate in different organs. NPD includes forms with low and high levels of sphingomyelin. We report a case of a 34 year-old man with a family history of NPD type B who presented with hepatosplenomegaly, neurological deficiency, bone abnormalities, and myositis ossificans. The clinical, biochemical, and imaging data confirmed the combined diagnosis of NPD type B with myositis ossificans.

  12. Hemolytic Disease of the Newborn Due to Anti-c Isoimmunization: A Case Report.

    Science.gov (United States)

    Sheeladevi, C S; Suchitha, S; Manjunath, G V; Murthy, Srinivas

    2013-09-01

    The Rhesus (Rh) blood group is one of the most complex blood groups known in humans. It has remained of primary importance in obstetrics, being the main cause of hemolytic disease of the newborn (HDN). Anti-D causes the most severe form of HDN. Other Rh allo antibodies that are capable of causing severe HDN include anti-c, which clinically is the most important Rh antigen after the D antigen. We report a case of hemolytic disease of the newborn due to Rh anti-c in an infant of an Rh positive mother.

  13. Kikuchi-Fujimoto Disease in the United States: Three Case Reports and Review of the Literature

    OpenAIRE

    Darcie M Deaver; Mojdeh Naghashpour; Lubomir Sokol

    2013-01-01

    Kikuchi-Fujimoto Disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a benign, self-limiting disease that manifests primarily as cervical lymphadenopathy but may include low-grade fever, headache, and fatigue.  There is a higher incidence of KFD in women aged 20-35 years and in Asian populations.  A PubMed search revealed 590 articles that described KFD.  Of these, 22 cases have been fully described in the United States.  Ten of the 22 (45%) patients were male and 12 (55%) ...

  14. A case of von Hippel-Lindau disease with exudative maculopathy

    Directory of Open Access Journals (Sweden)

    Basel T Ba′arah

    2009-01-01

    Full Text Available Von Hippel-Lindau (VHL disease is a rare multisystem familial tumor syndrome of autosomal dominant inheritance. Hallmark lesions include retinal, cerebellum and spinal cord hemangioblastomas, renal cell carcinomas, adrenal pheochromocytomas, angiomatous or cystic lesions of the kidneys, pancreas, and epididymis. We report a case of VHL disease in a 26-year-old patient who presented with exudative macular edema. Ocular and systemic studies revealed the presence of retinal and central nervous system hemangioblastomas, adrenal pheochromocytoma, multiple pancreatic, and kidney cysts. The retinal angiomas were successfully treated with argon laser photocoagulation and cryotherapy.

  15. Cases of Trichohepatoenteric Syndrome (Syndromic Diarrhea with Underlying Crohn’s Disease

    Directory of Open Access Journals (Sweden)

    Е. А. Roslavtseva

    2015-01-01

    Full Text Available Tricho-hepato-enteric syndrome (syndromic, phenotypic diarrhea, SD/THES is a rare inborn disease, which affects bowels. It is caused by the mutation of genes SKIV2L or TTC37. Manifestations include intrauterine hypotrophy, severe chronic diarrhea, which starts in infancy, characteristic facial features and hair growth abnormalities, immune disorders. There are data on two patients dealing with tricho-hepato-enteric syndrome with underlying Crohn’s disease. This is the first description of cases of aggravated tricho-hepatoenteric syndrome ever found in Russian medical literature. 

  16. [Rosai-Dorfman's disease: a propos of an interesting case study].

    Science.gov (United States)

    Córdova Ramos, Gustavo; Machin González, Victoriano; Benítez Tang, Som My

    2008-01-01

    We present a case with involvement of both nostrils by the Rosai-Dorfman's disease. Physical examination revealed tumorous lesions totally occupying the right nostril and partially the left one. The presence of proliferation of histiocytes was proved by the histological test, several of them full of non-disintegrated lymphoid nuclei, i.e. emperipolesis, the basic histological characteristic in Rosai-Dorfman's disease. The patient underwent surgical treatment with a combined approach by means of lateral rhinotomy and endoscopic nasal resection using a microdebrider.

  17. Mucormycosis in Patients with Inflammatory Bowel Disease: Case Series and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Maheen Z. Abidi

    2014-01-01

    Full Text Available Mucormycosis is a rare and often fatal invasive fungal infection mostly seen in immune-compromised individuals. A high index of clinical suspicion is necessary, so that effective preemptive therapy can be started, as timely intervention is crucial. In this series we present three cases of invasive mucormycosis in patients with underlying inflammatory bowel disease that had received therapy with immunomodulators prior to the infection. All three had varied clinical manifestations. We also review the literature of invasive mucormycosis in patients with inflammatory bowel disease.

  18. Homocyst(e)ine and cardiovascular disease: a systematic review of the evidence with special emphasis on case-control studies and nested case-control studies.

    Science.gov (United States)

    Ford, Earl S; Smith, S Jay; Stroup, Donna F; Steinberg, Karen K; Mueller, Patricia W; Thacker, Stephen B

    2002-02-01

    Elevated concentrations of homocyst(e)ine are thought to increase the risk of vascular diseases including coronary heart disease and cerebrovascular disease. We searched MEDLINE (1966-1999), EMBASE (1974-1999), SciSearch (1974- 1999), and Dissertation Abstracts (1999) for articles and theses about homocyst(e)ine concentration and coronary heart disease and cerebrovascular disease. We included 57 publications (3 cohort studies, 12 nested case-control studies, 42 case-control studies) that reported results on 5518 people with coronary heart disease (11,068 control subjects) and 1817 people with cerebrovascular disease (4787 control subjects) in our analysis. For coronary heart disease, the summary odds ratios (OR) for a 5-micromol/l increase in homocyst(e)ine concentration were 1.06 (95% CI : 0.99-1.13) for 2 publications of cohort studies, 1.23 (95% CI : 1.07-1.41) for 10 publications of nested case-control studies, and 1.70 (95% CI : 1.50-1.93) for 26 publications of case-control studies. For cerebrovascular disease, the summary OR for a 5-micromol/l increase in homocyst(e)ine concentration were 1.10 (95% CI : 0.94-1.28) for 2 publications of cohort studies, 1.58 (95% CI : 1.35-1.85) for 5 publications of nested case-control studies, and 2.16 (95% CI : 1.65-2.82) for 17 publications of case-control studies. Prospective studies offer weaker support than case-control studies for an association between homocyst(e)ine concentration and cardiovascular disease. Although other lines of evidence support a role for homocyst(e)ine in the pathogenesis of cardiovascular disease, more information from prospective epidemiological studies or clinical trials is needed to clarify this role.

