Psychogenic Purpura (Gardner-Diamond Syndrome)

Science.gov (United States)

Bhattacharya, Gaurav

2015-01-01

Psychogenic purpura, also known as Gardner-Diamond syndrome or autoerythrocyte sensitization syndrome, is a rare condition characterized by spontaneous development of painful edematous skin lesions progressing to ecchymosis over the next 24 hours. Severe stress and emotional trauma always precede the skin lesions. The condition is most commonly seen in women, but isolated cases have been reported in adolescents and in males. Psychodermatologic evaluation and dermatology and psychiatry liaison have been successful in the treatment of these patients. This report provides an overview of psychogenic purpura and presents the case of a 15-year-old girl. PMID:26137346

A case of immune thrombocytopenic purpura presenting with intracranial hemorrhage

Directory of Open Access Journals (Sweden)

Sinan Akbayram

2013-01-01

Full Text Available Immune thrombocytopenic purpura is an acute, generally considered a self-limiting benign disorder with a 60%-80% change of spontaneous recovery occurring usually within a few months after onset. Intracranial hemorrhage is a rare but life-threatening complication of childhood immune thrombocytopenic purpura. We report a 4-year-old girl who admitted with headache, vomiting, bleeding from noise and bruises on the extremities. Her neurological examination was normal. Based on laboratory finding she was diagnosed immune thrombocytopenic purpura and intracranial hemorrhage. We suggest that cranial imaging should be perform in patients with immune thrombocytopenic purpura admitted with bleeding symptoms, vomiting and headache even if they had no abnormal neurological signs.

Imatinib-induced postoperative periorbital purpura: GASP (Gleevec-Associated Surgical Purpura) in a woman with imatinib-treated chronic myelogenous leukemia.

Science.gov (United States)

Anzalone, C Lane; Cohen, Philip R; Kurzrock, Razelle; Cortes, Jorge E

2014-01-15

Imatinib mesylate is a selective tyrosine kinase inhibitor used in the treatment of chronic myelogenous leukemia. Ocular side effects of imatinib include periorbital edema, which may become so severe as to obstruct the visual field. The purpose of this case study is to describe the clinical characteristics of imatinib- induced postoperative periorbital purpura. We retrospectively reviewed the medical literature using PubMed, searching the terms edema, Gleevec, imatinib, periorbital, postoperative and purpura. Patient reports and previous reviews of the subject were critically assessed and the salient features are presented. Three patients have undergone surgery to reduce the imatinib-induced periorbital edema; two of these individuals have developed imatinib-induced postoperative periorbital purpura. We recommend discontinuing imatinib usage one week prior to periorbital surgery and not resuming therapy until the eighth postoperative day.

[Henoch-Schönlein Purpura Presenting as Intussusception].

Science.gov (United States)

Kim, Keun Young

2017-06-25

Henoch-Schönlein purpura (HSP) is systemic vasculitis disease with various clinical manifestations. Gastrointestinal symptoms in patients with HSP are usually common, with an incidence rate of 62-90%. Most of these gastrointestinal symptoms occur after typical skin purpura, which is a very important clinical evidence for making a diagnosis of HSP. It is difficult to diagnose HSP without skin rash. About 25% of patients may experience gastrointestinal symptoms as their first symptoms. Herein, we report a case of ileo-colic intussusception associated with HSP in a 5-years-old girl presented with diffuse abdominal distension. Our patient did present any symptoms of HSP, such as purpura, arthralgia or arthritis, before surgery.

Diagnosis of Henoch-Schonlein purpura: renal or skin biopsy?

NARCIS (Netherlands)

Davin, Jean-Claude; Weening, Jan J.

2003-01-01

Henoch-Schonlein purpura (HSP) is a form of systemic vasculitis characterized by vascular wall deposits of predominantly IgA, typically involving small vessels in skin, gut, and glomeruli and associated with purpura, intestinal colic, hematuria, and arthralgia or arthritis. HSP nephritis leads to

Acute infectious purpura fulminans due to probable spotted fever

Directory of Open Access Journals (Sweden)

A Kundavaram

2014-01-01

Full Text Available Purpura fulminans (PF is associated with several infections, most notably with meningococcus, staphylococcus, and streptococcus infections. However, there are few reports of association of this entity with spotted fever from India. We report the case of a 55-year-old man who presented with fever, headache, and myalgia. On the seventh day of fever he developed nonblanching purple hemorrhagic purpura on the trunk and most prominently on the extremities consistent with purpura fulminans. Immunofluorescent assay confirmed the diagnosis of spotted fever. PF though common with rocky mountain spotted fever (RMSF is rarely seen in association with Indian tick typhus, the usual cause of spotted fever in India.

Abdominal ultrasonographic manifestation of Henoch-schonlein purpura

International Nuclear Information System (INIS)

Eun, Hyo Won; Kim, Mi Sung; Kang, Beoung Chul; Lee, Sun Wha

1998-01-01

The purpose of this study was to describe the ultrasonographic features and assess the diagnostic value of sonography in the evaluation of children with Henoch-Schonlein purpura. Between October 1993, and Febuary 1998, 67 children with Henoch-Schonlein purpura underwent abdominal ultrasonography, which in 13 was used for follow up. Bowel wall thickness and location, pattern of color Doppler signal in the thickened bowel wall, the size and location of enlarged mesenteric lymph node and the presence of ascites were evaluated. In 42 cases(63%), sonographic findings were positive, and indicated mesenteric lymphadenopathy(n=3D21), small bowel wall thickening(n=3D20), and ascites(n=3D17). Thickened bowels were demonstrated at the ileum in 11 cases, the jejunum in five, the duodenum in one, and combined wall thickening at the duodenum and jejunum in two;thickening of the duodenum and ileum was seen in one case. Thickness varied from 3 to 10 mm(mean:6.5 mm). On follow-up sonography, regression of bowel wall thickening was observed earlier than that of mesenteric lymphadenopathy or ascites, and correlated well with improved abdominal symptoms. Abdominal ultrasonographic manifestations of Henoch-Schonlein purpura were bowel wall thickening, mesentric lymphadenopathy and ascites. Sonography was a simple and useful method for the evaluation of gastrointestinal manifestation of Henoch-Schonlein purpura.=20

Unilateral purpura annularis telangiectodes of majocchi in an elderly male: an atypical presentation.

Science.gov (United States)

Wang, Apphia; Shuja, Fareesa; Chan, Audrey; Wasko, Carina

2013-08-15

Purpura annularis telangiectodes (PAT), also known as Majocchi purpura, is a rare form of pigmented purpuric dermatosis characterized by non-palpable red-brown, occasionally pruritic patches which progress to hyperpigmented halos. Purpura annularis telangiectodes usually presents in female adolescents as benign symmetric lesions with a predilection for the lower extremities. We present an atypical case of unilateral PAT in an elderly male. To our knowledge, our patient at 85-years-old is the oldest PAT and first unilateral purpura annularis telangiectodes case described in the literature.

Penis Ödemi ile Kendini Gösteren Henoch-Schönlein Purpurası

OpenAIRE

UZUN, Hakan; ALAGÖZ, Demet; ?ENSES, Dursun Ali; D?K?C?, Bünyamin; KOCABAY, Kenan

2015-01-01

Henoch Schonlein purpurası artrit/artralji, gastrointestinal sistem ve renal tutulumun e?lik etti?i nontrombositopenik purpura ile karakterize bir sendromdur. Burada penis ödemi ile ba?vuran 3 ya??ndaki bir erkek hasta sunulmuştur. Bu ?ekilde nadir olan bağlangıç bulguları nedeni ile Henoch Schonlein purpurası tanıs?nda zorluk çekilebilmektedir.

Coma in thrombotic thrombocytopenic purpura

NARCIS (Netherlands)

F.J. de Jong (Fransina); P.A.W. te Boekhorst (Peter); D.W.J. Dippel (Diederik); B.C. Jacobs (Bart)

2010-01-01

textabstractThrombotic thrombocy topenic purpura (TTP) is characterised by a thrombotic, haemolytic microangiopathy leading to microvascular occlusion, haemolysis and ischaemic dysfunction of various organs including the brain. TTP may present with a variety of neurological symptoms, including

Acrally distributed dermatoses: Vascular dermatoses (purpura and vasculitis).

Science.gov (United States)

Kazandjieva, Jana; Antonov, Dimitar; Kamarashev, Jivko; Tsankov, Nikolai

Purpuric lesions appear in acral distribution in a variety of conditions and often provide clues to the clinical diagnosis. Purpuric means "hemorrhagic"-that is, the lesions do not blanch from pressure. This review focuses on dermatoses that produce hemorrhagic lesions in acral distribution from the large groups of the vasculitic diseases and their mimics. Cutaneous small vessel vasculitis is confined to the skin, involves mainly postcapillary venules, and has the hallmark manifestation of palpable purpura. Henoch-Schönlein purpura is an immune complex-mediated systemic vasculitis of the small vessels with manifestations from the skin, joints, kidneys, and gastrointestinal system. Only cases where the immune complexes contain immunoglobulin A type are classified as Henoch-Schönlein purpura. Cryoglobulinemic vasculitis is induced by the deposition of cold-precipitated immune complexes in the small vessels. Urticarial vasculitis comprises a spectrum of conditions with the characteristic course of chronic urticaria, with wheals that persist longer than 24 hours, leave hyperpigmentation, and have leukocytoclastic vasculitis on histologic examination. Polyarteritis nodosa is a rare multisystem, segmental necrotizing vasculitis of mainly the medium-sized vessels. Pigmented purpuric dermatoses are chronic benign dermatoses characterized by petechiae, purpura, and increased skin pigmentation. The hallmark of pigmented purpuric dermatoses is their orange-brown, speckled, cayenne pepper-like discoloration. Copyright © 2016 Elsevier Inc. All rights reserved.

Purpura fulminans

DEFF Research Database (Denmark)

Jordan, Karina; Kristensen, Kim

2010-01-01

Varicella-associated purpura fulminans (PF) is a rare complication to varicella infection. The condition is due to autoantibodies directed against protein S which forms part of the anticoagulation system. Lack of protein S leads to disseminated intravascular coagulation in the small vessels, whic...... causes thrombosis and ischemia. Despite early treatment, amputation and skin-grafting is often necessary. In this case story, we give a brief review of the pathogenesis and possible modes of treatment. Knowledge of PF is necessary since early treatment may be life-saving....

Anti-HPA-1b Mediated Posttransfusion Purpura: A Case Report

Directory of Open Access Journals (Sweden)

O. P. Arewa

2013-01-01

Full Text Available Posttransfusion purpura (PTP is an uncommon, but potentially fatal, transfusion reaction characterized by profound thrombocytopenia and bleeding. PTP is caused by alloimmunization to human platelet specific antigens following blood component transfusion. Although there is evidence of a wide serological spectrum of culprit antibodies implicated, Anti-human-platelet-antigen- (HPA- 1a is the most common antibody in cases reported. We report a case of posttransfusion purpura in an African American. The patient was negative for HPA-1a antibodies, but anti-HPA-1b was identified with a platelet phenotype of HPA-1a/HPA-1a. Although less common, HPA-1b antibody may be an important consideration in posttransfusion purpura diagnosed in patients of African descent.

Pulmonary manifestations in anaphylactoid purpura (Henoch-Schoenlein syndrome)

International Nuclear Information System (INIS)

Fiegler, W.; Siemoneit, K.D.

1981-01-01

Pulmonary manifestations in anaphylactoid purpura (Henoch-Schoenlein-syndrome). Radiological observations are reported on 2 female adults with Henoch-Schoenlein-syndrome accompanied by reversible pulmonary disease. This form of anaphylaktoid purpura has rarely been reported until now. The pulmonary opacities are regarded as intraalveolar bleeding probably with edem and interstitial perivascular infiltrations. They developed at the same time as the skin lesions and are probably part of the disease. These pulmonary changes are characterized by the following radiological criteria: 1. parahilar, butterfly-shaped opacities, 2. air-bronchograms, 3. reticulo-nodular pattern, 4. rapid change. (orig.) [de

The profile of adult onset Henoch-Schönlein purpura in an Asian population.

Science.gov (United States)

Yong, Adeline Mei-Yen; Lee, Shan-Xian; Tay, Yong-Kwang

2015-11-01

Henoch-Schönlein purpura (HSP) is less common in adults and has been linked with a more severe clinical syndrome as well as a higher frequency of renal disease and internal malignancy. Renal involvement in adult HSP has been significantly associated with antecedent infections, pyrexia at time of first presentation, and purpura above the waist. We aim to evaluate the frequency of cutaneous and extra-cutaneous features and identify the predictive factors for renal involvement in Asian adults with HSP. We performed a retrospective study of 48 adult Asian patients diagnosed with HSP based on the European League Against Rheumatism (EULAR) criteria at a tertiary hospital in Singapore between January 2000 and December 2011. The most common cutaneous manifestations were palpable purpura (73%), papules (31%), and petechiae (27%). Forty-percent had cutaneous lesions extending above the waist. Fifteen patients (31%) had gastrointestinal symptoms, 21 (44%) had joint involvement, and 27 (56%) had renal disease. Seventy-percent of patients with pyrexia at presentation experienced renal disease, whereas only 30% without pyrexia had renal involvement (P = 0.018). Sixty-six percent of patients with purpura had renal involvement as compared to 31% in those without purpura (P = 0.049). The frequency of renal involvement in patients with purpura above the waist (52%) was similar to those with purpura below the waist (55%). Our study confirms that HSP in adults tends to be more severe with a high incidence of extracutaneous manifestations, especially renal disease. Pyrexia at presentation and the presence of purpura were significant predictive factors for renal involvement. © 2015 The International Society of Dermatology.

Spectrum of purpura fulminans: report of three classical prototypes and review of management strategies.

Science.gov (United States)

Talwar, Ankur; Kumar, Sharath; Gopal, M G; Nandini, A S

2012-01-01

Purpura fulminans is a rare syndrome of intravascular thrombosis and hemorrhagic infarction of the skin that is rapidly progressive and is accompanied by vascular collapse and disseminated intravascular coagulation. It usually occurs in children, but this syndrome has also been noted in adults. The three forms of this disease are classified by the triggering mechanisms. We describe three classical cases of purpura fulminans of the three classical prototypes treated at our center and their varied clinical outcomes. We also describe a case of acute infectious purpura fulminans secondary to systemic leptospirosis which to our best knowledge is the first reported case in world literature. The various treatment options for purpura fulminans have also been reviewed.

Spectrum of purpura fulminans: Report of three classical prototypes and review of management strategies

Directory of Open Access Journals (Sweden)

Ankur Talwar

2012-01-01

Full Text Available Purpura fulminans is a rare syndrome of intravascular thrombosis and hemorrhagic infarction of the skin that is rapidly progressive and is accompanied by vascular collapse and disseminated intravascular coagulation. It usually occurs in children, but this syndrome has also been noted in adults. The three forms of this disease are classified by the triggering mechanisms. We describe three classical cases of purpura fulminans of the three classical prototypes treated at our center and their varied clinical outcomes. We also describe a case of acute infectious purpura fulminans secondary to systemic leptospirosis which to our best knowledge is the first reported case in world literature. The various treatment options for purpura fulminans have also been reviewed.

Diagnosis and Treatment of Thrombotic Thrombocytopenic Purpura ...

African Journals Online (AJOL)

Diagnosis and Treatment of Thrombotic Thrombocytopenic Purpura. ... by features of microangiopathic hemolytic anemia and thrombocytopenia. ... Current knowledge in the etiopathogenesis, epidemiology, trends in the diagnosis and ...

A case of eczematid-like purpura of Doucas and Kapetanakis in a child.

Science.gov (United States)

Vedak, Priyanka; Nazarian, Rosalynn M; Kroshinsky, Daniela

2015-01-01

Eczematid-like purpura of Doucas and Kapetanakis is a subtype of the pigmented purpuric dermatoses, a group of uncommon dermatoses of unclear etiology characterized by purpura, petechiae, and hyperpigmentation. The Doucas and Kapetanakis subtype is rare in children, and its subtle findings can initially be overlooked and mistaken for other, more common dermatologic disorders in this age group. We present a case eczematid-like purpura of Doucas and Kapetanakis in an 11-year-old boy initially treated as eczema. © 2015 Wiley Periodicals, Inc.

Purpura fulminans associated with Streptococcus pneumoniae septicemia in an asplenic pediatric patient.

Science.gov (United States)

Konda, S; Zell, D; Milikowski, C; Alonso-Llamazares, J

2013-09-01

Purpura fulminans is a rapidly progressive syndrome of small-vessel thrombosis and hemorrhagic necrosis of the skin accompanied by disseminated intravascular coagulation. We describe a case of Streptococcus pneumoniae septicemia in an asplenic 5-year-old boy on oral tacrolimus, with a past medical history of multivisceral organ transplantation and subsequent development of purpura fulminans on his chest and distal extremities. The acute infectious form of purpura fulminans is usually caused by gram-negative bacteria. Cases secondary to gram-positive encapsulated bacteria usually occur when individuals are immuno-suppressed or have anatomic or functional asplenia. Our patient had both, which likely increased his susceptibility, and he responded well to antimicrobial therapy in addition to prophylactic coverage in the setting of his immunosuppression. We review the literature for similar cases due to S. pneumoniae in the pediatric population and discuss the etiology and treatment of purpura fulminans. Copyright © 2011 Elsevier España, S.L. and AEDV. All rights reserved.

[Clinical characteristics of Henoch-Schönlein purpura in children].

Science.gov (United States)

Liu, Li-Jun; Yu, Jing; Li, Yu-Ning

2015-10-01

To explore the clinical characteristics of Henoch-Schönlein purpura (HSP) in children. The clinical data of 325 hospitalized children who were diagnosed with HSP between June 2012 and June 2014 were analyzed retrospectively. In the 325 children with HSP, the incidence of HSP was higher in winter and spring, with 33.9% and 27.4%, respectively. Infection was the major factor to induce HSP (57.2%). The incidence of renal damage in children with purpura accompanied by abdominal symptoms and children with purpura accompanied by abdominal and joint symptoms was 60.3% and 48.9%, respectively, with statistically significant differences compared with children with purpura alone (Ppurpura nephritis, the pathological grades of IIIa and IIIb were more common, accounting for 28% and 31%, respectively. In 325 children, an increased serum D-dimer level was observed in 260 children (80.0%), an increased peripheral IgA content in 101 children (46.3%), and a decreased CD4+ cell percentage in 62 children (56.4%). A high incidence of HSP is often seen in spring and winter. HSP is often induced by upper respiratory tract infection. Renal damage is more likely to occur in children with digestive tract symptoms, with IIIa and IIIb as the common pathological grades of renal damage.

Targeting study of gelatin adsorbed clodronate in reticuloendothelial system and its potential application in immune thrombocytopenic purpura of rat model

International Nuclear Information System (INIS)

Li, P.; Tan, Z.; Zhu, Y.

2007-01-01

Full text: Depletion of splenic and hepatic macrophages has potentials to alleviate hemorrhage in patients who suffered from immune thrombocytopenic purpura (ITP). This investigation was aimed to assess whether nanotechnology can play a role in this clinical setting by absorbing bisphosphonate clodronate (CLOD) to type A gelatin nanospheres (GNS) to form CLOD-GNS. First, the stability of CLOD-GNS was assessed in- vitro and up to 6 mg CLOD can be adsorbed in 1 mg GNS. The ability of CLOD-GNS to target the spleen and the liver was then evaluated by biodistribution assay and 99mTc-CLOD-GNS scintigraphy in rats. It showed that up to 70.6% of CLOD-GNS could be accumulated in the liver and spleen. The survival of the macrophages in vitro and the phagocytic ability of hepatic and splenic macrophage in vivo were reduced and later demonstrated by99mTc-phytic colloid scintigraphy. In rats with induced ITP, administration of CLOD-GNS successfully prevented peripheral platelet levels from decreasing. Our preliminary data demonstrate that CLOD-GNS can effectively target the reticuloendothelial system and has potentials in the treatment of ITP warrants further study. (author)

Fireogfyrre svangerskaber med idiopatisk trombocytopenisk purpura

DEFF Research Database (Denmark)

Clausen, Tine Dalsgaard; Jønsson, Viggo; Wiik, Allan

2002-01-01

INTRODUCTION: The aim of this project was to describe the course of pregnancy with idiopathic thrombocytopenic purpura (ITP) and to estimate risk factors and indications for treatment. MATERIAL AND METHODS: Birth, haematological, and neonatal files were examined retrospectively. RESULTS: Forty...

Purpura fulminans mimicking toxic epidermal necrolysis - additional value of 16S rRNA sequencing and skin biopsy.

Science.gov (United States)

Dautzenberg, K H W; Polderman, F N; van Suylen, R J; Moviat, M A M

2017-05-01

Both purpura fulminans and toxic epidermal necrolysis (TEN) are rare and life-threatening disorders with a high mortality. We present a case of suspected rapidly progressive, severe pneumococcal sepsis-induced purpura fulminans complicated by multiple organ failure, severe epidermolysis and cutaneous necrosis. We show the diagnostic challenge to differentiate between purpura fulminans and TEN, as the extensive epidermolysis in purpura fulminans may mimic TEN and we highlight the additional value of repeated skin biopsies and 16S rRNA gene sequencing.

[New marker in thrombotic thrombocytopenic purpura

DEFF Research Database (Denmark)

Hillarp, A.; Lindblom, A.; Bjork, P.

2008-01-01

Thrombotic microangiopathy can be caused by several conditions which are difficult to diagnose from the clinical presentation alone. Deficient enzyme activity of a newly-discovered enzyme, ADAMTS-13, can lead to thrombotic thrombocytopenic purpura (TTP). Lack of ADAMTS-13 activity causes increased...

Henoch-Schonlein purpura on the legs (image)

Science.gov (United States)

... children than adults and often occurs after an upper respiratory infection. It causes skin rashes that bleed into the skin (petechiae and purpura). Bleeding may also occur from the gastrointestinal tract and kidneys.

High-dose methylprednisolone pulse therapy for treatment of refractory intestinal involvement caused by Henoch-Schönlein purpura: a case report.

Science.gov (United States)

Kang, Hyun Sik; Chung, Hee Sup; Kang, Ki-Soo; Han, Kyoung Hee

2015-03-24

Henoch-Schönlein purpura is an immunoglobulin A-mediated, small vascular inflammatory disease that can be associated with palpable purpura, arthralgia, abdominal pain, or nephritis. The presence of purpura facilitates the diagnosis of Henoch-Schönlein purpura at the onset of associated symptoms, whereas the absence of purpura makes the diagnosis challenging. It is important to diagnose Henoch-Schönlein purpura with delayed-onset skin purpura to avoid unnecessary surgery for acute abdomen. Most cases of Henoch-Schönlein purpura with severe abdominal pain are treated with low-dose steroids and intravenous immunoglobulin. A 15-year-old Korean girl complained of severe abdominal pain and delayed-onset purpura on admission. Henoch-Schönlein purpura was diagnosed based on endoscopic findings of hemorrhagic duodenitis and duodenal vasculitis and abdominal computed tomography findings of edematous bowels. Two common initial treatments, a low-dose steroid and intravenous immunoglobulin, were administered, but there was no improvement for 1 month. Subsequently, we used high-dose intravenous methylprednisolone pulse therapy (30 mg/kg/day, with a maximum of 1g/day), which dramatically alleviated her abdominal symptoms. High-dose intravenous methylprednisolone pulse therapy can be used as the ultimate treatment for delayed-onset Henoch-Schönlein purpura with severe abdominal pain when symptoms do not improve after low-dose steroid and intravenous immunoglobulin treatments.

CT and US demonstration of gastrointestinal involvement in children with rheumatoid purpura

International Nuclear Information System (INIS)

Li Xin; Li Lin; Wang Chunxiang; Zhao Bin

2002-01-01

Objective: To evaluate the usefulness of CT and US in the diagnosis of gastrointestinal involvement in children with rheumatoid purpura. Methods: Abdominal CT and US findings in 14 cases of clinical and laboratory confirmed of rheumatoid purpura were analyzed. There were 9 males and 5 females with the age ranged from 6 months to 14 years. All patients was hospitalized because of acute abdominal emergency, and the clinical presentations included paroxysm of abdomen pain, vomiting, without skin rashes and subcutaneous hemorrhage. CT examination was done in 7 cases, and contrast enhancement was performed in 1 case. Abdominal ultrasound was done in 9 cases. Both CT and US were done in 2 cases. Results: All patients demonstrated multiple or single areas of bowel-wall thickening, localized intraluminal narrowing, mesenteric edema, and liquid in abdominal cavity by CT and ultrasound. Conclusion: Abdominal CT and US examination have important values in differential diagnosis of acute abdominal disorders in children patients with rheumatoid purpura. Before appearing skin rashes, classical picture can help for the diagnosis and clinical treatment. Abdominal CT and US examination can also find the intussusception, bowel obstruction, and perforation in rheumatoid purpura

Acute scrotal pathology in Schonlein-Henoch purpura: management by color Doppler ultrasound. A case report

International Nuclear Information System (INIS)

Jornet, J.; Cervera, J.; Marco, S.F.; Gonzalez, M.

1998-01-01

Schonlein-Henoch purpura is the most common form of purpura in children. It can affect a number of organs, but scrotal involvement is rare. We present a case of scrotal hemorrhage and epidymo-orchitis, diagnosed by color Doppler ultrasound, in which conservative treatment was employed. The differential diagnosis involves testicular torsion, which is also, although less frequently, associated with Schonlein-Henoch purpura. color Doppler ultrasound results in the correct diagnosis, ruling out testicular torsion and avoiding surgical exploration of the testicles. (Author) 8 refs

Henoch-Schönlein Purpura Complicated by Hepatocellular Carcinoma.

Science.gov (United States)

Akizue, Naoki; Suzuki, Eiichiro; Yokoyama, Masayuki; Inoue, Masanori; Wakamatsu, Toru; Saito, Tomoko; Kusakabe, Yuko; Ogasawara, Sadahisa; Ooka, Yoshihiko; Tawada, Akinobu; Maru, Yugo; Matsue, Hiroyuki; Chiba, Tetsuhiro

2017-11-15

Although Henoch-Schönlein purpura (HSP) is known to be accompanied by malignancies, cases with hepatobiliary cancer are extremely rare. A 62-year-old man with palpable purpura rapidly extending to both lower legs was admitted to our hospital. He was undergoing follow-up for cirrhosis caused by chronic hepatitis B virus infection and hepatocellular carcinoma (HCC). He had renal dysfunction with hematuria and proteinuria and abdominal pain. Based on the clinical presentation and skin biopsy findings, he was diagnosed with HSP. The administration of steroids resulted in the rapid improvement of the patient's symptoms and he was discharged 12 days after admission.

Henoch-schönlein purpura (HSP) in an adult

Science.gov (United States)

Negara, C. A.; Zubir, Z.

2018-03-01

Henoch-schönlein purpura (HSP) is vasculitis of the small vessels, the most common vasculitis of the childhood and is uncommon in adults. A case of HSP is reported in a 36-year-old female with ten days history of multiple palpable purpura on region antebrachii, region femoralis and cruris dextra et sinistra. Burn sensation in both legs, pain sensation on knees joint and ankles joint and bloody stools were found. History of a cough and sore throat are often to be a presentation. Laboratory examination was mild anemia, mild leukocytes, ASTO (antistreptolysin titer O): children due to an increased risk of disorders of the renal.

An 'inflammatory' variant of solar purpura: a simulant of leukocytoclastic vasculitis and neutrophilic dermatoses.

Science.gov (United States)

Wood, Benjamin A; LeBoit, Philip E

2013-08-01

To study the clinical and pathological features of cases of apparent solar purpura, with attention to the recently described phenomenon of inflammatory changes within otherwise typical lesions. We studied 95 cases diagnosed as solar purpura and identified 10 cases (10.5%) in which significant neutrophilic inflammation was present, potentially simulating a leukocytoclastic vasculitis or neutrophilic dermatosis. An additional three cases were identified in subsequent routine practice. The clinical features, including follow-up for subsequent development of vasculitis and histological features were studied. In all cases the histological features were typical of solar purpura, with the exception of inflammatory changes, typically associated with clefting of elastotic stroma. Clinical follow-up information was available for all patients and none developed subsequent evidence of a cutaneous or systemic vasculitis or neutrophilic dermatosis. Inflammatory changes appear to be more frequent in solar purpura than is generally recognised. Awareness of this histological variation and correlation with the clinical findings and evolution is important in avoiding misdiagnosis.

Henoch-Schonlein purpura on an infant's legs (image)

Science.gov (United States)

... children than adults and often occurs after an upper respiratory infection. It causes skin rashes that bleed into the skin (petechiae and purpura). Bleeding may also occur from the gastrointestinal tract and kidneys.

Henoch-Schonlein purpura on an infant's foot (image)

Science.gov (United States)

... children than adults and often occurs after an upper respiratory infection. It causes skin rashes that bleed into the skin (petechiae and purpura). Bleeding may also occur from the gastrointestinal tract and kidneys.

Idiopathic Thrombocytopenic Purpura Misdiagnosed as Hereditary Angioedema

DEFF Research Database (Denmark)

Andersen, Michelle Fog; Bygum, Anette

2015-01-01

however not always have to be caused by angioedema but can relate to other concomitant disorders. In this report we are focusing on misdiagnosis in a patient with known hereditary angioedema, whose bleeding episode caused by idiopathic thrombocytopenic purpura was mistaken for an acute attack...

Two cases of infectious purpura fulminans and septic shock caused by Capnocytophaga canimorsus transmitted from dogs

DEFF Research Database (Denmark)

Christiansen, Claus Behrend; Berg, Ronan Martin Griffen; Plovsing, Ronni R.

2012-01-01

We report 2 cases of Capnocytophaga canimorsus-induced septicaemia complicated by purpura fulminans in previously healthy individuals, both of whom had been exposed to dog saliva prior to disease. They both presented with purpuric skin lesions, as well as the tetrad of abdominal symptoms, haemoly......We report 2 cases of Capnocytophaga canimorsus-induced septicaemia complicated by purpura fulminans in previously healthy individuals, both of whom had been exposed to dog saliva prior to disease. They both presented with purpuric skin lesions, as well as the tetrad of abdominal symptoms......, haemolytic anaemia, metabolic acidosis, and renal failure, which may be common in C. canimorsus-associated purpura fulminans. The patients survived after treatment with broad-spectrum antibiotics and supportive intensive care. C. canimorsus should be considered as a possible cause of infectious purpura...

Primary Sjögren syndrome that initially presented with repeated hypergammaglobulinemic purpura after prolonged sitting: A case report.

Science.gov (United States)

Zhou, Zhihua; Jiang, Weiqiang; Wang, Ming; Liu, Yongyuan; Zhang, Wei; Huang, Manping; Liang, Donghui

2017-12-01

Purpura is a common dermatologic manifestation in Sjögren syndrome (SS). When a patient presents with sicca symptoms, the diagnosis of SS is not difficult. Here, we reported a case of a 52-year-old Chinese woman who initially presented with nonpalpable purpura on both lower extremities, and these lesions had developed soon after prolonged sitting. In the past 2 years, she had repeated cutaneous nonpalpable purpura 4 times. She had no sicca symptoms, dry eyes, or dry mouth. Combining the laboratory findings, Schirmer test, and labial gland biopsy, primary SS was confirmed. The patient was placed on a trial of hydroxychloroquine (200 mg once daily). The purpura on both lower extremities had faded at the sixth day after onset and at the third day after hydroxychloroquine treatment. These case was not easy to diagnosis primary SS because she had no sicca symptoms. A patient with primary SS who initially presented with recurrent purpura associated with prolonged sitting. Prolonged sitting had been a possible aggravating factor for the cutaneous purpura of this patient with primary SS. Copyright © 2017 The Authors. Published by Wolters Kluwer Health, Inc. All rights reserved.

[Changes in 25-hydroxyvitamin D3 level in children with Henoch-Schönlein purpura].

Science.gov (United States)

Zhang, Yuan-Da; Dong, Qing-Wei; Li, Rong-Min; Ji, Chao-Yu; Chu, Yong-Tao; Ma, Lei; Zhang, Yu

2017-03-01

To examine the changes in 25-hydroxyvitamin D 3 [25-(OH)D 3 ] level in children with Henoch-Schönlein purpura (HSP) and its clinical significance. A total of 92 HSP children were included in this study, and were divided into HSP nephritis (HSPN) group (31 cases) and HSP group (61 cases) based on the presence or absence of HSPN. Alternatively, the patients were divided into purpura alone group (22 cases), purpura with joint symptoms group (joint symptom group, 24 cases), purpura with gastrointestinal symptoms group (gastrointestinal symptom group, 20 cases), and purpura with joint and gastrointestinal symptoms (mixed group, 26 cases) based on their clinical symptoms. In addition, 42 healthy children were selected as healthy control group. The level of 25-(OH)D 3 in each group was measured using enzyme-linked immunoassay. The 25-(OH)D 3 level in the HSP and HSPN groups was significantly lower than that in the healthy control group (Ppurpura alone group (P<0.05). The level of 25-(OH)D 3 is reduced in children with HSP, particularly those with HSPN or with joint and gastrointestinal symptoms. Therefore, the reduction in 25-(OH)D 3 level may serve as a predictor of whether HSP is associated with other impairments.

[Psychogenic purpura with hematuria and sexual pain disorder: a case report].

Science.gov (United States)

Ozyildirim, Ilker; Yücel, Başak; Aktan, Melih

2010-01-01

Psychogenic purpura (Gardner-Diamond syndrome) is the occurrence and spontaneous recurrence of painful ecchymosis following emotional stress and minor trauma. Although the exact mechanism of this syndrome remains unknown, apart from skin lesions, different types of hemorrhaging have been reported, such as epistaxis, gastrointestinal bleeding, and bleeding from the ear canals and eyes. We report a psychogenic purpura case that presented with hematuria in addition to skin lesions. Based on the psychiatric evaluation she was diagnosed with major depressive disorder, generalized anxiety disorder, and obsessive-compulsive disorder. Additionally, sexual pain disorder accompanied these disorders. With the help of antidepressant and supportive psychotherapy, the patient's ecchymosis and bleeding disappeared. During 8 months of follow-up the symptoms did not return. Vaginismus has not been reported in patients with psychogenic purpura. The presence of vaginismus, which is seen more frequently in eastern cultures and is thought to be related to sociocultural determinants, suggests that some cultural factors may be common to both psychogenic purpura and vaginismus. The aim of this case report was to call attention to a syndrome that is rarely seen and diagnosed, and to discuss its relationship to psychosocial factors. This syndrome should be considered in the differential diagnosis of not only ecchymotic lesions, but also various types of bleeding, including hematuria. Despite the fact that its etiology and treatment are not clearly understood, it should be noted that psychological factors play a role in this disease and therefore, psychopharmacological and psychotherapeutic approaches can be effective.

Pregabalin- and azithromycin-induced rhabdomyolysis with purpura: An unrecognized interaction: A case report.

Science.gov (United States)

Kato, Kazuya; Iwasaki, Yoshiaki; Onodera, Kazuhiko; Higuchi, Mineko; Kato, Kimitaka; Kato, Yurina; Tsutsui, Masato; Taniguchi, Masahiko; Furukawa, Hiroyuki

2016-01-01

Rhabdomyolysis associated with the use of pregabalin or azithromycin has been demonstrated to be a rare but potentially life-threatening adverse event. Here, we report an extremely rare case of rhabdomyolysis with purpura in a patient who had used pregabalin and azithromycin. We present the case of a 75-year-old woman with a history of fibromyalgia who was admitted with mild limb weakness and lower abdominal purpura. She was prescribed pregabalin (75mg, twice daily) for almost 3 months to treat chronic back pain. Her medical history revealed that 3days before admission, she began experiencing acute bronchitis and was treated with a single dose of azithromycin (500mg). She had developed rapid onset severe myalgia, mild whole body edema, muscle weakness leading to gait instability, abdominal purpura and tender purpura on the lower extremities. Laboratory values included a white blood cell count of 25,400/mL and a creatinine phosphokinase (CPK) concentration of 1250 IU/L. Based on these findings and the patient's clinical history, a diagnosis of pregabalin- and azithromycin-induced rhabdomyolysis was made. The long-term use of pregabalin and the initiation azithromycin therapy followed by a rapid onset of rhabdomyolysis is indicative of a drug interaction between pregabalin and azithromycin. We report an extremely rare case of rhabdomyolysis with purpura caused by a drug interaction between pregabalin and azithromycin. However, the mechanisms of the interactions between azithromycin on the pregabalin are still unknown. Copyright © 2016 The Author(s). Published by Elsevier Ltd.. All rights reserved.

Ny markør ved trombotisk trombocytopenisk purpura

DEFF Research Database (Denmark)

Gøtze, Jens Peter; Hillarp, Andreas; Lindblom, Anders

2008-01-01

Thrombotic microangiopathy can be caused by several conditions which are difficult to diagnose from the clinical presentation alone. Deficient enzyme activity of a newly-discovered enzyme, ADAMTS-13, can lead to thrombotic thrombocytopenic purpura (TTP). Lack of ADAMTS-13 activity causes increased...

Coexisting Situs Inversus Totalis and Immune Thrombocytopenic Purpura.

Science.gov (United States)

Gundogdu, Kemal; Altintoprak, Fatih; Uzunoğlu, Mustafa Yener; Dikicier, Enis; Zengin, İsmail; Yağmurkaya, Orhan

2016-01-01

Situs inversus totalis is a rare congenital abnormality with mirror symmetry of mediastinal and abdominal organs. Immune thrombocytopenic purpura is an autoimmune disease with destruction of thrombocytes. This paper is presentation of surgical approach to a case with coexistence of these two conditions.

Recurrent Acute Myocardial Infarction in Patients with Immune Thrombocytopenic Purpura

Directory of Open Access Journals (Sweden)

Fengyi Shen

2014-01-01

Full Text Available Immune thrombocytopenic purpura (ITP, also known as idiopathic thrombocytopenic purpura, is an acquired immune-mediated disease of adults and children characterized by a transient or persistent decrease of platelets and, depending upon the degree of thrombocytopenia, an increased risk of bleeding. The use of standard treatments for acute myocardial infarction (AMI, such as antiplatelet agents and anticoagulants, pose serious problems in patients with ITP due to the potential higher risk of bleeding complications. There are no current guidelines available for management of ITP patients with AMI. In this brief review of the limited available literature, we discuss the proposed pathophysiological link between ITP and arterial thrombosis and the challenging medical and interventional treatment of these patients.