  19. [Myasthenia gravis, Graves-Basedow disease and other autoimmune diseases in patient with diabetes type 1 - APS-3 case report, therapeutic complications].

    Science.gov (United States)

    Klenczar, Karolina; Deja, Grażyna; Kalina-Faska, Barbara; Jarosz-Chobot, Przemysława

    2017-01-01

    Diabetes type 1(T1D) is the most frequent form of diabetes in children and young people, which essence is autoimmune destruction of pancreatic B cells islet. Co-occurrence of other autoimmune diseases is observed in children with T1D, the most often are: Hashimoto disease or coeliac disease. We report the case of the patient, who presents coincidence of T1D with other rare autoimmune diseases such as: Graves - Basedow disease, myasthenia gravis, vitiligo and IgA deficiency. All mentioned diseases significantly complicated both endocrine and diabetic treatment of our patient and they negatively contributed her quality of life. The clinical picture of the case allows to recognize one of the autoimmune polyendocrine syndromes: APS-3 and is associated with still high risk of developing another autoimmune disease. © Polish Society for Pediatric Endocrinology and Diabetology.

  20. Neuropsychiatric manifestation of celiac disease: A case-control study in North India

    Directory of Open Access Journals (Sweden)

    Mahendra Nimel

    2016-01-01

    Full Text Available Background: Celiac disease (CD is an immune-mediated disease dependent on gluten. Prevalence of CD is about 1% and beside gastrointestinal complaints, neuropsychiatric symptoms may represent an atypical feature of CD. Some studies suggest that a gluten-free diet is effective in treating them. Settings and Design: This case-control study of 49 cases was done during the period of January to March 2013. Aim: To know the spectrum of psychiatric manifestations and cognitive functions in children with CD. Materials and Methods: We took 49 diagnosed cases of CD (based on the demonstration of IgA tissue transglutaminase antibodies and duodenal biopsy and compared with demographically matched control group (n = 50 on Seguin Form Board Test for cognitive functions and Behavioral Summarized Evaluation-Revised scale for assessment of psychiatric and behavior disturbances. All possible psychiatric diagnosis was made on the basis of International Statistical Classification of Disease and Related Health Problems-Tenth Revision criteria. Statistical Analysis: Statistical analyses were done by using Chi-square test and two-tailed P-values. Results: Neuropsychiatric manifestations were seen in 29% of cases as against 4% of controls which was statistically significant (P=0.001. Only four cases and 1 control fount to be mild mental retardation (P = 0.16. Autism, dyslexia, developmental delay, disruptive behavior disorder, and tic disorder present in cases were not found. Conclusion: Clinical manifestations of CD vary from typical malabsorption syndrome to neuropsychiatric manifestations. Those psychiatric patients who are not responding to standard pharmacological modalities, a diagnosis of CD should be taken into consideration. Only behavioral problem can be the sole clinical manifestation of CD.

  1. Clinicopathological review of immunohistochemically defined Kikuchi-Fujimoto disease-including some interesting cases.

    Science.gov (United States)

    Seong, Gil Myeong; Kim, Jo-Heon; Lim, Gil Chai; Kim, Jinseok

    2012-10-01

    Kikuchi-Fujimoto Disease (KFD) is a benign, self-limited disease characterized by tender regional lymphadenopathy with fever. KFD remains a poorly defined disease, and no clear diagnostic criteria are available. Here, we assess the clinical, laboratory, and histopathologic findings of KFD cases and report two unusual cases. Forty KFD patients that underwent lymph node (LN) biopsy and diagnosed by immunohistochemical staining, from January 2003 to November 2010, were enrolled in this retrospective study. The patients had a mean age of 29.3 years, and 29 (72.5 %) were women. Affected LNs were mainly located unilaterally in the cervical area. Mean LN size was 15.3 mm. Twenty-eight (70 %) patients had LN tenderness, and 25 (62.5 %) patients had fever. Leukopenia was observed in 18 of 35 evaluable patients. C-reactive protein and erythrocyte sedimentation rate were elevated in most patients. Anti-nuclear antibody was positive in four of 19 evaluable patients, but all had been diagnosed with concurrent systemic lupus erythematosus. Histologically, the 40 cases were classified into three types, that is, as proliferative (37.5 %), necrotizing (55.0 %), or xanthomatous (7.5 %). Interesting cases: Case 1 was a 35-year-old female with KFD and uveitis, retinal vasculitis, and superior sagittal sinus thrombosis. Case 2 was a 47-year-old male with KFD and bone marrow involvement and presented with severe bicytopenia. Although KFD is an uncommon self-limited benign disorder, it must be included in the differential diagnosis of lymphadenopathy with fever and cytopenia. It is important that the clinical features of KFD be understood to reach a correct diagnosis.

  2. Misleading pustular plaques of the lower limbs during Crohn's disease: two case reports

    Directory of Open Access Journals (Sweden)

    Farhi David

    2007-10-01

    Full Text Available Abstract Background Extraintestinal manifestations of Crohn's disease may involve the skin, the eyes, the genital mucosa, and the joints. Dermatoses associated with Crohn's disease include neutrophilic dermatoses, erythema nodosum, granulomatous dermatitis, blistering dermatoses, and non-specific skin manifestations. Cutaneous Crohn's disease is characterized by skin non-caseating epithelioid granulomatas with giant cells, remote from the gastrointestinal tract. We report herein two new cases. Observations On both patients, differential diagnosis of neutrophilic dermatoses and infectious disease were evoked, and antimicrobial agents were introduced in one of them. Given the atypical presentation, the final diagnosis of cutaneous Crohn's disease could only be made with histological examination. In patient 1, the plaques decreased in size and infiltration by more than 75% after 3 weeks of treatment with bethametasone dipropionate 0.05% cream. In patient 2, the plaques decreased by more than 50% after 6 weeks of treatment with prednisolone (45 mg/day and azathioprine (100 mg/day. Discussion Cutaneous Crohn's disease may present as dusky, erythematous, infiltrated, and ulcerated plaques and nodules. Female-to-male sex ratio is about 2, and the mean age at onset is 35. Recurrently, the hypothesis of a skin mycobacterial or fungal infection greatly delays proper treatment. Rarity of cutaneous Crohn's disease hampers therapeutic assessment in controlled trials. Thus, available literature is limited to case reports and sparse small series, with contradictory results. These reports are subject to publication bias, and no definite evidence-based recommendations can be made on the most adequate therapeutic strategy.

  3. Stressful life events and Graves' disease: Results of a case control study

    International Nuclear Information System (INIS)

    Pintor, A.B.; Barrenechea, E.A.; Laureta, E.G.; Ligon, R.A.

    2003-01-01

    Prolonged worry has generally been acknowledged as one of the main precipitating factors of the onset of Graves' disease. A review of literature reveals that emotional stresses of considerable severity precede the onset of hyperthyroidism in about 90% of cases. However, not everyone subscribes to the Stress/Graves' disease hypothesis. Attempts to resolve this issue have tended to focus on whether a correlation can be shown between the magnitude of antecedent life events and the disease. Hence the main objective of the present study was to identify the possible association between stressful life events and Graves' disease. This paper presents the results of a case-control study, involving patents and subjects from the Veterans Memorial Medical Centre of Manila. A total of 224 patients of Graves' disease, newly or previously diagnosed, representing the 'patient's' arm were interviewed. All patients were questioned regarding various stresses, which greatly affected their life style spanning over a 12-month period prior to the onset of the disease. Simultaneously, 224 control subjects were also interviewed, and their stresses spanning over a similar period preceding the dates of interviews were recorded in quantifiable terms. Different stresses were given different intensity scores based on a social readjustment scale taking into consideration the life situations, emotions and diseases. In cases of multiple stresses, intensity scores were added to obtain the total stress intensity. Results were expressed as mean, standard deviation, median, frequency and percent distribution. Scatter plot was also constructed for intensity of stressful life events. To determine association of different factors with Graves' disease, Students t-test and chi-square tests were applied to the data. Odds ratio (OR) was also computed to determine risk attributed to each factor. Since there was significant difference in gender distribution between the patients and controls, stratified Mantel

  4. Neurological diseases and bullous pemphigoid: A case-control study in Iranian patients.

    Science.gov (United States)

    Daneshpazhooh, Maryam; Khorassani, Javad; Balighi, Kamran; Ghandi, Narges; Mahmoudi, Hamidreza; Tohidinik, Hamidreza; Hamzelou, Shahin; Chams-Davatchi, Cheyda

    2017-01-01

    Neurological diseases are important co-morbidities found in association with bullous pemphigoid. Various neurological conditions (stroke, Parkinson's disease, dementia, epilepsy and multiple sclerosis) have been reported as associations of this bullous disease; whether these are significant has not been definitely proved. However, the presence of neurological conditions is a predictor of poorer prognosis. Our aim was to examine the association of bullous pemphigoid and neurological diseases in Iranian bullous pemphigoid patients. The medical records of one hundred and sixty consecutive bullous pemphigoid patients who presented to the Autoimmune Bullous Diseases Research Center, Tehran, Iran, from 2006 to 2011 were examined for evidence of any neurological disease. The control group comprised of 317 age- and sex-matched subjects. Neurological diseases were seen in 42 (26.4%) patients with bullous pemphigoid and in 29 (9.1%) controls (odds ratio: 3.53 (2.1-5.9), P< 0.001). Comparing cases to controls, stroke was seen in 17.5% versus 4.1%, odds ratio 4.96 (2.49-9.88); dementia in 5.6% versus 1.9%, odds ratio 3.09 (1.08-8.84); Parkinson's disease in 2.5% versus 2.2%, odds ratio 1.14 (0.33-3.94); epilepsy in 2.5% versus 0.6%, odds ratio 4.04 (0.73-22.3); and multiple sclerosis in 0 versus 0.3% odds ratio 1.00 (0.98-1.01). The main limitations of our study were referral bias, retrospective design and a rather low sample size. Neurological diseases in general, and stroke and dementia in particular, were significantly associated with bullous pemphigoid in our study.

  5. Screening detected celiac disease in children with type 1 diabetes mellitus : Effect on the clinical course - (A case control study)

    NARCIS (Netherlands)

    Rami, B; Sumnik, Z; Schober, E; Waldhor, T; Battelino, T; Bratanic, N; Kurti, K; Lebl, J; Limbert, C; Madacsy, L; Odink, RJH; Paskova, M; Soltesz, G

    Objective: To investigate clinical and metabolic characteristics of diabetic children with screening detected celiac disease in a multicenter case-control study. Methods: Cases: 98 diabetic patients were diagnosed as having silent celiac disease by screening with endomysial antibodies and subsequent

  6. Similarities of Variant Creutzfeldt-Jakob Disease Strain in Mother and Son in Spain to UK Reference Case.

    Science.gov (United States)

    Diack, Abigail B; Boyle, Aileen; Ritchie, Diane; Plinston, Chris; Kisielewski, Dorothy; de Pedro-Cuesta, Jesús; Rábano, Alberto; Will, Robert G; Manson, Jean C

    2017-09-01

    We investigated transmission characteristics of variant Creutzfeldt-Jakob disease in a mother and son from Spain. Despite differences in patient age and disease manifestations, we found the same strain properties in these patients as in UK vCJD cases. A single strain of agent appears to be responsible for all vCJD cases to date.

  7. Congenital heart disease: a hard case for differential diagnosis and treatment

    Directory of Open Access Journals (Sweden)

    David Gonçalves Nordon

    2012-04-01

    Full Text Available ABSTRACT Congenital heart diseases are important malformations that might compromise not only the patient's survival, but also his/her quality of life. We present the case of a female newborn who presented cardiovascular unbalance and cianosis in spite of her previous month of life without any complication. Her differential diagnosis was rather difficult, due not only to restrictions of exams available for diagnosis, but also to their sensibility and specificity. We discuss such differential diagnosis and the complicated development of the case.

  8. Fifty Cases of Parkinson's Disease Treated by Acupuncture Combined with Madopar

    Institute of Scientific and Technical Information of China (English)

    REN Xiao-ming

    2008-01-01

    objective;To search for an effective therapy for trealing motor disorder due to Parkinson's disease(PD).Methods;Fifty cases in a treatrnent group were treated by acupuncture combined with madopar,and 30 cases in a control group treated by madopar only.Results;A total effective rate of 92%was achieved with obvious alleviation of motor disorder in the treatment group,which was significantly higher than that in the control group(P<0.05).Conclusion;Acupuncture can enhance therapeutic effects of western medicine and lessen the dose of the medicine needed.

  9. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): two case reports.