Thrombotic thrombocytopenic purpura or immune thrombocytopenia in a sickle cell/β+-thalassemia patient: a rare and challenging condition.

Science.gov (United States)

Vlachaki, Efthymia; Agapidou, Aleka; Neokleous, Nikolaos; Adamidou, Despoina; Vetsiou, Evaggelia; Boura, Panagiota

2014-10-01

The diagnosis of thrombotic thrombocytopenic purpura is one of the possible diagnosis when a patient is admitted with unexpected micro-angiopathic hemolytic anemia and thrombocytopenia. The combination of sickle cell/β(+)-thalassemia and thrombotic thrombocytopenic purpura is rare and triggering. This article describes the poor outcome of a patient with sickle cell/β(+)-thalassemia presenting with gingival bleeding, severe thrombocytopenia and anemia. The patient had normal renal function, no neurological deficit and he was initially treated as immune thrombocytopenic purpura. He eventually died due to multi-organ failure and brain hemorrhage even though he had started plasma exchange sessions. The co-existence of thrombotic thrombocytopenic purpura and sickle cell anemia is making the diagnosis of the former difficult. Early and rapid intervention is critical to the outcome. Copyright © 2014 Elsevier Ltd. All rights reserved.

Splenectomy for the treatment of thrombotic thrombocytopenic purpura

NARCIS (Netherlands)

Kappers-Klunne, MC; Wijermans, P; Fijnheer, R; Croockewit, AJ; van der Holt, B; de Wolf, JTM; Lowenberg, B; Brand, A

Plasma exchange is the treatment of choice for patients with thrombotic thrombocytopenic purpura (TTP) and results in remission in >80% of the cases. Treatment of patients who are refractory to plasma therapy or have relapsing disease is difficult. Splenectomy has been a therapeutic option in these

Coexisting Situs Inversus Totalis and Immune Thrombocytopenic Purpura

Directory of Open Access Journals (Sweden)

Kemal Gundogdu

2016-01-01

Full Text Available Situs inversus totalis is a rare congenital abnormality with mirror symmetry of mediastinal and abdominal organs. Immune thrombocytopenic purpura is an autoimmune disease with destruction of thrombocytes. This paper is presentation of surgical approach to a case with coexistence of these two conditions.

Thrombotic thrombocytopenic purpura

International Nuclear Information System (INIS)

Heyns, A. du P.; Badenhorst, P.N.; Lotter, M.G.; Minnaar, P.C.; Vorster, B.J.; Retief, F.P.

1979-01-01

A 34-year-old woman presented with the clinical and laboratory features of thrombotic thrombocytopenic purpura (TTP). Studies with isologous platelets labelled with 111 In-oxine revealed a short half-life or circulating platelets (18,5 hours) and destruction of the transfused platelets in the spleen, liver and bone marrow. There was no scintigraphic evidence of deposition of labelled platelets in the vasculature. The patient was treated with daily fresh frozen plasma transfusions, but no improvement in platelet count or serum urea level was noted. Although there was no clinical evidence of a bleeding tendency at the time, the patient had a fatal cerebrovascular haemorrhage. The findings in this case suggest that an immune type destruction of platelets may occur in TTP

Idiopathic thrombocytopenic purpura

Directory of Open Access Journals (Sweden)

L Kayal

2014-01-01

Full Text Available Idiopathic thrombocytopenic purpura (ITP is defined as a hematologic disorder, characterized by isolated thrombocytopenia without a clinically apparent cause. The major causes of accelerated platelet consumption include immune thrombocytopenia, decreased bone marrow production, and increased splenic sequestration. The clinical presentation may be acute with severe bleeding, or insidious with slow development with mild or no symptoms. The initial laboratory tests useful at the first visit to predict future diagnosis were erythrocyte count, leukocyte count, anti-glycoprotein IIb/IIIa antibodies, reticulated platelets, plasma thrombopoietin level. Treatment should be restricted to those patients with moderate or severe thrombocytopenia who are bleeding or at risk of bleeding. We present a case report on ITP with clinical presentation, diagnosis and management.

Penoscrotal edema and purpura in a 12-year-old boy: a case report and review of causes.

Science.gov (United States)

Dudley, Anne G; Fox, Janelle A; Reyes-Múgica, Miguel; Cannon, Glenn

2012-10-01

We report the case of a 12-year-old patient with previously diagnosed Crohn disease who presented with penile edema and purpura, with extension into the scrotum. Subsequent work-up including biopsy led to the diagnosis of extraintestinal Crohn disease, a rare manifestation in the genital region. Prompt treatment with steroids led to complete resolution of both penoscrotal edema and purpura. We describe our case, followed by a discussion of etiologies of penoscrotal edema and purpura as a review for the practicing pediatric urologist. Copyright © 2012 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.

A case of refractory thrombotic thrombocytopenic purpura treated ...

African Journals Online (AJOL)

Background. Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threaten- ing disorder that occurs due to deficiency of ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member. 13), which is a von Willebrand factor (VWF) cleaving protein.[1]. The absent or severely reduced activity ...

An unusual occurrence of Kleine-Levin syndrome in a man with refractory immune thrombocytopenic purpura: a case report.

Science.gov (United States)

Amirifard, Hamed; Barzkar, Farzaneh; Fazeli, Seyed Amirhossein; Hashemi, Seyed Mehdi

2015-04-01

Kleine-Levin syndrome is an extremely rare neurological entity characterized by recurrent episodes of hypersomnia which are sometimes associated with compulsive hyperphagia and behavioral changes. Autoimmunity has recently been proposed as a factor contributing to its pathogenesis. Immune thrombocytopenic purpura is a relatively common autoimmune disease showing a lot of complexity and uncertainty regarding its treatment regimens and its refractory nature in some cases. A 32-year-old Persian White man visited his private hematologist complaining of recent episodes of epistaxis and appearance of petechial lesions 24 hours after receiving a meningococcal vaccine. He had a history of immune thrombocytopenic purpura 13 years before his presentation. Based on his history and laboratory findings, his condition was diagnosed as a relapse of immune thrombocytopenic purpura and was managed accordingly. He did not respond to first-line corticosteroid regimens and later developed neurological symptoms as recurrent episodes of hypersomnia and hyperphagia. After a complete clinical and paraclinical evaluation and ruling out other possible conditions, he was given a diagnosis of Kleine-Levin syndrome. He was followed up for his immune thrombocytopenic purpura and received different treatment regimens none of which were adequately successful except intravenous immunoglobulin that was only temporarily effective. He has had 4 documented self-limited episodes of Kleine-Levin syndrome since his initial presentation. Immune thrombocytopenic purpura may be associated with meningococcal vaccination in adulthood. Responses to treatment in immune thrombocytopenic purpura vary among patients. Our patient only had a transient acceptable response to intravenous immunoglobulin while all other options failed to improve his platelet count. Concurrence of immune thrombocytopenic purpura and Kleine-Levin syndrome supports the role of autoimmunity as the proposed pathophysiological mechanism of

Henoch-Schönlein purpura with intracerebral haemorrhage in an adult patient: a case report

Directory of Open Access Journals (Sweden)

Karamadoukis Lazarus

2008-06-01

Full Text Available Abstract Introduction Henoch-Schönlein purpura is a small vessel vasculitis that affects mainly the skin, joints, gastrointestinal tract and kidneys. The central nervous system is also occasionally affected, although the majority of patients experience only mild symptoms such as headaches and behavioural changes. Intracerebral haemorrhage is a rare complication of Henoch-Schönlein purpura that so far has mainly been described in children and young adolescence. Case presentation We describe a 42-year-old man with Henoch-Schönlein purpura who developed an acute intracerebral haemorrhage that coincided with a reactivation of his vasculitis and the development of renal failure following discontinuation of steroids. In this patient, both the Henoch-Schönlein purpura and his neurological symptoms were successfully treated with intravenous cyclophosphamide and methylprednisolone, followed by a short course of oral cyclophosphamide and long-term oral prednisolone. His renal function also recovered sufficiently not to require renal replacement therapy. Conclusion The management of Henoch-Schönlein nephritis remains unclear, especially in the presence of severe complications such as intracerebral haemorrhage. We describe a successful outcome in such a patient.

Characterization of Pneumococcal Purpura-Producing Principle

OpenAIRE

Chetty, Chamroen; Kreger, Arnold

1980-01-01

Purpura was grossly observable in albino mice 6 to 8 h after the intraperitoneal injection of sterile, deoxyribonuclease-treated, cell-free extracts prepared by sodium deoxycholate-induced lysis, sonic disruption, Parr bomb treatment, autolysis without sodium deoxycholate, or alternate freezing and thawing of washed suspensions of Streptococcus pneumoniae type I. Cell-free extracts obtained from sonically disrupted, heat-killed cells (100°C for 20 min) did not contain purpurogenic activity. T...

Henoch-Schonlein purpura associated with primary active Epstein ...

African Journals Online (AJOL)

Henoch-Schönlein purpura (HSP) is the most common form of childhood vasculitis. Various viral and bacterial infections, drugs, vaccines, food allergy and even insect bites have been considered as triggering factors in pathogenesis of HSP. Epstein-Barr virus (EBV) infection, which is associated with HSP, have been rarely ...

A randomized, placebo-controlled, double-blind study to evaluate the efficacy of a citrus bioflavanoid blend in the treatment of senile purpura.

Science.gov (United States)

Berlin, Joshua M; Eisenberg, David P; Berlin, Mindy B; Sarro, Robert A; Leeman, Douglas R; Fein, Howard

2011-07-01

Senile purpura is a common, chronic skin condition affecting more than 10 percent of individuals over the age of 50. Despite being a benign condition, the continual development of purpura lesions in afflicted patients is frequently a source of significant visual and social concern. To date, there are no known effective treatments for this condition. To evaluate the efficacy of a novel nutraceutical citrus bioflavonoid blend in improving the skin's appearance in patients with senile purpura. A six-week, randomized, multicenter, placebo-controlled, double-blind study was conducted to determine whether a uniquely formulated, oral citrus bioflavonoid supplement could treat active lesions of senile purpura while preventing new lesions from arising. Seventy patients with senile purpura were enrolled and 67 completed the study. Subjects were randomized into two groups receiving either a citrus bioflavonoid blend or placebo medication, which was taken orally twice daily for six weeks. Clinical evaluations were performed by blinded investigators at two locations. A statistically significant reduction in the number of new purpura lesions in the skin area undergoing clinical study was documented. At the end of six weeks, the citrus bioflavonoid blend treated group showed a 50 percent reduction in purpura lesions from baseline. Patient self-assessment of the effectiveness of the medication echoed the results of an investigator global assessment with a statistically significant improvement in the skin's appearance noted by the patients receiving the active medication. No adverse effects were noted by either the patients or investigators. This new treatment appears to both safely and effectively diminish skin bruising in patients with senile purpura.

Limitations of ADAMTS-13 activity level in diagnosing thrombotic thrombocytopenic purpura in pregnancy.

Science.gov (United States)

Ehsanipoor, Robert M; Rajan, Priya; Holcombe, Randall F; Wing, Deborah A

2009-10-01

In pregnancy, it may be difficult to differentiate the syndrome of hemolysis, elevated liver enzymes, and low platelets from thrombotic thrombocytopenia purpura. Severely depressed (present a case of a patient that presented at 20 weeks gestation with elevated liver enzymes and thrombocytopenia. The diagnosis was unclear at the time of presentation. She underwent induction of labor, and during the postpartum course, she was eventually diagnosed with thrombotic thrombocytopenia purpura; however, her activity level of a disintegrin and metalloproteinase with thrombospondin motifs-13 was only moderately depressed at 15% (normal pregnancy value 41%-105%).

Clinical significance of the changes of serum IL-8 and IL-12 levels in pediatric patients with anaphylactoid purpura (AP)

International Nuclear Information System (INIS)

Liang Zhenming; Liu Xia

2005-01-01

Objective: To explore the role of IL-8 and IL-12 in the pathogenesis of anaphylactoid purpura (AP) and anaphylactoid purpura nephritis (APN). Methods: Serum IL-8 (with RIA) and IL-12 (with ELISA) levels were measured in 32 pediatric patients with anaphylactoid purpura (AP), 11 pediatric patients with anaphylactoid purpura nephritis (APN) and 15 controls. Results: During acute stage, serum IL-8 and IL-12 levels in both the AP and APN patients were significantly higher than those in controls and remained higher during convalescence. IL-8 and IL-12 levels were mutually positively correlated in acute stage. Conclusion: IL-8 and IL-12 participated in the pathogenesis of AP and APN. Theoretically, antagonist to those cytokines might be of clinical benefits. (authors)

Artefactual skin lesions in children and adolescents: review of the literature and two cases of factitious purpura.

Science.gov (United States)

Ring, Hans Christian; Miller, Iben M; Benfeldt, Eva; Jemec, Gregor B E

2015-01-01

Self harm is a great diagnostic and treatment challenge. In addition, psychocutaneous conditions are rare in the pediatric population and may therefore be misdiagnosed. Dermatitis artefacta is a psychocutaneous syndrome, which is a subgroup of the general spectrum of self-inflicted skin lesions. Dermatitis artefacta encompasses an array of different clinical manifestations, including purpura. Factitious purpura has rarely been reported in children. Case report and review of the literature. We describe two Caucasian patients (9-year-old boy and 10-year-old girl) with striking purpuric lesions diagnosed as factitious purpura. The clinical lesions were similar, but the underlying psychological problems differed significantly (depression and stress). The current state of knowledge of dermatitis artefacta in children and adolescents was reviewed. The presence of purpura in children and adolescents typically causes extensive intervention programs due to the possible serious pathological consequences. The two cases demonstrate a need for a high degree of attention to psychological disturbances, lesional evolution, and distribution once the suspicion is established. © 2014 The International Society of Dermatology.

Henoch-Schönlein purpura in an older man presenting as rectal bleeding and IgA mesangioproliferative glomerulonephritis: a case report

OpenAIRE

Howarth Charles B; Jirajariyavej Teeranun; Cheungpasitporn Wisit; Rosen Raquel M

2011-01-01

Abstract Introduction Henoch-Schönlein purpura is the most common systemic vasculitis in children. Typical presentations are palpable purpura, abdominal pain, arthritis, and hematuria. This vasculitic syndrome can present as an uncommon cause of rectal bleeding in older patients. We report a case of an older man with Henoch-Schönlein purpura. He presented with rectal bleeding and acute kidney injury secondary to IgA mesangioproliferative glomerulonephritis. Case presentation A 75-year-old Pol...

A dirty cause of vancomycin-mediated Henoch-Schonlein purpura: oxygen tubing is not a foley.

Science.gov (United States)

Shah, Nikhil H; Kline, Kristopher P; Shukla, Manas K

2017-06-20

A 59-year-old male presented with methicillin-resistant Staphylococcus aureus bacteraemia from a prostatic abscess and was treated with vancomycin. Two weeks into his treatment course, he developed severe joint pains, abdominal pain with bloody, mucinous stools and a diffuse palpable purpuric rash on his extremities. Biopsy of the rash showed IgA immune-complex deposition consistent with Henoch-Schönlein purpura. After treatment with glucocorticoids, his symptoms resolved completely. Vancomycin is an extremely commonly used antibiotic with certain well-known adverse effects. Henoch-Schönlein purpura, a vasculitis involving abdominal pain, arthralgias and palpable purpura, is a much less common side effect, as seen in this patient. Given that vancomycin is widely used internationally, clinicians should be aware of the risks entailed by its use. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

Idiopathic thrombocytopenic purpura during pregnancy

Directory of Open Access Journals (Sweden)

Tânia Regina Padovani

2012-04-01

Full Text Available ABSTRACT This essay is based on a medical case of idiopathic thrombocytopenic purpura (ITP during pregnancy. The cause of ITP is unknown, who suffer from this disorder, generate antibodies that destroy thrombocytes from their blood. ITP affects women of childbearing age and is associated to maternal and fetal complications. The management of a pregnant patient is difficult and requires the combined care of an obstetrician, a hematologist, and a neonatologist. The main therapeutic options for ITP in pregnant women include glucocorticoids and intravenous immunoglobulin. Splenectomy may be (performed in refractory cases. There is no concerning the management and treatment of pregnant women.

Trombotisk trombocytopenisk purpura hos barn med lavt ADAMTS13 enzymniveau

DEFF Research Database (Denmark)

Spangenberg, Katrine Bredsdorff; Clasen-Linde, Erik; Poulsen, Anja

2014-01-01

Thrombotic thrombocytopenic purpura (TTP) is a rare condition, but important to consider in case of thrombocytopenia and haemolysis. It is imperative to proceed with the correct treatment, in order to ensure a satisfactory outcome. TTP is either acquired or idiopathic. This case report shows...

A case of palpable purpura and nephropathy: Occam's Razor or Hickam's Dictum.

Science.gov (United States)

Mandhadi, Ranadeep; Kodumuri, Vamsi; Arora, Rohit; Puneet Singh, Param; Adigopula, Shashi; Chua, Serafin

2013-01-01

Vasculitis causing palpable purpura, nephropathy, and hematologic abnormalities is a well-known entity. However, sometimes, vasculitis may not be the primary cause but is part of a systemic disease. Literature suggests that infections like HIV can induce nephropathy and antineutrophilic cytoplasmic antibody-positive vasculitis, which is different from the well-known entity of "antineutrophilic cytoplasmic antibody-associated vasculitis." We present a 46-year-old female patient with a history of intravenous drug abuse who reported with a rash, swelling, and palpable purpura of the lower extremities. Peripheral smear showed no evidence of disseminated intravascular coagulation or thrombotic thrombocytopenic purpura; metabolic profile showed acute kidney injury. She was found to be HIV- and hepatitis C-positive. Immunologic workup was positive for both MPO and PR3 antineutrophilic cytoplasmic antibodies and negative for cryoglobulins; complement levels were low. Skin biopsy showed leukocytoclastic vasculitis but kidney biopsy was negative for any immunologic involvement; it showed only glomerulosclerosis. Thus, it was thought that nephropathy and vasculitis, in this case, are two distinct pathologic processes, both induced by infection (HIV and/or hepatitis C). The patient responded to low-dose steroid therapy. She was later started on the definitive therapy, the highly active antiretroviral therapy regimen. This case illustrates the fact that low-dose steroids can still be a good alternative in acute situations in patients at risk from immunosuppression.

Thrombotic Thrombocytopenic Purpura Associated with Pneumococcal Sepsis

Directory of Open Access Journals (Sweden)

Jeffrey R Schriber

1993-01-01

Full Text Available The first documented case of thrombotic thrombocytopenic purpura (TTP associated with pneumococcal septicemia is reported. This association has been previously demonstrated with hemolytic uremic syndrome. The patient presented with recurrent seizures, oliguric renal failure, fever, thrombocytopenia and microangiopathic hemolytic anemia; coagulation studies were normal. Blood and sputum cultures were positive for Streptococcus pneumoniae. The patient responded to therapy with plasmapheresis and antiplatelet agents as well as antibiotics. Coincident infection should be searched for in all cases of TTP.

A case of lupus-like glomerulonephritis in an HIV patient with nephrotic range proteinuria, purpura, and elevated IgA level.

Science.gov (United States)

Yang, Jihyun; Seo, Min Young; Kim, Ki Tae; Lee, Jun Yong; Kim, Sun-Chul; Kim, Myung-Gyu; Jo, Sang-Kyung; Cho, Won-Yong; Kim, Hyoung-Kyu; Won, Nam Hee; Cha, Ran-Hui; Cho, Eunjung

2014-01-01

Human immunodeficiency virus (HIV) infection is growing medical concern worldwide. There are many types of glomerulonephritis which are associated with HIV infection. We report a case of a 53-year-old Korean man with an HIV infection, who was developed nephritic range proteinuria and purpura with elevated IgA level rasing a possibility of Henoch-Schölein Purpura (H-S purpura). However, renal biopsy showed "lupus-like feature" glomerulonephritis without clinical or serologic evidence of systemic lupus erythematosus. Although baseline renal function was maintained without further need for maintenance dialysis following anti-retroviral therapy (ART) and steroid, patient died from uncontrolled gastrointestinal bleeding.

Forskelle i behandling af børn med idiopatisk trombocytopenisk purpura

DEFF Research Database (Denmark)

Edslev, Pernille Wendtland; Kjærsgaard, Mimi; Rosthøj, Steen

2010-01-01

INTRODUCTION: Idiopathic thrombocytopenic purpura (ITP) is a rare immune-mediated bleeding disorder that usually takes a self-limiting and benign course. Due to the risk of intracranial haemorrhage, treatment regimens tend to be active. We present treatment data from 17 paediatric departments...

A Case Associated with Comorbidities Among Cerebral Infarction, Idiopathic Thrombocytopenic Purpura, and Triple X Syndrome

Directory of Open Access Journals (Sweden)

Hanjun Kim

2014-06-01

Full Text Available A 46-year-old female presented to the emergency room due to the chief complaint of left-sided weakness. By imaging study, she was diagnosed with cerebral infarction. Thrombolytic and antiplatelet agents were not considered due to the “golden hour” for treatment having passed and a low platelet count. The peripheral blood smear, bone marrow biopsy, and aspirate findings were consistent with immune thrombocytopenic purpura. The chromosome analysis revealed the 47,XXX karyotype. To the best of our knowledge, this is the first case report associated with the comorbidities of cerebral infarction, idiopathic thrombocytopenic purpura, and triple X syndrome.

Goodpasture's Syndrome due to IgA in a patient with clinical diagnosis of Henoch Schonlein's purpura

International Nuclear Information System (INIS)

Restrepo Cesar A

2005-01-01

This is a case of a 23 year old woman with an initial clinical syndrome compatible with glomerulonephritis of uncertain origin, who later showed lesions of purpuric rash characteristics of Henoch- Schonlein Purpura and then complicated with a pulmonary hemorrhage and a rapidly progressive glomerulonephritis, with a mixed lung-kidney syndrome. The renal biopsy showed presence of linear deposits of immunoglobulin A and extra capillary proliferative changes. The case was concluded corresponding to Goodpasture's syndrome for antibodies antiglomerular basement membrane of the type of IgA in the context of a Henoch-Schonlein Purpura.

Expression of CD markers' in immune thrombocytopenic purpura: prognostic approaches.

Science.gov (United States)

Behzad, Masumeh Maleki; Asnafi, Ali Amin; Jaseb, Kaveh; Jalali Far, Mohammad Ali; Saki, Najmaldin

2017-12-01

Immune Thrombocytopenic Purpura (ITP) is a common autoimmune bleeding disorder characterized by a reduction in peripheral blood platelet counts. In this disease, autoantibodies (Auto-Abs) are produced against platelet GPIIb/GPIIIa by B cells, which require interaction with T cells. In this review, the importance of B and T lymphocytes in ITP prognosis has been studied. Relevant literature was identified by a PubMed search (1990-2016) of English-language papers using the terms B and T lymphocyte, platelet, CD markers and immune thrombocytopenic purpura. T and B lymphocytes are the main immune cells in the body. Defective function causes disrupted balance of different subgroups of lymphocytes, and abnormal expression of surface markers of these cells results in self-tolerance dysfunction, as well as induction of Auto-Abs against platelet glycoproteins (PG). Given the role of B and T cells in production of autoantibodies against PG, it can be stated that the detection of changes in CD markers' expression in these cells can be a good approach for assessing prognosis in ITP patients. © 2017 APMIS. Published by John Wiley & Sons Ltd.

Henoch-Schönlein purpura nephritis occurring postpartum in a patient with anti-PL-7 anti-synthetase syndrome.

Science.gov (United States)

Nagai, Kojiro; Kishi, Jun; Morizumi, Shun; Minakuchi, Jun; Bando, Yoshimi; Nishioka, Yasuhiko; Doi, Toshio

2017-09-01

A 37-year-old pregnant woman developed purpura which was subsequently diagnosed as Henoch-Schönlein purpura (HSP). After childbirth, the patient developed proteinuria and hematuria. Further examination revealed that the HSP nephritis (HSPN) was associated with anti-threonyl-tRNA synthetase anti-synthetase syndrome. The onset of HSPN during pregnancy or after childbirth is rare. Moreover, to our knowledge, this is the first case to describe renal involvement in anti-synthetase syndrome.

Glatiramer Acetate-associated Refractory Immune Thrombocytopenic Purpura

Directory of Open Access Journals (Sweden)

Iftach Sagy

2016-04-01

Full Text Available We present a case of glatiramer acetate-associated refractory immune thrombocytopenic purpura (ITP in a female patient with multiple sclerosis. A search of MEDLINE/PubMed did not find any connection between glatiramer acetate and thrombocytopenia, specifically ITP. The autoimmune reaction was resistant to conservative ITP treatment, and was eventually managed only by splenectomy. To the best of our knowledge, this is the first report of glatiramer acetate-associated ITP. Physicians should be aware of this condition, and consider performing routine blood counts at the beginning of glatiramer acetate treatment.

Acquired thrombotic thrombocytopenic purpura: new therapeutic options and their optimal use.

Science.gov (United States)

Cataland, S R; Wu, H M

2015-06-01

Advances in our understanding of the pathophysiology of both congenital and acquired thrombotic thrombocytopenic purpura (TTP) have led to both an increased understanding of the disease and novel approaches to therapy. The efficacy of rituximab in acquired TTP has led to consideration of rituximab as a prophylactic therapy to prevent relapse of TTP. Novel therapies that target the A1 domain of von Willebrand factor (VWF) to block the formation of microthrombotic disease have also entered clinical study and have demonstrated promise as potential therapeutic options. Additionally, a recombinant ADAMTS13 protease has been developed which may be an important therapeutic option for both congenital and acquired TTP. The development of these new therapeutic options for patients diagnosed with TTP has increased the importance of conducting prospective, randomized studies with these agents to both confirm their efficacy and more importantly understand their most appropriate role in the treatment of patients with TTP. © 2015 International Society on Thrombosis and Haemostasis.

Oral purpura as the first manifestation of primary systemic amyloidosis.

Science.gov (United States)

McCormick, Robert Stuart; Sloan, Philip; Farr, David; Carrozzo, Marco

2016-07-01

Oral blood blisters and purpura are rare features of primary systemic amyloidosis (amyloid light-chain (AL) amyloidosis). We report a case in which these unusual presentations led to a diagnosis of amyloidosis, which enabled effective treatment before organ failure. Copyright © 2015 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.

Obesity increases the risk of renal involvement in children with Henoch-Schönlein purpura.

Science.gov (United States)

Zhao, Yong-Li; Liu, Zheng-Juan; Bai, Xue-Mei; Wang, Yu-Chuan; Li, Guo-Hua; Yan, Xue-Yan

2015-10-01

The main aim of this study was to evaluate the relationship between obesity and renal involvement in children with Henoch-Schönlein purpura (HSP). A retrospective study of 141 pediatric patients with HSP was conducted in our hospital. The clinical data of all patients were collected from the electronic medical record management system from January 2010 to June 2014. The possible risk factors of renal involvement, especially obesity, were analyzed using univariate and multivariate analyses. Renal involvement occurred in 45/141 of the patients. A univariate analysis showed that an age more than 7 years at onset, persistent purpura, obesity, time from symptoms onset to diagnosis more than 14 days, and decreased C3 all increased the risk of renal involvement in HSP. The forward stepwise logistic regression analysis indicated obesity (odds ratio (OR) 4.43, 95 % confidence interval (CI) 1.896 to 10.358), age more than 7 years at onset (OR 2.81, 95 % CI 1.142 to 6.907), and persistent purpura (OR 2.57, 95 % CI 1.119 to 5.909) were independent risk factors for renal involvement. Our results show that obesity can increase the hazard of renal involvement in children with HSP and reconfirm that older age at onset and persistent purpura are the independent risk factors for renal involvement. • There have been some reports that obesity was associated with the development of renal injury. • It is not clear whether obesity can increase the risk of renal involvement in children with HSP. What is New: • The main finding of this study is that obesity can increase the hazard of renal involvement in children with HSP.

Purpura fulminans and anticardiolipin antibodies in a patient with Grave's disease.

Science.gov (United States)

Ligier, Sophie; Pham, Cuong D; Watters, A Kevin; Kassis, Jeannine; Fortin, Paul R

2002-01-01

We describe a patient with Grave's discase who developed purpura fulminans and who was found to have anticardiolipin antibodies after being started on propylthiouracil (PTU). We discuss the potential role of the antiphospholipid antibody in this woman's presentation, and its association to both PTU and autoimmune thyroid disease.

Pregabalin- and azithromycin-induced rhabdomyolysis with purpura: An unrecognized interaction: A case report

Directory of Open Access Journals (Sweden)

Kazuya Kato, MD

2016-01-01

Conclusion: We report an extremely rare case of rhabdomyolysis with purpura caused by a drug interaction between pregabalin and azithromycin. However, the mechanisms of the interactions between azithromycin on the pregabalin are still unknown.

Characterization of pneumococcal purpura-producing principle.

Science.gov (United States)

Chetty, C; Kreger, A

1980-07-01

Purpura was grossly observable in albino mice 6 to 8 h after the intraperitoneal injection of sterile, deoxyribonuclease-treated, cell-free extracts prepared by sodium deoxycholate-induced lysis, sonic disruption, Parr bomb treatment, autolysis without sodium deoxycholate, or alternate freezing and thawing of washed suspensions of Streptococcus pneumoniae type I. Cell-free extracts obtained from sonically disrupted, heat-killed cells (100 degrees C for 20 min) did not contain purpurogenic activity. The reaction was maximal at approximately 24 h postinjection, started to fade slowly after 24 to 48 h, and usually was not grossly observable by 4 to 6 days postinjection. The purpura-producing principle (PPP) in the cell-free extract was purified by sequential ammonium sulfate precipitation, protamine sulfate precipitation, Sepharose 6B gel filtration, wheat germ lectin-Sepharose 6MB affinity chromatography, ribonuclease and trypsin treatment, and a second Sepharose 6B gel filtration step. The final preparation (i) contained glucosamine (5.6%), muramic acid (8.0%), neutral carbohydrate (12.8%), phosphate (8.0%), orcinol-reactive material (6.0%), and Lowry-reactive material (1.6%), and (ii) was free of detectable amounts of deoxyribonucleic acid, capsular polysaccharide, neuraminidase, cytolysin, and hyaluronidase. The isoelectric point and molecular size of the PPP were approximately pI 3.0 and several million daltons, respectively, and the activity remained in the supernatant fluid after centrifugation for 1 day at 105,000 x g. PPP activity was destroyed by incubation with egg white lysozyme and sodium metaperiodate but was resistant to trypsin, pronase, alpha-amylase, deoxyribonuclease, ribonuclease, alkaline phosphatase, pancreatic lipase, 7% trichloroacetic acid, 6 M urea, autoclaving (121 degrees C) for 30 min, and mild acid and alkali exposure. Our observations indicate that the PPP requires intact beta-1,4-glucosidic linkages for activity and support the working

Association of endothelial nitric oxide synthase gene polymorphism with the risk of Henoch-Schönlein purpura/Henoch-Schönlein purpura nephritis.

Science.gov (United States)

Zhong, Weiqiang; Zhou, Tian-Biao; Jiang, Zongpei

2015-04-01

Association between endothelial nitric oxide synthase (eNOS) gene polymorphism and Henoch-Schönlein purpura (HSP)/Henoch-Schönlein purpura nephritis (HSPN) risk is still controversial. A meta-analysis was performed to evaluate the association between eNOS gene polymorphism and HSP/HSPN susceptibility. A predefined literature search and selection of eligible relevant studies were performed to collect data from electronic database. Three articles were identified for the analysis of association between eNOS gene polymorphism and HSPN/HSP risk. eNOS G894T gene polymorphism was not associated with HSPN susceptibility and the risk of patients with HSP developing into HSPN. Interestingly, eNOS G894T T allele and GG genotype were associated with HSP susceptibility, but not the TT genotype. eNOS T786C TT genotype was associated with HSPN susceptibility, but not C allele and CC genotype. Furthermore, eNOS T786C gene polymorphism was not associated with HSP risk and the risk of patients with HSP developing into HSPN. In conclusion, eNOS T786C TT genotype was associated with and eNOS G894T T allele and GG genotype were associated with HSP susceptibility. However, more studies should be performed in the future.

Anaphylactoid Purpura Manifested after Acute Gastroenteritis with Severe Dehydration in an 8-Year-Old Male Child: A Case Report.

Science.gov (United States)

Thakkar, Umang G; Vanikar, Aruna V; Trivedi, Hargovind L

2015-12-01

Anaphylactoid purpura, also known as Henoch-Schönleinpurpura (HSP), is an IgA-mediated vasculitis that tends to be a benign disease of childhood. Up to 50% of cases are preceded by an upper tract respiratory infection caused by group-A beta-hemolytic streptococcus and present with the common tetrad of abdominal pain, arthritis, purpuric rash, and renal involvement. The majority of patients recover completely. Here we document a rare case of anaphylactoid purpura which manifested with skin lesions in the form of palpable purpura following about of acute gastroenteritis with severe dehydration; it was treated with a short regimen of steroid therapy, which resulted in the complete remission of the disease. We conclude that prompt diagnosis and multidisciplinary intervention will lead to appropriate management-consisting of the installation of early short-course steroid therapy and thus, prevent further complications and the recurrence of the disease.

Immune thrombocytopenic purpura: A rare side effect in a patient treated with Imiquimod for lentigo maligna

Directory of Open Access Journals (Sweden)

E.A. Whatling

2017-09-01

Full Text Available Topical Imiquimod is increasingly used in the treatment of lentigo maligna (LM for patients not suitable or willing for surgery. It acts as an immune response modifier and is recommended for use with caution in patients with autoimmune disorders. A review of the literature did not reveal any reported cases of Idiopathic Thrombocytopenic Purpura (ITP with the use of Imiquimod so far. We report a case of a 55-year-old woman with a history of Addison's and Graves' disease who had been diagnosed with lentigo maligna on the right nasal ala. Within two days of commencing of topical Imiquimod treatment, she was admitted to the hospital with extensive cutaneous and mucosal bruising and treated as acute Idiopathic Throbocytopenic Purpura. Deranged platelets were reversed promptly with treatment and Imiquimod discontinued. We demonstrate that Idiopathic Throbocytopenic Purpura is a potential life-threatening side effect of Imiquimod treatment and patients who have autoimmune disorders are at increased risk of developing this side effect. We recommend that Imiquimod is contraindicated in patients with autoimmune disease. Here we describe the clinical presentation and review the relevant literature on Imiquimod treatment for lentigo maligna and its potential side effects.

Platelet survival in idiopathic thrombocytopenic purpura and response to splenectomy

International Nuclear Information System (INIS)

Monteiro, M.E.; Verhaeghe, R.; Devos, P.

Platelet survival combined with surface counting was performed in 9 patients with idiopathic thrombocytopenic purpura, resistent to steroid therapy. All patients had a markedly enhanced platelet turnover, five of them showed an augmented trapping of radioactivity over the spleen compared to liver and heart. These five patients underwent splenectomy: the platelet count increased in all of them but this increase was not always sustained. (Author) [pt

Henoch-Schönlein purpura in an older man presenting as rectal bleeding and IgA mesangioproliferative glomerulonephritis: a case report.

Science.gov (United States)

Cheungpasitporn, Wisit; Jirajariyavej, Teeranun; Howarth, Charles B; Rosen, Raquel M

2011-08-10

Henoch-Schönlein purpura is the most common systemic vasculitis in children. Typical presentations are palpable purpura, abdominal pain, arthritis, and hematuria. This vasculitic syndrome can present as an uncommon cause of rectal bleeding in older patients. We report a case of an older man with Henoch-Schönlein purpura. He presented with rectal bleeding and acute kidney injury secondary to IgA mesangioproliferative glomerulonephritis. A 75-year-old Polish man with a history of diverticulosis presented with a five-day history of rectal bleeding. He had first noticed colicky left lower abdominal pain two months previously. At that time he was treated with a 10-day course of ciprofloxacin and metronidazole for possible diverticulitis. He subsequently presented with rectal bleeding to our emergency department. Physical examination revealed generalized palpable purpuric rash and tenderness on his left lower abdomen. Laboratory testing showed a mildly elevated serum creatinine of 1.3. Computed tomography of his abdomen revealed a diffusely edematous and thickened sigmoid colon. Flexible sigmoidoscopy showed severe petechiae throughout the colon. Colonic biopsy showed small vessel acute inflammation. Skin biopsy resulted in a diagnosis of leukocytoclastic vasculitis. Due to worsening kidney function, microscopic hematuria and new onset proteinuria, he underwent a kidney biopsy which demonstrated IgA mesangioproliferative glomerulonephritis. A diagnosis of Henoch-Schönlein purpura was made. Intravenous methylprednisolone was initially started and transitioned to prednisone tapering orally to complete six months of therapy. There was marked improvement of abdominal pain. Skin lesions gradually faded and gastrointestinal bleeding stopped. Acute kidney injury also improved. Henoch-Schönlein purpura, an uncommon vasculitic syndrome in older patients, can present with lower gastrointestinal bleeding, extensive skin lesions and renal involvement which responds well to

Epidemiology and pathophysiology of adulthood-onset thrombotic microangiopathy with severe ADAMTS13 deficiency (thrombotic thrombocytopenic purpura): a cross-sectional analysis of the French national registry for thrombotic microangiopathy.