    Science.gov (United States)

    El Kohen, A; Planquart, X; Al Hamany, Z; Bienvenu, L; Kzadri, M; Herman, D

    2001-12-01

    Sinus histiocytosis with massive lymphadenopathy or Destombes-Rosai-Dorfman's syndrome is a rare benign disease of unknown etiology, usually seen in younger patients. The cases reported concerned a 15-month old Caucasian boy and an 8 year old black boy with unilateral cervical enlargement, occasional fever and without any extranodal involvement. Diagnosis was performed by superficial lymph node biopsy. No immunodeficiency was found. The patients received no therapy and a complete spontaneous resolution was seen after a few months in the two cases. The clinical presentation, histologic characteristics, pathogenesis and treatment of the Destombes-Rosai-Dorfman's syndrome are discussed.

  10. Kikuchi – Fujimoto Disease. Case report and a brief review of the literature

    Directory of Open Access Journals (Sweden)

    Manuel Valdebran

    2014-10-01

    Full Text Available Kikuchi Fujimoto disease (KFD was first described in Japan in 1972 almost simultaneously by Kikuchi and Fujimoto. It is a rare, self-limiting, benign form of histiocytic necrotizing lymphadenitis, which can be mistaken for tuberculosis, lymphoma or systemic lupus erythematosus. Although the pathogenesis of KFD is not fully understood, infectious and autoimmune etiologies have been proposed. It generally presents as cervical lymphadenopathy with associated systemic signs and symptoms. Definitive diagnosis requires histopathological examination of the affected lymph nodes. There are only few cases described in the literature, as far as we are aware we report the first case of KFD in the Dominican Republic.

  11. Inclusion and exclusion in the globalisation of genomics; the case of rare genetic disease in Brazil

    Science.gov (United States)

    Gibbon, Sahra

    2018-01-01

    ABSTRACT Within the context of a globalising agenda for genetic research where ‘global health’ is increasingly seen as necessarily informed by and having to account for genomics, the focus on rare genetic diseases is becoming prominent. Drawing from ethnographic research carried out separately by both authors in Brazil, this paper examines how an emerging focus on two different arenas of rare genetic disease, cancer genetics and a class of degenerative neurological diseases known as Ataxias, is subject to and a product of the dynamics of inclusion and exclusion as this concerns participation in research and access to health care. It examines how in these different cases ‘rarenesss’ has been diversely situated and differently politicised and how clinicians, patients and their families grapple with the slippery boundaries between research, rights to health and the limits of care, therapy or prevention. It illustrates how attention to rare genetic disease in Brazil emerges at the intersection of a particular history of genetic research and public health infrastructure, densely complicated feedback loops between clinical care and research, patient mobilisation around the ‘judicialisation’ of health and recent state legislation regarding rare disease in Brazil. It highlights the relevance of local configurations in the way rare genetic disease is being made relevant for and by different communities. PMID:29533091

  12. Looking for Celiac Disease in Italian Women with Endometriosis: A Case Control Study

    Directory of Open Access Journals (Sweden)

    Luca Santoro

    2014-01-01

    Full Text Available In the last years, a potential link between endometriosis and celiac disease has been hypothesized since these disorders share some similarities, specifically concerning a potential role of oxidative stress, inflammation, and immunological dysfunctions. We investigated the prevalence of celiac disease among Italian women with endometriosis with respect to general population. Consecutive women with a laparoscopic and histological confirmed diagnosis of endometriosis were enrolled; female nurses of our institution, without a known history of endometriosis, were enrolled as controls. IgA endomysial and tissue transglutaminase antibodies measurement and serum total IgA dosage were performed in both groups. An upper digestive endoscopy with an intestinal biopsy was performed in case of antibodies positivity. Presence of infertility, miscarriage, coexistence of other autoimmune diseases, and family history of autoimmune diseases was also investigated in all subjects. Celiac disease was diagnosed in 5 of 223 women with endometriosis and in 2 of 246 controls (2.2% versus 0.8%; P=0.265. Patients with endometriosis showed a largely higher rate of infertility compared to control group (27.4% versus 2.4%; P<0.001. Our results confirm that also in Italian population an increased prevalence of celiac disease among patients with endometriosis is found, although this trend does not reach the statistical significance.

  13. Inclusion and exclusion in the globalisation of genomics; the case of rare genetic disease in Brazil.

    Science.gov (United States)

    Gibbon, Sahra; Aureliano, Waleska

    2018-04-01

    Within the context of a globalising agenda for genetic research where 'global health' is increasingly seen as necessarily informed by and having to account for genomics, the focus on rare genetic diseases is becoming prominent. Drawing from ethnographic research carried out separately by both authors in Brazil, this paper examines how an emerging focus on two different arenas of rare genetic disease, cancer genetics and a class of degenerative neurological diseases known as Ataxias, is subject to and a product of the dynamics of inclusion and exclusion as this concerns participation in research and access to health care. It examines how in these different cases 'rarenesss' has been diversely situated and differently politicised and how clinicians, patients and their families grapple with the slippery boundaries between research, rights to health and the limits of care, therapy or prevention. It illustrates how attention to rare genetic disease in Brazil emerges at the intersection of a particular history of genetic research and public health infrastructure, densely complicated feedback loops between clinical care and research, patient mobilisation around the 'judicialisation' of health and recent state legislation regarding rare disease in Brazil. It highlights the relevance of local configurations in the way rare genetic disease is being made relevant for and by different communities.

  14. Disease flare of ankylosing spondylitis presenting as reactive arthritis with seropositivity: a case report

    Directory of Open Access Journals (Sweden)

    Manoj EM

    2012-02-01

    Full Text Available Abstract Introduction Concurrent rheumatoid factor seropositivity is occasionally detected in ankylosing spondylitis and often causes confusion in clinical routine. Overlap between various seronegative arthritides is a known but uncommon association. Differentiation of spondyloarthropathy from rheumatoid arthritis is important, since the natural history, complications, treatments and prognosis of the two diseases differ significantly. Case presentation Here, we report the case of a 47-year-old Sri Lankan man who had a long history of intermittent joint pains worsening following a recent episode of self-resolving non-bloody diarrhea. Subsequently, he developed a skin rash suggestive of keratoderma blenorrhagica and circinate balanitis. He had classical radiological evidence of ankylosing spondylosis (previously undiagnosed associated with human leukocyte antigen B27 antigen, but was positive for rheumatoid factor. Conclusions A disease flare of ankylosing spondylitis prompted by a minor diarrheal illness showing well documented features of reactive arthritis is remarkable. The prognostic implications of seropositivity in spondyloarthritis are discussed.

  15. Five cases of a Joseph disease family with non-REM sleep apnea and MRI study

    International Nuclear Information System (INIS)

    Kitamura, Junichi; Tsuruta, Kazuhito; Yamamura, Yoshinori; Kurihara, Teruyuki; Matsukura, Shigeru

    1987-01-01

    Four male and one female patients of a new Joseph disease family in southern Kyushu are presented. This disorder is inherited by autosomal dominant trait. The clinical symptoms are characterized by bulging eyes, ophthalmoplegia, dysarthria, rigospasticity of the lower limbs, marked dystonia and bradykinesia. In our cases, extrapyramidal symptoms were improved by amantadine and L-Dopa therapy. CSF homovanilic acid (HVA) was markedly reduced. Muscle biopsy and electromyographic studies revealed neurogenic changes. MRI revealed mild atrophy of frontal lobe and cerebellum, and marked atrophy of brain stem. These findings were consistent with the clinical manifestations. Our case had central type sleep apnea by sleep EEG and polygraphic studies. This is the first report about sleep apnea and MRI of Joseph disease. (author)

  16. Iatrogenic Creutzfeldt-Jakob disease following human growth hormone therapy: case report.

    Science.gov (United States)

    Caboclo, Luís Otávio Sales Ferreira; Huang, Nancy; Lepski, Guilherme Alves; Livramento, José Antônio; Buchpiguel, Carlos Alberto; Porto, Cláudia Sellitto; Nitrini, Ricardo

    2002-06-01

    We report the case of a 41-year-old man with iatrogenic Creutzfeldt-Jakob disease (CJD) acquired after the use of growth hormone (GH) obtained from a number of pituitary glands sourced from autopsy material. The incubation period of the disease (from the midpoint of treatment to the onset of clinical symptoms) was rather long (28 years). Besides the remarkable cerebellar and mental signs, the patient exhibited sleep disturbance (excessive somnolence) from the onset of the symptoms, with striking alteration of the sleep architecture documented by polysomnography. 14-3-3 protein was detected in the CSF, and MRI revealed increased signal intensity bilaterally in the striatum, being most evident in diffusion-weighted (DW-MRI) sequences. This is the second case of iatrogenic CJD associated with the use of GH reported in Brazil.

  17. Three Co-Existing Sexually Transmitted Diseases in a Heterosexual Male Youth: A Case Report

    Directory of Open Access Journals (Sweden)

    Theetat M. Surawan

    2016-03-01

    Full Text Available Most sexually transmitted diseases (STDs are asymptomatic, leading to widespread underdiagnoses estimated at 50% or higher. The presence of one STD significantly indicates an individual’s sexual health risk since an STD contributes to the transmission and acquisition of other STDs, including human immunodeficiency virus (HIV infection. Multiple co-existing STDs, thus, further increase the susceptibility of acquiring and transmitting HIV by twofold or more. Therefore, the comprehensive STD prevention strategies play a major role in reducing the transmission of HIV infection. We report an interesting case of a heterosexual male youth who presented at dermatology clinic with three concurrent sexually transmitted diseases: gonococcal urethritis, genital wart, and late latent syphilis. The case demonstrated significant issues for appropriate approaches and management of multiple co-existing STDs. Also, it reinforced the necessity for STD counselling for the patient, his partners, and family.

  18. A rare case of haemolytic disease of newborn with Bombay phenotype mother

    Directory of Open Access Journals (Sweden)

    Shamee Shastry

    2013-01-01

    Full Text Available We are reporting a rare case of severe hemolytic disease of newborn (HDN with Bombay phenotype mother. A retrospective study of a case with severe haemolytic disease of newborn with Bombay phenotype mother was done. Blood grouping, antibody screening, and lectin study was done on the blood sample of the baby and mother to confirm the diagnosis. Hematological and biochemical parameters were obtained from the hospital laboratory information system for the analysis. Blood group of the baby was A positive, direct antiglobulin test was negative. Blood group of the mother was confirmed to be Bombay phenotype, Hematological parameters showed all the signs of ongoing hemolysis and the bilirubin level was in the zone of exchange transfusion. Due to the unavailability of this rare phenotype blood unit, baby was managed conservatively. Anticipating the fetal anemia and HDN with mothers having Bombay phenotype and prior notification to the transfusion services will be of great help in optimizing the neonatal care and outcome.

  19. Five cases of a Joseph disease family with non-REM sleep apnea and MRI study

    Energy Technology Data Exchange (ETDEWEB)

    Kitamura, Junichi; Tsuruta, Kazuhito; Yamamura, Yoshinori; Kurihara, Teruyuki; Matsukura, Shigeru

    1987-09-01

    Four male and one female patients of a new Joseph disease family in southern Kyushu are presented. This disorder is inherited by autosomal dominant trait. The clinical symptoms are characterized by bulging eyes, ophthalmoplegia, dysarthria, rigospasticity of the lower limbs, marked dystonia and bradykinesia. In our cases, extrapyramidal symptoms were improved by amantadine and L-dopa therapy. CSF homovanilic acid (HVA) was markedly reduced. Muscle biopsy and electromyographic studies revealed neurogenic changes. MRI revealed mild atrophy of frontal lobe and cerebellum, and marked atrophy of brain stem. These findings were consistent with the clinical manifestations. Our case had central type sleep apnea by sleep EEG and polygraphic studies. This is the first report about sleep apnea and MRI of Joseph disease.

  20. Creutzfeldt-Jakob disease: A great masquerade in neurology, a rare case report from South India

    Directory of Open Access Journals (Sweden)

    Sivaprakash Varadan

    2015-01-01

    Full Text Available Creutzfeldt-Jakob disease (CJD is a rare, fatal neurodegenerative disease caused by an infectious protein called prion and is characterized by spongiform changes, neuronal loss, reactive astrocytic proliferation, and accumulation of pathologic cellular protein. Clinical presentation of CJD is characterized by rapidly progressive dementia, neurologic symptoms and visual impairment, and the development of akinetic mutism, which can mimic many neurological conditions. The diagnosis is based on clinical presentation, electroencephalogram, and typical cerebrospinal fluid and magnetic resonance imaging (MRI findings. Literature on the incidence and prevalence of CJD is lacking in South India. We report the case of a 57-year-old woman with progressive dementia and typical neurologic symptoms, myoclonic jerks, and MRI findings of CJD. This case highlights the need for a high index of suspicion to diagnose CJD.