Science.gov (United States)

Mariotte, Eric; Azoulay, Elie; Galicier, Lionel; Rondeau, Eric; Zouiti, Fouzia; Boisseau, Pierre; Poullin, Pascale; de Maistre, Emmanuel; Provôt, François; Delmas, Yahsou; Perez, Pierre; Benhamou, Ygal; Stepanian, Alain; Coppo, Paul; Veyradier, Agnès

2016-05-01

Thrombotic thrombocytopenic purpura is a thrombotic microangiopathy related to a severe deficiency of ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13; activity purpura at initial presentation. Between Jan 1, 1999, and Dec 31, 2013, we did a cross-sectional analysis of the French national registry for thrombotic microangiopathy to identify all patients with adult-onset thrombotic microangiopathy (first episode after age 18 years) who had severe ADAMTS13 deficiency at presentation. ADAMTS13 activity, anti-ADAMTS13 IgG, and ADAMTS13 gene mutations were investigated by a central laboratory. We collected patients' clinical data for correlation with their ADAMTS13 phenotype and genotype. We used logistic regression analysis to identify variables significantly associated with idiopathic thrombotic thrombocytopenic purpura, as measured by estimated odds ratios (ORs) and 95% CIs. This study is registered with ClinicalTrials.gov, number NCT00426686. We enrolled 939 patients with adult-onset thrombotic thrombocytopenic purpura, of whom 772 (82%) patients had available data and samples at presentation and comprised the cohort of interest. The prevalence of thrombotic thrombocytopenic purpura in France was 13 cases per million people. At presentation, 378 (49%) patients had idiopathic thrombotic thrombocytopenic purpura, whereas 394 (51%) patients had disease associated with miscellaneous clinical situations (infections, autoimmunity, pregnancy, cancer, organ transplantation, and drugs). Pathophysiologically, three distinct forms of thrombotic thrombocytopenic purpura were observed: 585 (75%) patients had autoimmune disease with anti-ADAMTS13 IgG, 166 (22%) patients had acquired disease of unknown cause and 21 (3%) patients had inherited disease (Upshaw-Schulman syndrome) with mutations of the ADAMTS13 gene. Idiopathic thrombotic thrombocytopenic purpura were mainly autoimmune (345 [91%] cases), whereas non-idiopathic diseases were

Thrombotic thrombocytopenic purpura and myoglobinuric acute renal failure following radiation therapy in a patient with polymyositis and cervical cancer

International Nuclear Information System (INIS)

Makino, Hirofumi; Nagake, Yoshio; Moriwaki, Kazuhiko; Hirakawa, Shuzo; Katayama, Takaaki; Yanai, Hiroyuki; Takahashi, Kiyoshi; Akagi, Tadaatsu; Ota, Zensuke

1995-01-01

A 73-year-old woman was admitted to receive radiation treatment for uterine cervical cancer, however a complex series of events ensued, leading to death. She developed an acute exacerbation of polymyositis complicated by thrombocytopenic purpura, rhabdomyolysis and acute renal failure. Radiation therapy may have produced an immune disturbance leading to the acute exacerbation of polymyositis. Auto-immune-mediated endothelial damage might have triggered a series of events leading to thrombotic thrombocytopenic purpura. Rhabdomyolysis seemed to be the main cause of acute renal failure. (author)

Thrombotic thrombocytopenic purpura and myoglobinuric acute renal failure following radiation therapy in a patient with polymyositis and cervical cancer

Energy Technology Data Exchange (ETDEWEB)

Makino, Hirofumi; Nagake, Yoshio; Moriwaki, Kazuhiko; Hirakawa, Shuzo; Katayama, Takaaki; Yanai, Hiroyuki; Takahashi, Kiyoshi; Akagi, Tadaatsu; Ota, Zensuke [Okayama Univ. (Japan). School of Medicine

1995-01-01

A 73-year-old woman was admitted to receive radiation treatment for uterine cervical cancer, however a complex series of events ensued, leading to death. She developed an acute exacerbation of polymyositis complicated by thrombocytopenic purpura, rhabdomyolysis and acute renal failure. Radiation therapy may have produced an immune disturbance leading to the acute exacerbation of polymyositis. Auto-immune-mediated endothelial damage might have triggered a series of events leading to thrombotic thrombocytopenic purpura. Rhabdomyolysis seemed to be the main cause of acute renal failure. (author).

Guillain-Barre syndrome in an adult patient with Henoch-Schonlein purpura.

Science.gov (United States)

Aggarwal, Puneet; Sahoo, Ratnakar; Gadpayle, A K; Kumar, Gunjan

2013-04-03

A 42-year-old man presented with pain in the abdomen, massive haematemesis and rashes over the body and development of bilateral lower limb weakness the next day. The patient was later diagnosed with Guillain-Barre syndrome with Henoch-Schonlein purpura. He was treated with intravenous immunoglobulins. At 3 months of follow-up and rehabilitation, patient showed complete recovery from weakness.

Guillain-Barre syndrome in an adult patient with Henoch-Schonlein purpura

OpenAIRE

Aggarwal, Puneet; Sahoo, Ratnakar; Gadpayle, A K; Kumar, Gunjan

2013-01-01

A 42-year-old man presented with pain in the abdomen, massive haematemesis and rashes over the body and development of bilateral lower limb weakness the next day. The patient was later diagnosed with Guillain-Barre syndrome with Henoch-Schonlein purpura. He was treated with intravenous immunoglobulins. At 3 months of follow-up and rehabilitation, patient showed complete recovery from weakness.

Recurrent purpura due to alcohol-related Schamberg's disease and its association with serum immunoglobulins: a longitudinal observation of a heavy drinker.

Science.gov (United States)

Bonnet, Udo; Selle, Claudia; Isbruch, Katrin; Isbruch, Katrin

2016-10-31

It is unusual for purpura to emerge as a result of drinking alcohol. Such a peculiarity was observed in a 55-year-old man with a 30-year history of heavy alcohol use. The Caucasian patient was studied for 11 years during several detoxification treatments. During the last 2 years of that period, purpuric rashes were newly observed. The asymptomatic purpura was limited to both lower limbs, self-limiting with abstinence, and reoccurring swiftly with alcohol relapse. This sequence was observed six times, suggesting a causative role of alcohol or its metabolites. A skin biopsy revealed histological features of purpura pigmentosa progressiva (termed Schamberg's disease). Additionally, alcoholic fatty liver disease markedly elevated serum immunoglobulins (immunoglobulin A and immunoglobulin E), activated T-lymphocytes, and increased C-reactive protein. In addition, moderate combined (cellular and humoral) immunodeficiency was found. Unlike the patient's immunoglobulin A level, his serum immunoglobulin E level fell in the first days of abstinence, which corresponded to the time of purpura decline. Systemic vasculitis and clotting disorders were excluded. The benign character of the purpura was supported by missing circulating immune complexes or complement activation. An alcohol provocation test with vinegar was followed by the development of fresh "cayenne pepper" spots characteristic of Schamberg's disease. This case report demonstrates that Schamberg's disease can be strongly related to alcohol intake, in our patient most likely as a late complication of severe alcoholism with alcoholic liver disease. Immunologic disturbances thereby acquired could have constituted a basis for a hypersensitivity-like reaction after ingestion of alcohol. Schamberg's disease induction by vinegar may point to an involvement of acetate, a metabolite of ethanol.

[Henoch-Schönlein purpura in a cocaine consumer man with HIV infection and ANCA-p positivity].

Science.gov (United States)

De Paoli, María C; Moretti, Dino; Scolari Pasinato, Carlos M; Buncuga, Martín G

The Henoch-Schönlein purpura (HSP) is a small vessel vasculitis with IgA immune complex deposition. The presentation in adults is rare and severe. Reported cases of HSP in patients infected with HIV are scarce. Neutrophil cytoplasmic antibodies (ANCA) are commonly found in other systemic vasculitis, but rarely in HSP and even more unusual the perinuclear pattern. Beside small vessel vasculitis, positivity of ANCA can be detected in a number of different pathological conditions in association with infectious processes, including HIV, or cocaine use, and especially the pattern of ANCA-p, associated with drugs, inflammatory bowel or autoimmune diseases. We report the case of a 35 years old man with toxic habits (cocaine, marijuana) who consulted for abdominal pain, hematochezia and purpura on lower extremities, and later fever, joint pain and progression of purpura associated with nephritic syndrome and ANCA-p (+). During hospitalization HIV infection was detected. Renal biopsy showed IgA nephropathy with favorable response to corticosteroid and antiproteinuric treatment. The communication of the case is due to the rarity of the presentation and therapeutic diagnostic challenge. It remains to elucidate the role of ANCA in the pathogenesis and management of adult PSH.

Korean Herbal Medicine for Treating Henoch-Schonlein Purpura with Yin Deficiency: Five Case Reports

Directory of Open Access Journals (Sweden)

Sung-Kyung Kim

2014-12-01

Full Text Available Objectives: The purpose of this study is to report the clinical effect of Korean medicine (KM treatment for Henoch-Schonlein purpura (HSP. Methods: Five HSP patients who demonstrated a Yin deficiency and who had a history of a previous upper respiratory tract infection were included in this study. Four patients had arthritis and three had severe stomachache. One of them appeared to have proteinuria and hematuria before starting KM treatment. Results: All patients were improved with only herbal medicine, Jarotang (JRT. Purpura in the lower extremities and abdominal pain, which were not treated by using a corticosteroid, disappeared and had not recurred after 6 months. Conclusion: These cases indicate that JRT may be effective in treating HSP in patients who demonstrate Yin deficiency, even though the number of cases was limited to five.

Thrombotic thrombocytopenic purpura presenting with pathologic fracture: a case report.

Science.gov (United States)

Berber, Ilhami; Erkurt, Mehmet Ali; Kuku, Irfan; Kaya, Emin; Unlu, Serkan; Ertem, Kadir; Nizam, Ilknur

2014-08-01

Thrombotic thrombocytopenic purpura is an acute syndrome with abnormalities in multiple organ systems, which becomes manifest with microangiopathic hemolytic anemia and thrombocytopenia. The hereditary or acquired deficiency of ADAMTS-13 activity leads to an excess of high molecular weight von Willebrand factor multimers in plasma, leading to platelet aggregation and diffuse intravascular thrombus formation, resulting in thrombotic thrombocytopenic purpura. Thrombotic lesions occurring in TTP leads to ischemia and convulsion. Depending on the properties of the bony tissue, fractures are divided into three groups as traumatic, pathological, and stress fractures. A pathologic fracture is a broken bone caused by disease leading to weakness of the bone. This process is most commonly due to osteoporosis, but may also be due to other pathologies such as cancer, infections, inherited bone disorders, or a bone cyst. We herein report a case with a pathologic fracture due to convulsion secondary to thrombotic thrombocytopenic pupura. Thrombotic lesions occurring in TTP may lead to ischemia and convulsion, as in our patient and pathological fractures presented in our case report may occur as a result of severe muscle contractions associated with convulsive activity. Thrombotic thrombocytopenic pupura is a disease that involves many organ systems and thus may have a very wide spectrum of clinical presentations. Copyright © 2014. Published by Elsevier Ltd.

Henoch-Schönlein purpura without systemic involvement beginning with acute scrotum and mimicking torsion of testis

Directory of Open Access Journals (Sweden)

Levent Verim

2013-04-01

Full Text Available Henoch-Schönlein purpura (HSP is the most common systemic vasculitis in children. Typical presentations of HSP are palpable purpura of the small vessels in the hips and lower limbs, abdominal pain, arthritis, and hematuria. Scrotal involvement manifested by the presence of scrotal pain and swelling during the course of the disease is rarely seen. HSP without systemic involvement with acute scrotum mimicking testicular torsion is even rare in the medical literature. In most cases, patients with this disease achieve complete recovery. Herein, we report an interesting HSP case with skin symptoms but without systemic involvement and then progression to acute scrotum resembling torsion of testis.

Homeostatic responses to amino acid insufficiency

Directory of Open Access Journals (Sweden)

Tracy G. Anthony

2015-09-01

Full Text Available This article provides a brief overview describing how two key signaling pathways, namely the integrated stress response and the mammalian target of rapamycin complex 1, work together to facilitate cellular adaptation to dietary amino acid insufficiency. A deeper understanding of these mechanisms is leading to identification of novel targets which aid in disease treatments, improve stress recovery and increase health span through slowed aging and enhanced metabolic fitness.

Systemic Lupus Erythematosus Presenting as Thrombotic Thrombocytopenia Purpura: How Close Is Close Enough?

Directory of Open Access Journals (Sweden)

Cesar A. Perez

2011-01-01

Full Text Available Thrombotic thrombocytopenic purpura (TTP is an uncommon life-threatening disease characterized by microangiopathic hemolytic anemia and thrombocytopenia, commonly associated with infections, malignancy, drugs, and autoimmune diseases. We report a case of 19-year-old previously healthy female that presents with anemia and thrombocytopenia diagnosed with thrombotic thrombocytopenic purpura that was treated successfully with plasmapheresis and corticosteroids. Laboratory findings also revealed antinuclear antibodies and antibodies to double-stranded DNA. Two weeks after presentation developed inflammatory arthritis, fulfilling diagnostic criteria for systemic lupus erythematosus (SLE. Prompt diagnosis and treatment with plasma exchange and corticosteroids should be instituted as soon as the diagnosis of TTP is suspected, even if other diagnoses, including lupus, are possible. When present, the coexistence of these two etiologies can have a higher mortality than either disease alone. An underlying diagnosis of SLE should be considered in all patients presenting TTP and the study of this association may provide a better understanding of their immune-mediated pathophysiology.

Novel platelet-agglutinating protein from a thrombotic thrombocytopenic purpura plasma.

OpenAIRE

Siddiqui, F A; Lian, E C

1985-01-01

A novel platelet-agglutinating protein (PAP) was purified approximately 2,000-fold from the plasma of a patient with thrombotic thrombocytopenic purpura (TTP) by ammonium sulfate fractionation, DEAE-Sephacel and concanavalin A-Sepharose chromatographies. On sodium dodecyl sulfate-polyacrylamide gel electrophoresis, with and without reduction, this preparation revealed a major protein band with a molecular weight of 37,000, and a minor band with a molecular weight of 32,000-34,000. After eluti...

Pituitary apoplexy with optic tract oedema and haemorrhage in a patient with idiopathic thrombocytopenic purpura

International Nuclear Information System (INIS)

Lenthall, R.; Jaspan, T.

2001-01-01

Bilateral optic tract oedema, left optic tract haemorrhage and subarachnoid haemorrhage occurred in a 70-year-old man with pituitary apoplexy associated with idiopathic thrombocytopenic purpura. Left optic tract haemorrhage was confirmed on MRI. (orig.)

Henoch-Schönlein purpura in an older man presenting as rectal bleeding and IgA mesangioproliferative glomerulonephritis: a case report

Directory of Open Access Journals (Sweden)

Howarth Charles B

2011-08-01

Full Text Available Abstract Introduction Henoch-Schönlein purpura is the most common systemic vasculitis in children. Typical presentations are palpable purpura, abdominal pain, arthritis, and hematuria. This vasculitic syndrome can present as an uncommon cause of rectal bleeding in older patients. We report a case of an older man with Henoch-Schönlein purpura. He presented with rectal bleeding and acute kidney injury secondary to IgA mesangioproliferative glomerulonephritis. Case presentation A 75-year-old Polish man with a history of diverticulosis presented with a five-day history of rectal bleeding. He had first noticed colicky left lower abdominal pain two months previously. At that time he was treated with a 10-day course of ciprofloxacin and metronidazole for possible diverticulitis. He subsequently presented with rectal bleeding to our emergency department. Physical examination revealed generalized palpable purpuric rash and tenderness on his left lower abdomen. Laboratory testing showed a mildly elevated serum creatinine of 1.3. Computed tomography of his abdomen revealed a diffusely edematous and thickened sigmoid colon. Flexible sigmoidoscopy showed severe petechiae throughout the colon. Colonic biopsy showed small vessel acute inflammation. Skin biopsy resulted in a diagnosis of leukocytoclastic vasculitis. Due to worsening kidney function, microscopic hematuria and new onset proteinuria, he underwent a kidney biopsy which demonstrated IgA mesangioproliferative glomerulonephritis. A diagnosis of Henoch-Schönlein purpura was made. Intravenous methylprednisolone was initially started and transitioned to prednisone tapering orally to complete six months of therapy. There was marked improvement of abdominal pain. Skin lesions gradually faded and gastrointestinal bleeding stopped. Acute kidney injury also improved. Conclusion Henoch-Schönlein purpura, an uncommon vasculitic syndrome in older patients, can present with lower gastrointestinal bleeding

Splenectomy in patients with idiopathic thrombocytopenic purpura: Analysis of 109 cases

Directory of Open Access Journals (Sweden)

Enver Ay

2012-03-01

Full Text Available Objectives: Splenectomy is performed in order to provide the treatment in the patients with severe idiopathic thrombocytopenic purpura, refractory to medical treatment. In this study, we aimed to investigate the postoperatif and longterm outcomes in the patients who underwent splenectomy with the diagnosis of idiopathic thrombocytopenic purpura.Materials and Methods: Between 2001-2010 at Dicle University Medical Faculty, General Surgery Department, a retrospective review of the 109 patients who had undergone splenectomy for ITP was reviewed. Age, gender, presence of accessory spleens and location, duration of the operation, number of preoperative platelet tranfusion, number of preoperative and postoperative blood transfusion, length of hospital stay, long-term outcomes, morbidity and mortality were recorded.Results: The mean age was 37.10 ± 16.62 (16-72, and there were 88 (80.7% female and 21 (19.3% male patients. The mean operation time was 44.87 ± 10:32 (30-120 minutes. The average postoperative blood and preoperative platelet transfusion were 1.63 ± 0.85 (0-3 and 2.01 ± 0.71 (1-3 units, respectively. The accessory spleens were encountered in 20 (18.3% patients at the ultrasonographic examination. And also the accessory spleens were encountered in 23 (21.1% patients during operation and confirmed with histopathologic examination. The most common localization of accessory spleens were splenic hilus. The postoperative complications were occurred in 16 patients (14.7% and the most complication was atelectasia. The mean length of hospital stay was 4:56 ± 2:45 (2-12 days. Patients were followed for an average of 28 (9-48 months. At the follow-up period, 1 (0.9 % patient had died.Conclusion: Splenectomy can be performed safely in the treatment of the patients with idiopathic thrombocytopenic purpura unresponsive to medical treatment. Long-term good results can be obtained with splenectomy in these patients. The accessory spleens should not be

Postpartum plasma exchange in a woman with suspected thrombotic thrombocytopenic purpura (TTP) vs. hemolysis, elevated liver enzymes, and low platelet syndrome (HELLP): a case study.

Science.gov (United States)

Myers, Linda

2010-01-01

The occurrence of a hypercoagulable state and decreasing concentration of ADAMTS 13 in late pregnancy and during the postpartum period increases the risk for a woman to develop life-threatening thrombotic thrombocytopenic purpura (TTP). This is also the time of great risk for the more common obstetric complications of preeclampsia; eclampsia; and hemolysis, elevated liver functions tests, low platelets (HELLP) syndrome. These conditions are associated with high maternal and perinatal mortality. Differential diagnosis may be difficult due to the overlapping of clinical and laboratory findings, including thrombocytopenia, microangiopathic hemolytic anemia, neurologic symptoms, and renal insufficiency, making it difficult or impossible to distinguish them from TTP. Management of microangiopathic disorders encountered during pregnancy differ; therefore, an accurate diagnosis is required. Outcomes of TTP without plasma exchange therapy (TPE) are almost uniformly fatal. Early recognition and management of symptoms with prompt and aggressive TPE is essential when TTP is suspected.

Post-transfusion purpura treated with plasma exchange by haemonetics cell separator. A case report

DEFF Research Database (Denmark)

Laursen, B; Morling, N; Rosenkvist, J

1978-01-01

A case of post-transfusion purpura in a 61-year-old, multiparous female with a platelet alloantibody (anti-Zwa) in her serum is reported. The patient was successfully treated with plasma exchange by means of a Haemonetics 30 cell separator and corticosteroids. Compared with other therapeutic...

Petechiae and purpura: the ominous and the not-so-obvious?

Science.gov (United States)

Block, Stan L

2014-08-01

Petechiae and purpura are among the most alarming findings a pediatrician will commonly observe in the office. Severity of illness can range from a temper tantrum, to common viral infections, to the most deadly infections and diseases. To avoid many of the pitfalls in diagnosis, practitioners will need to be thorough in history taking, assessing fever and immunization status, and physical examination. In addition, a few simple laboratory tests will usually be needed and possibly a manual differential. Copyright 2014, SLACK Incorporated.

Aortic insufficiency

Science.gov (United States)

... page, please enable JavaScript. Aortic insufficiency is a heart valve disease in which the aortic valve does not close ... aortic insufficiency Images Aortic insufficiency References Carabello BA. Valvular heart disease. In: Goldman L, Schafer AI, eds. Goldman-Cecil ...

Kinetic study of indium-111 labelled platelets in idiopathic thrombocytopenic purpura

International Nuclear Information System (INIS)

Reiffers, J.; Vuillemin, L.; Broustet, A.; Ducassou, D.

1982-01-01

Labelling platelets with 111 Indium-oxine has advantages over the conventional 51 chromium method: labelling is more efficient and the radiations emitted almost exclusively consist of gamma-rays. Owing to these advantages, autologous platelets can be used for kinetic studies in patients with idiopathic thrombocytopenic purpura, even when thrombocytopenia is severe. 111 Indium labelling also provides accurate information on the sites of platelet destruction, which may help to predict the patient's response to splenectomy [fr

Efficacy of romiplostim in patients with chronic immune thrombocytopenic purpura : a double-blind randomised controlled trial

NARCIS (Netherlands)

Kuter, David J.; Bussel, James B.; Lyons, Roger M.; Pullarkat, Vinod; Gernsheimer, Terry B.; Senecal, Francis M.; Aledort, Louis M.; George, James N.; Kessler, Craig M.; Sanz, Miguel A.; Liebman, Howard A.; Slovick, Frank T.; de Wolf, J. Th M.; Bourgeois, Emmanuelle; Guthrie, Troy H.; Newland, Adrian; Wasser, Jeffrey S.; Hamburg, Solomon I.; Grande, Carlos; Lefrere, Francois; Lichtin, Alan Eli; Tarantino, Michael D.; Terebelo, Howard R.; Viallard, Jean-Francois; Cuevas, Francis J.; Go, Ronald S.; Henry, David H.; Redner, Robert L.; Rice, Lawrence; Schipperus, Martin R.; Guo, D. Matthew; Nichol, Janet L.

2008-01-01

Background Chronic immune thrombocytopenic purpura (ITP) is characterised by accelerated platelet destruction and decreased platelet production. Short-term administration of the thrombopoiesis-stimulating protein, romiplostim, has been shown to increase platelet counts in most patients with chronic

Location of skin lesions in Henoch-Schönlein purpura and its association with significant renal involvement.

Science.gov (United States)

St John, Jessica; Vedak, Priyanka; Garza-Mayers, Anna Cristina; Hoang, Mai P; Nigwekar, Sagar U; Kroshinsky, Daniela

2018-01-01

Henoch-Schönlein purpura (HSP) is a small vessel IgA-predominant vasculitis. To describe adult patients with HSP and determine if the distribution of skin lesions (ie, purpura above the waist or purpura below the waist only), is a predictor of significant renal involvement at the time of the skin biopsy and the months following. A retrospective study on renal function from 72 adult patients with skin-biopsy proven HSP. Longitudinal renal data were analyzed after HSP diagnosis by using baseline renal function for comparison. Statistical analysis adjusted for sex, age, and baseline creatinine revealed a trend between HSP lesions only on the upper and lower extremities and long-term renal involvement (4.767, P = .067). Moreover, in another analysis adjusted for age and baseline creatinine, lesions located only on the upper and lower extremities significantly increased the odds of having long-term significant renal involvement (6.55, P = .049) in men. This retrospective study used patient information that was subject to selection bias. In patients with HSP, skin lesion distribution on the extremities might be predictive of significant long-term renal involvement and might be critical for risk stratification and development of personalized diagnostics and therapeutics. Copyright © 2017 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.

Congenital thrombotic thrombocytopenic purpura caused by new compound heterozygous mutations of the ADAMTS13 gene

DEFF Research Database (Denmark)

Rank, Cecilie Utke; Kremer Hovinga, Johanna; Taleghani, Magnus Mansouri

2014-01-01

, causing intravascular platelet clumping and thrombotic microangiopathy. Our patient, a 26-year-old man, had attacks of thrombotic thrombocytopenic purpura (TTP) with thrombocytopenia and a urine dipstick positive for hemoglobin (4+), often as the only sign of hemolytic activity. He had ADAMTS13 activity...

Gastrointestinal involvement revealing Henoch Schonlein purpura in adults: Report of three cases and review of the literature

Directory of Open Access Journals (Sweden)

Hamzaoui Amira

2011-09-01

Full Text Available Abstract The diagnosis of Henoch-Schönlein purpura (HSP is difficult, especially when abdominal symptoms precede cutaneous lesions. We report three cases of adult HSP revealed by gastrointestinal (GI involvement.

[Localized purpura revealing vascular prosthetic graft infection].

Science.gov (United States)

Boureau, A S; Lescalie, F; Cassagnau, E; Clairand, R; Connault, J

2013-07-01

Prosthetic graft infection after vascular reconstruction is a rare but serious complication. We report a case of infection occurring late after implantation of an iliofemoral prosthetic vascular graft. The Staphylococcus aureus infection was revealed by vascular purpura localized on the right leg 7 years after implantation of a vascular prosthesis. This case illustrates an uncommonly late clinical manifestation presenting as an acute infection 7 years after the primary operation. In this situation, the presentation differs from early infection, which generally occurs within the first four postoperative months. Diagnosis and treatment remain a difficult challenge because prosthetic graft infection is a potentially life-threatening complication. Morbidity and mortality rates are high. Here we detail specific aspects of the clinical and radiological presentation. Copyright © 2013 Elsevier Masson SAS. All rights reserved.

Acute immune thrombocytopenic purpura in an adolescent with 2009 novel H1N1 influenza A virus infection

Directory of Open Access Journals (Sweden)

Chun-Yi Lee

2011-09-01

Full Text Available Although both leukopenia and thrombocytopenia are not uncommon hematological findings among patients with novel 2009 H1N1 influenza virus infection, immune thrombocytopenic purpura has rarely been shown to be associated with this novel influenza A infection. Here, we describe a previously healthy adolescent who presented with fever, influenza-like symptoms and acute onset of generalized petechiae and active oral mucosa bleeding on the third day of his illness. Severe leukopenia and thrombocytopenia were found. There was neither malignancy nor blast cells found by bone marrow aspiration. Real-time reverse transcriptase polymerase chain reaction was positive for novel 2009 H1N1 influenza infection. Novel influenza-associated atypical immune thrombocytopenic purpura was diagnosed. The patient recovered uneventfully after oseltamivir and methylprednisolone therapy.

Immune Thrombocytopenic Purpura and Gastritis by H. pylori Associated With Type 1 Diabetes Mellitus.

Science.gov (United States)

Culquichicón-Sánchez, Carlos; Correa, Ricardo; Flores-Guevara, Igor; Espinoza Morales, Frank; Mejia, Christian R

2016-02-24

We present the 15th case reported worldwide and 3rd case reported in Latin America of immune thrombocytopenic purpura associated with Type 1 diabetes mellitus in Scopus, MEDLINE, and SciELO. An 11-year-old male patient of mixed ethnicity with immune thrombocytopenic purpura, Type 1 diabetes mellitus, and gastritis due to H. pylori presented to the emergency room with petechiae, ecchymosis, and gingival and conjunctival bleeding that had been worsening for the past three months. The patient had a body mass index of 18.85 kg/m(2) (P75). A biochemical analysis showed 1×10(9) platelets/L, increased prothrombin time, increased partial thromboplastin time, and an HbA1C of 7.84% on admission. He was prescribed a single dose of intravenous methylprednisolone 750 mg in 100 mL of NaCl and daily oral 50 mg prednisolone, with intravenous 250 mg tranexamic acid every eight hours. The patient's glycemic control was continued with the administration of insulin glargine (30 units every 24 hours) and prandial insulin glulisine (five to eight units per meal). Before admission, the patient was on a prescribed treatment of sitagliptin 50 mg and metformin 850 mg, but this was suspended in the emergency room. For the eradication of H. pylori he was prescribed amoxicillin 500 mg every eight hours, oral clarithromycin 335 mg every 12 hours, and IV omeprazole 40 mg. After 15 days, he showed disease resolution and he was discharged to his home with orders to follow-up with pediatrics, hematology, and endocrinology services. The first-line treatment for immune thrombocytopenic purpura patients with active bleeding and a platelet count < 30,000 platelets/μl is the administration of corticosteroids and inmunoglobulin.

A Case of Systemic Lupus Erythematosus developing Two years after Remission of Thrombotic Thrombocytopenic Purpura

Science.gov (United States)

Myung, Seung-Jae; Yoo, Bin; Lee, Kyoo-Hyung; Yoo, Mi-Ran; Choi, Seung-Won; Yoo, Eun-Sil; Chi, Hyun-Sook; Moon, Hee-Bom

1996-01-01

We describe a 17-year-old male who presented with thrombotic thrombocytopenic purpura (TTP) and 2 years thereafter developed central nervous system lupus and nephritis. The association of TTP and systemic lupus erythematosus has been described, but the unusual sequence and chronological separation is very rare. PMID:8854658

Purpura, petechiae, and bullae as first signs of juvenile granulomatosis with polyangiitis.

Science.gov (United States)

Rawn, Saara; Miettunen, Paivi; Brown, Holly A; Schmeling, Heinrike

2014-12-01

We present a case of a 14-year-old girl who had a severe form of granulomatosis with polyangiitis (GPA) with extensive dermatological involvement, whose initial presentation was nonspecific leading to diagnostic confusion and initial consideration of infectious and other vasculitis causes. The patient presented with fever, congestion, malaise, and sinus pain. She was diagnosed with bacterial sinusitis and treated with antibiotics. Within weeks, she developed abdominal pain, hematuria, migratory arthritis, and palpable purpura and was diagnosed with Henoch-Schonlein purpura. She went on to develop hemoptysis and progression of the rash into erosive bullae. Investigations revealed that she was ANCA positive and had pauci-immune glomerulonephritis. Given her upper airway, pulmonary and renal involvement, and antineutrophil cytoplasmic antibodies positivity, a definitive diagnosis of a severe form of GPA was made. GPA is a chronic relapsing, life threatening vasculitis that predominantly affects small vessels. Our case demonstrates that GPA can present initially with nonspecific symptoms, including extensive dermatological involvement, leading to diagnostic confusion, and delays in treatment. In the case of a severe peripheral rash in the juvenile population and/or resistant upper airway symptoms, it is vital to consider a diagnosis of GPA to avoid serious organ or life threatening consequences.

Cardiac surgery in a patient with immunological thrombocytopenic purpura: Complications and precautions

Directory of Open Access Journals (Sweden)

Vivek Chowdhry

2013-01-01

Full Text Available Immune thrombocytopenic purpura (ITP patients are at high-risk for bleeding complications during and after cardiac surgeries involving cardiopulmonary bypass. We report a patient with ITP with severe coronary artery disease and mitral valve regurgitation who underwent uncomplicated coronary artery bypass grafting and mitral valve replacement. Three weeks later, the patient was readmitted in a very low general condition with signs of pericardial tamponade. We describe our experience of managing the case.

Bullous Henoch–Schonlein purpura with involvement of face

Directory of Open Access Journals (Sweden)

Devdeep Mukherjee

2017-01-01

Full Text Available Henoch–Schonlein purpura (HSP with facial involvement with bullous rashes are extremely rare. A 12-year-old boy presented with abdominal pain and features of arthritis. He also had multiple purpuric rashes over his lower limbs. Gradually, he developed bullous rashes which were seen on his legs and hands and progressed to involve the face. He was confirmed to be suffering from HSP from clinical presentation and skin biopsy. The child responded well to oral steroids. Bullous lesions may be seen in HSP. However, there is neither prognostic significance of this nor does it alter the management. Other causes of bullous lesions should be ruled out. As facial involvement is associated with renal and gastrointestinal involvement, these children should be monitored for sequelae.

A case of thrombotic thrombocytopenic purpura induced by acute pancreatitis

Directory of Open Access Journals (Sweden)

Arimoto M

2012-03-01

Full Text Available Miyoko Arimoto1, Yutaka Komiyama2, Fumiko Okamae1, Akemi Ichibe1, Setsuko Teranishi1, Hirohiko Tokunaga1, Keiko Nakaya3, Michie Fujiwara3, Manabu Yamaoka4, Shuji Onishi4, Rie Miyamoto5, Naoto Nakamichi5, Shosaku Nomura51Blood Transfusion Unit, Kansai Medical University Takii Hospital, 2Department of Clinical Sciences and Laboratory Medicine, Kansai Medical University, 3Clinical Medical Technology Unit, Kansai Medical University Takii Hospital, 4Blood Transfusion Unit, Kansai Medical University Hirakata Hospital, 5First Department of Internal Medicine, Kansai Medical University, Moriguchi, JapanAbstract: Thrombotic thrombocytopenic purpura (TTP is a multisystemic microvascular disorder that may be caused by an imbalance between unusually large von Willebrand factor multimers and the cleaving protease ADAMTS13. In acquired TTP, especially in secondary TTP with various underlying diseases, the diagnosis is difficult because there are many cases that do not exhibit severe deficiency of ADAMTS13 or raised levels of ADAMST13 inhibitors. It is well known that collagen disease, malignancy, and hematopoietic stem cell transplantation can be underlying conditions that induce TTP. However, TTP induced by acute pancreatitis, as experienced by our patient, has rarely been reported. Our patient completely recovered with treatments using steroids and plasma exchange (PE only. In cases where patients develop acute pancreatitis with no apparent causes for hemolytic anemia and thrombocytopenia, the possibility of TTP should be considered. Treatments for TTP including PE should be evaluated as soon as a diagnosis is made.Keywords: thrombotic thrombocytopenic purpura, ADAMTS13, acute pancreatitis, plasma exchange

Study on the relationship between peripheral blood red blood cells immunofunction status and serum TNF-α levels in pediatric patients with Henoch-Schoenlein purpura

International Nuclear Information System (INIS)

Feng Yue; He Haoming

2005-01-01

Objective: To investigate the relationship between changes of peripheral RBC immuno-function and serum TNF-α levels in pediatric patients with Henoch-Schoenlein purpura. Methods: RBC immuno-function status was studied with immunologic methods and serum TNF-α levels were measured with RIA in 31 pediatric patients with Henoch-Schoenlein purpura and 35 controls, Results: Levels of RBC-C3bRR were significantly lower and levels of serum TNF-α were significantly higher in the patients than those in controls (P<0.01). These two variables were significantly negatively correlated (r=-0.3018, P<0.05). On the contrary, the RBC-ICRRR levels were significantly higher in the patients (P<0.01) and were positively correlated with levels of TNF-α (r=0.3588, P<0.05). Conclusion: There were disturbances of RBC immuno-regulation with suppressed immuno-function in the purpura patients, which were related to the high levels of TNF-α. (authors)

Hematological long-term results of laparoscopic splenectomy for patients with idiopathic thrombocytopenic purpura: a case control study

NARCIS (Netherlands)

Berends, F. J.; Schep, N.; Cuesta, M. A.; Bonjer, H. J.; Kappers-Klunne, M. C.; Huijgens, P.; Kazemier, G.

2004-01-01

Laparoscopic splenectomy (LS) for idiopathic thrombocytopenic purpura (ITP) appears, when compared to open splenectomy (OS), associated with immediate important advantages. However, in a number of patients splenectomy does not lead to an adequate response, or after initial adequate response a

Recurrent Tricuspid Insufficiency

Science.gov (United States)

Kara, Ibrahim; Koksal, Cengiz; Cakalagaoglu, Canturk; Sahin, Muslum; Yanartas, Mehmet; Ay, Yasin; Demir, Serdar

2013-01-01

This study compares the medium-term results of De Vega, modified De Vega, and ring annuloplasty techniques for the correction of tricuspid insufficiency and investigates the risk factors for recurrent grades 3 and 4 tricuspid insufficiency after repair. In our clinic, 93 patients with functional tricuspid insufficiency underwent surgical tricuspid repair from May 2007 through October 2010. The study was retrospective, and all the data pertaining to the patients were retrieved from hospital records. Functional capacity, recurrent tricuspid insufficiency, and risk factors aggravating the insufficiency were analyzed for each patient. In the medium term (25.4 ± 10.3 mo), the rates of grades 3 and 4 tricuspid insufficiency in the De Vega, modified De Vega, and ring annuloplasty groups were 31%, 23.1%, and 6.1%, respectively. Logistic regression analysis revealed that chronic obstructive pulmonary disease, left ventricular dysfunction (ejection fraction, tricuspid insufficiency. Medium-term survival was 90.6% for the De Vega group, 96.3% for the modified De Vega group, and 97.1% for the ring annuloplasty group. Ring annuloplasty provided the best relief from recurrent tricuspid insufficiency when compared with DeVega annuloplasty. Modified De Vega annuloplasty might be a suitable alternative to ring annuloplasty when rings are not available. PMID:23466680

Surgical treatment of chronic idiopathic thrombocytopenic purpura: results in 107 cases

International Nuclear Information System (INIS)

Cola, B.; Tonielli, E.; Sacco, S.; Brulatti, M.; Franchini, A.

1986-01-01

Between 1972 and 1985, 107 patients with chronic Idiopathic Thrombocytopenic Purpura underwent splenectomy. Platelet life span and sites of sequestration were studied with labelled platelets and external scanning. Medical treatment was always of scarce and transient effectiveness and had considerable side effects. Splenectomy had minimal complications and mortality and caused no hazard of overwhelming sepsis in adults. The results of splenectomy were very satisfying, especially when platelet sequestration was mainly splenic (remission in about 90% of patients). Surgical treatment is at present the most effective in patients with chronic ITP

Treatment of immune thrombocytopenic purpura: focus on eltrombopag

Directory of Open Access Journals (Sweden)

Lawrence Rice

2009-03-01

Full Text Available Lawrence RiceWeill Cornell Medical College, The Methodist Hospital, Houston, Texas, USAAbstract: Immune thrombocytopenic purpura (ITP is a relatively common autoimmune disorder in which antibodies are produced to circulating platelets. Symptoms can be mild, but for most patients the risk of severe bleeding is unacceptable and treatment is required. Glucocorticoids followed by splenectomy had been the mainstays of therapy. High dose intravenous immunoglobulin and anti-RhD therapy are available for patients with severe illness, but produce only temporary benefit. Rituximab may provide more durable responses, danazol may be underutilized, and immunosuppressants and cytotoxic agents are less often required. Recently the pathophysiology of ITP has been more clearly elucidated, particularly the importance of decreased production of platelets in most patients and the very blunted rise that occurs in serum thrombopoietin (TPO. The isolation of TPO and better understanding of its role in thrombopoiesis has led to the development of new highly effective treatments. TPO analogs had some successes in treating highly refractory ITP patients but were taken out of development due to TPO-antibody induction. Two second-generation TPO-mimetics, romiplostim and the orally available eltrombopag, have recently been licensed in some territories for the treatment of ITP. Approval of eltrombopag was based on results from Phase II and III placebo-controlled clinical trials and a long-term extension study. About 80% of patients achieve significant increases in platelet count (11% of placebo patients, with reduced bleeding and reduced use of concomitant medications; responses are often durable with no tachyphylaxis. The side effects of eltrombopag are generally mild and not worse than placebo, although there are concerns about hepatic dysfunction, and the potentials for thromboses, marrow reticulin fibrosis, rebound thrombocytopenia and cataracts. This is an important new

Classical management of refractory adult immune (idiopathic) thrombocytopenic purpura.

Science.gov (United States)

McMillan, R

2002-03-01

Treatment of chronic immune (idiopathic) thrombocytopenic purpura with corticosteroids and/or splenectomy results in safe platelet counts in over 70% of patients without additional treatment. Therapy of patients who are refractory to these two treatments may be difficult. The treatment approach to refractory ITP patients, described in this report, is arbitrarily divided into four levels: levels 1 through 3 represent treatments with increasing side effects; level 4 therapy may be tried when the others have failed. Patients undergoing these treatments may require concomitant intravenous gammaglobulin, high-dose corticosteroids or platelets, to maintain the platelet count in the setting of mucosal bleeding or severe thrombocytopenia. Copyright 2002, Elsevier Science Ltd. All rights reserved.

Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS: a 24-year clinical experience with 178 patients

Directory of Open Access Journals (Sweden)

Lara Primo

2008-12-01

Full Text Available Abstract Background Thrombotic thrombocytopenic purpura and the hemolytic uremic syndrome (TTP-HUS are related and uncommon disorders with a high fatality and complication rate if untreated. Plasma exchange therapy has been shown to produce high response rates and improve survival in patients with many forms of TTP-HUS. We performed a retrospective cohort study of 178 consecutively treated patients with TTP-HUS and analyzed whether clinical or laboratory characteristics could predict for important short- and long-term outcome measures. Results Overall 30-day mortality was 16% (n = 27. 171 patients (96% received plasma exchange as the principal treatment, with a mean of 8 exchanges and a mean cumulative infused volume of 42 ± 71 L of fresh frozen plasma. The rate of complete response was 65% or 55% depending on whether this was defined by a platelet count of 100,000/μl or 150,000/μl, respectively. The rate of relapse was 18%. The Clinical Severity Score did not predict for 30-day mortality or relapse. The time to complete response did not predict for relapse. Renal insufficiency at presentation was associated with a decreased risk of relapse, with each unit increase in serum creatinine associated with a 40% decreased odds of relapse. 72% of our cohort had an idiopathic TTP-sporadic HUS, while 17% had an underlying cancer, received a solid organ transplant or were treated with a mitomycin-based therapy. The estimated overall 5-year survival was 55% and was significantly better in those without serious underlying conditions. Conclusion Plasma exchange therapy produced both high response and survival rates in this large cohort of patients with TTP-HUS. The Clinical Severity Score did not predict for 30-day mortality or relapse, contrary to our previous findings. Interestingly, the presence of renal insufficiency was associated with a decreased risk of relapse. The most important predictor of mortality was the presence or absence of a serious

Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS): a 24-year clinical experience with 178 patients

Science.gov (United States)

Levandovsky, Mark; Harvey, Danielle; Lara, Primo; Wun, Ted

2008-01-01

Background Thrombotic thrombocytopenic purpura and the hemolytic uremic syndrome (TTP-HUS) are related and uncommon disorders with a high fatality and complication rate if untreated. Plasma exchange therapy has been shown to produce high response rates and improve survival in patients with many forms of TTP-HUS. We performed a retrospective cohort study of 178 consecutively treated patients with TTP-HUS and analyzed whether clinical or laboratory characteristics could predict for important short- and long-term outcome measures. Results Overall 30-day mortality was 16% (n = 27). 171 patients (96%) received plasma exchange as the principal treatment, with a mean of 8 exchanges and a mean cumulative infused volume of 42 ± 71 L of fresh frozen plasma. The rate of complete response was 65% or 55% depending on whether this was defined by a platelet count of 100,000/μl or 150,000/μl, respectively. The rate of relapse was 18%. The Clinical Severity Score did not predict for 30-day mortality or relapse. The time to complete response did not predict for relapse. Renal insufficiency at presentation was associated with a decreased risk of relapse, with each unit increase in serum creatinine associated with a 40% decreased odds of relapse. 72% of our cohort had an idiopathic TTP-sporadic HUS, while 17% had an underlying cancer, received a solid organ transplant or were treated with a mitomycin-based therapy. The estimated overall 5-year survival was 55% and was significantly better in those without serious underlying conditions. Conclusion Plasma exchange therapy produced both high response and survival rates in this large cohort of patients with TTP-HUS. The Clinical Severity Score did not predict for 30-day mortality or relapse, contrary to our previous findings. Interestingly, the presence of renal insufficiency was associated with a decreased risk of relapse. The most important predictor of mortality was the presence or absence of a serious underlying disorder. PMID

Effects of prednisone and splenectomy in patients with idiopathic thrombocytopenic purpura : only splenectomy induces a complete remission

NARCIS (Netherlands)

Louwes, H; Vellenga, E; Houwerzijl, EJ; de Wolf, JTM

2001-01-01

Idiopathic thrombocytopenic purpura (ITP) is a heterogeneous disease, whereby it is unclear if and in which way prednisone and splenectomy affect the platelet kinetics leading to a complete remission. To determine the effects of prednisone and splenectomy on the mean platelet life (MPL) and platelet

A dengue infection without bleeding manifestations in an adult with immune thrombocytopenic purpura

OpenAIRE

Ehelepola, N. D. B.; Gunawardhana, M. B. K.; Sudusinghe, T. N.; Sooriyaarachchi, S. K. D.; Manchanayake, S. P.; Kalupahana, K. L. R.

2016-01-01

Background Dengue is the most prevalent and fast spreading arboviral infection affecting people. No specific drug is available to treat dengue. Thrombocytopenia with potential of serious hemorrhages is one of the hall mark features of dengue. Immune thrombocytopenic purpura is an autoimmune disease causing thrombocytopenia. If a patient with that gets dengue, we expect severe thrombocytopenia with bleeding manifestations. Only a handful of such cases were reported before, and they were manage...

Henoch schonlein purpura--a 5-year review and proposed pathway.

Directory of Open Access Journals (Sweden)

Louise Watson

Full Text Available Henoch Schonlein Purpura (HSP is the commonest systemic vasculitis of childhood typically presenting with a palpable purpuric rash and frequently involving the renal system. We are the first group to clinically assess, critically analyse and subsequently revise a nurse led monitoring pathway for this condition.A cohort of 102 children presenting with HSP to a secondary/tertiary level UK paediatric hospital over a five year period, were monitored using a nurse led care pathway. Using this cohort, the incidence (6.21 cases per 100,000 children per year and natural disease course of HSP nephritis (46% initial renal inflammation; 9% subsequent renal referral; 1% renal biopsy and immunosuppression was determined. Older patients were at higher risk of requiring a renal referral (renal referral 12.3 (8.4-13.5 years vs. normal outcome 6.0 (3.7-8.5 years; p<0.01. A normal urinalysis on day 7 had a 97% (confidence interval 90 to 99% negative predictive value in predicting a normal renal outcome.Using this data and existing literature base, The Alder Hey Henoch Schonlein Purpura Pathway was developed, a revised pathway for the screening of poor renal outcome in HSP. This is based on a six-month monitoring period for all patients presenting with HSP, which importantly prioritises patients according to the urine findings on day 7 and thus intensively monitors those at higher risk of developing nephritis. The pathway could be easily adapted for use in different settings and resources.The introduction of a standardised pathway for the monitoring of HSP will facilitate the implementation of disease registries to further our understanding of the condition and permit future clinical trials.

Clinical significance of measurement of changes of serum IL-2, SIL-2R levels after treatment in patients with thrombocytopenic purpura

International Nuclear Information System (INIS)

Feng Yue

2005-01-01

Objective: To study the changes of serum IL-2 and SIL-2R levels after treatment in 31 patients with thrombocytopenic purpura. Methods: Serum IL-2 (with RIA) and SIL-2R (with ELISA) levels were measured in 31 patients with thrombocytopenic purpura both before and after treatment as well as in 35 controls. Results: Before treatment, in the patients the serum IL-2 levels were significantly lower and serum SIL-2R levels were significantly higher than those in the controls ( P 0.05). Conclusion: Cytokines IL-2 and SIL-2R levels changes could reflect the immunostatus of the patients as well as the progress of diseases and could be of prognostic values. (authors)

Purpura fulminans in a patient with mixed connective tissue disease.

LENUS (Irish Health Repository)

Murad, Aizuri A

2013-01-01

A 43-year-old lady was admitted to the intensive care unit with sepsis. She had a history of mixed connective tissue disease, Raynaud\\'s syndrome and hypothyroidism. 2 days later, she developed a purpuric rash on her face and extremities with a livedoid background. Few days later, her distal fingers and toes became gangrenous which then had to be amputated. Laboratory investigations showed that she was coagulopathic and had multiple organ dysfunctions. Antiphospholipid antibodies were negative; however, protein C and antithrombin III levels were low. A skin biopsy showed fibrinoid necrosis in the vessel wall with microthrombi and red-cell extravasation. A diagnosis of purpura fulminans was made.

Accessory spleen compromising response to splenectomy for idiopathic thrombocytopenic purpura

International Nuclear Information System (INIS)

Ambriz, P.; Munoz, R.; Quintanar, E.; Sigler, L.; Aviles, A.; Pizzuto, J.

1985-01-01

Accessory spleens were sought in 28 patients who had undergone splenectomy for chronic idiopathic thrombocytopenic purpura (ITP), using a variety of techniques. Abdominal scintigraphy with autologous erythrocytes labeled with Tc-99m and opsonized with anit-D IgG (radioimmune method) proved to be most useful, clearly demonstrating one or more accessory spleens in 12 cases (43%). Computed tomography (CT) was also helpful. Four out of five patients demonstrated an increased platelet count following surgery, the effectiveness of which was illustrated by the radioimmune scan. Patients who have had splenectomy for chronic ITP should be scanned using radioimmune techniques and CT to determine whether an accessory spleen is present

Age of onset as a risk factor of renal involvement in Henoch-Schönlein purpura

Science.gov (United States)

Ledika, Masayu Amanda; Sapartini, Gartika; Setiabudiawan, Budi

2014-01-01

Background Henoch-Schönlein purpura (HSP) is the most common vasculitis in children, characterized by triad of symptoms; palpable purpura without thrombocytopenia, abdominal pain, and arthritis. Renal involvement often occur in children with HSP. No data on the renal involvement of children with HSP in Indonesia, especially West Java. Objective To evaluate renal involvement in children with HSP. Methods Retrospective study was conducted in children with HSP in Department of Child Health, Hasan Sadikin Hospital, from 2006 to 2011. Characteristics and clinical manifestations was reviewed from medical record. HSP was diagnosed by American College of Rheumatology 1990 criteria or European League Against Rheumatism/Pediatric Rheumatology International Trials Organization/Pediatric Rheumatology European Society 2008. Results There were 128 patients, consisting of 82 male (64.9%) and 46 female (35.1%) with ratio 1.8:1. Mean age was 7.9 ± 2.9 years old which range from 6 month to 15 years. Peak morbidity was between 5-10 years old. Prevalence of HSP in Hasan Sadikin Hospital tend to raise from 2.7/100,000 in 2008 to 5.2/100,000 in 2010. In most patients (71%) purpura was the first symptom. Seventy-one patients (44.5%) had arthritis and 89 patients (69.5%) had abdominal pain, while renal involvement was in 28 patients (21.8%). Gastrointestinal manifestations tend to manifest in patients less than 5 years old (p = 0.267), while renal involvement tend to manifest in age group 11-15 years old (p = 0.015) with odds ratio 3.1 (95% confidence interval, 1.2-8.1). Conclusion Renal involvement in children with HSP is more common in age group 11 to 15 years old. PMID:24527410

Sertraline-induced periorbital purpura: a case report.

Science.gov (United States)

Kayhan, Fatih; Eken, Zahide Eriş; Uguz, Faruk

2015-08-01

The incidence of mild to severe levels of spontaneous bleeding due to the usage of selective serotonin reuptake inhibitors (SSRIs) is relatively low. Although the exact mechanism is not known, it is thought that inhibition of the serotonin transporter together with a decrease in platelet serotonin could be responsible for the bleeding. Therefore, the use of SSRIs in conjunction with anti-aggregants may predispose to or exacerbate the risk of bleeding. In this case report, we describe a 44-year-old female patient with a diagnosis of anxiety disorder who spontaneously developed periorbital purpura during treatment with sertraline. Abnormal bleeding after treatment with an SSRI should be kept in mind, and alternative non-SSRI drugs of choice in such cases would be more appropriate. More extensive and comprehensive studies focusing on hemostasis and bleeding disorders are needed for SSRIs such as sertraline. © The Royal Australian and New Zealand College of Psychiatrists 2015.

Exudative Retinal Detachment Treatment in a Patient with Thrombotic Thrombocytopenic Purpura

Directory of Open Access Journals (Sweden)

Magali Sampo

2016-02-01

Full Text Available Purpose: We report a case of unilateral exudative retinal detachment in a patient with thrombotic thrombocytopenic purpura (TTP, without associated hypertension, successfully treated with plasmapheresis. Case Report: A 46-year-old woman with a medical history of TTP presented with unilateral exudative retinal detachment. Biological and radiological assessment eliminated other causes of exudative retinal detachment, including hypertension. Plasma exchange was performed, followed by a rapid improvement in visual acuity and total disappearance of serous detachment. Conclusion: Exudative unilateral retinal detachment is a rare complication of TTP and can be successfully treated by plasma exchange.

EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part I: Overall methodology and clinical characterisation

DEFF Research Database (Denmark)

Ruperto, Nicolino; Ozen, Seza; Pistorio, Angela

2010-01-01

To report methodology and overall clinical, laboratory and radiographic characteristics for Henoch-Schönlein purpura (HSP), childhood polyarteritis nodosa (c-PAN), c-Wegener granulomatosis (c-WG) and c-Takayasu arteritis (c-TA) classification criteria.......To report methodology and overall clinical, laboratory and radiographic characteristics for Henoch-Schönlein purpura (HSP), childhood polyarteritis nodosa (c-PAN), c-Wegener granulomatosis (c-WG) and c-Takayasu arteritis (c-TA) classification criteria....

Early diagnostic value of determination of urinary excretion amount of proteins for renal lesions in pediatric patients with anaphylactoid purpura (AP)

International Nuclear Information System (INIS)

Xu Guocheng; Li Zhiqi; Guo Benbiao; Shen Yina; Mao Shuanggen; Zhang Xinlu

2009-01-01

Objective: To study the early diagnostic value of determination of urinary excretion amourt of proteins for renal damage in pediatric patients with AP. Methods: Morning arine specimen contents of albumin, IgG and β 2 -m (with RIA) as well as serum BUN and creatinine levels were measured in 25 pediatric patients with simple A P, 27 pediatric patients with purpura nephritis (PN) and 32 controls. Results: The urinary contents of proteins in all the patients were significantly higher than those in controls (P 0.05). Conclusion: Changes of urinary excretion of proteins occurred much earlier than changes of BUN and creatinine in purpura patients complicated with renal involvement and might be used as an indicator for early diagnosis. (authors)

Henoch-Schönlein purpura: a long-term prospective study in Greek children.

Science.gov (United States)

Fretzayas, Andrew; Sionti, Irini; Moustaki, Maria; Papadimitriou, Anastasios; Nicolaidou, Polyxeni

2008-12-01

Henoch-Schönlein purpura is a common vasculitis of childhood. The present study, comprising 74 children enrolled during a 9-year period, aimed to delineate the clinical spectrum and the long term follow-up of the disease. Patients were diagnosed strictly on the criteria established by the American College of Rheumatology. The diagnosis was further strengthened by a skin biopsy performed in more than half of cases (42/74), which revealed leukocytoclastic vasculitis in all patients, whereas IgA deposits were found in 37/42 children. There was a predominance of boys (41 males), and the mean age of study population was 5.2 +/- 2.54 years. Palpable purpura was identified in 73/74 children, whereas transient arthritis manifested in 68/74 children. Gastrointestinal blood loss was elicited in 30 children, 11 of whom had also renal involvement. The latter was seen in 19/74 (25.7%) children, and in the long term follow-up (extended to 2004, from 4-12 years, mean duration 7.3 years), 2 children remained with persistent occult hematuria. Relapses occurred in 49 children (66%), and half of them experienced more than 1 recurrence. Arthritis was less common in the relapsed episodes, and this difference was statistically significant (P period. Recurrences often differ to some extent from the initial episode with arthritis being less common with relapses.

Young Ischemic Stroke as Presentation of Thrombotic Thrombocytopenic Purpura: A Case Report

Directory of Open Access Journals (Sweden)

Ahmad Najib Azmi

2017-12-01

Full Text Available Thrombotic thrombocytopenic purpura (TTP is a rare disorder with an estimated incidence of 3 - 7/1,000,000. It is an autoimmune disorder characterized by fever, neurological signs, microangiopathic hemolytic anemia, thrombocytopenia and renal failure. This case report will describe a young lady who presented with acute middle cerebral artery infarct and was subsequently diagnosed to have TTP. Therapeutic plasma exchange (TPE did not improve the neurological deficit. This case highlights the importance of recognizing TTP as a possible differential diagnosis in young onset stroke.

Imaging of insufficiency fractures

Energy Technology Data Exchange (ETDEWEB)

Krestan, Christian [Department of Radiology, Medical University of Vienna, Vienna General Hospital, Waehringerstr. 18-20, 1090 Vienna (Austria)], E-mail: christian.krestan@meduniwien.ac.at; Hojreh, Azadeh [Department of Radiology, Medical University of Vienna, Vienna General Hospital, Waehringerstr. 18-20, 1090 Vienna (Austria)

2009-09-15

This review focuses on the occurrence, imaging and differential diagnosis of insufficiency fractures. Prevalence, the most common sites of insufficiency fractures and their clinical implications are discussed. Insufficiency fractures occur with normal stress exerted on weakened bone. Postmenopausal osteoporosis is the most common cause of insufficiency fractures. Other conditions which affect bone turnover include osteomalacia, hyperparathyroidism, chronic renal failure and high-dose glucocorticoid therapy. It is a challenge for the radiologist to detect and diagnose insufficiency fractures, and to differentiate them from other bone lesions. Radiographs are still the most widely used imaging method for identification of insufficiency fractures, but sensitivity is limited, depending on the location of the fractures. Magnetic resonance imaging (MRI) is a very sensitive tool to visualize bone marrow abnormalities associated with insufficiency fractures. Thin section, multi-detector computed tomography (MDCT) depicts subtle fracture lines allowing direct visualization of cortical and trabecular bone. Bone scintigraphy still plays a role in detecting fractures, with good sensitivity but limited specificity. The most important differential diagnosis is underlying malignant disease leading to pathologic fractures. Bone densitometry and clinical history may also be helpful in confirming the diagnosis of insufficiency fractures.

Quantifying the reduction in immunoglobulin use over time in patients with chronic immune thrombocytopenic purpura receiving romiplostim (AMG 531)

NARCIS (Netherlands)

Pullarkat, Vinod A.; Gernsheirner, Terry B.; Wasser, Jeffrey S.; Newland, Adrian; Guthrie, Troy H.; de Wolf, Joost Th. M.; Stewart, Ron; Berger, Dietmar

Patients with Immune thrombocytopenic purpura (ITP) often require immunoglobulin (Ig) therapy with intravenous 19 (IVIG) or anti-D to prevent or treat the serious bleeding events. Because the thrombopoietin (TPO) mimetic romiplostim (AMG 531; Nplate) elevates platelet counts in patients with chronic

Splenectomy in children with idiopathic thrombocytopenic purpura : A prospective study of 134 children from the Intercontinental Childhood ITP Study Group

NARCIS (Netherlands)

Kuehne, Thomas; Blanchette, Victor; Buchanan, George R.; Ramenghi, Ugo; Donato, Hugo; Tamminga, Rienk Y. J.; Rischewski, Johannes; Berchtold, Willi; Imbach, Paul

2007-01-01

Background. Splenectomy is an effective procedure for children and adults with severe or refractory idiopathic thrombocytopenic purpura (ITP). Data regarding pediatric patients are limited. Procedure. Sixty-eight Intercontinental Childhood ITP Study Group (ICIS) investigators from 57 institutions in

Kampo medicines improved blood test and QOL in two vasculitis cases of Churg–Strauss syndrome and Henoch–Shönlein purpura after inadequate treatment with conventional therapies

Directory of Open Access Journals (Sweden)

Yasuyo Hijikata

2010-02-01

Full Text Available Yasuyo Hijikata1, Yumiko Ikemoto2, Urara Kohdera31Toyodo Hijikata Clinic, Osaka, Japan; 2St Mary’s Hospital, Himeji, Japan; 3Nakano Children’s Hospital, Osaka, JapanObjective: Based on the tenets of traditional Chinese medicine (TCM theory, Kampo medicines were selected and applied to two cases of Churg–Strauss syndrome and Henoch–Shönlein purpura. Two vasculitis syndrome patients exhibited persistent symptoms and abnormal blood tests after treatment with conventional therapies.Methods: As the two cases had “blood stagnation” and “damps and heat” and one had a “yang deficiency” in terms of TCM theory, we applied certain selected Kampo medicines.Results: In case 1, the patient presented with hypereosinophilia, venous thrombosis, pulmonary infarction, decreased platelet count, ulner nerve palsy and Raynaud’s phenomena, which led to a diminished quality of life. After starting the Kampo medicines, the patient improved quickly and recovered within 11 months. In case 2, persistent purpura, abdominal pain, and bloody feces quickly improved and disappeared after Kampo treatment. After starting the Kampo medicines, prednisolone was stopped at 21 days without any sign of relapse to date.Conclusion: Kampo medicines helped clear the persistent abnormal symptoms and laboratory findings of vasculitis syndromes, Churg–Strauss syndrome and Henoch–Shönlein purpura, which had responded inadequately to the conventional therapies administered.Keywords: Kampo medicine, vasculitis, Churg–Strauss syndrome, Henoch–Shönlein purpura

Bula Hemoragik dengan Komplikasi Perforasi Gaster Sebagai Manifestasi Klinis Purpura Henoch-Schonlein yang Tidak Biasa pada Anak

Directory of Open Access Journals (Sweden)

Budi Setiabudiawan

2016-11-01

Full Text Available Purpura Henoch-Schonlein (PHS merupakan vaskulitis pada pembuluh darah kecil tersering terjadi pada anak. Penyakit ini ditandai dengan purpura palpablenontrombositopenia disertai salah satu gejala nyeri perut, artritis atau atralgia, glomerulonefritis, dan hasil biopsi jaringan berupa gambaran vaskulitis leukositoklastik. Bula hemoragik disertai edema jaringan subkutan merupakan gambaran yang tidak umum pada PHS dan sering terlewatkan. Manifestasi klinis vesikobulosa PHS sering ditemukan pada pasien dewasa, 16%–60% kasus, sedangkan pada anak kurang dari 2% kasus. Walaupun PHS secara tipikal merupakan penyakit selflimiting, tetapi komplikasi serius dapat terjadi. Perforasi gaster sangat jarang dilaporkan sebagai komplikasi PHS. Kami melaporkan 2 kasus PHS dengan manifestasi kulit yang berat, yaitu timbulnya bula hemoragik disertai dengan perforasi gaster. Pada kedua kasus dilakukan tindakan operatif dengan keluaran yang berbeda, pada kasus pertama pasien dipulangkan dalam kondisi baik pascaoperasi setelah dilakukan laparatomi eksplorasi, walaupun masih menderita nefritis. Sedangkan pasien kedua meninggal setelah tindakan diagnostic peritoneal lavagedisebabkan sepsis berat. Simpulan, bula hemoragik dapat dipertimbangkan sebagai prediktor komplikasi perforasi gaster pada PHS yang akan meningkatkan kewaspadaan dalam tata laksana PHS

Fournier's gangrene in a patient receiving treatment for idiopathic thrombocytopenic purpura.

Science.gov (United States)

Yuda, Junichiro; Honma, Riko; Yahagi, Tomoyasu; Omoto, Eijiro

2011-01-01

We report the case of a 68-year-old man who was diagnosed with Fournier's gangrene (FG), which developed during immunosuppresive treatment for idiopathic thrombocytopenic purpura (ITP). The patient was administered steroids for ITP but on the 36th day, he developed FG and septic shock. We initiated antibiotic treatment and drained a periproctal abscess immediately. On day 53, extensive drainage to progressive FG and a splenectomy was performed, following which both FG and thrombocytopenia improved. This is the first case of FG has developing in a ITP patient. It appears that high-dose immunoglobulin therapy and splenectomy should be considered earlier especially for a patient complicated with FG.

Mesenteric vasculitis in adults with Henoch-Schonlein purpura: a not-so-benign condition.

LENUS (Irish Health Repository)

Sibartie, V

2009-02-07

INTRODUCTION: The gastrointestinal manifestations of Henoch-Schonlein purpura (HSP) are well characterised, but their recognition can be difficult when they occur in isolation. Furthermore, HSP can run a more serious course in adults, compared to children, in whom the disease usually occurs. MATERIALS AND METHODS: We describe two cases that illustrate the challenges of HSP with mesenteric vasculitis and the outcome in adults. CONCLUSION: Although self-limiting in most patients, the outcome of HSP in adults can be far from benign and even fatal.

Long-term outcomes of laparoscopic splenectomy versus open splenectomy for idiopathic thrombocytopenic purpura.

Science.gov (United States)

Qu, Yikun; Xu, Jian; Jiao, Chengbin; Cheng, Zhuoxin; Ren, Shiyan

2014-01-01

The long-term outcomes of laparoscopic splenectomy (LS) versus open splenectomy (OS) in patients with idiopathic thrombocytopenic purpura (ITP) are not known. A retrospective analysis of 73 patients who underwent splenectomy (32 LS and 41 OS) for refractory ITP between April 2003 and June 2012 was conducted. LS was associated with shorter hospital stay (P = 0.01), less blood loss and blood transfusion during surgery, quicker resumption of oral diet (P splenectomy is not different from that of open splenectomy for patients with ITP.

Anti-alpha-galactosyl antibodies and immune complexes in children with Henoch-Schönlein purpura or IgA nephropathy

NARCIS (Netherlands)

Davin, J. C.; Malaise, M.; Foidart, J.; Mahieu, P.

1987-01-01

Episodes of hematuria in IgA nephropathy or Henoch-Schönlein purpura are frequently associated with microbial infections. Some of those infectious agents bear alpha-galactosyl residues on their cell surface. These observations prompted us to determine, by passive hemagglutination, the titers of

Successful Treatment of Aggressive Mature B-cell Lymphoma Mimicking Immune Thrombocytopenic Purpura.

Science.gov (United States)

Ono, Koya; Onishi, Yasushi; Kobayashi, Masahiro; Ichikawa, Satoshi; Hatta, Shunsuke; Watanabe, Shotaro; Okitsu, Yoko; Fukuhara, Noriko; Ichinohasama, Ryo; Harigae, Hideo

2018-03-30

A 55-year-old woman suffered from hemorrhagic tendency. She had severe thrombocytopenia without any hematological or coagulatory abnormalities, and a bone marrow examination revealed an increased number of megakaryocytes without any abnormal cells or blasts. No lymphadenopathy or hepatosplenomegaly was observed on computed tomography. She was initially diagnosed with immune thrombocytopenic purpura (ITP). None of the treatments administered for ITP produced a response. However, abnormal cells were eventually found during the third bone marrow examination. The pathological diagnosis was mature B-cell lymphoma. Rituximab-containing chemotherapy produced a marked increase in the patient's platelet count, and her lymphoma went into complete remission.

Epidemiologi, sygdomspræsentation og forløb af idiopatisk trombocytopenisk purpura hos børn i Danmark 1998-2000

DEFF Research Database (Denmark)

Kjaersgaard, Mimi; Edslev, Pernille Wendtland; Rosthøj, Steen

2008-01-01

INTRODUCTION: Idopathic Thrombocytopenic Purpura (ITP) is a condition with isolated thrombocytopenia and bleeding symptoms in skin and mucous membranes. It is easy to establish the diagnosis, but treatment is controversial, possibly due to different estimates of the risk for serious bleeding. We...

Pulmonary Hyalinizing Granuloma Associated with Idiopathic Thrombocytopenic Purpura

Directory of Open Access Journals (Sweden)

Christopher Coleman

2014-01-01

Full Text Available Pulmonary hyalinizing granuloma (PHG is a rare, benign lung disease of unknown etiology. It manifests as discrete, rounded nodules within the lung parenchyma. A 39-year-old woman presented for investigation after pulmonary nodules were found incidentally. Chest computed tomography showed multiple, discrete, non-enhancing pulmonary nodules bilaterally. Positron emission tomography (PET was negative. Biopsy demonstrated a non-specific lymphoplasmacytic infiltrate. Open resection yielded two nodules consistent with hyalinizing granulomas. The differential for multiple pulmonary nodules is broad. PET scan can help rule out metastatic disease, although some cancers are not hypermetabolic on PET. Furthermore, some non-malignant conditions, including hyalinizing granuloma, can show increased activity on PET. PHG should be included in the differential of multiple pulmonary nodules, especially if nodule stability can be demonstrated and/or needle biopsies are non-diagnostic. Associated immune-mediated conditions, such as idiopathic thrombocytopenic purpura (ITP in our patient, may also favor HG. In this case report we find an association between PHG and ITP.

Cytomegalovirus-associated Immune Thrombocytopenic Purpura After Liver Transplantation

Directory of Open Access Journals (Sweden)

Shu-Hao Wei

2007-01-01

Full Text Available Immune thrombocytopenic purpura (ITP is a rare complication after liver transplantation. Infection with cytomegalovirus (CMV is a frequent complication of organ transplantation and may induce autoimmune diseases, such as ITP. We report a case of ITP after primary CMV infection in a 3-year-old boy recipient of living-related orthotopic liver transplantation (OLT. The ITP developed 2 years after OLT in this patient who had received tacrolimus as an immunosuppressive agent, with nadir platelet counts of 5000/mm3 in 2 weeks. The patient was treated with two courses of intravenous gamma globulin (1 g/kg/day for 2 days and subsequent oral prednisolone (1.3 mg/kg/day for 2 weeks. He recovered from thrombocytopenia 4 weeks later. An inadequate immunosuppression, as evident by the low serum tacrolimus level (5.8 ng/mL before the episode of ITP in this patient, may allow the development of ITP after CMV infection. [J Formos Med Assoc 2007;106(4:327-329

Thrombotic thrombocytopenic purpura possibly triggered by Graves’ disease

Science.gov (United States)

Chitnis, Saurabh D; Mene-Afejuku, Tuoyo O; Aujla, Amandeep; Shady, Ahmed; Gil, Gaby S; Cativo, Eder Hans; Popescu-Martinez, Andrea

2017-01-01

Abstract Thrombotic thrombocytopenic purpura (TTP) is a part of a spectrum of thrombotic microangiopathy syndromes which are mainly characterized by platelet aggregation causing microangiopathic hemolytic anemia, thrombocytopenia and microvascular occlusion. In literature, very few cases expressing a direct association between pre-existing Grave’s disease and TTP have been described. A 37-year-old African–American woman with past medical history of Grave’s disease and polysubstance abuse who presented with complaints of dyspnoea at rest and chest pain was diagnosed to have TTP on further evaluation. Patient also showed severely elevated thyroid hormones and suppressed thyroid stimulating hormone levels indicating severe thyrotoxicosis. Initiation of prompt management of TTP and thyrotoxicosis led to a favorable patient outcome. In conclusion, patients presenting with thyrotoxicosis, thrombocytopenia and microangioapthic hemolytic anemia without an alternative cause should be treated and screened for TTP due to the high fatality associated with untreated or untimely detection of this disease. PMID:29744115

Neonatal purpura fulminans caused by rare Citrobacter species

Directory of Open Access Journals (Sweden)

Sanjiv Vijay Choudhary

2018-01-01

Full Text Available A 23-day-old neonate, born of nonconsangious marriage, admitted to Neonatal Intensive Care Unit for hypernatremic dehydration with petechiae and ecchymotic patches and necrotic skin lesions for 10–12 days was referred to dermatology department. On the general examination, pulse was 158/min, and respiratory rate was 52/min, and systemic examination was normal. Hematological investigations showed pancytopenia. Bleeding time was normal but prothrombin time and activated partial thromboplastin time reports were prolonged. D-dimer levels were elevated. Urine and stool were normal. Blood culture and sensitivity report revealed the growth of Citrobacter species with sensitivity to ciprofloxacin, amikacin, tetracycline, and resistance to Imipenem. Histopathology revealed epidermal hyperkeratosis with epidermal-dermal splitting, vessels showing fibrin occlusion with red blood cell extravasation into the perivascular areas in dermis along with dermal necrosis. To the best of our knowledge, this might be the first case of purpura fulminans in a neonate caused by rare Citrobacter species.

Study of acute renal insufficiency and chronic renal insufficiency using radioisotopes

International Nuclear Information System (INIS)

Raynaud, C.

1976-01-01

Radioisotopic renal function tests are of assistance to diagnose and follow-up the course of renal insufficiency. The radioisotopic renogram is useful in assessing the response to therapy of child obstructive uropathies and evaluating renal transplant function. The renal scan is helpful, in an emergency service, to differenciate chronic renal insufficiency from acute renal insufficiency. Hg renal uptake test provides informations on physiopathological problems. Among them, the following problems are emphasized: evolution of a nonfunctioning kidney, control of the success of a reparative surgery and of bilateral obstructive uropathies with unilateral symptoms [fr

[Addison's disease : Primary adrenal insufficiency].

Science.gov (United States)

Pulzer, A; Burger-Stritt, S; Hahner, S

2016-05-01

Adrenal insufficiency, a rare disorder which is characterized by the inadequate production or absence of adrenal hormones, may be classified as primary adrenal insufficiency in case of direct affection of the adrenal glands or secondary adrenal insufficiency, which is mostly due to pituitary or hypothalamic disease. Primary adrenal insufficiency affects 11 of 100,000 individuals. Clinical symptoms are mainly nonspecific and include fatigue, weight loss, and hypotension. The diagnostic test of choice is dynamic testing with synthetic ACTH. Patients suffering from chronic adrenal insufficiency require lifelong hormone supplementation. Education in dose adaption during physical and mental stress or emergency situations is essential to prevent life-threatening adrenal crises. Patients with adrenal insufficiency should carry an emergency card and emergency kit with them.

Pulmonary hyalinizing granuloma: Bilateral pulmonary nodules associated with chronic idiopathic thrombocytopenic purpura

International Nuclear Information System (INIS)

Satti, Mohamed B.; Batouk, Abdelnasir; Ahmad, Mohamed F.; Abdelaal, Mohamed A.; Abdelaziz, Muntasir M.

2005-01-01

We report a case of a 30-year-old female who had been treated periodically with steroids for idiopathic thrombocytopenic purpura ICTP over the last 10 years. Recently, during the course of investigation, she was found to have incidental asymptomatic multiple pulmonary nodules on chest CT. Following a needle biopsy to exclude malignancy, 2 nodules were excised and were histologically confirmed as pulmonary hyalinizing granuloma PHG. The remaining 2 nodules regressed on increasing her dose of steroids. The case is discussed with emphasis on the histological and radiological differential diagnosis, in addition to including ITP among the spectrum of immunologic conditions associated with PHG. (author)

Henoch-Schonlein purpura: ultrasonography of scrotal and penile involvement

Directory of Open Access Journals (Sweden)

Youngsik Lim

2015-04-01

Full Text Available Testicular or scrotal involvement has been reported in children with Henoch-Schonlein purpura (HSP, but there are very few reports on penile involvement. We report the initial and follow-up ultrasonographic findings of scrotal and penile involvement of HSP in a 5-year-old boy. On ultrasonography, scrotal soft tissue thickening and epididymal swelling with increased vascularity were noted, and on the penis, a focal mass-like lesion appeared on the dorsal surface of the distal penis, having a hypoechoic mass-like appearance without visible vascular flow on a Doppler study. After 2 days of treatment, follow-up ultrasonography showed normal scrotum and penis with a resolved soft tissue mass-like lesion. Therefore, we think that HSP ultrasonographic findings involving the scrotum and penis might help to diagnose scrotal and penile involvement in a case of HSP and to avoid unnecessary medication and/or surgical procedures.

Idiopathic combined, autoantibody-mediated ADAMTS-13/factor H deficiency in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome in a 17-year-old woman: a case report

Directory of Open Access Journals (Sweden)

Patschan Daniel

2011-12-01

Full Text Available Abstract Introduction Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome is a life-threatening condition with various etiopathogeneses. Without therapy approximately 90% of all patients die from the disease. Case presentation We report the case of a 17-year-old Caucasian woman with widespread hematomas and headache. Due to hemolytic anemia, thrombocytopenia, and schistocytosis, thrombotic thrombocytopenic purpura-hemolytic uremic syndrome was suspected and plasma exchange therapy was initiated immediately. Since her thrombocyte level did not increase during the first week of therapy, plasma treatment had to be intensified to a twice-daily schedule. Further diagnostics showed markedly reduced activities of both ADAMTS-13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 - also known as von Willebrand factor-cleaving protease and factor H. Test results for antibodies against both proteins were positive. While plasma exchange therapy was continued, rituximab was given once weekly for four consecutive weeks. After the last dose, thrombocytes and activities of ADAMTS-13 and factor H increased into the normal range. Our patient improved and was discharged from the hospital. Conclusions Since no clinical symptoms/laboratory findings indicated a malignant or specific autoimmune-mediated disorder, the diagnosis made was thrombotic thrombocytopenic purpura-hemolytic uremic syndrome due to idiopathic combined, autoantibody-mediated ADAMTS-13/factor H deficiency.

Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS): a 24-year clinical experience with 178 patients

OpenAIRE

Lara Primo; Harvey Danielle; Levandovsky Mark; Wun Ted

2008-01-01

Abstract Background Thrombotic thrombocytopenic purpura and the hemolytic uremic syndrome (TTP-HUS) are related and uncommon disorders with a high fatality and complication rate if untreated. Plasma exchange therapy has been shown to produce high response rates and improve survival in patients with many forms of TTP-HUS. We performed a retrospective cohort study of 178 consecutively treated patients with TTP-HUS and analyzed whether clinical or laboratory characteristics could predict for imp...