  1. Iatrogenic Creutzfeldt-Jakob disease following human growth hormone therapy: case report

    Directory of Open Access Journals (Sweden)

    Caboclo Luís Otávio Sales Ferreira

    2002-01-01

    Full Text Available We report the case of a 41-year-old man with iatrogenic Creutzfeldt-Jakob disease (CJD acquired after the use of growth hormone (GH obtained from a number of pituitary glands sourced from autopsy material. The incubation period of the disease (from the midpoint of treatment to the onset of clinical symptoms was rather long (28 years. Besides the remarkable cerebellar and mental signs, the patient exhibited sleep disturbance (excessive somnolence from the onset of the symptoms, with striking alteration of the sleep architecture documented by polysomnography. 14-3-3 protein was detected in the CSF, and MRI revealed increased signal intensity bilaterally in the striatum, being most evident in diffusion-weighted (DW-MRI sequences. This is the second case of iatrogenic CJD associated with the use of GH reported in Brazil.

  2. Kikuchi-Fujimoto Disease in the United States: Three Case Reports and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Darcie M Deaver

    2013-12-01

    Full Text Available Kikuchi-Fujimoto Disease (KFD, also known as histiocytic necrotizing lymphadenitis, is a benign, self-limiting disease that manifests primarily as cervical lymphadenopathy but may include low-grade fever, headache, and fatigue.  There is a higher incidence of KFD in women aged 20-35 years and in Asian populations.  A PubMed search revealed 590 articles that described KFD.  Of these, 22 cases have been fully described in the United States.  Ten of the 22 (45% patients were male and 12 (55% were female, with 20% Caucasian, 20% Asian American, and the remaining 60% of other ethnic backgrounds.  In this study, we describe an additional 3 cases of KFD and discuss the diagnosis, pathology, and management of KFD.

  3. Diverticular disease of the colon presenting as pyometra: a case report.

    Science.gov (United States)

    Pankaja, Susmita; Rrukaj, Astrit; Bathula, Uma

    2014-05-04

    Pyometra can be caused by various etiologies. We present a rare case of diverticular disease of the colon presenting as pyometra. This type of presentation can be challenging even for an astute clinician. A 74-year-old Caucasian woman with a history of pyometra was referred to our gynecology clinic as an urgent case. She was obese, diabetic and hypertensive. Due to the patient profile and the clinical presentation, clinicians were misled toward a diagnosis of possible endometrial cancer. After further investigations, she was found to have colouterine fistula secondary to a diverticular abscess of the sigmoid colon. Persistent vaginal discharge due to pyometra can be caused by diverticular disease of the colon. Clinicians should be aware of this important differential diagnosis.

  4. Carpal spasm in a girl as initial presentation of celiac disease: a case report.

    Science.gov (United States)

    Ramosaj-Morina, Atifete; Keka-Sylaj, A; Hasbahta, V; Baloku-Zejnullahu, A; Azemi, M; Zunec, R

    2017-09-04

    Celiac disease is an immune-mediated disorder elicited by ingestion of gluten in genetically susceptible persons. This disorder is characterized by specific histological changes of the small intestine mucosa resulting in malabsorption. This case was written up as it was an unusual and dramatic presentation of celiac disease. We report the case of a 3-year-old Albanian girl who presented at our clinic with carpal spasms and hand paresthesia. A physical examination at admission revealed a relatively good general condition and body weight of 10.5 kg (10 percentile). Carpal spasms and paresthesias of her extremities were present. Neuromuscular irritability was demonstrated by positive Chvostek and Trousseau signs. Blood tests showed severe hypocalcemia with a total serum calcium of 1.2 mmol/L (normal range 2.12 to 2.55 mmol/L), ionized calcium of 0.87 (normal range 1.11 to 1.30 mmol/L), and 24-hour urine calcium excretion of 9.16 mmol (normal range female celiac disease was performed: antigliadin immunoglobulin A, anti-tissue transglutaminase, and anti-endomysial immunoglobulin A antibodies were positive. A duodenal biopsy revealed lymphocyte infiltration, crypt hyperplasia, and villous atrophy compatible with celiac disease grade IIIb according to the Marsh classification. Following the diagnosis of celiac disease, human leukocyte antigen typing was performed, giving a definite diagnosis of celiac disease. She was started on a gluten-free diet. Due to failure to follow a gluten-free diet, episodes of carpal spasms appeared again. Unfortunately, at the age of 7 years she presents with delayed psychophysical development. Although hypocalcemia is a common finding in celiac disease, hypocalcemic carpal spasm is a rare initial manifestation of the disease. Therefore, the possibility of celiac disease should be considered in patients with repeated carpal spasms that seem unduly difficult to treat. This should be evaluated even in the absence of gastrointestinal

  5. An unusual case of Extranodal Rosai-Dorfman Disease manifesting as a bilateral Epibulbar mass.

    OpenAIRE

    Dr. Swati Ravani; Dr. Jaideep Sharma

    2017-01-01

    Purpose: To report an unusual presentation of a 24-year-old man with bilateral epibulbar mass, whose presentation was confirmed histopathologically as Rosai Dorfman disease. Method: We studied a retrospective and interventional case report. Results: A 24-year-old man presented with a mass over both eyes, gradually and, progressively increasing over one year. Visual acuity and intraocular pressures were normal in both eyes. Examination revealed bilateral non tender mobile soft in consistency ...

  6. Myositis complicating benzathine penicillin-G injection in a case of rheumatic heart disease

    Directory of Open Access Journals (Sweden)

    Joshua R. Francis

    2016-01-01

    Full Text Available A 7-year old boy developed myositis secondary to intramuscular injection of benzathine penicillin-G in the context of secondary prophylaxis for rheumatic heart disease. Side effects of intramuscular delivery of benzathine penicillin-G are well described and include injection site pain and inflammation, but myositis, as depicted on magnetic resonance imaging in this case, has not previously been described.

  7. A Case of Miller Fisher Syndrome, Thromboembolic Disease, and Angioedema: Association or Coincidence?

    OpenAIRE

    Salehi, Nooshin; Choi, Eric D.; Garrison, Roger C.