Partial splenic embolization combined with vincristine infusion for the treatment of refractory idiopathic thrombocytopenic purpura and Evans syndrome: observation of its long-term efficacy

International Nuclear Information System (INIS)

Chen Shibing

2011-01-01

Objective: To observe the long-term efficacy of partial spleen embolization combined with vincristine infusion in treating refractory idiopathic thrombocytopenic purpura (ITP) and Evans syndrome. Methods: During the period of 2000-2007, partial spleen embolization together with vincristine infusion was carried out in 30 patients with refractory idiopathic thrombocytopenic purpura (n=24) or Evans syndrome (n=6). Vincristine infusion (2 mg) via splenic artery was performed before partial spleen embolization procedure. The long-term effectiveness was observed and analyzed. Results: One week after the treatment, the platelet count was increased from preoperative (10.23±8.28) × 10 9 /L to (140.28±85.45) × 10 9 /L in patients with ITP, while the platelet count was increased from preoperative (12±8) × 10 9 /L to (210±60) × 10 9 /L in patients with Evans syndrome. Meanwhile, the hemoglobin level showed an increase in different degrees, from preoperative (63.00±13.62) g/L to postoperative (123.00±13.14) g/L. The therapeutic effectiveness was 100%. During the follow-up time lasting for 3-5 years, recurrence was seen in 11 patients (36.7%) and the overall efficacy rate was 63.3%. Conclusion: For the treatment of refractory idiopathic thrombocytopenic purpura and Evans syndrome, partial spleen embolization combined with vincristine infusion carries reliable long-term efficacy. (author)

[Henoch-Schönlein Purpura with lung abscess].

Science.gov (United States)

Nakazawa, Junji; Watanabe, Atsushi; Nakajima, Tomohiro; Mishina, Taijiro; Miyajima, Masahiro; Higami, Tetsuya

2013-09-01

A 72-year-old man had underwent left lower lobectomy for squamous cell carcinoma in our hospital in 2008. Postoperative stage was I A (T1N0M0). In 2010, follow-up chest computed tomography (CT) images showed similar cavitary nodules in segments 2 and 8 of the right lung with positive uptake on fluorodeoxyglucose-positron emission tomography (FDG-PET) images. Physical examination, blood tests, and levels of serum tumor markers showed no abnormality. Transbronchial lung biopsy revealed the absence of malignant cells. Segment 8 of the right lower lobe with the nodule was partially resected, and pathological examination demonstrated lung abscess. He was discharged but was hospitalized in another hospital for purpuric rash, fever, and arthralgia. Microscopic albuminuria was noted, and renal biopsy revealed nephritis with immunoglobulin A( IgA)deposition. He was made a diagnosis of Henoch-Schönlein purpura. Oral steroid therapy( prednisolone 60 mg/d) was initiated, resulting in the improvement of symptoms and disapearance of the cavitary nodule in the right lung segment 2.

Risk Factors Associated with Renal Involvement in Childhood Henoch-Schönlein Purpura: A Meta-Analysis

Science.gov (United States)

Chan, Han; Tang, Yan-Ling; Lv, Xiao-Hang; Zhang, Gao-Fu; Wang, Mo; Yang, Hai-Ping; Li, Qiu

2016-01-01

Background and objective Henoch-Schönlein purpura (HSP) is an important cause of chronic kidney disease in children. This meta-analysis identified risk factors associated with renal involvement in childhood HSP. Methods PubMed, Embase, and Web of Science were searched. The quality of all eligible studies was assessed using the Newcastle-Ottawa scale criteria. An analysis of possible risk factors was conducted to report the odds ratio (OR) and weighted mean difference (WMD). Results Thirteen studies (2398 children) revealed 20 possible and 13 significant risk factors associated with renal involvement in HSP, with the following meta-analysis estimates of OR and WMD, with 95% confidence intervals: older age (0.90, 0.61–1.19); age > 10 y (3.13, 1.39–7.07); male gender (1.36, 1.07–1.74); abdominal pain (1.94,1.24–3.04); gastrointestinal bleeding (1.86, 1.30–2.65); severe bowel angina (3.38, 1.17–9.80); persistent purpura (4.02, 1.22–13.25); relapse (4.70, 2.42–9.14); WBC > 15 × 109/L (2.42, 1.39–4.22); platelets > 500 × 109/L (2.98, 1.22–7.25); elevated antistreptolysin O (ASO) (2.17, 1.29–3.64); and decreased complement component 3 (C3) (3.13, 1.62–6.05). Factors not significantly associated with renal involvement were: blood pressure; orchitis; elevated C-reactive protein; elevated erythrocyte sedimentation rate (ESR); and elevated serum IgA/IgE or IgG. Arthritis/arthralgia may be a risk factor according to the criteria of the American College of Rheumatology (1.41, 1.01–1.96). Conclusion The following are associated with renal involvement in pediatric HSP: male gender; > 10 y old; severe gastrointestinal symptoms (abdominal pain, gastrointestinal bleeding, and severe bowel angina); arthritis/arthralgia; persistent purpura or relapse; WBC > 15 × 109/L; platelets > 500 × 109/L; elevated ASO; and low C3. Relevant clinical interventions for these risk factors may exert positive effects on the prevention of kidney disease during the early

ST-segment elevation myocardial infarction treated with thrombolytic therapy in a patient with thrombotic thrombocytopenic purpura.

Science.gov (United States)

Doll, Jacob A; Kelly, Jacob P

2014-07-01

Acute myocardial infarction is a common complication of thrombotic thrombocytopenic purpura (TTP), but rarely the presenting manifestation. Anti-thrombotic therapy for myocardial infarction is rarely utilized in the setting of TTP because of elevated bleeding risk. We report a case of TTP presenting with ST-segment elevation myocardial infarction and treated with thrombolytic therapy. The resultant cardiac and neurological complications highlight the challenges of using evidence-based therapy for myocardial infarction in the setting of TTP.

Pregnancy shortly after an acute episode of severe acquired thrombotic thrombocytopenic purpura.

Science.gov (United States)

Panaitescu, Anca M; Stoia, Razvan; Ciobanu, Anca M; Demetrian, Mihaela; Peltecu, Gheorghe

2016-12-01

Thrombotic thrombocytopenic purpura (TTP) is a rare but potentially fatal condition. In women with a previous history of TTP there is increased risk of recurrence during pregnancy and the puerperium. There is some evidence that the risk of relapse during pregnancy is increased if the interval between the event and conception is short. We present a case in which pregnancy was achieved a few days after full recovery from an acute episode of severe acquired TTP (ADAMTS13 activity <0.1%) which was successfully treated with four courses of plasma exchange. There was no relapse of TTP during pregnancy and a healthy baby was delivered at term; the puerperium was uneventful. Copyright © 2016 Elsevier Ltd. All rights reserved.

Clinical outcomes in children with Henoch-Schönlein purpura nephritis without crescents.

Science.gov (United States)

Delbet, Jean Daniel; Hogan, Julien; Aoun, Bilal; Stoica, Iulia; Salomon, Rémi; Decramer, Stéphane; Brocheriou, Isabelle; Deschênes, Georges; Ulinski, Tim

2017-07-01

Henoch-Schönlein purpura is the most common vasculitis in children. Its long-term prognosis depends on renal involvement. The management of Henoch-Schönlein purpura nephritis (HSPN) remains controversial. This study reports the prognosis of children with HSPN presenting with class 2 International Study of Kidney Disease in Children (ISKDC) nephritis. All children with HSPN class 2 diagnosed between 1995 and 2015 in four pediatric nephrology centers were included, and clinical and biological data were collected from the medical files. The primary endpoint was proteinuria remission defined as a proteinuria 3 g/L, 52% proteinuria between 1 and 3 g/L, and 18% proteinuria <1 g/L. Forty-seven percent of patients received orally treatment with steroids alone, 37% received methylprednisolone pulses followed by steroids orally, 18% received no steroids. Although 85% reached remission during follow-up, 12% did not maintain complete remission over time so that only 75% remained in complete remission by the end of the follow-up. Univariate analysis found a higher likelihood of remission in patients with higher proteinuria at disease onset (p = 0.009). This trend was not found in the multivariate analysis after adjusting for treatments, as patients with higher proteinuria were most often treated with steroids. Our study shows that one fourth of patients with HSPN class 2 remain proteinuric and thus carry the risk of developing chronic kidney disease over the long term. This finding, together with the better outcome of patients treated with steroids, is in favor of using high-dose steroids orally or IV in these patients.

Cocaine-associated retiform purpura: a C5b-9-mediated microangiopathy syndrome associated with enhanced apoptosis and high levels of intercellular adhesion molecule-1 expression.

Science.gov (United States)

Magro, Cynthia M; Wang, Xuan

2013-10-01

Cocaine-associated retiform purpura is a recently described entity characterized by striking hemorrhagic necrosis involving areas of skin associated with administration of cocaine. Levamisole, an adulterant in cocaine, has been suggested as the main culprit pathogenetically. Four cases of cocaine-associated retiform purpura were encountered in the dermatopathology practice of C. M. Magro. The light microscopic findings were correlated with immunohistochemical and immunofluorescence studies. All 4 cases showed a very striking thrombotic diathesis associated with intravascular macrophage accumulation. Necrotizing vasculitis was noted in 1 case. Striking intercellular adhesion molecule-1 (ICAM-1)/CD54 expression in vessel wall along with endothelial expression of caspase 3 and extensive vascular C5b-9 deposition was observed in all biopsies examined. Cocaine-induced retiform purpura is a C5b-9-mediated microvascular injury associated with enhanced apoptosis and prominent vascular expression of ICAM-1, all of which have been shown in prior in vitro and in vivo murine models to be a direct effect of cocaine metabolic products. Antineutrophilic cytoplasmic antibody and antiphospholipid antibodies are likely the direct sequelae of the proapoptotic microenvironment. The inflammatory vasculitic lesion could reflect the downstream end point reflective of enhanced ICAM-1 expression and the development of antineutrophilic cytoplasmic antibody. Levamisole likely works synergistically with cocaine in the propagation of this syndromic complex.

Diagnostic value of polymerase chain reaction analysis of skin biopsies in purpura fulminans.

Science.gov (United States)

Beau, Caroline; Vlassova, Natalia; Sarlangue, Jean; Brissaud, Olivier; Léauté-Labrèze, Christine; Boralevi, Franck

2013-01-01

Even though prompt diagnosis and treatment of purpura fulminans (PF) is essential to reduce mortality, early administration of antibiotics may preclude identification of the causative agent by standard bacterial cultures and thus render definitive diagnosis impossible. Here we present a case of an infant with PF and negative bacterial cultures for whom polymerase chain reaction (PCR) analysis of a cutaneous biopsy specimen obtained 4 days after initiation of antibiotics identified the genomic sequence of Neisseria meningitidis genogroup C. When bacterial cultures fail to provide useful information, PCR of skin biopsy specimens can be a valuable diagnostic tool in PF. © 2013 Wiley Periodicals, Inc.

Extension of the Calvert formula to patients with severe renal insufficiency.

Science.gov (United States)

Oguri, Tomoyo; Shimokata, Tomoya; Ito, Isao; Yasuda, Yoshinari; Sassa, Naoto; Nishiyama, Masami; Hamada, Akinobu; Hasegawa, Yoshinori; Ando, Yuichi

2015-07-01

The Calvert formula was derived from the study among patients with glomerular filtration rates (GFRs) of 33-135 ml/min, and it remains unclear whether the formula can be used to calculate optimal and safe dosages of carboplatin in patients with severe renal insufficiency. We evaluated the utility of this formula in patients with severe renal insufficiency. For pharmacokinetic analysis, we studied nine adult Japanese patients with advanced cancer who had an estimated GFR of lower than 30 ml/min/1.73 m(2), as calculated by the Japanese equation for estimating GFR, or who were receiving hemodialysis. The dose of carboplatin was calculated with the Calvert formula, in which GFR was measured by inulin clearance or was assumed to be 0 in patients requiring hemodialysis. Hemodialysis was started 23 h after the end of carboplatin infusion. Although there was a significant correlation between the estimated and measured carboplatin clearance, the estimated clearance was consistently higher than the measured clearance [mean prediction error ± standard deviation = 41.0 ± 26.3 %] in all seven patients with renal insufficiency (GFR, median 21.4, range 7.8-31.4 ml/min) and in the two hemodialysis patients. Actual areas under the concentration-time curve (AUC) (mg/ml min) were 5.4, 5.7, 6.2, and 9.0 for the four patients with a target AUC (mg/ml min) of 5; 5.7, 6.2, and 7.1 for the three patients with a target AUC (mg/ml min) of 4; and 5.1 and 8.7 for the two hemodialysis patients with a target AUC (mg/ml min) of 5. The measured clearance of carboplatin ranged from 23.0 to 51.3 ml/min in the seven patients not receiving hemodialysis. The pre-hemodialysis carboplatin clearance in the hemodialysis patients was 20.5 and 11.1 ml/min, respectively. For adult patients with severe renal insufficiency, the Calvert formula causes carboplatin overdosing by overestimating the carboplatin clearance.

Nocardia transvalensis Disseminated Infection in an Immunocompromised Patient with Idiopathic Thrombocytopenic Purpura

Directory of Open Access Journals (Sweden)

Jorge García-Méndez

2016-01-01

Full Text Available Nocardia transvalensis complex includes a wide range of microorganisms with specific antimicrobial resistance patterns. N. transvalensis is an unusual Nocardia species. However, it must be differentiated due to its natural resistance to aminoglycosides while other Nocardia species are susceptible. The present report describes a Nocardia species involved in an uncommon clinical case of a patient with idiopathic thrombocytopenic purpura and pulmonary nocardiosis. Microbiological and molecular techniques based on the sequencing of the 16S rRNA gene allowed diagnosis of Nocardia transvalensis sensu stricto. The successful treatment was based on trimethoprim-sulfamethoxazole and other drugs. We conclude that molecular identification of Nocardia species is a valuable technique to guide good treatment and prognosis and recommend its use for daily bases diagnosis.

EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria

DEFF Research Database (Denmark)

Ozen, Seza; Pistorio, Angela; Iusan, Silvia M

2010-01-01

To validate the previously proposed classification criteria for Henoch-Schönlein purpura (HSP), childhood polyarteritis nodosa (c-PAN), c-Wegener granulomatosis (c-WG) and c-Takayasu arteritis (c-TA)....

Bir olgu nedeniyle immün trombositopenik purpura ve çocuğun fiziksel istismarı

OpenAIRE

Çolak, Başar; Karakaya, Işık; Şentürk, Ekrem; Yanal, Servet; Şişmanlar, Şahika G.; Biçer, Ümit

2014-01-01

To the Editor Physical abuse of children can be diagnosed more easily compared to other cases of abuse On the other hand difficulties may be experienced in the diagnosis in cases where multidiciplinary evaluation is not made A 8 year old girl with immune thrombocytopenic purpura ITP who had ecchymoses on the body and a history of falling in the school was diagnosed as physical abuse as a result of evaluation by the departments of pediatric psychiatry and forensic medicine We decided to discus...

Preclinical assessment of a new recombinant ADAMTS-13 drug product (BAX930) for the treatment of thrombotic thrombocytopenic purpura.

Science.gov (United States)

Kopić, A; Benamara, K; Piskernik, C; Plaimauer, B; Horling, F; Höbarth, G; Ruthsatz, T; Dietrich, B; Muchitsch, E-M; Scheiflinger, F; Turecek, M; Höllriegl, W

2016-07-01

Essentials ADAMTS-13-deficiency is a cause of thrombotic thrombocytopenic purpura (TTP). Preclinical safety of recombinant human ADAMTS-13 (BAX930) was shown in animal models. Preclinical efficacy of BAX930 was shown in a mouse model of TTP. BAX930 showed advantageous efficacy over fresh frozen plasma, the current standard of care. Click to hear Dr Cataland and Prof. Lämmle present a seminar on Thrombotic Thrombocytopenic Purpura (TTP): new Insights in Pathogenesis and Treatment Modalities. Background Thrombotic thrombocytopenic purpura (TTP) is a rare blood disorder characterized by microthrombosis in small blood vessels of the body, resulting in a low platelet count. Baxalta has developed a new recombinant ADAMTS-13 (rADAMTS-13) product (BAX930) for on-demand and prophylactic treatment of patients with hereditary TTP (hTTP). Objectives To evaluate the pharmacokinetics, efficacy and safety of BAX930 in different species, by use of an extensive preclinical program. Methods The prophylactic and therapeutic efficacies of BAX930 were tested in a previously established TTP mouse model. Pharmacokinetics were evaluated after single intravenous bolus injection in mice and rats, and after repeated dosing in cynomolgus monkeys. Toxicity was assessed in rats and monkeys, safety pharmacology in monkeys, and local tolerance in rabbits. Results BAX930 was shown to be efficacious, as demonstrated by a stabilized platelet count in ADAMTS-13 knockout mice that were thrombocytopenic when treated. Prophylactic efficacy was dose-dependent and comparable with that achieved by treatment with fresh frozen plasma, the mainstay of hTTP treatment. Therapeutic efficacy was treatment interval-dependent. Safety pharmacology evaluation did not show any deleterious effects of BAX930 on cardiovascular and respiratory functions in monkeys. The compound's pharmacokinetics were similar and dose-proportional in mice, rats, and monkeys. BAX930 was well tolerated in rats, monkeys, and rabbits, even

[Henoch-Schönlein purpura in the adult].

Science.gov (United States)

Pillebout, E; Verine, J

2014-06-01

Henoch-Schönlein purpura is a systemic vasculitis of the small vessels characterized by perivascular leucocyte infiltrates. It is an immunoglobulin A-related immune complex-mediated disease involving the skin, the joints and the gastrointestinal system. Renal disease may sometimes be associated to these clinical manifestations. Prevalence of the nephritis is highly variable, depending on the series. More rarely, other organs such as the lungs, the heart or the nervous system may be involved. The clinical diagnosis is confirmed by histopathology of the skin (leukocytoclastic vasculitis) and kidney (endo-capillary proliferative glomerulonephritis), showing IgA deposits in these tissues. Short-term prognosis depends on the severity of digestive involvement, but long-term prognosis depends on the renal disease. Recent publications of pediatric and adult series show that the chronic renal failure may progress, sometimes more than ten years after the initial flare. Treatment is usually supportive. The benefit of more specific treatments (corticosteroids or immunosuppressive drugs) in severe visceral forms (usually abdominal or kidney) has not yet been established. Copyright © 2013 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.

Nonsteroidal Anti-inflammatory Drug Induced Thrombotic Thrombocytopenic Purpura

Directory of Open Access Journals (Sweden)

Karlos Z. Oregel

2013-01-01

Full Text Available A 21-year-old male presented to the emergency department after a 5-day history of recurrent vomiting and decreased urine output. History revealed ingestion of ibuprofen. During the diagnostic workup, the following was identified: white blood cell count 13.4 (x10(3/mcL, hemoglobin 11.9 (x10(6/mcL with an MCV of 73 fL, hematocrit 34% and platelets were 31,000/mcL, sodium of 130 mmol/L, potassium of 5.1 mmol/L, chloride of 83 mmol/L, bicarbonate of 21 mmol/L, blood urea nitrogen of 184 mg/dL and creatinine of 19.1 mg/dL. He was later diagnosed with thrombotic thrombocytopenic purpura (TTP based on the fact that he presented with most components of the TTP pentad (except for fever, which included altered mental status, acute kidney injury, thrombocytopenia, and evidence of red cell fragmentation and his ADAMTS13 level was found to be less than 10% prior to therapy. The patient then received plasma exchange, oral corticosteroids, and hemodialysis, which led to a full recovery of platelet count and renal function.

Itching Papules in a child; Scabies or Henoch Schonlein Purpura?

Directory of Open Access Journals (Sweden)

Zahra Soheili Rad

2015-12-01

Full Text Available Introduction: Henoch schonlein purpura (HSP is a self-limited systemic disease with multiorgan involvement. While cutaneous involvement may wary, presenting as itching papules is not a common manifestation. In this report we discuss a case of HSP which was presented solely with itching papules; mistaken for scabies. Case presentation: A 7 year old boy presented with itchy palpable papules without any gastrointestinal or urinary problem which was primary taught to be an ectoparasitic infestation. During the hospitalization the patient developed abdominal pain as well as hematuria and the diagnosis became more evident. The skin biopsy showed perivascular inflammatory cells infiltration in epidermis, perivascular fibrinoid necrosis and leukocytoclastic vasculitis. The patient received corticosteroids and discharged healthy.Conclusion: While HSP may have different cutaneous manifestation, presenting as only itchy papules may complicate the diagnosis and physicians should always keep in mind that HSP might be presented as other common infestations unresponsive to treatment.

Rapid improvement of Henoch-Schonlein purpura associated with the treatment of Helicobacter pylori infection

Directory of Open Access Journals (Sweden)

Turgay Ulas

2012-01-01

Full Text Available Helicobacter pylori (H. pylori are one of the most common bacterial infections, seen in humans, worldwide and their possible relationships to different diseases are a focus of attention nowadays. H. pylori may cause some extra intestinal manifestations some of which are dermatological conditions, including Henoch-Schönlein purpura (HSP, chronic urticaria and atopic dermatitis. We describe a 49-year-old man who presented with HSP triggered by gastric H. pylori infection. Treatment of H. Pylori infection was accompanied by prompt resolution of the gastrointestinal manifestations and purpuric rashes. These findings suggest a causative role for H. pylori in the occurrence of HSP.

Chinese herbal medicine for the treatment of Henoch-Schönlein purpura nephritis in children: A prospective cohort study protocol.

Science.gov (United States)

Zhang, Jun; Lv, Jing; Pang, Shuang; Bai, Xiaohong; Yuan, Fang; Wu, Yubin; Jiang, Hong; Yang, Guanqi; Zhang, Shaoqing

2018-06-01

Henoch-Schönlein purpura nephritis (HSPN) involves the renal impairment of Henoch-Schönlein purpura and can easily relapse into life-threatening late nephropathy in severe cases. Although there is a lack of validated evidence for its effectiveness, Chinese herbal medicine (CHM) is one of the most commonly used methods in China to treat HSPN. It is thus need to report the protocol of a prospective cohort trial using CHM to investigate the effectiveness, safety and advantages for children with HSPN. This large, prospective, multicenter cohort study started in May 2015 in Shenyang. Six hundred children diagnosed with HSPN were recruited from 3 institutions and are followed-up every 2 to 4 weeks till May 2020. Detailed information of participants includes general information, history of treatment, physical examination, and symptoms of TCM is taken face-to-face at baseline. This study has received ethical approval from the ethics committee of institutional review board of the Affiliated Hospital of Liaoning University of Traditional Chinese Medicine (No.2016CS(KT)-002-01). Articles summarizing the primary results and ancillary analyses will be published in peer-reviewed journals. Clinical Trials Registration: NCT02878018.

Caplacizumab reduces the frequency of major thromboembolic events, exacerbations and death in patients with acquired thrombotic thrombocytopenic purpura.

Science.gov (United States)

Peyvandi, F; Scully, M; Kremer Hovinga, J A; Knöbl, P; Cataland, S; De Beuf, K; Callewaert, F; De Winter, H; Zeldin, R K

2017-07-01

Essentials Acquired thrombotic thrombocytopenic purpura (aTTP) is linked with significant morbidity/mortality. Caplacizumab's effect on major thromboembolic (TE) events, exacerbations and death was studied. Fewer caplacizumab-treated patients had a major TE event, an exacerbation, or died versus placebo. Caplacizumab has the potential to reduce the acute morbidity and mortality associated with aTTP. Background Acquired thrombotic thrombocytopenic purpura (aTTP) is a life-threatening autoimmune thrombotic microangiopathy. In spite of treatment with plasma exchange and immunosuppression, patients remain at risk for thrombotic complications, exacerbations, and death. In the phase II TITAN study, treatment with caplacizumab, an anti-von Willebrand factor Nanobody ® was shown to reduce the time to confirmed platelet count normalization and exacerbations during treatment. Objective The clinical benefit of caplacizumab was further investigated in a post hoc analysis of the incidence of major thromboembolic events and exacerbations during the study drug treatment period and thrombotic thrombocytopenic purpura-related death during the study. Methods The Standardized Medical Dictionary for Regulatory Activities (MedDRA) Query (SMQ) for 'embolic and thrombotic events' was run to investigate the occurrence of major thromboembolic events and exacerbations in the safety population of the TITAN study, which consisted of 72 patients, of whom 35 received caplacizumab and 37 received placebo. Results Four events (one pulmonary embolism and three aTTP exacerbations) were reported in four patients in the caplacizumab group, and 20 such events were reported in 14 patients in the placebo group (two acute myocardial infarctions, one ischemic stroke, one hemorrhagic stroke, one pulmonary embolism, one deep vein thrombosis, one venous thrombosis, and 13 aTTP exacerbations). Two of the placebo-treated patients died from aTTP during the study. Conclusion In total, 11.4% of caplacizumab

A rare association of localized scleroderma type morphea, vitiligo, autoimmune hypothyroidism, pneumonitis, autoimmune thrombocytopenic purpura and central nervous system vasculitis. Case report.

Science.gov (United States)

Bonilla-Abadía, Fabio; Muñoz-Buitrón, Evelyn; Ochoa, Carlos D; Carrascal, Edwin; Cañas, Carlos A

2012-12-20

The localized scleroderma (LS) known as morphea, presents a variety of clinical manifestations that can include systemic involvement. Current classification schemes divide morphea into categories based solely on cutaneous morphology, without reference to systemic disease or autoimmune phenomena. This classification is likely incomplete. Autoimmune phenomena such as vitiligo and Hashimoto thyroiditis associated with LS have been reported in some cases suggesting an autoimmune basis. To our knowledge this is the first case of a morphea forming part of a multiple autoimmune syndrome (MAS) and presenting simultaneously with autoimmune thrombocytopenic purpura and central nervous system vasculitis. We report an uncommon case of a white 53 year old female patient with LS as part of a multiple autoimmune syndrome associated with pneumonitis, autoimmune thrombocytopenic purpura and central nervous system vasculitis presenting a favorable response with thrombopoietin receptor agonists, pulses of methylprednisolone and cyclophosphamide. Is likely that LS have an autoimmune origin and in this case becomes part of MAS, which consist on the presence of three or more well-defined autoimmune diseases in a single patient.

A rare association of localized scleroderma type morphea, vitiligo, autoimmune hypothyroidism, pneumonitis, autoimmune thrombocytopenic purpura and central nervous system vasculitis. Case report

Directory of Open Access Journals (Sweden)

Bonilla-Abadía Fabio

2012-12-01

Full Text Available Abstract Background The localized scleroderma (LS known as morphea, presents a variety of clinical manifestations that can include systemic involvement. Current classification schemes divide morphea into categories based solely on cutaneous morphology, without reference to systemic disease or autoimmune phenomena. This classification is likely incomplete. Autoimmune phenomena such as vitiligo and Hashimoto thyroiditis associated with LS have been reported in some cases suggesting an autoimmune basis. To our knowledge this is the first case of a morphea forming part of a multiple autoimmune syndrome (MAS and presenting simultaneously with autoimmune thrombocytopenic purpura and central nervous system vasculitis. Case presentation We report an uncommon case of a white 53 year old female patient with LS as part of a multiple autoimmune syndrome associated with pneumonitis, autoimmune thrombocytopenic purpura and central nervous system vasculitis presenting a favorable response with thrombopoietin receptor agonists, pulses of methylprednisolone and cyclophosphamide. Conclusion Is likely that LS have an autoimmune origin and in this case becomes part of MAS, which consist on the presence of three or more well-defined autoimmune diseases in a single patient.

Insufficiency fracture after radiation therapy

Energy Technology Data Exchange (ETDEWEB)

Oh, Dong Ryul; Huh, Seung Jae [Dept.of Radiation Oncology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul (Korea, Republic of)

2014-12-15

Insufficiency fracture occurs when normal or physiological stress applied to weakened bone with demineralization and decreased elastic resistance. Recently, many studies reported the development of IF after radiation therapy (RT) in gynecological cancer, prostate cancer, anal cancer and rectal cancer. The RT-induced insufficiency fracture is a common complication during the follow-up using modern imaging studies. The clinical suspicion and knowledge the characteristic imaging patterns of insufficiency fracture is essential to differentiate it from metastatic bone lesions, because it sometimes cause severe pain, and it may be confused with bone metastasis.

Bilateral large subconjunctival haemorrhages unmasking immune thrombocytopenic purpura during retinopathy of prematurity screening.

Science.gov (United States)

Chandra, Parijat; Kumawat, Devesh; Kumar, Vinod; Tewari, Ruchir

2017-10-04

Although thrombocytopenia is known to be associated with pathogenesis of retinopathy of prematurity (ROP), immune thrombocytopenic purpura (ITP) is rare in infancy and not reported to occur with ROP. A preterm infant with aggressive posterior ROP developed bilateral massive subconjunctival haemorrhage after scleral indentation during screening. On evaluation, the infant was found to have severe ITP. Following intravenous transfusion of platelets and immunoglobulin, platelet counts improved and subconjunctival haemorrhage resolved over time. This case highlights the unusual presentation of ITP and also discusses the association of thrombocytopenia with ROP. Ophthalmologists should get prompt haematological work-up of such occurrences. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

Insufficient control of heart rate in stable coronary artery disease patients in Latvia

Directory of Open Access Journals (Sweden)

Inga Balode

2014-01-01

Conclusions: Despite the wide use of beta-blockers, HR is insufficiently controlled in the analyzed sample of stable CAD patients in Latvia. Target HR ≤60 bpm is achieved only in 25% of the patients while more than one third have increased HR ≥70 bpm.

[Treatment of chronic refractory idiopathic thrombocytopenia purpura. 10 years experience at the Salvador Zubiran National Institute of Nutrition].

Science.gov (United States)

Pita-Ramírez, L; Hurtado-Monroy, R; Labardini-Méndez, J

1992-01-01

A total of 126 patients with chronic idiopathic thrombocytopenic purpura were diagnosed from January 1980 to January 1990 in our institute. In this group of patients, 21 were refractory to prednisone therapy, splenectomy or both, or had had a relapse after a good response with these treatments. They were given other therapies. There was enough information for evaluation in 16 of the 21 patients. The treatment responses were classified according to the post-therapy platelet counts: complete response (CR) = > 150 x 10(9)/L for more than three months; partial response (PR) = 50-150 x 10(9)/L for more than three months; any response (AR) = CR + PR; no response (NR) = < 50 x 10(9)/L. There were 15 women and one male. The median age was 41 years (range 11 to 65). 6-mercaptopurine was given in all patients with CR = 31.2%, PR = 18.8%, AR = 50% and NR = 50%. Seven patients received cyclophosphamide with CR = 28.6%, PR = 14.3%, AR = 42.9% and NR = 57%. Vincristine was given in four patients with only one PR. Interferon alpha 2B was given in four patients with two transitory PR. One patient received colchicine and vitamin C without response. It is concluded that 6-mercaptopurine and cyclophosphamide are useful drugs in refractory thrombocytopenic purpura.

Optimal glucocorticoid replacement in adrenal insufficiency.

Science.gov (United States)

Øksnes, Marianne; Ross, Richard; Løvås, Kristian

2015-01-01

Adrenal insufficiency (glucocorticoid deficiency) comprises a group of rare diseases, including primary adrenal insufficiency, secondary adrenal insufficiency and congenital adrenal hyperplasia. Lifesaving glucocorticoid therapy was introduced over 60 years ago, but since then a number of advances in treatment have taken place. Specifically, little is known about short- and long-term treatment effects, and morbidity and mortality. Over the past decade, systematic cohort and registry studies have described reduced health-related quality of life, an unfavourable metabolic profile and increased mortality in patients with adrenal insufficiency, which may relate to unphysiological glucocorticoid replacement. This has led to the development of new modes of replacement that aim to mimic normal glucocorticoid physiology. Here, evidence for the inadequacy of conventional glucocorticoid therapy and recent developments in treatment are reviewed, with an emphasis on primary adrenal insufficiency. Copyright © 2014 Elsevier Ltd. All rights reserved.

Immunochip analysis identifies novel susceptibility loci in the human leukocyte antigen region for acquired thrombotic thrombocytopenic purpura.

Science.gov (United States)

Mancini, I; Ricaño-Ponce, I; Pappalardo, E; Cairo, A; Gorski, M M; Casoli, G; Ferrari, B; Alberti, M; Mikovic, D; Noris, M; Wijmenga, C; Peyvandi, F

2016-12-01

Essentials Genetic predisposition to acquired thrombotic thrombocytopenic purpura (aTTP) is mainly unknown. Genetic risk factors for aTTP were studied by Immunochip analysis and replication study. Human leukocyte antigen (HLA) variant rs6903608 conferred a 2.5-fold higher risk of developing aTTP. rs6903608 and HLA-DQB1*05:03 may explain most of the HLA association signal in aTTP. Click to hear Dr Cataland's presentation on acquired thrombotic thrombocytopenic purpura SUMMARY: Background Acquired thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening thrombotic microangiopathy associated with the development of autoantibodies against the von Willebrand factor-cleaving protease ADAMTS-13. Similarly to what has been found for other autoimmune disorders, there is evidence of a genetic contribution, including the association of the human leukocyte antigen (HLA) class II complex with disease risk. Objective To identify novel genetic risk factors in acquired TTP. Patients/Methods We undertook a case-control genetic association study in 190 European-origin TTP patients and 1255 Italian healthy controls by using the Illumina Immunochip. Replication analysis in 88 Italian cases and 456 controls was performed with single-nucleotide polymorphism (SNP) TaqMan assays. Results and conclusion We identified one common variant (rs6903608) located within the HLA class II locus that was independently associated with acquired TTP at genome-wide significance and conferred a 2.6-fold increased risk of developing a TTP episode (95% confidence interval [CI] 2.02-3.27, P = 1.64 × 10 -14 ). We also found five non-HLA variants mapping to chromosomes 2, 6, 8 and X that were suggestively associated with the disease: rs9490550, rs115265285, rs5927472, rs7823314, and rs1334768 (nominal P-values ranging from 1.59 × 10 -5 to 7.60 × 10 -5 ). Replication analysis confirmed the association of HLA variant rs6903608 with acquired TTP (pooled P = 3.95 × 10 -19 ). Imputation of classic

High Prevalence of Sinusitis in Children with Henoch-Schönlein Purpura

Directory of Open Access Journals (Sweden)

Haruna Nakaseko

2011-01-01

Full Text Available We evaluated the prevalence and the types of infectious foci in oral as well as ear, nose, and throat diseases, and we examined incidence of renal involvement with active treatment for focal infection in children with Henoch-Schönlein Purpura. A total of 96 children who presented at Aichi Children's Health and Medical Center and were diagnosed as having HSP were evaluated for infectious foci in the ear, nose, throat, and oral cavities. Seventy-one of 96 children (74.0% had some type of infectious lesion, such as sinusitis or tonsillitis, and the prevalence of sinusitis was the highest (51 cases, 53.7%. In 44 HSP patients without renal involvement at the first examination, the incidence of nephritis was lower (13.6% than in previous reports (17–54% due to our aggressive intervention for infectious foci.

Henoch–Schönlein purpura: a clinical case with dramatic presentation

Science.gov (United States)

Bento, João; Magalhães, Adriana; Moura, Conceição Souto; Hespanhol, Venceslau

2010-01-01

A case study involving a 55-year-old Caucasian male with end-stage glomerulosclerosis is presented here. Kidney biopsies showed no deposits on imunofluorescent microscopy. Relapsing massive haemoptysis and suspected bronchovascular malformation required lung lobectomy which revealed malformative and tortuous small blood vessels, with no vasculitis. Blood antinuclear antibodies, antineutrophil cytoplasmic antibodies and antiglomerular basement membrane antibodies were undetectable. Seric immunoglobulins and complement levels were normal. Three months later, arthralgia and joint oedema first appeared. Skin biopsy revealed vasculitis immune-reactive to immunoglobulin A. Systemic corticotherapy was then started. Two weeks later, the patient presented with abdominal pain melena and rectal bleeding (haematoquesia). Endoscopic study showed diffuse gastrointestinal haemorrhage. Angiographic study revealed diffuse lesions compatible with vasculitis and haemorrhage from multiple spots. Cyclophosphamide and then intravenous immunoglobulin were associated to treatment without response. Increasing blood loss occurred, with massive gastrointestinal haemorrhage and haemorrhagic ascitis. Death occurred due to uncontrolled diffuse bleeding. Necropsy findings showed generalised small vessels vasculitis compatible with Henoch–Schönlein purpura. PMID:22778211

Severe Henoch-Schönlein purpura with infliximab for ulcerative colitis.

Science.gov (United States)

Song, Yang; Shi, Yan-Hong; He, Chong; Liu, Chang-Qin; Wang, Jun-Shan; Zhao, Yu-Jie; Guo, Yan-Min; Wu, Rui-Jin; Feng, Xiao-Yue; Liu, Zhan-Ju

2015-05-21

Infliximab (IFX) is an anti-tumor necrosis factor chimeric antibody that is effective for treatment of autoimmune disorders such as Crohn's disease and ulcerative colitis (UC). IFX is well tolerated with a low incidence of adverse effects such as infections, skin reactions, autoimmunity, and malignancy. Dermatological manifestations can appear as infusion reaction, vasculitis, cutaneous infections, psoriasis, eczema, and skin cancer. Here, we present an unusual case of extensive and sporadic subcutaneous ecchymosis in a 69-year-old woman with severe UC, partial colectomy and cecostomy, following her initial dose of IFX. The reaction occurred during infliximab infusion, and withdrawal of IFX led to gradual alleviation of her symptoms. We concluded that Henoch-Schönlein purpura, a kind of leukocytoclastic vasculitis, might have contributed to the development of the bruising. Although the precise mechanisms of the vasculitis are still controversial, such a case highlights the importance of subcutaneous adverse effects in the management of UC with IFX.

Stroke in thrombotic thrombocytopenic purpura induced by thyrotoxicosis: a case report.

Science.gov (United States)

Bellante, Flavio; Redondo Saez, Patricia; Springael, Cecile; Dethy, Sophie

2014-07-01

Thrombotic thrombocytopenic purpura (TTP) is a hematologic disease involving the platelet aggregation and resulting in hemolytic anemia, thrombocytopenia, and microvascular occlusion. Although frequent neurologic features are headache and confusion, focal deficit is described in 30% of the cases. There are a lot of causes inducing thrombotic thrombocytopenic, but reports are lacking when associated with Grave disease. We describe the case of a 51-year-old Caucasian woman presenting a 24-hour story of sudden onset of dysarthria and left superior limb palsy. Four months before, she developed severe hyperthyroidism associated with petechiae, hemolytic anemia, thrombocytopenia, and schistocytes at blood film examination. Relapse of TTP in association with Grave disease was diagnosed. There are few reports describing association between Grave disease and TTP with only mild neurologic involvement. We described, to our knowledge, the first case of acute ischemic stroke secondary to thrombotic thrombocytopenic induced by thyrotoxicosis. Copyright © 2014 National Stroke Association. Published by Elsevier Inc. All rights reserved.