    2017-01-01

    Patient: Male, 32 Final Diagnosis: Miller Fisher syndrome Symptoms: Ataxia ? headache ? ophthalmoplegia Medication: ? Clinical Procedure: Plasmapheresis Specialty: Neurology Objective: Rare co-existance of disease or pathology Background: Miller Fisher Syndrome is characterized by the clinical triad of ophthalmoplegia, ataxia, and areflexia, and is considered to be a variant of Guillain-Barre Syndrome. Miller Fisher Syndrome is observed in approximately 1?5% of all Guillain-Barre cases in Wes...

  8. Laryngeal Rosai-Dorfman Disease (Sinus Histiocytosis with Massive Lymphadenopathy): A Retrospective Study of 5 Cases

    OpenAIRE

    Yanyan Niu; Yongjin Li; Jian Wang; Xiaofeng Jin; Dahai Yang; Hong Huo; Wuyi Li

    2017-01-01

    This study was performed to investigate the clinical manifestations, treatment methods, and prognosis of Rosai-Dorfman disease (RDD) with laryngeal involvement. Five clinical cases of RDD with laryngeal involvement diagnosed between 1986 and 2015 were retrospectively analyzed. The laryngeal lesions of these 5 patients mostly involved the glottis and subglottis, with the main symptoms being a hoarse voice and airway obstruction. In addition, the patients mostly exhibited a unilateral or asymme...

  9. Madelung's disease: a case report and literature review

    Energy Technology Data Exchange (ETDEWEB)

    Vidal, Maria da Graca Caminha; Londero, Thiza Massaia [Universidade Federal de Santa Maria (UFSM), RS (Brazil); Haygert, Carlos Jesus Pereira; Zagoury, Andre Rivas [Universidade Federal de Santa Maria (UFSM), RS (Brazil). Hospital Universitario; Adaime, Samia Braga Ramos, E-mail: sbra5@hotmail.co [Hospital Orencio Freitas, Niteroi, RJ (Brazil); Carrion, Rodrigo Previdello [Hospital de Clinicas de Porto Alegre, RS (Brazil)

    2010-07-15

    The authors report the case of a patient with Madelung's disease, which is clinically described as the presence of multiple and symmetric non-encapsulated masses of fatty tissue, usually involving the neck and the upper region of the trunk. Computed tomography was selected as the imaging method for diagnosis confirmation, considering its lower cost and higher availability as compared with magnetic resonance imaging. (author)

  10. Case report 632: Parosteal osteochondromatous hamartoma associated with Trevor's disease (dysplasia epiphysealis hemimelica)

    International Nuclear Information System (INIS)

    Abrahams, T.G.; Whitten, C.G.; Jones, M.; Dorfman, H.J.

    1991-01-01

    The current case represents a patient with classic plain film findings of Trevor's disease (DEH) seen in the foot and ankle, who presented with a large, eccentric metaphyseal lesion in the ipsilateral distal femur. The radiographic and pathologic characteristics of this large metaphyseal lesion were considered atypical for a simple osteochondroma; therefore, we propose the term 'parosteal osteochondromatous hamartoma' as a descriptive term that best fits its unique characteristic. (orig.)

  11. Radiological features in congenital erythropoietic porphyria (Gunther's disease). Report of three cases

    Energy Technology Data Exchange (ETDEWEB)

    Levesque, M.; Legmann, P.; Le Cloirec, A.; Deybach, J.C.; Nordmann, Y.

    1988-01-01

    Gunther's disease or congenital erythropoietic porphyria is a rare and severe disorder comprising cutaneous and haemolytic symptoms. Photocutaneous lesions are responsible for scleroderma-like calcifications and deformities of the extremities visible on X-rays. Hemolytic manifestations lead to diffuse major osteopenia. Soft tissue calcifications of the fingers can be seen even in young patients. One case reported here is the first illustration of intracranial calcifications located on dura-mater and calvarium.

  12. Case report of imaging analysis of the dysplasia epiphysealis hemimelic (Trevor's disease)

    International Nuclear Information System (INIS)

    Shu, Jang Ho; Cho, Kil Ho

    2013-01-01

    Trevor's disease, also known as dysplasia epiphysealis hemimelica, is a rare developmental disorder presented with epiphyseal overgrowth involving one or multiple epiphyses. Here we report the radiologic findings of two cases of dysplasia epiphysealis hemimelica in a 4-year-old boy in the knee without symptom and a 10-year-old boy in the ankle with pain. The former was observed for eight years and the latter was treated with surgical resection.

  13. Case report of imaging analysis of the dysplasia epiphysealis hemimelic (Trevor's disease)

    Energy Technology Data Exchange (ETDEWEB)

    Shu, Jang Ho; Cho, Kil Ho [Dept. of Diagnostic Radiology, College of Medicine, Yeungnam University, Daegu (Korea, Republic of)

    2013-08-15

    Trevor's disease, also known as dysplasia epiphysealis hemimelica, is a rare developmental disorder presented with epiphyseal overgrowth involving one or multiple epiphyses. Here we report the radiologic findings of two cases of dysplasia epiphysealis hemimelica in a 4-year-old boy in the knee without symptom and a 10-year-old boy in the ankle with pain. The former was observed for eight years and the latter was treated with surgical resection.

  14. Castleman's disease presenting as a pleural tumor: a case report with CT findings.

    Science.gov (United States)

    Doo, Kyung Won; Kim, Bomi

    2018-02-01

    Castleman's disease (CD) is an uncommon benign lymphoproliferative disorder which most commonly involves the mediastinum but rarely affects the pleura. We report a case of unicentric CD that presents as a pleural mass in a 45-year-old man, which was subsequently resected followed by an unexpected diagnosis on histologic examination. Although rare, CD should be included in the differential diagnosis of well-enhancing pleural mass.

  15. Creutzfeldt-Jakob’s disease: case report with emphasis on the electroencephalographic features

    OpenAIRE

    Patrícia, Coral; Germiniani, Francisco M. B.; Silvado, Carlos E.

    2005-01-01

    Creuzfeldt-Jakob’s Disease is a rare neurodegenerative disorder that is included among the transmissible spongiform encephalopathies. The clinical features are those of a rapid progressive dementia with mioclonic jerks, which progresses to death in less than one year. We report the case of a 79 years old woman, with initial complaints of vertigo, visual and gait compromise, with an initial brain MRI, EEG and CSF that had no specific features of CJD. After 12 days she was again admitted to the...