Food insufficiency is associated with high-risk sexual behavior among women in Botswana and Swaziland.

Directory of Open Access Journals (Sweden)

Sheri D Weiser

2007-10-01

Full Text Available Both food insufficiency and HIV infection are major public health problems in sub-Saharan Africa, yet the impact of food insufficiency on HIV risk behavior has not been systematically investigated. We tested the hypothesis that food insufficiency is associated with HIV transmission behavior.We studied the association between food insufficiency (not having enough food to eat over the previous 12 months and inconsistent condom use, sex exchange, and other measures of risky sex in a cross-sectional population-based study of 1,255 adults in Botswana and 796 adults in Swaziland using a stratified two-stage probability design. Associations were examined using multivariable logistic regression analyses, clustered by country and stratified by gender. Food insufficiency was reported by 32% of women and 22% of men over the previous 12 months. Among 1,050 women in both countries, after controlling for respondent characteristics including income and education, HIV knowledge, and alcohol use, food insufficiency was associated with inconsistent condom use with a nonprimary partner (adjusted odds ratio [AOR] 1.73, 95% confidence interval [CI] 1.27-2.36, sex exchange (AOR 1.84, 95% CI 1.74-1.93, intergenerational sexual relationships (AOR 1.46, 95% CI 1.03-2.08, and lack of control in sexual relationships (AOR 1.68, 95% CI 1.24-2.28. Associations between food insufficiency and risky sex were much attenuated among men.Food insufficiency is an important risk factor for increased sexual risk-taking among women in Botswana and Swaziland. Targeted food assistance and income generation programs in conjunction with efforts to enhance women's legal and social rights may play an important role in decreasing HIV transmission risk for women.

An adult patient with Henoch-Schönlein purpura and non-occlusive mesenteric ischemia

Science.gov (United States)

2013-01-01

Background Onset of Henoch-Schönlein purpura (HSP) in middle age is uncommon, and adults with renal or gastrointestinal involvement present with more severe disease than do similar pediatric patients. Case presentation We present the case of a 69-year-old male with HSP who, after treatment with steroids, cyclophosphamide, and continuous intravenous prostaglandin E1 (PGE1), died as a result of severe gastrointestinal involvement with non-occlusive mesenteric ischemia (NOMI). Vascular narrowing associated with the NOMI improved after catheter injection of PGE1 and prednisolone, but the patient died of bleeding from an exposed small vessel. At autopsy there was no active vasculitis in the jejunal submucosa. Conclusion Treatment with PGE1 and prednisolone might improve small-vessel vasculitis associated with NOMI. PMID:23343144

Cervical cerclage placement decreases local levels of proinflammatory cytokines in patients with cervical insufficiency.

Science.gov (United States)

Monsanto, Stephany P; Daher, Silvia; Ono, Erika; Pendeloski, Karen Priscilla Tezotto; Trainá, Évelyn; Mattar, Rosiane; Tayade, Chandrakant

2017-10-01

Cervical insufficiency is characterized by premature, progressive dilation and shortening of the cervix during pregnancy. If left unattended, this can lead to the prolapse and rupture of the amniotic membrane, which usually results in midtrimester pregnancy loss or preterm birth. Previous studies have shown that proinflammatory cytokines such as interleukin-1β, interleukin-6, interleukin-8, and tumor necrosis factor alpha are up-regulated in normal parturition but are also associated with preterm birth. Studies evaluating such markers in patients with cervical insufficiency have evaluated only their diagnostic potential. Even fewer studies have studied them within the context of cerclage surgery. The objective of the study was to evaluate the impact of local and systemic inflammatory markers on the pathogenesis of cervical insufficiency and the effect of cerclage surgery on the local immune microenvironment of women with cervical insufficiency. We recruited 28 pregnant women (12-20 weeks' gestation) diagnosed with insufficiency and referred for cerclage surgery and 19 gestational age-matched normal pregnant women as controls. Serum and cervicovaginal fluid samples were collected before and after cerclage surgery and during a routine checkup for normal women and analyzed using a targeted 13-plex proinflammatory cytokine assay. Before surgery, patients with cervical insufficiency had higher levels of interleukin-1β, interleukin-6, interleukin-12, monocyte chemoattractant protein-1 and tumor necrosis factor alpha in cervicovaginal fluid compared to controls, but after surgery, these differences disappeared. No differences were found in serum of insufficiency versus control women. In patients with insufficiency, the levels of interleukin-1β, interleukin-6, interleukin-8, monocyte chemoattractant protein-1, and interferon gamma in cervicovaginal fluid declined significantly after cerclage compared with before intervention, but these changes were not detected in serum

Response to rituximab in a refractory case of thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus

Directory of Open Access Journals (Sweden)

Niaz Faraz

2010-01-01

Full Text Available Thrombotic thrombocytopenic purpura (TTP is a serious disorder with a significant morbidity and mortality. Majority of cases of TTP are idiopathic, but some cases may be secon-dary to connective tissue diseases. TTP has been rarely associated with systemic lupus erythe-matosus (SLE and may be refractory to treatment with plasma exchange, requiring immuno-suppressive therapy. We describe a patient with TTP and SLE who was refractory to plasma exchange and corticosteroids but responded to anti-CD20 antibody rituximab with continued re-mission after eight months of follow-up. Rituximab appears to be an effective treatment in re-fractory cases of TTP associated with SLE.

A report of three cases with thrombotic thrombocytopenic purpura (TTP) secondary to an occult gastric adenocarcinoma

International Nuclear Information System (INIS)

Forat, Y.M.; Hashemian, Z.; Nazmieh, H.; Ghadimi, H.R.

2009-01-01

Thrombotic thrombocytopenic purpura (TTP) is a disseminated form of thrombotic microangiopathy with clinical findings consisting of fever, microangiopathic hemolytic anemia (MAHA), thrombocytopenia, fluctuating neurologic impairment and renal dysfunction. However, Microangiopathic hemolytic anemia has been described in association with disseminated malignancies, most commonly adenocarcinoma of the breast or stomach. We present three patients with microangiopathic anemia in whom metastatic cancer was finally diagnosed; however, they died of refractory hemolytic anemia in the end. The occurrence of microangiopathic hemolytic anemia and thrombocytopenia in patients with disseminated malignant in gastric adenocarsinoma is well documented. Therefore, the diagnosis of tumor-associated TTP should be considered in unresponsive TTP patient treated with plasmapheresis. (author)

Identification of the first homozygous 1-bp deletion in GDF9 gene leading to primary ovarian insufficiency by using targeted massively parallel sequencing.

Science.gov (United States)

França, M M; Funari, M F A; Nishi, M Y; Narcizo, A M; Domenice, S; Costa, E M F; Lerario, A M; Mendonca, B B

2018-02-01

Targeted massively parallel sequencing (TMPS) has been used in genetic diagnosis for Mendelian disorders. In the past few years, the TMPS has identified new and already described genes associated with primary ovarian insufficiency (POI) phenotype. Here, we performed a targeted gene sequencing to find a genetic diagnosis in idiopathic cases of Brazilian POI cohort. A custom SureSelect XT DNA target enrichment panel was designed and the sequencing was performed on Illumina NextSeq sequencer. We identified 1 homozygous 1-bp deletion variant (c.783delC) in the GDF9 gene in 1 patient with POI. The variant was confirmed and segregated using Sanger sequencing. The c.783delC GDF9 variant changed an amino acid creating a premature termination codon (p.Ser262Hisfs*2). This variant was not present in all public databases (ExAC/gnomAD, NHLBI/EVS and 1000Genomes). Moreover, it was absent in 400 alleles from fertile Brazilian women screened by Sanger sequencing. The patient's mother and her unaffected sister carried the c.783delC variant in a heterozygous state, as expected for an autosomal recessive inheritance. Here, the TMPS identified the first homozygous 1-bp deletion variant in GDF9. This finding reveals a novel inheritance pattern of pathogenic variant in GDF9 associated with POI, thus improving the genetic diagnosis of this disorder. © 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Glottal insufficiency with aspiration risk in dysphagia.

Science.gov (United States)

Giraldez-Rodriguez, Laureano A; Johns, Michael

2013-12-01

Glottal closure is an important part of the mechanism that protects the airway during the normal swallow. Glottal insufficiency disrupts glottal closure and therefore puts patients at risk of aspiration. Treatment of glottal insufficiency can be classified as surgical or nonsurgical. The objective of treating glottal insufficiency is to avoid aspiration or penetration of secretions or food into the airway. Nonsurgical treatment consists of swallowing maneuvers and other measures. Surgical treatment of glottal insufficiency includes injection laryngoplasty, medialization thyroplasty with or without arytenoid adduction or with arytenopexy and cricothyroid subluxation, hypopharyngoplasty, cricopharynx muscle dilation, and cricopharynx myotomy. Copyright © 2013 Elsevier Inc. All rights reserved.

Life after acquired thrombotic thrombocytopenic purpura: morbidity, mortality, and risks during pregnancy.

Science.gov (United States)

Vesely, S K

2015-06-01

Patients who have recovered from their acute episode of acquired ADAMTS13-deficient thrombotic thrombocytopenic purpura (TTP) were once thought to have complete recovery except for risk of relapse. Data from previous publications from the Oklahoma TTP-hemolytic uremic syndrome (HUS) Registry are summarized. Patients have decreased cognitive function and increased prevalence of hypertension, systemic lupus erythematosus, major depression, and albuminuria as compared to the expected values from the US population. The proportion of patients that died during the follow-up period was greater than expected based on the US population reference population. Among women who had a pregnancy following recovery from TTP, relapse during pregnancy or postpartum is uncommon, but the occurrence of preeclampsia may be increased. Thirteen of 16 pregnancies in these women resulted in healthy children. Increased morbidity and mortality in TTP patients following recovery suggest that TTP may be more of a chronic disorder than a disorder with acute episodes and complete recovery. © 2015 International Society on Thrombosis and Haemostasis.

IgA Nephropathy and Henoch-Schoenlein Purpura Nephritis: Aberrant Glycosylation of IgA1, Formation of IgA1-Containing Immune Complexes, and Activation of Mesangial Cells

DEFF Research Database (Denmark)

Novak, J.; Moldoveanu, Z.; Renfrow, M.B.

2007-01-01

IgA1 in the circulation and glomerular deposits of patients with IgA nephropathy (IgAN) is aberrantly glycosylated; the hinge-region O-linked glycans are galactose-deficient. The circulating IgA1 of patients with Henoch-Schoenlein purpura nephritis (HSPN) has a similar defect. This aberrancy...

Should we Investigate Gastroenterology Patients for Pancreatic Exocrine Insufficiency? A Dual Centre UK Study.

Science.gov (United States)

Campbell, Jennifer A; Sanders, David S; Francis, Katherine A; Kurien, Matthew; Lee, Sai; Taha, Hatim; Ramadas, Arvind; Joy, Diamond; Hopper, Andrew D

2016-09-01

Pancreatic exocrine insufficiency may be under recognised in gastroenterological practice. We aimed to identify the prevalence of pancreatic insufficiency in secondary care gastroenterology clinics and determine if co-morbidity or presenting symptoms could predict diagnosis. A secondary aim was to assess response to treatment. A dual centre retrospective analysis was conducted in secondary care gastroenterology clinics. Patients tested for pancreatic exocrine insufficiency with faecal elastase-1 (FEL-1) between 2009 and 2013 were identified in two centres. Demographics, indication and co-morbidities were recorded in addition to dose and response to pancreatic enzyme replacement therapy. Binary logistic regression was used to assess if symptoms or co-morbidities could predict pancreatic insufficiency. 1821 patients were tested, 13.1% had low FEL-1 (<200µg/g). This prevalence was sub-analysed with 5.4% having FEL-1 100-200µg/g (mild insufficiency) and 7.6% having faecal elastase readings <100µg/g. Low FEL-1 was most significantly associated with weight loss or steatorrhoea. Co-morbidity analysis showed that low levels were significantly associated with excess alcohol intake, diabetes mellitus or human immunodeficiency virus; 80.0% treated with enzyme supplements reported symptomatic benefit with no difference in response between high and low dose supplementation (p=0.761). Targeting the use of FEL-1 in individuals with specific symptoms and associated conditions can lead to improved recognition of pancreatic exocrine insufficiency in a significant proportion of secondary care patients. Intervening with lifestyle advice such as smoking cessation and minimising alcohol intake could improve outcomes. In addition, up to 80% of patients with low faecal elastase respond to supplementation.

Splenic dynamics of indium-111 labeled platelets in idiopathic thrombocytopenic purpura

International Nuclear Information System (INIS)

Syrjaelae, M.T.Sa.; Savolainen, S.; Nieminen, U.; Gripenberg, J.; Liewendahl, K.; Ikkala, E.

1989-01-01

Splenic dynamics of 111 In-labeled platelets and platelet-associated IgG in 33 patients with idiopathic thrombocytopenic purpura (ITP) were studied. Two half-lives were calculated for the biexponential splenic time-activity curve after i.v. injection of 111 In-labeled platelets. There was no difference in the mean half-life of the rapid component of the splenic curve (ST1) when patients with negative or slightly positive platelet suspension immunofluorescence test (PSIFT) were compared to those with strongly positive PSIFT (3.0 ± 0.7 min vs. 3.6 ± 0.4, p greater than 0.05). Mean half-life of the slow component of the splenic curve (ST2) was found to be longer in patients with a strongly positive than a negative or weakly positive PSIFT (26 ± 5 min vs. 13.2 ± 1.0 min, p less than 0.01). It seems that determination of the two components of the splenic time-activity curve provides a useful method for studying platelet kinetics in ITP

Two cases of eczematid-like purpura of Doucas and Kapetanakis responsive to narrow band ultraviolet B treatment.

Science.gov (United States)

Karadag, Ayse Serap; Bilgili, Serap Gunes; Onder, Sevda; Calka, Omer

2013-04-01

Eczematid-like purpura of Doucas and Kapetanakis is a type of pigmented purpuric dermatoses (PPDs) with eczematous changes in the purpuric surface. A 10-year-old male and a 44-year-old male patients were admitted to our clinics for itching and flaking of the skin rashes. Based on the clinical and histopathological evaluations, the rashes were identified as eczematid-like PPDs of Doucas and Kapetanakis. Both patients were treated with narrow band ultraviolet B. The lesions were remarkably regressed following the treatment. These cases reported due its rarity and good response to narrow band ultraviolet B. © 2013 John Wiley & Sons A/S.

Thrombotic thrombocytopenic purpura and deep vein thrombosis as the presenting manifestations of systemic lupus erythematosus: A case report and review of literature

Directory of Open Access Journals (Sweden)

Mohammad AH Mashhadi

2011-01-01

Full Text Available Systemic lupus erythematosus (SLE, is sometimes complicated by the rare fatal syndrome, Thrombotic thrombocyto-penic purpura (TTP, but the occurrence of TTP as the initial manifestation of SLE is very rare. Since they have similar-ities in some features, the differentiation of TTP from SLE may be missed. SLE patients are also more prone to throm-botic events. Here we report a case with TTP and deep vein thrombosis as the presenting symptoms of SLE.

Thrombocytosis following splenectomy and aortic valve replacement for idiopathic thrombocytopaenic purpura with bicuspid aortic valve

Directory of Open Access Journals (Sweden)

Sarika Katiyar

2015-01-01

Full Text Available Idiopathic thrombocytopaenic purpura (ITP patients are at high risk for complications during and after cardiac surgeries involving cardiopulmonary bypass. The main clinical problem of primary ITP is an increased risk of bleeding although bleeding may not always be present. More recently, thrombosis has become appreciated as another potential complication of the procedure. We report a 22-year-old female patient with ITP with bicuspid aortic valve and splenomegaly, who underwent uncomplicated aortic valve replacement and splenectomy simultaneously. She was readmitted with chest pain due to coronary thrombosis following splenectomy which made the management difficult. We describe our experience in managing this patient who presented with thrombotic complication rather than bleeding in post-operative period and the challenges met in maintaining appropriate anticoagulation for aortic valve replacement as well as thrombosis, post-splenectomy

[Variety of thrombotic thrombocytopenic purpura clinical course in Polish family members with ADAMTS 13 gene mutation].

Science.gov (United States)

Hyla-Klekot, Lidia; Kucharska, Grazyna; Słonka, Karina

2013-03-01

The congenital form of thrombotic thrombocytopenic purpura (Upshaw-Schulman syndrom) is a result of genetically conditioned dysfunction of protease ADAMTS 13 enzyme which is responsible for von Wiellebrand factor multimer disintegration. The disease is inherited autosomally and recessively. The decrease of ADAMTS 13 activity results in intravascular clotting process activation with rapid lowering of platelet count, haemolytic anaemia, and occurence of schistocytes. Clinically, the disease is characterized by a range of symptoms such as severe jaundice in neonatal period, embolicthrombotic incidents of nervous system and progressive dysfunction of kidneys and other organs. Delaying diagnosis and hence administering of freshly frozen plasma leads to death. Molecular diagnosis allows for identification of genetical profile of the patient, and showing lowered enzyme activity is a basis for regular prophylactic plasma administration which is the protease donor. In our study we present members of a Polish family identified with ADAMTS 13 mutation. 52 old male with heterozygotic mutation of exon 29 (4143_4144insA) and in exon 19 (c2281G>A; Gly761Ser), experienced a few episodes of ischaemic stroke with ongoing neurological deficiency and developed chronic kidney disease. His 16-year old daughter with double homozygotic mutation in exon 29 (4143_4144insA) after severe episode of TTP at the age of 4 has been receiving plasma every 2 weeks for 12 years, which prevented her from other disorders. Target treatment introduced to clinical practice by means of ADAMTS 13 obtained by genetic recombination technology raises hopes.

[Clinical investigation and mutation analysis of a child with citrin deficiency complicated with purpura, convulsive seizures and methioninemia].

Science.gov (United States)

Wen, Peng-qiang; Wang, Guo-bing; Chen, Zhan-ling; Liu, Xiao-hong; Cui, Dong; Shang, Yue; Li, Cheng-rong

2013-12-01

To analyze the clinical features and SLC25A13 gene mutations of a child with citrin deficiency complicated with purpura, convulsive seizures and methioninemia. The patient was subjected to physical examination and routine laboratory tests. Blood amino acids and acylcarnitines, and urine organic acids and galactose were analyzed respectively with tandem mass spectrometry and gas chromatographic mass spectrometry. SLC25A13 gene mutation screening was conducted by high resolution melt (HRM) analysis. The petechiae on the patient's face and platelet count (27×10(9)/L, reference range 100×10(9)/L-300×10(9)/L) supported the diagnosis of immunologic thrombocytopenic purpura (ITP). Laboratory tests found that the patient have abnormal coagulation, cardiac enzyme, liver function and liver enzymes dysfunction. Tandem mass spectrometry also found methionine to be increased (286 μmol/L, reference ranges 8-35 μmol/L). The patient did not manifest any galactosemia, citrullinemia and tyrosinemia. Analysis of SLC25A13 gene mutation found that the patient has carried IVS16ins3kb, in addition with abnormal HRM result for exon 6. Direct sequencing of exon 6 revealed a novel mutation c.495delA. The same mutation was not detected in 100 unrelated healthy controls. Further analysis of her family has confirmed that the c.495delA mutation has derived from her farther, and that the IVS16ins3kb was derived from her mother. The clinical features and metabolic spectrum of citrin deficiency can be variable. The poor prognosis and severity of clinical symptoms of the patient may be attributed to the novel c.495delA mutation.

Peliosis hepatis presenting with massive hepatomegaly in a patient with idiopathic thrombocytopenic purpura

Directory of Open Access Journals (Sweden)

Sun Bean Kim

2015-12-01

Full Text Available Peliosis hepatis is a rare condition that can cause hepatic hemorrhage, rupture, and ultimately liver failure. Several authors have reported that peliosis hepatis develops in association with chronic wasting disease or prolonged use of anabolic steroids or oral contraceptives. In this report we describe a case in which discontinuation of steroid therapy improved the condition of a patient with peliosis hepatis. Our patient was a 64-year-old woman with a history of long-term steroid treatment for idiopathic thrombocytopenic purpura . Her symptoms included abdominal pain and weight loss; the only finding of a physical examination was hepatomegaly. We performed computed tomography (CT and magnetic resonance imaging (MRI of the liver and a liver biopsy. Based on these findings plus clinical observations, she was diagnosed with peliosis hepatis and her steroid treatment was terminated. The patient recovered completely 3 months after steroid discontinuation, and remained stable over the following 6 months.

Acquired Thrombotic Thrombocytopenic Purpura in a Patient with Pernicious Anemia

Directory of Open Access Journals (Sweden)

Ramesh Kumar Pandey

2017-01-01

Full Text Available Introduction. Acquired thrombotic thrombocytopenic purpura (TTP has been associated with different autoimmune disorders. However, its association with pernicious anemia is rarely reported. Case Report. A 46-year-old male presented with blood in sputum and urine for one day. The vitals were stable. The physical examination was significant for icterus. Lab tests’ results revealed leukocytosis, macrocytic anemia, severe thrombocytopenia, renal dysfunction, and unconjugated hyperbilirubinemia. He had an elevated LDH, low haptoglobin levels with many schistocytes, nucleated RBCs, and reticulocytes on peripheral smear. Low ADAMTS13 activity (<10% with elevated ADAMTS13 antibody clinched the diagnosis of severe acquired TTP, and plasmapheresis was started. There was an initial improvement in his hematological markers, which were however not sustained on discontinuation of plasmapheresis. For his refractory TTP, he was resumed on daily plasmapheresis and Rituximab was started. Furthermore, the initial serum Vitamin B12 and reticulocyte index were low in the presence of anti-intrinsic factor antibody. So with the concomitant diagnosis of pernicious anemia, Vitamin B12 was supplemented. The rest of the immunological workups were negative. Subsequently, his symptoms resolved and his hematological parameters improved. Discussion. While pernicious anemia can masquerade as TTP, an actual association between the two can also occur and needs further evaluation and characterization.

A Case of Thrombotic Thrombocytopenia Purpura Associated with Systemic Lupus Erythematosus: Diagnostic Utility of ADAMTS-13 Activity

Directory of Open Access Journals (Sweden)

Risa Yamada

2011-01-01

Full Text Available Thrombotic thrombocytopenia purpura (TTP caused by a deficiency in ADAMTS-13 activity is considered to involve a subset of thrombotic microangiopathy (TMA. Although concept of TTP is included under the umbrella of TMA, discrimination of TTP from TMA is occasionally difficult in an autoimmune disorder. Herein, we report a case with TTP associated with systemic lupus erythematosus (SLE. In this case, it was difficult to discriminate TTP from TMA and the measurement of ADAMTS-13 activity was useful for obtaining an accurate diagnosis. SLE patients having thrombocytopenia in complication with anemia should be considered a monitoring of ADAMTS-13 activity even though the patients lacked symptoms of TTP related to the microvascular coagulation.

Hypothalamic functions in patients with pituitary insufficiency

NARCIS (Netherlands)

Borgers, A.J.F.

2013-01-01

The main objective of this thesis is to increase our understanding of hypothalamic (dys)function in patients with pituitary insufficiency. This goal is driven by the clinical experience of persisting symptoms in patients adequately treated for pituitary insufficiency. We focus primarily on patients

Thrombotic Thrombocytopenic Purpura Associated with Mixed Connective Tissue Disease: A Case Report

Directory of Open Access Journals (Sweden)

João Tadeu Damian Souto Filho

2011-01-01

Full Text Available Thrombotic thrombocytopenic purpura (TTP is a multisystemic disorder characterized by microangiopathic hemolytic anemia and thrombocytopenia, which may be accompanied by fever, renal, or neurologic abnormalities. Cases are divided into acute idiopathic TTP and secondary TTP. Autoimmune diseases, especially systemic lupus erythematosus, in association with TTP have been described so far in many patients. In contrast, TTP occurring in a patient with mixed connected tissue disease (MCTD is extremely rare and has only been described in nine patients. We describe the case of a 42-year-old female with MCTD who developed thrombocytopenia, microangiopathic hemolytic anemia, fever, and neurological symptoms. The patient had a good clinical evolution with infusion of high volume of fresh frozen plasma, steroid therapy, and support in an intensive care unit. Although the occurrence of TTP is rare in MCTD patients, it is important to recognize TTP as a cause of thrombocytopenia and hemolytic anemia in any patient with autoimmune diseases. Prompt institution of treatment remains the cornerstone of treatment of TTP even if plasma exchange is not available like what frequently happens in developing countries.

Platelet production, clearance and distribution in patients with idiopathic thrombocytopenic purpura

International Nuclear Information System (INIS)

Isaka, Yoshinari; Kambayashi, Junichi; Kimura, Kazufumi

1990-01-01

We have studied 8 normal subjects, and 12 patients with idiopathic thrombocytopenic purpura whose platelet counts ranged from 9x10 9 /L to 40x10 9 /L. Autologous platelets labeled with 111 In-tropolone were used for evaluation of mean platelet survival, platelet turnover, platelet sequestration sites, and platelet production (turnover) to clearance (sum of platelet uptake in the liver and the spleen) ratio. Platelet survival correlated directly with platelet counts. There was no significant correlation between the platelet sequestration pattern and platelet count, survival, or turnover. Sum of platelet uptake in the liver and the spleen showed a significant inverse correlation with platelet survival. No significant correlation was found between platelet turnover and platelet count. There was a significant correlation between the platelet production and clearance index when all subjects were analyzed. The distribution of platelet turnover showed considerable individual variation; eight of twelve patients showed platelet turnover less than mean minus 2SD of the control value, but others showed normal range. We conclude that although platelet destruction mechanism in RES shows a primary role of thrombocytopenia, impaired rate of effective thrombopoiesis may also contribute to disease severity in ITP. (author)

Clinical significance of the serum biomarker index detection in children with Henoch-Schonlein purpura.

Science.gov (United States)

Purevdorj, Narangerel; Mu, Yun; Gu, Yajun; Zheng, Fang; Wang, Ran; Yu, Jinwei; Sun, Xuguo

2018-02-01

To explore a panel of serum biomarkers for laboratory diagnosis of pediatric Henoch-Schönlein purpura (HSP). The blood white blood cells (WBC) and serum levels of serum amyloid A (SAA), interleukin 6 (IL-6), immunoglobulin A (IgA), immunoglobulin G (IgG), immunoglobulin M (IgM), immunoglobulin E (IgE), C-reactive protein (CRP), complement component 3 (C3), complement component 4 (C4), and ASO (anti-streptolysin O) were detected in 127 patients with Henoch-Schonlein purpura (HSP), 110 cases of septicemia patients, and 121 healthy volunteers. The diagnostic ability of biomarkers selected from HSP and septicemia patients was analyzed by ROC curve. By designing the calculation model, the biomarker index was calculated for laboratory diagnosis of HSP and differential diagnosis between HSP and septicemia. The levels of serum WBC, CRP, IL-6 and SAA in the septicemia patients were significantly higher than those in the control group (p<0.05). Compared with the healthy individuals, serum levels of WBC, CRP, IL-6, SAA, IgA and IgM were significantly increased in patients with HSP (p<0.05). The area under the curve (AUC) of SAA, IgA, IgM, WBC, IL-6, and CRP in the patients with HSP was 0.964, 0.855, 0.849, 0.787, 0.765, and 0.622, respectively. The values of SAA, IgA, IgM, WBC, IL-6, and CRP in septicemia patients were 0.700, 0.428, 0.689, 0.682, 0.891, and 0.853, respectively. Biomarker index=SAA+IgA/4000+IgM/4000×0.4CRPmean valueCRPi . The biomarker index in HSP patients was significantly higher than that of the healthy controls. However, the biomarker index in septicemia patients was significantly lower than the control. The biomarker index of HSP patients is higher than that of the control group. While in the infectious disease represented by septicemia, it is decreased. The detection of biomarker index could exclude the interference of infection as the auxiliary examination to HSP patients. Copyright © 2017 The Canadian Society of Clinical Chemists. Published by

Helicobacter Pylori – A Moving Target | Lambiotte | South African ...

African Journals Online (AJOL)

Pylori) continues to grow. Testing is also now advised for patients with immune thrombocytopenia purpura, unexplained vitamin B12 or iron deficiency anemia. Despite the indications for treatment of Helicobacter pylori infection widening, definitive ...

Convergence Insufficiency, Accommodative Insufficiency, Visual Symptoms, and Astigmatism in Tohono O'odham Students.

Science.gov (United States)

Davis, Amy L; Harvey, Erin M; Twelker, J Daniel; Miller, Joseph M; Leonard-Green, Tina; Campus, Irene

2016-01-01

Purpose. To determine rate of convergence insufficiency (CI) and accommodative insufficiency (AI) and assess the relation between CI, AI, visual symptoms, and astigmatism in school-age children. Methods. 3rd-8th-grade students completed the Convergence Insufficiency Symptom Survey (CISS) and binocular vision testing with correction if prescribed. Students were categorized by astigmatism magnitude (no/low: <1.00 D, moderate: 1.00 D to <3.00 D, and high: ≥3.00 D), presence/absence of clinical signs of CI and AI, and presence of symptoms. Analyses determine rate of clinical CI and AI and symptomatic CI and AI and assessed the relation between CI, AI, visual symptoms, and astigmatism. Results. In the sample of 484 students (11.67 ± 1.81 years of age), rate of symptomatic CI was 6.2% and symptomatic AI 18.2%. AI was more common in students with CI than without CI. Students with AI only (p = 0.02) and with CI and AI (p = 0.001) had higher symptom scores than students with neither CI nor AI. Moderate and high astigmats were not at increased risk for CI or AI. Conclusions. With-the-rule astigmats are not at increased risk for CI or AI. High comorbidity rates of CI and AI and higher symptoms scores with AI suggest that research is needed to determine symptomatology specific to CI.

Adolescent Premature Ovarian Insufficiency Following Human Papillomavirus Vaccination

Directory of Open Access Journals (Sweden)

Deirdre Therese Little MBBS, DRANZCOG, FACRRM

2014-10-01

Full Text Available Three young women who developed premature ovarian insufficiency following quadrivalent human papillomavirus (HPV vaccination presented to a general practitioner in rural New South Wales, Australia. The unrelated girls were aged 16, 16, and 18 years at diagnosis. Each had received HPV vaccinations prior to the onset of ovarian decline. Vaccinations had been administered in different regions of the state of New South Wales and the 3 girls lived in different towns in that state. Each had been prescribed the oral contraceptive pill to treat menstrual cycle abnormalities prior to investigation and diagnosis. Vaccine research does not present an ovary histology report of tested rats but does present a testicular histology report. Enduring ovarian capacity and duration of function following vaccination is unresearched in preclinical studies, clinical and postlicensure studies. Postmarketing surveillance does not accurately represent diagnoses in adverse event notifications and can neither represent unnotified cases nor compare incident statistics with vaccine course administration rates. The potential significance of a case series of adolescents with idiopathic premature ovarian insufficiency following HPV vaccination presenting to a general practice warrants further research. Preservation of reproductive health is a primary concern in the recipient target group. Since this group includes all prepubertal and pubertal young women, demonstration of ongoing, uncompromised safety for the ovary is urgently required. This matter needs to be resolved for the purposes of population health and public vaccine confidence.

Longitudinal study of microvascular involvement by nailfold capillaroscopy in children with Henoch-Schönlein purpura.

Science.gov (United States)

Zampetti, Anna; Rigante, Donato; Bersani, Giulia; Rendeli, Claudia; Feliciani, Claudio; Stabile, Achille

2009-09-01

The aim of this study is to describe by video-nailfold capillaroscopy the microvascular involvement and capillary changes in children with Henoch-Schönlein purpura (HSp) and to establish a possible correlation with clinical outcome. Thirty-one patients underwent capillaroscopic evaluation through a videomicroscope during the acute phase and after 6 months. Twenty sex/age-matched controls were also examined. All capillaroscopic variables were statistically examined in combination with laboratoristic/clinical data. Architectural and morphological changes recorded during the acute phase were statistically significant in comparison to the controls (p capillaroscopy changes, laboratoristic/clinical data, and outcome. Video-nailfold capillaroscopy can be a simple tool to evaluate microvascular abnormalities in the acute phase of HSp, and the persistence of oedema could suggest an incomplete disease resolution at a microvascular level.

A case report of uncompensated alkalosis induced by daily plasmapheresis in a patient with thrombotic thrombocytopenic purpura.

Science.gov (United States)

Nagai, Yoshiko; Itabashi, Mitsuyo; Mizutani, Mayuko; Ogawa, Tetsuya; Yumura, Wako; Tsuchiya, Ken; Nitta, Kosaku

2008-02-01

Plasmapheresis (PP) is widely known as the standard therapy for thrombotic thrombocytopenic purpura (TTP). Citrate is used as an anticoagulant in fresh frozen plasma, and the large amount of citrate infused during PP induces metabolic alkalosis. A 29-year-old woman was diagnosed with TTP associated with systemic lupus erythematosus, and was treated by daily PP in addition to a steroid, an immunosuppressant, vincristine, and cyclophosphamide. Uncompensated alkalosis caused by a combination of metabolic and respiratory alkalosis developed after artificial ventilation was discontinued. Her metabolic status improved after controlling her respiratory status and the activity of the TTP. Metabolic alkalosis is a common complication in TTP patients treated by frequent PP, but several factors that affect metabolic status may aggravate the alkalosis and induce uncompensated alkalosis.

Platelet antibody in prolonged remission of childhood idiopathic thrombocytopenic purpura

International Nuclear Information System (INIS)

Ware, R.; Kinney, T.R.; Rosse, W.

1985-01-01

Evaluations were performed in 20 patients with childhood idiopathic thrombocytopenic purpura (ITP) who remained in remission longer than 12 months. The mean duration of follow-up from diagnosis was 39 months (range 17 to 87 months). Eleven patients (four girls) in group 1 had an acute course of ITP, defined as platelet count greater than 150 X 10(9)/L within 6 months of diagnosis. Nine patients (five girls) in group 2 had a chronic course, defined as platelet count less than 150 X 10(9)/L for greater than or equal to 1 year or requiring splenectomy in an attempt to control hemorrhagic symptoms. Platelet count and serum (indirect) platelet-associated IgG (PAIgG) levels were normal in all 20 patients at follow-up. Both direct and indirect PAIgG levels were measured using a 125 I-monoclonal anti-IgG antiglobulin assay. All had normal direct PAIgG levels, except for one patient in group 1 who had a borderline elevated value of 1209 molecules per platelet. These data suggest that the prevalence of elevated platelet antibodies is low during sustained remission without medication in patients with a history of childhood ITP. These data may be relevant for pregnant women with a history of childhood ITP, with regard to the risk of delivering an infant with thrombocytopenia secondary to transplacental passage of maternal platelet antibody

Pancreatic Exocrine Insufficiency in Pancreatic Cancer

Directory of Open Access Journals (Sweden)

Miroslav Vujasinovic

2017-02-01

Full Text Available Abstract: Cancer patients experience weight loss for a variety of reasons, commencing with the tumor’s metabolism (Warburg effect and proceeding via cachexia to loss of appetite. In pancreatic cancer, several other factors are involved, including a loss of appetite with a particular aversion to meat and the incapacity of the pancreatic gland to function normally when a tumor is present in the pancreatic head. Pancreatic exocrine insufficiency is characterized by a deficiency of the enzymes secreted from the pancreas due to the obstructive tumor, resulting in maldigestion. This, in turn, contributes to malnutrition, specifically a lack of fat-soluble vitamins, antioxidants, and other micronutrients. Patients with pancreatic cancer and pancreatic exocrine insufficiency have, overall, an extremely poor prognosis with regard to surgical outcome and overall survival. Therefore, it is crucial to be aware of the mechanisms involved in the disease, to be able to diagnose pancreatic exocrine insufficiency early on, and to treat malnutrition appropriately, for example, with pancreatic enzymes.

Pancreatic Exocrine Insufficiency in Pancreatic Cancer.

Science.gov (United States)

Vujasinovic, Miroslav; Valente, Roberto; Del Chiaro, Marco; Permert, Johan; Löhr, J-Matthias

2017-02-23

Abstract : Cancer patients experience weight loss for a variety of reasons, commencing with the tumor's metabolism (Warburg effect) and proceeding via cachexia to loss of appetite. In pancreatic cancer, several other factors are involved, including a loss of appetite with a particular aversion to meat and the incapacity of the pancreatic gland to function normally when a tumor is present in the pancreatic head. Pancreatic exocrine insufficiency is characterized by a deficiency of the enzymes secreted from the pancreas due to the obstructive tumor, resulting in maldigestion. This, in turn, contributes to malnutrition, specifically a lack of fat-soluble vitamins, antioxidants, and other micronutrients. Patients with pancreatic cancer and pancreatic exocrine insufficiency have, overall, an extremely poor prognosis with regard to surgical outcome and overall survival. Therefore, it is crucial to be aware of the mechanisms involved in the disease, to be able to diagnose pancreatic exocrine insufficiency early on, and to treat malnutrition appropriately, for example, with pancreatic enzymes.

Thirty-day outcomes underestimate endocrine and exocrine insufficiency after pancreatic resection.

Science.gov (United States)

Lim, Pei-Wen; Dinh, Kate H; Sullivan, Mary; Wassef, Wahid Y; Zivny, Jaroslav; Whalen, Giles F; LaFemina, Jennifer

2016-04-01

Long-term incidence of endocrine and exocrine insufficiency after pancreatectomy is poorly described. We analyze the long-term risks of pancreatic insufficiency after pancreatectomy. Subjects who underwent pancreatectomy from 2002 to 2012 were identified from a prospective database (n = 227). Subjects who underwent total pancreatectomy or pancreatitis surgery were excluded. New post-operative endocrine and exocrine insufficiency was defined as the need for new pharmacologic intervention within 1000 days from resection. 28 (16%) of 178 subjects without pre-existing endocrine insufficiency developed post-operative endocrine insufficiency: 7 (25%) did so within 30 days, 8 (29%) between 30 and 90 days, and 13 (46%) after 90 days. 94 (43%) of 214 subjects without pre-operative exocrine insufficiency developed exocrine insufficiency: 20 (21%) did so within 30 days, 29 (31%) between 30 and 90 days, and 45 (48%) after 90 days. Adjuvant radiation was associated with new endocrine insufficiency. On multivariate regression, pancreaticoduodenectomy and chemotherapy were associated with a greater risk of exocrine insufficiency. Reporting 30-day functional outcomes for pancreatic resection is insufficient, as nearly 45% of subjects who develop disease do so after 90 days. Reporting of at least 90-day outcomes may more reliably assess risk for post-operative endocrine and exocrine insufficiency. Copyright © 2016 International Hepato-Pancreato-Biliary Association Inc. Published by Elsevier Ltd. All rights reserved.

MRI of occult sacral insufficiency fractures following radiotherapy

International Nuclear Information System (INIS)

Mammone, J.F.; Schweitzer, M.E.

1995-01-01

Following radiation therapy, marrow abnormalities noted on magnetic resonance imaging (MRI) are frequent and may mimic metastases. Specific radiotherapy changes are usually easily identifiable; however, traumatic lesions cause more interpretive difficulties. We assessed the incidence and MRI characteristics of insufficiency fractures in this population. During a 5-year span (1987-1991), 546 patients received pelvic radiotherapy for primary malignancies. MRI was performed in 25 of these patients at least 3 months after treatment. The mean dose in this group was 53 Gy. These MRI scans were retrospectively reviewed for the appearance of the sacrum with particular attention to the presence of insufficiency fractures. This was correlated with clinical course and scintigraphic findings. Presumed insufficiency fractures on MRI paralleled the sacral side of the sacroiliac joint, enhanced with Gd-DTPA, were most prominent or initially seen anteriorly, and had ill-defined margins on all imaging sequences. The incidence of occult sacral insufficiency fractures was at least 20%. Insufficiency fractures of the sacrum in the post-radiotherapy patient are a relatively frequent occurrence which can mimic metastases. Consideration of this phenomenon and knowledge of differential features may avoid overdiagnosis of osseous metastases. (orig.)

Predictable efficacy for splenic artery embolization with sites of sequestration using 99mTc labeled platelets in immunogenic idiopathic thrombocytopenic purpura

International Nuclear Information System (INIS)

Tian Aiping

1992-01-01

28 patients with chronic Immunogenic (Idiopathic) Thrombocytopenic purpura (ITP) were determined of the site of sequestration using 99m Tc labeled platelets. Among them, partial splenic artery embolization were done in 20 patients. 7/8 spleen type cases and 1/6 hepato-spleen hepatic type cases have their platelets counts raised above 100 x 10 9 /L. The separation of platelets and its 99m Tc labelling method were also discussed. Above results suggested that the determination of sequestration site of the 99m Tc labelled platelets have some help for the splenic artery embolization therapy in ITP

Long-term follow-up of acute isolated accommodation insufficiency.

Science.gov (United States)

Lee, Jung Jin; Baek, Seung-Hee; Kim, Ungsoo Samuel

2013-04-01

To define the long-term results of accommodation insufficiency and to investigate the correlation between accommodation insufficiency and other factors including near point of convergence (NPC), age, and refractive errors. From January 2008 to December 2009, 11 patients with acute near vision disturbance and remote near point of accommodation (NPA) were evaluated. Full ophthalmologic examinations, including best corrected visual acuity, manifest refraction and prism cover tests were performed. Accommodation ability was measured by NPA using the push-up method. We compared accommodation insufficiency and factors including age, refractive errors and NPC. We also investigated the recovery from loss of accommodation in patients. Mean age of patients was 20 years (range, 9 to 34 years). Five of the 11 patients were female. Mean refractive error was -0.6 diopters (range, -3.5 to +0.25 diopters) and 8 of 11 patients (73%) had emmetropia (+0.50 to -0.50 diopters). No abnormalities were found in brain imaging tests. Refractive errors were not correlated with NPA or NPC (rho = 0.148, p = 0.511; rho = 0.319, p = 0.339; respectively). The correlation between age and NPA was not significant (rho = -395, p = 0.069). However, the correlation between age and NPC was negative (rho = -0.508, p = 0.016). Three of 11 patients were lost to follow-up, and 6 of 8 patients had permanent insufficiency of accommodation. Accommodation insufficiency is most common in emmetropia, however, refractive errors and age are not correlated with accommodation insufficiency. Dysfunction of accommodation can be permanent in the isolated accommodation insufficiency.

Adrenal insufficiency in pakistani hiv infected patients

International Nuclear Information System (INIS)

Afreen, B.; Khan, K.A.; Riaz, A.

2017-01-01

Background: Adrenal insufficiency (AI) is the most common endocrine complication among patients with AIDS/HIV infection and there are number of causes of AI in HIV patients. Human immunodeficiency virus directly as well as indirectly destroys adrenal glands. The estimates of its prevalence and severity vary. AI is the most life threatening but readily correctable endocrine complication that occurs in persons with HIV infection. This study was carried out to determine the frequency of Adrenal Insufficiency in HIV patients and their clinical features as proper diagnosis and timely treatment have been shown to improve quality of life and long-term mortality in AIDS patients. Methods: It was a cross sectional survey conducted at HIV clinic and Jinnah Allama Iqbal Institute of Diabetes and Endocrinology, Jinnah Hospital Lahore. Sixty-four HIV positive patients, both male and female, aged above 15 years were included in the study. HIV patients who had recently taken steroids, ketoconazole or rifampicin, determined on history, were excluded from the study. The data was collected on a structured proforma and analysis was performed in SPSS-21.0. Frequency and percentages for adrenal insufficiency and its characteristics were calculated. Chi-square test was used with p<0.05 as statistically significant. Results: In this study, 9 (14.06%) HIV patients were diagnosed with adrenal insufficiency, male to female ratio was 3.5:1 and AI was found statistically significantly associated with fatigue (p<0.008) and weight loss (p<0.001). Conclusion: Adrenal insufficiency was high among the patients with HIV, it was not gender specific but it was found to be associated with fatigue and weight loss. (author)

Characterization of pituitary cells targeted by antipituitary antibodies in patients with isolated autoimmune diseases without pituitary insufficiency may help to foresee the kind of future hypopituitarism.

Science.gov (United States)

De Bellis, A; Dello Iacovo, A; Bellastella, G; Savoia, A; Cozzolino, D; Sinisi, A A; Bizzarro, A; Bellastella, A; Giugliano, D

2014-10-01

Detection of antipituitary antibodies (APA) at high levels and with a particular immunofluorescence pattern in patients with autoimmune polyendocrine syndromes may indicate a possible future autoimmune pituitary involvement. This longitudinal study was aimed at characterizing in patients with a single organ-specific autoimmune disease the pituitary cells targeted by APA at start, verifying whether this characterization allows to foresee the kind of possible subsequent hypopituitarism. Thirty-six APA positive and 40 APA negative patients with isolated autoimmune diseases participated in the study. None of them had pituitary dysfunction at entry. Characterization by four-layer immunofluorescence of pituitary cells targeted by APA in APA positive patients at entry and study of pituitary function in all patients were performed every 6 months during a 5 year follow-up. Antipituitary antibodies immunostained selectively one type of pituitary-secreting cells in 21 patients (58.3 %, group 1), and several types of pituitary cells in the remaining 15 (41.7 %, group 2). All patients in group 1 showed subsequently a pituitary insufficiency, corresponding to the type of cells targeted by APA in 18 of them (85.7 %). Only 8 out of 15 patients in group 2 (53.3 %) showed a hypopituitarism, isolated in 7 and combined in the other one. None of APA negative patients showed hypopituitarism. The characterization of pituitary cells targeted by APA in patients with isolated autoimmune diseases, when the pituitary function is still normal, may help to foresee the kind of subsequent hypopituitarism, especially when APA immunostained selectively only one type of pituitary cells. A careful follow-up of pituitary function in these patients is advisable to allow an early diagnosis of hypopituitarism, even in subclinical phase and a consequent timely replacement therapy.

Subchondral insufficiency fractures of the femoral head

Energy Technology Data Exchange (ETDEWEB)

Davies, M.; Cassar-Pullicino, V.N. [Department of Radiology, Robert Jones and Agnes Hunt Orthopaedic and District Hospital, Oswestry, SY10 7AG, Shropshire (United Kingdom); Darby, A.J. [Department of Pathology, Robert Jones and Agnes Hunt Orthopaedic and District Hospital, Oswestry, SY10 7AG, Shropshire (United Kingdom)

2004-02-01

The aim of this study was to increase awareness of, and to show the variable clinical and radiological features of, subchondral insufficiency fractures of the femoral head. The clinical and radiological findings in 7 patients with subchondral insufficiency fractures of the femoral head were reviewed retrospectively. The diagnosis was confirmed histologically in 4 patients. Radiographs were performed in all patients, MRI in 5 and scintigraphy in 4 patients. Radiographs showed varying degrees of femoral head collapse in 4 patients. In the remaining 3 patients radiographs showed a normal femoral head, regional osteoporosis and focal sclerosis, respectively. Magnetic resonance imaging showed a low-signal band on T1- and T2-weighted images in the subchondral bone adjacent or parallel to the articular surface associated with bone marrow oedema. Scintigraphy showed increased uptake in the femoral head. Insufficiency fractures of the femoral head are easily overlooked or confused with avascular necrosis and, when there is significant joint destruction, osteoarthritis. Unsuspected insufficiency fracture of the femoral head can lead to significant and rapid loss of bone stock in osteoporotic patients waiting for arthroplasty for osteoarthritis. Increased awareness of this condition will hopefully lead to earlier diagnosis and a successful outcome of conservative treatment. (orig.)

Adrenal Insufficiency and Addison's Disease

Science.gov (United States)

... These conditions can lead to an adrenal crisis. Pregnancy Women with adrenal insufficiency who become pregnant are ... can benefit from following a diet rich in sodium. A health care provider or a dietitian can ...

Pneumococcal Sepsis Complicated by Splenic Abscesses and Purpura Fulminans in a 15-Month-Old Child

Directory of Open Access Journals (Sweden)

Scott Pangonis MD

2016-02-01

Full Text Available Streptococcus pneumoniae is an invasive organism that causes a wide range of common diseases, including sinusitis, acute otitis media, and pneumonia. Splenic abscesses and purpura fulminans (PF are rare complications of pneumococcal disease. Splenic abscesses caused by S pneumoniae have only been reported in the adult literature. PF has been described in the pediatric population as a rare complication in patients with invasive pneumococcal disease (IPD with and without underlying immunological disorders such as asplenia. Here, we report a patient with IPD complicated by splenic abscesses and PF. Our patient initially presented with bacteremia, septic shock, and disseminated intravascular coagulation. She subsequently developed PF and splenic abscesses. She survived her illness after receiving a total of 8 weeks of antibiotic therapy. This case highlights 2 rare complications of IPD and demonstrates the need to keep pneumococcal disease in the differential diagnosis even in children whose vaccination status is up to date.

Household food insufficiency is associated with dietary intake in Korean adults.

Science.gov (United States)

Lee, Sang Eun; Song, Yoon Ju; Kim, Young; Choe, Jeongsook; Paik, Hee-Young

2016-04-01

To examine the association of food insufficiency with dietary intake and eating and health behaviours. A cross-sectional study. Data were obtained from a secondary source, the Fifth Korea National Health and Nutrition Examination Survey (2010-2012). The sample size consisted of 15 603 adults over 19 years of age (8898 households). Significant differences in socio-economic factors were observed according to food insufficiency level (Pfood-insufficient group, but we found no association with fat intake. Regarding micronutrients, Ca, Fe, vitamin A, thiamin, riboflavin niacin and vitamin C intakes were negatively associated with food insufficiency level (P trendfood groups, such as meat, fish, eggs and beans, vegetables and fruits, was significantly lower as food insufficiency level decreased after controlling for all possible variables; food group consumption also differed by sex. Overall eating and health behaviours were poorer in the mildly and severely food-insufficient groups, who received more food assistance but less nutritional education. Our results showed that dietary intake as well as eating and health behaviours are adversely associated with food insufficiency. These findings suggest that specific strategies to help food-insufficient individuals should be developed in order to improve their dietary quality and health status.

Primary ovarian insufficiency: an update

Directory of Open Access Journals (Sweden)

Cox L

2014-02-01

Full Text Available Leticia Cox, James H LiuUH Case Medical Center, MacDonald Women's Hospital, Department of Obstetrics and Gynecology, Case Western Reserve University School of Medicine, Department of Reproductive Biology, Cleveland, OH, USAAbstract: Primary ovarian insufficiency is a condition that represents impaired ovarian function on a continuum with intermittent ovulation. This condition commonly leads to premature menopause, defined as cessation of ovulation prior to the age of 40 years. Because there are potential immediate and long-term consequences of hypoestrogenism, a timely diagnosis is invaluable. This comprehensive review will discuss identifiable causes for primary ovarian insufficiency, including genetic disorders and metabolic abnormalities, as well as review current strategies for diagnosis, evaluation, and management of women with this condition.Keywords: premature ovarian failure, premature menopause, ovarian dysfunction

[Adrenal insufficiency of the adult].

Science.gov (United States)

Jublanc, C; Bruckert, E

2016-12-01

Adrenal insufficiency is a rare but life-threatening disorder. Clinical manifestations include fatigue, weight loss, gastrointestinal manifestations and skin hyperpigmentation, the latter being specific of primary adrenal failure. Because of non-specific clinical features of this rare disorder, diagnosis can be delayed and adrenal failure be revealed by an acute crisis. Adrenal insufficiency can be primary (Addison disease), most frequently autoimmune, or secondary, resulting from long term administration of exogenous glucocorticoids or more rarely from pituitary disorders. Monitoring of substitutive treatment is now well codified. Patient education is very important in this chronic disease that remains associated with a persistent high risk of adrenal crisis. Copyright © 2016 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.

[Treatment and results of therapy in chronic idiopathic thrombocytopenic purpura].

Science.gov (United States)

Tasić, J; Milenović, M; Drasković, S; Vukicević, T; Macukanović, L; Kitić, Lj; Bakić, M

1994-01-01

Basic principles in the therapy of chronic idiopathic thrombocytopenic purpura are glucocorticoides and splenectomy. Other measures: Intravenous high doses gamma globulin therapy, attenuated androgenes, immunosupresive drugs and plasmaferesis are less effective. During the period of 1989-1992 we treated 34 patients. From 34 patients, 23 were women and 11 were men. We treated patients primarily by prednisolon approximaly for 2 - 4 weeks. Rarely we use doses of 3 mg/kg per day for short periods of time (5 to 10 days) or "pulse therapy" of 500 mg per day. Those doses may be effective in elevating platelet count if the response is poor. If response occurs, high dosages of steroides should be tareped to determine the amount that will maintain the platelet count in the range of 30x10(9)/l to 50x10(9)/l (to minimaze the toxic sade effects of steroides). If steroides are ineffective, we perform splenectomy. From 34 treated patients by glucocorticoides, in 16 we got remission and in 11 partial response. We discussed in detailes relationship duration of treatment with glucocorticoides and level of platelets, and also correlation duration of treatment with prognosis. From 6 splenectomized patients 3 were successful. In two patients we applied intravenous gamma globulin therapy and attenuated androgen successfuly. In one patients therapy with gamma globulin, immunosupresive drugs, androgen and other measures was ineffective. In one patients without splenectomy we administrated successfuly gamma globulin therapy and androgen for peroid of two years.

Genetics Home Reference: fragile X-associated primary ovarian insufficiency

Science.gov (United States)

... Share: Email Facebook Twitter Home Health Conditions FXPOI Fragile X-associated primary ovarian insufficiency Printable PDF Open All ... Javascript to view the expand/collapse boxes. Description Fragile X-associated primary ovarian insufficiency ( FXPOI ) is a condition ...

Púrpura em paciente com estrongiloidíase disseminada Purpura in patient with disseminated strongiloidiasis

Directory of Open Access Journals (Sweden)

Luciano C. Ribeiro

2005-05-01

Full Text Available A infecção pelo Strongyloides stercoralis em associação com imunosupressão pode manifestar-se com lesões em múltiplos órgãos e sistemas, caracterizando a forma disseminada da doença. Lesões cutâneas não são freqüentemente relatadas e, se presentes, manifestam-se como rash e petéquias. Púrpuras bem definidas são pouco descritas. No presente trabalho é descrito um caso de estrongiloidíase disseminada, com acometimento cutâneo em forma de púrpura, que se desenvolveu em um paciente timectomizado e usuário crônico de corticosteróide devido à miastenia gravis.The association of systemic corticosteroid therapy and dissemination of Strongyloides stercoralis has been increasingly documented in the literature. Skin involvement in disseminated strongyloidiasis has been reported and the most commonly described cutaneous manifestations are rash and petechial eruptions. We present a case of an immunosuppressed man that developed disseminated strongyloidiasis with extensive purpura.

Acute renal failure and severe rhabdomyolysis in a patient with resistant thrombotic thrombocytopenic purpura

Directory of Open Access Journals (Sweden)

Al Qahtani S

2011-10-01

Full Text Available Saad Al Qahtani Intensive Care Department, Critical Care Response Team, King Abdulaziz Medical City (KAMC, National Guard Health Affairs; King Saud Bin Abdulaziz University for Health Sciences, College of Medicine, Riyadh, Kingdom of Saudi Arabia Abstract: Thrombotic thrombocytopenic purpura (TTP is a rare, life-threatening disorder. This paper describes the case of a 39-year-old Sudanese male who presented to the emergency room with fever, jaundice, decreased level of consciousness, and worsening kidney function for 7 days, a high lactate dehydrogenase level (1947, severe thrombocytopenia (platelets 8, and numerous schistocytes in the peripheral blood smear. The patient was admitted with a diagnosis of TTP for plasma exchange. Fourteen days later, his creatinine kinase (CK level rose to >50,000 IU; rhabdomyolysis was suggested. Continuous venovenous hemodialysis (CVVHD was started. The patient's CK level remained high, despite CVVHD, until the 6th day, after which this parameter gradually started to decrease. This report highlights a resistant case of TTP that presented with concomitant severe rhabdomyolysis, which demanded aggressive, continuous intervention. Keywords: TTP, CVVHD, continuous venovenous hemodialysis

Recurrent tricuspid insufficiency: is the surgical repair technique a risk factor?

Science.gov (United States)

Kara, Ibrahim; Koksal, Cengiz; Cakalagaoglu, Canturk; Sahin, Muslum; Yanartas, Mehmet; Ay, Yasin; Demir, Serdar

2013-01-01

This study compares the medium-term results of De Vega, modified De Vega, and ring annuloplasty techniques for the correction of tricuspid insufficiency and investigates the risk factors for recurrent grades 3 and 4 tricuspid insufficiency after repair. In our clinic, 93 patients with functional tricuspid insufficiency underwent surgical tricuspid repair from May 2007 through October 2010. The study was retrospective, and all the data pertaining to the patients were retrieved from hospital records. Functional capacity, recurrent tricuspid insufficiency, and risk factors aggravating the insufficiency were analyzed for each patient. In the medium term (25.4 ± 10.3 mo), the rates of grades 3 and 4 tricuspid insufficiency in the De Vega, modified De Vega, and ring annuloplasty groups were 31%, 23.1%, and 6.1%, respectively. Logistic regression analysis revealed that chronic obstructive pulmonary disease, left ventricular dysfunction (ejection fraction, tricuspid insufficiency. Medium-term survival was 90.6% for the De Vega group, 96.3% for the modified De Vega group, and 97.1% for the ring annuloplasty group. Ring annuloplasty provided the best relief from recurrent tricuspid insufficiency when compared with DeVega annuloplasty. Modified De Vega annuloplasty might be a suitable alternative to ring annuloplasty when rings are not available.

Pubic insufficiency fracture: MRI findings

International Nuclear Information System (INIS)

Min, Tae Kyu; Lee, Yeon Soo; Park, Jeong Mi; Kim, Jee Young; Chung, Hong Jun; Lee, Eun Hee; Lee, Eun Ja; Kang, So Won; Han Tae Il

2000-01-01

To evaluate the characteristic MRI findings of pubic insufficiency fracture. In nine cases of pubic insufficiency fracture, the findings of plain radiography (n=9), MRI (n=9), and bone scintigraphy (n=8) were reviewed. We retrospectively analyzed, with regard to fracture site, the destructive pattern revealed by plain radiography, and uptake by other pelvic bones, as demonstrated by RI bone scanning. The MR findings evaluated were the fracture gap and its signal intensity, the site and signal intensity of the soft tissue mass, and other pelvic bone fractures. Plain radiography revealed osteolysis and sclerosis of pubic bone in eight of nine cases (89%), and parasymphyseal fractures in seven (78%). RI indicated uptake by the sacrum in six cases (66%), and by the ilium in three (33%). MR findings of fracture gap (seven cases, 78%) were hypo to isointensity on T1WI, hyper intensity on T2WI and the absence of contrast enhancement. Soft tissue masses were found in seven cases (78%); in four of these the location was parasymphyseal, and in three, surrounding muscle was involved. Hypo to isointensity was revealed by T1WI, hyperintensity by T2WI, and there was peripheral enhancement. Other associated pelvic bone fractures involved the sacrum in seven cases and the ilium in four. The characteristic MR findings of pubic insufficiency fracture were parasymphyseal location, fracture gap, peripherally enhanced soft tissue mass formation, and fractures of other pelvic bones, namely the sacrum and ilium

MANAGEMENT OF CHRONIC IDIOPATHIC THROMBOTIC THROMBOCYTOPENIC PURPURA WITH SPLENECTOMY

Directory of Open Access Journals (Sweden)

Mojca Modic

2002-07-01

Full Text Available Background. Treatment of thrombotic thrombocytopenic purpura (TTP with plasma exchange (PE has reduced mortality rates from 90% in untreated patients to less than 20%. Despite primary effective PE, relapses occur in up to 40% of patients. Beside PE, second line treatment options are corticosteroids, vincristine, defibrotide, prostacycline. We describe two patients with relapsing TTP treated successfully with splenectomy.Patients and methods. Case 1. A 20–year female was diagnosed in October 2000 with idiopathic chronic relapsing TTP. First episode of PE plus methylprednisolone treatment led to complet remission. Subsequent relapses occured 3, 6 and 8 months after first TTP episode. Every relaps was effectivelly treated with PE and methylprednisolone. Because of more and more frequent TTP relapses laparoscopic splenectomy was performed. Currently, 7 months after splenectomy she is in complet TTP remission.Case 2. A 18–year female, was diagnosed in January 1997 with idiopathic TTP. After PE and methylprednisolone complete remission was established. Relaps occured after nearly three years. Later on, TTP got chronic course with neurological clinical symptoms and plateled drops as soon as PE treatment was withdrawn. Besides PE and methylprednisolone she received also defibrotide. Because of chronic, PE dependant TTP, laparoscopic splenectomy was performed. Today, 15 months after splenectomy she is in complete TTP remission.Conclusions. In idiopathic relapsing and chronic TTP, PE treatment only is usually not sufficient for durable remission. Second line TTP treatment drugs have limited efficacy. Based on our experience in patients with relapsing and/or chronic TTP, laparoscopic splenectomy should be considered as a first treatment option, especialy in junger patients.

[Association between Williams syndrome and adrenal insufficiency].

Science.gov (United States)

Rchachi, Meryem; Larwanou, Maazou Mahamane; El Ouahabi, Hanan; Ajdi, Farida

2017-01-01

Williams syndrome is a developmental disorder including dysmorphia, cardiovascular malformations and a specific neuropsychological profile together with other associated disorders. We report the case of a 17-year old girl, born of a non-inbred marriage, with Williams syndrome discovered during an assessment of degree of failure to thrive. Its association with primary adrenal insufficiency makes it unique. Diagnosis is confirmed by cytogenetic and molecular analysis. Its management consists of the implementation of treatment for adrenal insufficiency associated with a clinico-biological monitoring.

Modified Extended Kalman Filtering for Tracking with Insufficient and Intermittent Observations

Directory of Open Access Journals (Sweden)

Pengpeng Chen

2015-01-01

Full Text Available This paper is concerned with the Kalman filtering problem for tracking a single target on the fixed-topology wireless sensor networks (WSNs. Both the insufficient anchor coverage and the packet dropouts have been taken into consideration in the filter design. The resulting tracking system is modeled as a multichannel nonlinear system with multiplicative noise. Noting that the channels may be correlated with each other, we use a general matrix to express the multiplicative noise. Then, a modified extended Kalman filtering algorithm is presented based on the obtained model to achieve high tracking accuracy. In particular, we evaluate the effect of various parameters on the tracking performance through simulation studies.

Magnetic resonance imaging in osteomalacic insufficiency fractures of the pelvis

Energy Technology Data Exchange (ETDEWEB)

Kanberoglu, K. [Department of Radiology, Cerrahpasa Medical Faculty, Istanbul University, Istanbul (Turkey)]. E-mail: zkanber@istanbul.edu.tr; Kantarci, F. [Department of Radiology, Cerrahpasa Medical Faculty, Istanbul University, Istanbul (Turkey); Cebi, D. [Department of Radiology, Cerrahpasa Medical Faculty, Istanbul University, Istanbul (Turkey); Yilmaz, M.H. [Department of Radiology, Cerrahpasa Medical Faculty, Istanbul University, Istanbul (Turkey); Kurugoglu, S. [Department of Radiology, Cerrahpasa Medical Faculty, Istanbul University, Istanbul (Turkey); Bilici, A. [Department of Internal Medicine, Cerrahpasa Medical Faculty, Istanbul University, Istanbul (Turkey); Koyuncu, H. [Department of Physical Medicine and Rehabilitation, Cerrahpasa Medical Faculty, Istanbul University, Istanbul (Turkey)

2005-01-01

AIMS: To report the magnetic resonance imaging (MRI) findings of osteomalacic insufficiency fractures of the pelvis. MATERIALS AND METHODS: In all, 12 persons presenting with chronic pelvic pain and with a definitive diagnosis of osteomalacia (OM) were enrolled in this study. Radiological work-up included direct radiographs (n=12), computed tomography (n=5), scintigraphy n=12) and MRI (n=12). The location of the insufficiency fractures and corresponding MRI appearances were evaluated. Depending on the presence or absence of signal intensity around the fractures, the lesions were grouped into active and chronic forms. RESULTS: A total of 34 insufficiency fractures were depicted on imaging studies. MRI demonstrated 33 of the lesions. All the insufficiency fractures were seen as a hypointense lines or fissures on T1- and T2-weighted and STIR MR images. There was a high frequency of insufficiency fractures at the sacrum and iliac bone (16/34, 47%). Overall, 11 chronic-type (no abnormal signal intensity around the fissure) and 22 active-type (abnormal signal intensity around the fissure) insufficiency fractures were detected by MRI. Follow-up MR examinations of 2 subjects showed that abnormal signal intensity had disappeared after appropriate treatment. CONCLUSION: The results of this study showed that the iliac and sacral bones are frequently involved in patients with OM. MRI can determine the clinical activity of the disease, and can monitor the response to treatment of the active type of insufficiency fractures.

Magnetic resonance imaging in osteomalacic insufficiency fractures of the pelvis

International Nuclear Information System (INIS)

Kanberoglu, K.; Kantarci, F.; Cebi, D.; Yilmaz, M.H.; Kurugoglu, S.; Bilici, A.; Koyuncu, H.

2005-01-01

AIMS: To report the magnetic resonance imaging (MRI) findings of osteomalacic insufficiency fractures of the pelvis. MATERIALS AND METHODS: In all, 12 persons presenting with chronic pelvic pain and with a definitive diagnosis of osteomalacia (OM) were enrolled in this study. Radiological work-up included direct radiographs (n=12), computed tomography (n=5), scintigraphy n=12) and MRI (n=12). The location of the insufficiency fractures and corresponding MRI appearances were evaluated. Depending on the presence or absence of signal intensity around the fractures, the lesions were grouped into active and chronic forms. RESULTS: A total of 34 insufficiency fractures were depicted on imaging studies. MRI demonstrated 33 of the lesions. All the insufficiency fractures were seen as a hypointense lines or fissures on T1- and T2-weighted and STIR MR images. There was a high frequency of insufficiency fractures at the sacrum and iliac bone (16/34, 47%). Overall, 11 chronic-type (no abnormal signal intensity around the fissure) and 22 active-type (abnormal signal intensity around the fissure) insufficiency fractures were detected by MRI. Follow-up MR examinations of 2 subjects showed that abnormal signal intensity had disappeared after appropriate treatment. CONCLUSION: The results of this study showed that the iliac and sacral bones are frequently involved in patients with OM. MRI can determine the clinical activity of the disease, and can monitor the response to treatment of the active type of insufficiency fractures

Predictive factors for exocrine pancreatic insufficiency after pancreatoduodenectomy with pancreaticogastrostomy.

Science.gov (United States)

Nakamura, Hiroyuki; Murakami, Yoshiaki; Uemura, Kenichiro; Hayashidani, Yasuo; Sudo, Takeshi; Ohge, Hiroki; Sueda, Taijiro

2009-07-01

The aim of this study was to determine risk factors for exocrine pancreatic insufficiency after pancreatoduodenectomy (PD) with pancreaticogastrostomy (PG). A (13)C-labeled mixed triglyceride breath test was performed in 61 patients after PD to assess exocrine pancreatic function. Percent (13)CO(2) cumulative dose at 7 h pancreatic insufficiency. Abdominal computed tomography scans were utilized to assess the dilatation of the main pancreatic duct (MPD dilatation) in the remnant. Thirty-eight of 61 patients (62.3%) were diagnosed with exocrine pancreatic insufficiency. Univariate analysis identified significant associations between two preoperative factors (preoperative impaired endocrine function and a hard pancreatic texture induced by preexisting obstructive pancreatitis), plus one postoperative factor (MPD dilatation caused by PG stricture) and exocrine pancreatic insufficiency (P pancreatic insufficiency after PD may be partly explainable by preexisting obstructive pancreatitis prior to surgery, surgeons desiring to obtain better postoperative exocrine pancreatic function after PD would be well-advised to devote considerable attention to preventing PG stricture.

Pancreatic insufficiency after different resections for benign tumours.

Science.gov (United States)

Falconi, M; Mantovani, W; Crippa, S; Mascetta, G; Salvia, R; Pederzoli, P

2008-01-01

Pancreatic resections for benign diseases may lead to long-term endocrine/exocrine impairment. The aim of this study was to compare postoperative and long-term results after different pancreatic resections for benign disease. Between 1990 and 1999, 62 patients underwent pancreaticoduodenectomy (PD), 36 atypical resection (AR) and 64 left pancreatectomy (LP) for benign tumours. Exocrine and endocrine pancreatic function was evaluated by 72-h faecal chymotrypsin and oral glucose tolerance test. The incidence of pancreatic fistula was significantly higher after AR than after LP (11 of 36 versus seven of 64; P = 0.028). The long-term incidence of endocrine pancreatic insufficiency was significantly lower after AR than after PD (P insufficiency was more common after PD (P endocrine and exocrine insufficiency was higher for PD and LP than for AR (32, 27 and 3 per cent respectively at 1 year; 58, 29 and 3 per cent at 5 years; P pancreatic resections are associated with different risks of developing long-term pancreatic insufficiency. AR represents the best option in terms of long-term endocrine and exocrine function, although it is associated with more postoperative complications. Copyright (c) 2007 British Journal of Surgery Society Ltd.

Renal tubular acidosis complicated with hyponatremia due to cortisol insufficiency

OpenAIRE

Izumi, Yuichiro; Nakayama, Yushi; Onoue, Tomoaki; Inoue, Hideki; Mukoyama, Masashi

2015-01-01

Adrenocortical insufficiency such as occurs in Addison's disease causes hyponatremia and renal tubular acidosis (RTA). Hyponatremia results from both aldosterone and cortisol insufficiency. RTA is due to aldosterone insufficiency. The involvement of cortisol in RTA is unclear. Here, we report a woman in her 70s who was admitted to our hospital with severe hyponatremia (106 mEq/l) and RTA. The patient exhibited low plasma cortisol levels with little response to rapid adrenocorticotropic hormon...

[A report of two children with fever, headache, and purpura].

Science.gov (United States)

Xu, Hong-Bo; Tan, Mei; Lu, Jian; Tian, Mao-Qiang; Chen, Yan

2017-09-01

In this study, two school-aged children had an acute onset in spring and had the manifestations of fever, headache, vomiting, disturbance of consciousness, purpura and ecchymosis, and positive meningeal irritation sign. There were increases in peripheral white blood cells and neutrophils, but reductions in the hemoglobin level and platelet count in the two children. They had a significant increase in C-reactive protein. There were hundreds or thousands of white blood cells in the cerebrospinal fluid, mainly neutrophils. Increased protein contents but normal levels of glucose and chloride in the cerebrospinal fluid were found. Head CT scan showed multiple hematomas in the right cerebellum and both hemispheres in one child. Bone marrow cytology indicated infection in the bone marrow, and both blood culture and bone marrow culture showed methicillin-resistant Staphylococcus aureus (MRSA). Both patients had cardiac murmurs and progressive reductions in the hemoglobin level and platelet count during treatment, and echocardiography showed the formation of vegetation in the aortic valve. Therefore, the patients were diagnosed with infectious endocarditis (IE). Vancomycin was used as the anti-infective therapy based on the results of drug sensitivity test. One child was cured after 6 weeks, and the other child was withdrawn from the treatment and then died. Dynamic monitoring of cardiac murmurs should be performed for children with unexplained fever, and echocardiography should be performed in time to exclude IE. IE should also be considered for children with purulent meningitis and skin and mucosal bleeding which cannot be explained by the reduction in platelet count.

Adrenal Insufficiency Caused by Locally Applied Glucocorticoids-Myth or Fact?

DEFF Research Database (Denmark)

Dinsen, Stina; Klose, Marianne; Rasmussen, Åse Krogh

2015-01-01

Case-reports have made it evident that both inhaled, percutaneous, intranasal, intraarticular and ophthalmic administered glucocorticoids have the potential to cause life threatening adrenal insufficiency. With few and sometimes conflicting data and study methodology the prevalence of adrenal...... insufficiency secondary to locally applied glucocorticoids is not clear. Adrenal insufficiency can only be correctly evaluated by a stimulation test, and has by this procedure been reported in up to 40-50% of patients treated with high-dose inhaled glucocorticoids. Medium- to low-dose inhaled glucocorticoids...... have been shown to cause adrenal suppression in 0-16% of patients. Glucocorticoid creams and nasal glucocorticoids can cause adrenal insufficiency, also when used within prescribed doses, but the frequency seems to be less than with inhaled glucocorticoids. Intraarticularly administered glucocorticoids...

Pancreatic insufficiency secondary to abdominal radiotherapy

International Nuclear Information System (INIS)

Dookeran, K.A.; Thompson, M.M.; Allum, W.H.

1993-01-01

Delayed post-irradiation steatorrhoea secondary to acute pancreatic insufficiency is rare. The authors describe a case occurring in a patient 23 years following radical abdominal radiotherapy for testicular seminoma. (Author)

Pancreatic insufficiency secondary to abdominal radiotherapy

Energy Technology Data Exchange (ETDEWEB)

Dookeran, K.A.; Thompson, M.M.; Allum, W.H. (Leicester Royal Infirmary (United Kingdom). Dept. of Surgery)

1993-02-01

Delayed post-irradiation steatorrhoea secondary to acute pancreatic insufficiency is rare. The authors describe a case occurring in a patient 23 years following radical abdominal radiotherapy for testicular seminoma. (Author).

Henoch-Schönlein Purpura in Northern Spain

Science.gov (United States)

Calvo-Río, Vanesa; Loricera, Javier; Mata, Cristina; Martín, Luis; Ortiz-Sanjuán, Francisco; Alvarez, Lino; González-Vela, M. Carmen; González-Lamuño, Domingo; Rueda-Gotor, Javier; Fernández-Llaca, Héctor; González-López, Marcos A.; Armesto, Susana; Peiró, Enriqueta; Arias, Manuel; González-Gay, Miguel A.; Blanco, Ricardo

2014-01-01

Abstract The severity of clinical features and the outcomes in previous series of patients reported with Henoch-Schönlein purpura (HSP) vary greatly, probably due to selection bias. To establish the actual clinical spectrum of HSP in all age groups using an unselected and wide series of patients diagnosed at a single center, we performed a retrospective review of 417 patients classified as having HSP according to the criteria proposed by Michel et al. Of 417 patients, 240 were male and 177 female, with a median age at the time of disease diagnosis of 7.5 years (interquartile range [IQR], 5.3–20.1 yr). Three-quarters of the patients were children or young people aged 20 years or younger (n = 315), and one-quarter were adults (n = 102). The most frequent precipitating events were a previous infection (38%), usually an upper respiratory tract infection, and/or drug intake (18.5%) shortly before the onset of the vasculitis. At disease onset the most common manifestations were skin lesions (55.9%), nephropathy (24%), gastrointestinal involvement (13.7%), joint symptoms (9.1%), and fever (6.2%). Cutaneous involvement occurring in all patients, mainly purpuric skin lesion, was the most common manifestation when the vasculitis was fully established, followed by gastrointestinal (64.5%), joint (63.1%), and renal involvement (41.2%). The main laboratory findings were leukocytosis (36.7%), anemia (8.9%), and increased serum IgA levels (31.7%). The most frequent therapies used were corticosteroids (35%), nonsteroidal antiinflammatory drugs (14%), and cytotoxic agents (5%). After a median follow-up of 12 months (IQR, 2–38 mo), complete recovery was observed in most cases (n = 346; 83.2%), while persistent, usually mild, nephropathy was observed in only 32 (7.7%) cases. Relapses were observed in almost a third of patients (n = 133; 31.9%). In conclusion, although HSP is a typical vasculitis affecting children and young people, it is not uncommon in adults. The prognosis is

Platelet antibodies of the IgM class in immune thrombocytopenic purpura

International Nuclear Information System (INIS)

Cines, D.B.; Wilson, S.B.; Tomaski, A.; Schreiber, A.D.