  16. Magnetic Resonance Finding of Acute Marchiafava-Bignami Disease with Diffuse Involvement: A Case Report

    International Nuclear Information System (INIS)

    Heo, Young Jin; Jeong, Hae Woong; In, Hyun Sin

    2011-01-01

    Marchiafava-Bignami disease (MBD) is a rare toxic disorder strongly associated with chronic alcoholism. It is characterized by progressive demyelination and necrosis of the corpus callosum. The process may extend to neighboring white matter and subcortical regions. We report a case of MBD in which fluid-attenuated inversion recovery and diffusion-weighted imaging revealed symmetrical hyperintense lesions with diffuse involvement of the corpus callosum, white matter, corticospinal tract, internal capsule, and middle cerebellar peduncle.

  17. Magnetic Resonance Finding of Acute Marchiafava-Bignami Disease with Diffuse Involvement: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Heo, Young Jin; Jeong, Hae Woong; In, Hyun Sin [Dept. of Radiology, Busan Paik Hospital, Inje University College of Medicine, Busan (Korea, Republic of)

    2011-11-15

    Marchiafava-Bignami disease (MBD) is a rare toxic disorder strongly associated with chronic alcoholism. It is characterized by progressive demyelination and necrosis of the corpus callosum. The process may extend to neighboring white matter and subcortical regions. We report a case of MBD in which fluid-attenuated inversion recovery and diffusion-weighted imaging revealed symmetrical hyperintense lesions with diffuse involvement of the corpus callosum, white matter, corticospinal tract, internal capsule, and middle cerebellar peduncle.

  18. Behcet disease combined with Sjogren syndrome: A unique case report and literature review.

    Science.gov (United States)

    Ju, Fang-He; Xu, Ting-Zhen; Hong, Hui-Hua; Mao, He; Wang, Meng; Wang, Zhen

    2018-03-01

    Behcet disease(BD) and Sjogren syndrome(SS) are separate conditions that rarely concomitantly affect an individual. In theory,mild symptoms of patients with BD or SS are easy to igore and,thus,remain undiagnosed. There,it is reasonable to believe there may be some clinical cases of combined diseases that go undiscovered and which needs to be taken seriously. In addition,it has been suggested that herpes simplex virus(HSV) types 1 and 2 are associated with BD,but have not been shown to be correlated to the direct pathogenesis of BD. The role of HSV in BD needs more research and attention. Here,we report a young woman who had both BD and SS. The first symptom of the disease was fever. However,the HSV type 1 IgG and HSV type 2 IgM antibody results were positive in our case and,which rendered this case unique. BD and SS concomitantly affect the individual,and BD was the acute type. IV methylprednisolone was used for 9 days and then oral glucocorticoids was used to instead,and the treatment works very well. BD and SS can concomitantly affect an individual,and we believe that HSV-2 may be directly related to the pathogenesis of BD. The nature of BD as an auto-inflammatory disorder, autoimmune disorder, or both, is controversial. If we can find more patients who combined affected these two disease, it might helpful for us to understand the nature of BD. For patients with clinical diagnosis of BD or SS,we need to be alert that it may combinded the other disease. Long term follow up and detailed inspection are important means to avoid undiscovered.

  19. The work of nurse case managers in a cancer and cardiovascular disease risk screening program.

    Science.gov (United States)

    Fawcett, Jacqueline; Schutt, Russell K; Gall, Gail B; Cruz, Elizabeth Riley; Woodford, Mary Lou

    2007-01-01

    The purpose of this conceptual model of nursing and health policy-based study was to identify the frequency and correlates of activities performed by nurse case managers. Massachusetts Women's Health Network (WHN) contracting organization sites for breast and cervical cancer and cardiovascular disease risk screening. Twenty nurse case managers were interviewed. More time was spent performing client service activities than bureaucratic activities. Frequently performed client service activities were tracking test results, finding/connecting with clients, assessing client needs, and educating clients. The most frequently performed activity was documenting services; the least, discharging clients. Client service activity frequency was correlated with client caseload size, social barriers, overall workload, satisfaction with the way activities are carried out in the WHN, special training in WHN policies and procedures, and contracting organization service delivery arrangements. Bureaucratic activity frequency was correlated with caseload size, workload, months as a WHN case manager, system barriers, satisfaction with the way activities were carried out in the WHN, and special training. Documentation requires a great deal of WHN nurse case managers' time, which perhaps could be more productively spent with clients. Thus, more efficient ways to document services need to be identified. Additional research is needed to determine similarities and differences in activities performed by WHN nurse case managers and other case managers working in cancer and cardiovascular disease screening programs. Strategies need to be identified to remove all barriers that interfere with performance of case manager practice activities. Strategies are needed to reduce client fear of bills, overcome scheduling constraints, and improve translation services to lessen language barriers to effective communication.

  20. Accuracy of Zika virus disease case definition during simultaneous Dengue and Chikungunya epidemics.

    Science.gov (United States)

    Braga, José Ueleres; Bressan, Clarisse; Dalvi, Ana Paula Razal; Calvet, Guilherme Amaral; Daumas, Regina Paiva; Rodrigues, Nadia; Wakimoto, Mayumi; Nogueira, Rita Maria Ribeiro; Nielsen-Saines, Karin; Brito, Carlos; Bispo de Filippis, Ana Maria; Brasil, Patrícia

    2017-01-01

    Zika is a new disease in the American continent and its surveillance is of utmost importance, especially because of its ability to cause neurological manifestations as Guillain-Barré syndrome and serious congenital malformations through vertical transmission. The detection of suspected cases by the surveillance system depends on the case definition adopted. As the laboratory diagnosis of Zika infection still relies on the use of expensive and complex molecular techniques with low sensitivity due to a narrow window of detection, most suspected cases are not confirmed by laboratory tests, mainly reserved for pregnant women and newborns. In this context, an accurate definition of a suspected Zika case is crucial in order for the surveillance system to gauge the magnitude of an epidemic. We evaluated the accuracy of various Zika case definitions in a scenario where Dengue and Chikungunya viruses co-circulate. Signs and symptoms that best discriminated PCR confirmed Zika from other laboratory confirmed febrile or exanthematic diseases were identified to propose and test predictive models for Zika infection based on these clinical features. Our derived score prediction model had the best performance because it demonstrated the highest sensitivity and specificity, 86·6% and 78·3%, respectively. This Zika case definition also had the highest values for auROC (0·903) and R2 (0·417), and the lowest Brier score 0·096. In areas where multiple arboviruses circulate, the presence of rash with pruritus or conjunctival hyperemia, without any other general clinical manifestations such as fever, petechia or anorexia is the best Zika case definition.