1985-01-01

The clinical course and response to therapy of patients with immune thrombocytopenic purpura (ITP) are not completely determined by the level of IgG present on the platelet surface. It is possible that antibodies of other immunoglobulin classes also play a role in platelet destruction in some of these patients. Therefore, the authors studied 175 patients with ITP for the presence of IgM anti-platelet antibodies using radiolabeled polyclonal or monoclonal anti-IgM. They observed that 57% of patients with clinical ITP had increased levels of IgM on their platelets, compared with normal controls and patients with thrombocytopenia who did not have ITP. They obtained similar results using either radiolabeled polyclonal or monoclonal anti-IgM, reagents whose integrity was first characterized using erythrocytes coated with defined amounts of IgM antibody. Among patients with increased platelet-IgM there was a significant correlation both with the presence of increased platelet-C3 as well as the amount of platelet-C3. The authors demonstrated the presence of warm-reacting IgM anti-platelet antibodies in the plasma of two of these patients who were further studied. These studies demonstrate the presence of warm-reacting IgM anti-platelet antibodies in some patients with ITP. They suggest that the binding of complement to platelets by IgM antibodies may initiate platelet clearance as well as enhance the effect of IgG antibodies in ITP

Thrombotic thrombocytopenic purpura (TTP or Moschowitz syndrome: a true hematologic emergency

Directory of Open Access Journals (Sweden)

Deborah Melis

2012-01-01

Full Text Available Introduction: Thrombotic thrombocytopenic purpura (TTP is a thrombotic microangiopathy caused by congenital or inherited disorders involving the processing of the ultra-large forms of von Willebrand factor. As a result, platelet-rich microthrombi form in the small arterial vessels of various organs, particularly those of the brain, heart, and kidneys. The idiopathic autoimmune form of TTP is the most common. There are various subgroups of acquired TTP associated with HIV infection, sepsis, pregnancy, autoimmune disease, various disseminated malignancies, and drugs. If not promptly treated, TTP is associated with high mortality, making it a true medical emergency. Materials and methods: The article is based on a review of the literature published between January and October of 2009. Its aim is to clarify the diagnosis, treatment, and follow-up of TTP. Results: Diagnostic criteria include the presence of microangiopathic hemolytic anemia associated with thrombocytopenia in the absence of other obvious causes. Assays of ADAMTS13 activity and titration of acquired antibodies against this enzyme are indicated in the follow-up of disease and as prognostic indicators. Treatment centers around daily plasma exchange associated with immunosuppressant drug therapy, particularly steroids and more recently the monoclonal anti-CD20 antibody rituximab. Discussion: Despite improved treatment, TTP is still associated with significant mortality (10—20%, particularly when plasma exchange is initiated late. Relapse also occurs in a substantial proportion of patients (10—40% although the frequency of this outcome may be reduced by rituximab therapy.

Antineoplastic treatment of patients with renal insufficiency

International Nuclear Information System (INIS)

Rajec, J.; Mego, M.; Rajec, J.

2011-01-01

Kidneys are the main route of elimination for many antineoplastic drugs and their metabolites. The kidney dysfunction may lead to the drug cumulation in organism with the resulting increased systemic toxicity. A lot of used cytostatics requires a dose modification at different levels of renal insufficiency. Due to the lack of data from clinical trials, the limiting of systemic toxicity is difficult especially in patients with severe renal impairment or patients undergoing chronic hemodialysis. The following article is focused on the preventive strategies dealing with recommended dosing modification of various antineoplastic agents in patients with renal insufficiency. (author)

Factors associated with the effect of open splenectomy for immune thrombocytopenic purpura.

Science.gov (United States)

Li, Ying; Zhang, Dawei; Hua, Fanli; Gao, Song; Wu, Yangjiong; Xu, Jianmin

2017-01-01

To assess the effect and complications of open splenectomy (OS) for immune thrombocytopenic purpura (ITP) and determine preoperative factors associated with surgical effect. This was a retrospective analysis of ITP patients who failed medical therapy and were treated with OS between 1997 and 2014 at the Jinshan Hospital, China. Follow-up was 60 months. Surgical effect was determined from platelet counts and bleeding episodes. Complications were assessed including bleeding episodes. Preoperative factors were identified by logistic regression analysis. Fifty-six patients (48.2 ± 16.2 yr old; 39 females) were included. Disease course was 31.2 ± 48.2 months; 91.1% patients had preoperative platelet count effect at 1 wk, 1 month, 1 yr, and 5 yrs was in 91.1%, 92.9%, 91.1%, and 89.3% patients, respectively. Pneumonia or lower extremity thrombosis occurred in 7.1% patients. Postoperative mild, moderate, and severe bleeding occurred in 33.9%, 50.0%, and 16.1% patients, respectively. No patients required blood transfusion. Mortality was zero. Larger spleen size associated with surgical effect at 1 wk, 1 month, and 1 yr, and lower preoperative minimum platelet count associated with effect at 5 yrs (P effective treatment with less complications for the management of ITP. Lower preoperative minimum platelet count associated with successful OS at 5 yrs. © 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Life-threatening autoimmune hemolytic anemia and idiopathic thrombocytopenic purpura: successful seletive splenic artery embolization

Directory of Open Access Journals (Sweden)

matteo molica

2016-04-01

Full Text Available Selective splenic artery embolization (SSAE is a nonsurgical intervention characterized by the transcatheter occlusion of the splenic artery and/or its branch vessels using metallic coils or other embolic devices. It has been applied for the management of splenic trauma, hypersplenism with portal hypertension, hereditary spherocytosis, thalassemia and splenic hemangioma. We hereby describe a case of a patient affected by idiopathic thrombocytopenic purpura (ITP and warm auto-immune hemolytic anemia (AIHA both resistant to immunosuppressive and biological therapies, not eligible for a surgical intervention because of her critical conditions. She underwent SSAE and achieved a hematologic complete response within a few days without complications. SSAE is a minimally invasive procedure to date not considered a standard option in the management of AIHA and ITP. However, following the progressive improvement of the techniques, its indications have been extended, with a reduction in morbidity and mortality compared to splenectomy in patients with critical clinical conditions. SSAE was a lifesaving therapeutic approach for our patient and it may represent a real alternative for the treatment of resistant AIHA and ITP patients not eligible for splenectomy.

[A 65-year-old man with history of claudication, palpable purpura and livedo reticularis].

Science.gov (United States)

Braun, N; Kimmel, M; Grabner, A; Ott, G; Alscher, M D

2010-04-01

A 65-year-old man was admitted with history of claudication symptoms and painful skin lesions of the lower legs. Physical examination showed palpable purpura of the lower legs and livedo reticularis, most marked at the forefoot and toes. Computed tomography (CT) showed an aortic mass 2 cm above the bifurcation. This was treated after angiography with a covered stent. Biopsy of the skin lesions showed no sign of vasculitis and no cholesterol crystals. The patient was discharged and remained symptom-free for 9 months. He was readmitted at that time with recurrent complaints. CT revealed a subtotal stenosis of the aortic stent. A skin biopsy showed CD31-positive tumor cells in small arteries. Biopsy of a new osteolytic lesion in the ileum confirmed the diagnosis of angiosarcoma of the aorta. The patient decided in favor of palliative care and was discharged from the hospital. Primary tumors of the aorta, although they are rare, should be considered in the presence of an intravascular mass with stenosis to blood flow. A skin biopsy is easy to conduct and often leads to the final diagnosis. Georg Thieme Verlag KG Stuttgart. New York.

Chronic workplace stress and insufficient physical activity: a cohort study.

Science.gov (United States)

Kouvonen, Anne; Vahtera, Jussi; Oksanen, Tuula; Pentti, Jaana; Väänänen, Ari K P; Heponiemi, Tarja; Salo, Paula; Virtanen, Marianna; Kivimäki, Mika

2013-01-01

To examine whether exposure to workplace stressors predicts changes in physical activity and the risk of insufficient physical activity. Prospective data from the Finnish Public Sector Study. Repeated exposure to low job control, high job demands, low effort, low rewards and compositions of these (job strain and effort-reward imbalance) were assessed at Time 1 (2000-2002) and Time 2 (2004). Insufficient physical activity (workplace stressors on change in physical activity was examined using fixed-effects (within-subject) logistic regression models (N=6665). In addition, logistic regression analysis was applied to examine the associations between repeated exposure to workplace stressors and insufficient physical activity (N=13 976). In these analyses, coworker assessed workplace stressor scores were used in addition to individual level scores. The proportion of participants with insufficient physical activity was 24% at baseline and 26% at follow-up. 19% of the participants who were sufficiently active at baseline became insufficiently active at follow-up. In the fixed-effect analysis, an increase in workplace stress was weakly related to an increase in physical inactivity within an individual. In between-subjects analysis, employees with repeated exposure to low job control and low rewards were more likely to be insufficiently active at follow-up than those with no reports of these stressors; fully adjusted ORs ranged from 1.11 (95% CI 1.00 to 1.24) to 1.21 (95% CI 1.05 to 1.39). Workplace stress is associated with a slightly increased risk of physical inactivity.

Why Sleep Matters—The Economic Costs of Insufficient Sleep

Science.gov (United States)

Hafner, Marco; Stepanek, Martin; Taylor, Jirka; Troxel, Wendy M.; van Stolk, Christian

2017-01-01

Abstract The Centers for Disease Control and Prevention (CDC) in the United States has declared insufficient sleep a “public health problem.” Indeed, according to a recent CDC study, more than a third of American adults are not getting enough sleep on a regular basis. However, insufficient sleep is not exclusively a US problem, and equally concerns other industrialised countries such as the United Kingdom, Japan, Germany, or Canada. According to some evidence, the proportion of people sleeping less than the recommended hours of sleep is rising and associated with lifestyle factors related to a modern 24/7 society, such as psychosocial stress, alcohol consumption, smoking, lack of physical activity and excessive electronic media use, among others. This is alarming as insufficient sleep has been found to be associated with a range of negative health and social outcomes, including success at school and in the labour market. Over the last few decades, for example, there has been growing evidence suggesting a strong association between short sleep duration and elevated mortality risks. Given the potential adverse effects of insufficient sleep on health, well-being and productivity, the consequences of sleep-deprivation have far-reaching economic consequences. Hence, in order to raise awareness of the scale of insufficient sleep as a public-health issue, comparative quantitative figures need to be provided for policy- and decision-makers, as well as recommendations and potential solutions that can help tackling the problem. PMID:28983434

Púrpura trombocitopênica idiopática e linfoma não-Hodgkin de células T na infância Idiopathic thrombocytopenic purpura and T-cell non-Hodgkin's lymphoma in childhood

Directory of Open Access Journals (Sweden)

Alessandra C. Borges

2006-03-01

Full Text Available Os linfomas representam 10% de todos os tumores malignos da infância e, destes, os linfomas não-Hodgkin são os mais freqüentes. Crianças com doenças auto-imunes apresentam maior probabilidade de desenvolver doenças linfoproliferativas, podendo ocorrer antes, durante ou após o aparecimento da neoplasia. A associação de púrpura trombocitopênica idiopática e linfomas é infreqüente (3%, principalmente na faixa etária pediátrica. Duas teorias tentam explicar a origem desta associação. Na primeira, a trombocitopenia seria decorrente da produção de auto-anticorpos antiplaquetas pelo clone tumoral. Na segunda, a PTI seria resultado de um estímulo antigênico persistente, secundário a uma desordem na proliferação linfóide. O objetivo do presente trabalho foi relatar a associação infreqüente na infância entre púrpura trombo-citopênica idiopática e linfoma não-Hodgkin de células T.Lymphomas represent 10% of all malignant tumors in childhood and from these non-Hodgkin's lymphomas are the most frequent. Children who have autoimmune diseases have a higher probability of developing lymphoproliferative diseases, which can happen before, during or after the appearance of the neoplasia. The association between idiopathic thrombocytopenic purpura and lymphomas is not common (3% especially in children. Two theories try to explain the origin of this association. In the first one, the thrombocytopenia would be a result of an autoantibody anti-blood platelet production by the tumoral clone. In the second one, the idiopathic thrombocytopenic purpura would be a result of a persistent antigenic stimulus subordinate to a disorder in the lymphoid proliferation. The aim of this work is to report the unusual association between idiopathic thrombocytopenic purpura and T-cell non-Hodgkin's lymphoma in childhood.

Physiologic AV valvular insufficiency in cine MR imaging

Energy Technology Data Exchange (ETDEWEB)

Jang, Yoon Hyung; Kang, Eun Joo; Baik, Seung Kug; Ahn, Woo Hyun; Choi, Han Yong; Kim, Bong Gi [Wallace Memorial Baptist Hospital, Pusan (Korea, Republic of)

1994-05-15

To give a help in the interpretation of cardiac cine-MR examination, the extent, shape, and timing of appearance of signal void regions near atrioventricular(A-V) valve prospectively evaluate in the healthy population. Using an axial gradient-echo technique with small flip angle, repetition time(TR) of 36 msec and echo time(TE) of 22 msec, 20 volunteers without known valvular abnormalities undertook cardiac cine-MR imaging including atrioventricular valve areas. Transient signal void was observed within the near the tricuspid(13/20 = 65%) and mitral valves(9/20 = 45%), respectively, which is so called {sup p}hysioloic atrioventricular valvular insufficiency{sup .} Eight subjects revealed the signal void areas near both tricuspid and mitral valves but, 5 subjects did not show any evidence of physiologic insufficiency. This physiologic condition does not extend more than 1 cm proximal to A-V valve plane and is generally observed only during early systole. Its morphology is semilunar or triangular configuration with the base to the valve plane in most cases of normal tricuspid insufficiency and small globular appearance in most cases of normal mitral insufficiency. Awareness of normal signal void areas near the A-V valve and their characteristics is critical in the interpretation of cardiac cine MR examinations and maybe helpful in the study of the normal cardiac physiology.

Physiologic AV valvular insufficiency in cine MR imaging

International Nuclear Information System (INIS)

Jang, Yoon Hyung; Kang, Eun Joo; Baik, Seung Kug; Ahn, Woo Hyun; Choi, Han Yong; Kim, Bong Gi

1994-01-01

To give a help in the interpretation of cardiac cine-MR examination, the extent, shape, and timing of appearance of signal void regions near atrioventricular(A-V) valve prospectively evaluate in the healthy population. Using an axial gradient-echo technique with small flip angle, repetition time(TR) of 36 msec and echo time(TE) of 22 msec, 20 volunteers without known valvular abnormalities undertook cardiac cine-MR imaging including atrioventricular valve areas. Transient signal void was observed within the near the tricuspid(13/20 = 65%) and mitral valves(9/20 = 45%), respectively, which is so called p hysioloic atrioventricular valvular insufficiency . Eight subjects revealed the signal void areas near both tricuspid and mitral valves but, 5 subjects did not show any evidence of physiologic insufficiency. This physiologic condition does not extend more than 1 cm proximal to A-V valve plane and is generally observed only during early systole. Its morphology is semilunar or triangular configuration with the base to the valve plane in most cases of normal tricuspid insufficiency and small globular appearance in most cases of normal mitral insufficiency. Awareness of normal signal void areas near the A-V valve and their characteristics is critical in the interpretation of cardiac cine MR examinations and maybe helpful in the study of the normal cardiac physiology

Endovenous Laser Ablation as a Treatment for Postsurgical Recurrent Saphenous Insufficiency

International Nuclear Information System (INIS)

Anchala, Praveen R.; Wickman, Christopher; Chen, Richard; Faundeen, Tonya; Pearce, William; Narducy, Lisa; Resnick, Scott A.

2010-01-01

The purpose of this study was to investigate the safety and efficacy of endovenous laser ablation as a treatment for recurrent symptomatic saphenous insufficiency occurring after saphenous vein ligation and stripping. A single-center retrospective review of patients who received endovenous laser ablation as a treatment for recurrent symptomatic saphenous insufficiency after ligation and stripping between November 2003 and October 2006 was performed. Fifty-six insufficient saphenous systems were identified in 38 patients. Follow-up consisted of a clinical examination in all patients as well as selective lower-extremity duplex ultrasound as clinically indicated. All 38 patients demonstrated complete closure of the insufficient saphenous vein by clinical examination and/or duplex ultrasound evaluation. Preoperative symptoms resolved after treatment in all 38 patients. No major complications were identified. Endovenous laser ablation of recurrent symptomatic saphenous venous insufficiency is a safe and effective treatment in patients who develop recurrent symptoms after saphenous vein ligation and stripping.

Hereditary myopathies with early respiratory insufficiency in adults.

Science.gov (United States)

Naddaf, Elie; Milone, Margherita

2017-11-01

Hereditary myopathies with early respiratory insufficiency as a predominant feature of the clinical phenotype are uncommon and underestimated in adults. We reviewed the clinical and laboratory data of patients with hereditary myopathies who demonstrated early respiratory insufficiency before the need for ambulatory assistance. Only patients with disease-causing mutations or a specific histopathological diagnosis were included. Patients with cardiomyopathy were excluded. We identified 22 patients; half had isolated respiratory symptoms at onset. The diagnosis of the myopathy was often delayed, resulting in delayed ventilatory support. The most common myopathies were adult-onset Pompe disease, myofibrillar myopathy, multi-minicore disease, and myotonic dystrophy type 1. Single cases of laminopathy, MELAS (mitochondrial encephalomyopathy with lactic acidosis and strokelike events), centronuclear myopathy, and cytoplasmic body myopathy were identified. We highlighted the most common hereditary myopathies associated with early respiratory insufficiency as the predominant clinical feature, and underscored the importance of a timely diagnosis for patient care. Muscle Nerve 56: 881-886, 2017. © 2017 Wiley Periodicals, Inc.

Ping-pong champion with adrenal insufficiency

Science.gov (United States)

Arima, Hiroshi; Imamine, Rui; Oiso, Yutaka

2009-01-01

A 62-year-old Japanese man, a bronze medal winner in the World Championship of table tennis when in his 20s, was diagnosed with secondary adrenocortical insufficiency due to isolated adrenocorticotropic hormone (ACTH) deficiency, and steroid administration was started. About 1 year after the diagnosis, he took part in a table tennis championship which was open to those 40 years or older. He took 10 mg hydrocortisone after breakfast as usual, played 10 matches, each of which took 20–30 min, and won the championship in about 8 h. Since the man could not always win the gruelling competition even when in his 50s, it is suggested that extra steroid hormone is not necessary for patients with adrenocortical insufficiency due to ACTH deficiency in order to successfully engage in sports requiring such intensity and endurance. PMID:21686433

[Traumatic tricuspid insufficiency].

Science.gov (United States)

Vayre, F; Richard, P; Ollivier, J P

1996-04-01

Traumatic tricuspid insufficiency is a rare condition. The diagnosis is difficult because of the slow progression of this pathology and the presence of more clinically acute lesions. Non-penetrating chest trauma is responsible for 90% of cases. Echocardiography is the investigation of choice for assessing the mechanism of the tricuspid regurgitation and for diagnosing associated lesions. It should be performed systematically in patients with multiple trauma. The surgical indications are difficult to determine and depend on the patients' symptoms and the type of anatomical lesions. It should be undertaken before right ventricular myocardial dysfunction. Several techniques may be used from valvuloplasty to valve replacement mainly with bioprostheses in symptomatic patients.

Pancreatic fibrosis correlates with exocrine pancreatic insufficiency after pancreatoduodenectomy.

Science.gov (United States)

Tran, T C K; van 't Hof, G; Kazemier, G; Hop, W C; Pek, C; van Toorenenbergen, A W; van Dekken, H; van Eijck, C H J

2008-01-01

Obstruction of the pancreatic duct can lead to pancreatic fibrosis. We investigated the correlation between the extent of pancreatic fibrosis and the postoperative exocrine and endocrine pancreatic function. Fifty-five patients who were treated for pancreatic and periampullary carcinoma and 19 patients with chronic pancreatitis were evaluated. Exocrine pancreatic function was evaluated by fecal elastase-1 test, while endocrine pancreatic function was assessed by plasma glucose level. The extent of fibrosis, duct dilation and endocrine tissue loss was examined histopathologically. A strong correlation was found between pancreatic fibrosis and elastase-1 level less than 100 microg/g (p pancreatic insufficiency. A strong correlation was found between pancreatic fibrosis and endocrine tissue loss (p pancreatic fibrosis nor endocrine tissue loss were correlated with the development of postoperative diabetes mellitus. Duct dilation alone was neither correlated with exocrine nor with endocrine function loss. The majority of patients develop severe exocrine pancreatic insufficiency after pancreatoduodenectomy. The extent of exocrine pancreatic insufficiency is strongly correlated with preoperative fibrosis. The loss of endocrine tissue does not correlate with postoperative diabetes mellitus. Preoperative dilation of the pancreatic duct per se does not predict exocrine or endocrine pancreatic insufficiency postoperatively. Copyright 2008 S. Karger AG, Basel.

Diagnosis and management of pancreatic exocrine insufficiency.

Science.gov (United States)

Nikfarjam, Mehrdad; Wilson, Jeremy S; Smith, Ross C

2017-08-21

In 2015, the Australasian Pancreatic Club (APC) published the Australasian guidelines for the management of pancreatic exocrine insufficiency (http://pancreas.org.au/2016/01/pancreatic-exocrine-insufficiency-guidelines). Pancreatic exocrine insufficiency (PEI) occurs when normal digestion cannot be sustained due to insufficient pancreatic digestive enzyme activity. This may be related to a breakdown, at any point, in the pancreatic digestive chain: pancreatic stimulation; synthesis, release or transportation of pancreatic enzymes; or synchronisation of secretions to mix with ingested food. Main recommendations: The guidelines provide advice on diagnosis and management of PEI, noting the following: A high prevalence of PEI is seen in certain diseases and conditions, such as cystic fibrosis, acute and chronic pancreatitis, pancreatic cancer and pancreatic surgery. The main symptoms of PEI are steatorrhoea or diarrhoea, abdominal pain, bloating and weight loss. These symptoms are non-specific and often go undetected and untreated. PEI diagnosis is predominantly based on clinical findings and the presence of underlying disease. The likelihood of PEI in suspected patients has been categorised into three groups: definite, possible and unlikely. If left untreated, PEI may lead to complications related to fat malabsorption and malnutrition, and have an impact on quality of life. Pancreatic enzyme replacement therapy (PERT) remains the mainstay of PEI treatment with the recommended adult initial enzyme dose being 25 000-40 000 units of lipase per meal, titrating up to a maximum of 75 000-80 000 units of lipase per meal. Adjunct acid-suppressing therapy may be useful when patients still experience symptoms of PEI on high dose PERT. Nutritional management by an experienced dietitian is essential. Changes in management as a result of these guidelines: These are the first guidelines to classify PEI as being definite, possible or unlikely, and provide a diagnostic algorithm to

Health consequences of shift work and insufficient sleep.

Science.gov (United States)

Kecklund, Göran; Axelsson, John

2016-11-01

This review summarises the literature on shift work and its relation to insufficient sleep, chronic diseases, and accidents. It is based on 38 meta-analyses and 24 systematic reviews, with additional narrative reviews and articles used for outlining possible mechanisms by which shift work may cause accidents and adverse health. Evidence shows that the effect of shift work on sleep mainly concerns acute sleep loss in connection with night shifts and early morning shifts. A link also exists between shift work and accidents, type 2 diabetes (relative risk range 1.09-1.40), weight gain, coronary heart disease (relative risk 1.23), stroke (relative risk 1.05), and cancer (relative risk range 1.01-1.32), although the original studies showed mixed results. The relations of shift work to cardiometabolic diseases and accidents mimic those with insufficient sleep. Laboratory studies indicate that cardiometabolic stress and cognitive impairments are increased by shift work, as well as by sleep loss. Given that the health and safety consequences of shift work and insufficient sleep are very similar, they are likely to share common mechanisms. However, additional research is needed to determine whether insufficient sleep is a causal pathway for the adverse health effects associated with shift work. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.

Postsplenectomy recurrence of idiopathic thrombocitopenic purpura: role of laparoscopic splenectomy in the treatment of accessory spleen.

Science.gov (United States)

Leo, C A; Pravisani, R; Bidinost, S; Baccarani, U; Bresadola, V; Risaliti, A; Terrosu, G

2015-01-01

Idiopatic thrombocytopenic purpura (ITP) is the most common indication for splenectomy. The failure rate of surgery is about 8% and the failure rate after splenectomy is approximately 28% for all patients. When the presence of an accessory spleen is diagnosed, splenectomy is recommended. Laparoscopic approach is considered the first choice. At our Department, between July and November 2011 two patients underwent laparoscopic accessory splenectomy for recurrence of ITP. Both patients had a previously laparoscopic splenectomy. Preoperative Magnetic Resonance (MR) was performed in both the cases revealing the presence of an accessory spleen. The operative time was 105 and 100 minutes respectively. No perioperative complications occured. Hospital stay was four days in both cases. The first patient had a disease free period of two months; the second one of one month. Both patients restarted immunosuppressive therapy. The relapse of thrombocytopenia post-splenectomy can be associated with the presence of an accessory spleen. The laparoscopic accessory splenectomy should be considered the first choice approach. Surgical accessory splenectomy allows a transitory remission of the disease.

Association Between Vitamin D Insufficiency and Metabolic Syndrome in Patients With Psychotic Disorders.

Science.gov (United States)

Yoo, Taeyoung; Choi, Wonsuk; Hong, Jin-Hee; Lee, Ju-Yeon; Kim, Jae-Min; Shin, Il-Seon; Yang, Soo Jin; Amminger, Paul; Berk, Michael; Yoon, Jin-Sang; Kim, Sung-Wan

2018-04-01

This study examined the association between vitamin D and metabolic syndrome in patients with psychotic disorders. The study enrolled 302 community-dwelling patients with psychotic disorders. Sociodemographic and clinical characteristics, including blood pressure, physical activity, and dietary habit were gathered. Laboratory examinations included vitamin D, lipid profile, fasting plasma glucose, HbA1c, liver function, and renal function. Vitamin D insufficiency was defined as vitamin D insufficiency were identified. Among the 302 participants, 236 patients (78.1%) had a vitamin D insufficiency and 97 (32.1%) had metabolic syndrome. Vitamin D insufficiency was significantly associated with the presence of metabolic syndrome (p=0.006) and hypertension (p=0.017). Significant increases in triglycerides and alanine transaminase were observed in the group with a vitamin D insufficiency (p=0.002 and 0.011, respectively). After adjusting for physical activity and dietary habit scores, vitamin D insufficiency remained significantly associated with metabolic syndrome and hypertension. Vitamin D insufficiency was associated with metabolic syndrome and was particularly associated with high blood pressure, although the nature, direction and implications of this association are unclear.

Effectiveness of vision therapy in school children with symptomatic convergence insufficiency

Directory of Open Access Journals (Sweden)

Jung Un Jang

2017-01-01

Conclusion: Among convergence insufficiency symptoms, the following improved in particular: near point of convergence, exophoria, and near positive fusional vergence. These findings suggest that vision therapy is very effective to recover from symptomatic convergence insufficiency.

Leptomeningeal dissemination of an astrocytoma causing hypophyseal insufficiency

International Nuclear Information System (INIS)

Suzan, S.; Cigdem, O.; Furkan, U.; Baki, A.

2012-01-01

Full text: Introduction: Hypophyseal insufficiency is an unusual clinical presentation of metastatic disease. Objectives: In this report, a case of leptomeningeal metastasis of an astrocytoma to the infundibular recess, causing hypophyseal insufficiency is presented with its magnetic resonance imaging (MRI) findings. Materials and methods: A 27-year-old woman presented with nausea, vomiting and generalized weakness. Her laboratory results were consistent with hypopituitarism. She had an operation history for astrocytoma. She was referred to radiology department for brain MRI study. Contrast-enhanced MRI scan showed extensive wall enhancement of ventricles consistent with leptomeningeal metastases. A nodular mass with pronounced contrast enhancement was also detected at the infundibular stalk. Results: Because suprasellar cistern was normal and extensive leptomeningeal metastases was detected, the nodular mass at the infundibular stalk thought to be secondary to leptomeningeal involvement of the infundibular recess. Conclusion: When a patient with a known malignancy presented with hypophyseal insufficiency, it should be thought that leptomeningeal metastases to the infundibular recess may also be a cause. The neuroimaging, especially contrast-enhanced studies, is necessary for the confirmation

[Thrombotic thrombocytopenia purpura in Martinique: Retrospective study between 2008 and 2015].

Science.gov (United States)

Patient, M; Fuseau, P; Deligny, C

2017-08-01

Some studies suggest that thrombotic thrombocytopenic purpura (TTP) occurs more often in African Americans. However there is low evidence for this in the literature. The aim of our study was to describe the clinical and biological characteristics of TTP in the Afro-Caribbean population of Martinique. We retrospectively analysed all patients with TTP diagnosed at the Fort-de-France hospital between 2008, January 1st and 2015, December 31st. Diagnosis was confirmed if ADAMTS-13 activity was<10 %. Ten patients were included, corresponding to an average annual incidence of 3.2 cases/year/million individuals. None of the patient presented with the association of the five characteristic features of TTP. Microangiopathic haemolytic anaemia and severe peripheral thrombocytopenia (median: 13G/L) was the main presentation leading to diagnosis. There was no kidney involvement in 90 % of all patients, but severe neurological manifestations occurred in 70 %. Classical management including corticosteroids and plasma exchanges allowed clinical remission in 6 out of the 10 cases. If necessary, rituximab or cyclophosphamide was used. The overall survival rate was 90 %. In Martinique, the incidence of TTP is twice that reported in similar studies in France. Clinical manifestations seem to differ by more common and more severe neurological involvement. Mortality is low, in part, due to optimal care. Copyright © 2017 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier SAS. All rights reserved.

Visualization of traumatic tricuspid insufficiency by three-dimensional echocardiography.

Science.gov (United States)

Nishimura, Kazuhisa; Okayama, Hideki; Inoue, Katsuji; Saito, Makoto; Nagai, Takayuki; Suzuki, Jun; Ogimoto, Akiyoshi; Ohtsuka, Tomoaki; Higaki, Jitsuo

2010-01-01

A 19-year-old male was admitted to the emergency room of our hospital after a motor vehicle accident. During his first physical examination, a holosystolic murmur was heard at the fourth left parasternal border. Transthoracic echocardiography showed severe tricuspid insufficiency, but the cause of tricuspid insufficiency was unclear. Therefore, three-dimensional echocardiography was performed and demonstrated flail anterior, posterior and septal leaflets of the tricuspid valve. The diagnosis was tricuspid insufficiency due to papillary muscle rupture secondary to chest blunt trauma. Surgical repair of the tricuspid valve was performed in this patient. After surgery, the signs and symptoms of right ventricular heart failure were relieved. In this case, three-dimensional echocardiography was very useful for the evaluation of spatial destruction of the tricuspid valve and papillary muscle. 2009 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.

Joint Affection in Henoch-Schönlein Purpura

Directory of Open Access Journals (Sweden)

O.V. Syniachenko

2016-10-01

Full Text Available Background. Hemorrhagic vasculitis, or Henoch-Schönlein purpura (HSP, in children has its onset in the form of the articular syndrome in the vast number of patients, which is a part of the diagnostic criteria of this disease. The nature of the arthritis after the disease transformation from the juvenile HSP remains unexplored. In adulthood, articular patho­logy can manifest as migratory intermittent or chronic arthritis, but this information should be clarified. The objective was to study the clinical, X-ray and sonographic features of arti­cular syndrome course in patients with HSP and to evaluate the features of arthropathy in the presence of the disease that began in childhood and adulthood. Materials and methods. The study included 174 patients with HSP (83 % of men and 47 % of women. Childhood-onset vasculitis (on average in 12 years was diagnosed in 92 patients, and adulthood-onset (on avera­ge in 25 years — in 82; I, II and III degree of activity of the pathological process are established at a ratio of 1 : 2 : 2. Results. The joint affection is observed in every second patient with HPS, which is closely related to the patient’s age at the onset of the disease, duration and degree of the activity of pathological process, seropositivity by the high content of immunoglobulin A and rheumatoid factor. At that, clinical, radiological and sonographic nature of the articular syndrome’s course (involving the wrist, hip and sacroiliac joints, the development of aseptic osteonecrosis, bone erosion, meniscitis and intraarti­cular cartilage flaps depend on extraarticular manifestations of the di­sease (lesion of heart, kidney, pancreas, central nervous system. They are accompanied by an increased concentration in the blood of β2-microglobulin and circulating immune complexes that together with the values of fibrinogenemia have predictive value. Dimorphism of HSP, transformed from a juvenile one, is characterized by the less serum levels

Long-Term Outcomes of Laparoscopic Splenectomy Versus Open Splenectomy for Idiopathic Thrombocytopenic Purpura

Science.gov (United States)

Qu, Yikun; Xu, Jian; Jiao, Chengbin; Cheng, Zhuoxin; Ren, Shiyan

2014-01-01

The long-term outcomes of laparoscopic splenectomy (LS) versus open splenectomy (OS) in patients with idiopathic thrombocytopenic purpura (ITP) are not known. A retrospective analysis of 73 patients who underwent splenectomy (32 LS and 41 OS) for refractory ITP between April 2003 and June 2012 was conducted. LS was associated with shorter hospital stay (P = 0.01), less blood loss and blood transfusion during surgery, quicker resumption of oral diet (P < 0.0001), and earlier drain removal (P < 0.01). Conversion to OS was required in 4 patients (12.5%). Operation time was significantly longer in LS (P < 0.0001). Deep venous thrombosis (DVT) was observed in 1 patient after LS and in 4 patients after OS (P = 0.52). One patient died from intraperitoneal bleeding after OS, another patient developed pulmonary embolism. Median follow-up of 36 months was performed in LS group (29 of 32, 91%) and of 46 months in OS group (35 of 41, 85%), 25 patients (86%) in LS group and 32 (91%) in OS group reached sustained complete response (P = 0.792). Kaplan-Meier analysis showed that there was no significant difference in the relapse-free survival rate between the groups (P = 0.777). In conclusion, the long-term outcome of laparoscopic splenectomy is not different from that of open splenectomy for patients with ITP. PMID:24833154

Platelet count evolution as a predictor of outcome after splenectomy for immune thrombocytopenic purpura.

Science.gov (United States)

Kim, Moonhwan; Park, Keun Myoung; Shin, Woo Young; Choe, Yun-Mee; Lee, Keon-Young; Ahn, Seung-Ik

2017-04-01

Splenectomy is the definitive second-line therapy for refractory immune thrombocytopenic purpura (ITP), and has a reported response rate of 50-80%. Medical attention should be reconsidered when there is no evidence of accessory spleen in refractory ITP patients after splenectomy. The purpose of this study was to determine whether platelet count evolution differs between patients with a successful or unsuccessful result after splenectomy for ITP. Archived records of 104 consecutive patients that underwent splenectomy for ITP were reviewed. Patients were divided into two groups (failures and successes) using a final follow-up platelet count of 100,000/μL as a cut-off. Platelet count evolutions in these two groups were compared using the Student's t test. Successes and failures were found to have significantly different platelet counts from two days postoperatively (P = 0.016). The area under the receiver operating characteristic curve was 0.630 (95% confidence interval, 0.518-0.741, P = 0.030), and when a cut-off value of 100,000/μL was used, sensitivity and specificity were 68.2 and 51.2%, respectively. To obtain positive and negative predictive values exceeding 50%, additional platelet counts were required at one week and one month after splenectomy. We propose a protocol for ITP follow-up after splenectomy.

Insufficiency fracture of the pelvis after the radiotherapy for carcinoma of the uterine cervix

International Nuclear Information System (INIS)

Nishimura, Tetsuo; Shimizu, Teppei; Sugiyama, Akira; Ichinohe, Kenji; Teshima, Takeshi; Takahashi, Motoichiro; Takai, Michikatsu; Kaneko, Masao

1990-01-01

Bone injury after radiotherapy for carcinoma of the uterine cervix has been reported since early days of this century. Recently, the concept of insufficiency fracture has been confirmed. Insufficiency fracture is one of the stress fractures and occurs when the elastic resistance of bone is inadequate to withstand normal or physiological stress. In the American and European studies, radiotherapy is known as a cause of insufficiency fracture. There are no reports on insufficiency fracture in the Japanese literatures. Four cases of carcinomas of the uterine cervix presented pelvic insufficiency fractures following radiotherapy. In the pubic bone, a vertical parasymphyseal fracture with mixed lytic and sclerotic changes of surrounding tissue is characteristic. Sacral insufficiency fracture shows a vertical or horizontal line with lytic and sclerotic appearance. Bone scintigraphy is a sensitive modality for the early detection. H-shaped configuration is diagnostic for sacral insufficiency fracture. CT is an accurate technique demonstrating a vertical fracture and excluding the malignant bony lesion. Although radiological findings of insufficiency fracture are similar to bone malignancy, differential diagnosis from malignant lesions is possible. Bony symptoms of all patients disappeared without specific treatments. In the follow-up study of patients with carcinomas of the uterine cervix after radiotherapy, it is important to have the consideration on insufficiency fracture. (author)

Recurrent Tricuspid Insufficiency: Is the Surgical Repair Technique a Risk Factor?

OpenAIRE

Kara, Ibrahim; Koksal, Cengiz; Cakalagaoglu, Canturk; Sahin, Muslum; Yanartas, Mehmet; Ay, Yasin; Demir, Serdar

2013-01-01

This study compares the medium-term results of De Vega, modified De Vega, and ring annuloplasty techniques for the correction of tricuspid insufficiency and investigates the risk factors for recurrent grades 3 and 4 tricuspid insufficiency after repair.

Autoimmune progesterone dermatitis: Case report with history of urticaria, petechiae and palpable pinpoint purpura triggered by medical abortion.

Science.gov (United States)

Mbonile, Lumuli

2016-03-17

Autoimmune progesterone dermatitis (APD) is a rare autoimmune response to raised endogenous progesterone levels that occur during the luteal phase of the menstrual cycle. Cutaneous, mucosal lesions and other systemic manifestations develop cyclically during the luteal phase of the menstrual cycle when progesterone levels are elevated. APD symptoms usually start 3 - 10 days before menstruation and resolve 1 - 2 days after menstruation ceases. A 30-year-old woman presented with urticaria, petechiae and palpable pinpoint purpura lesions of the legs, forearms, neck and buttocks 1 week prior to her menses starting and 2 months after a medical abortion. She was diagnosed with allergic contact dermatitis and topical steroids were prescribed. Her skin conditions did not improve and were associated with her menstrual cycle. We performed an intradermal test using progesterone, which was positive. She was treated with oral contraceptive pills and the symptoms were resolved. This is a typical case of APD triggered by increased sensitivity to endogenous progesterone induced a few months after medical abortion.

Warfarin-induced toxic epidermal necrolysis in combination therapy of Henoch-Schönlein purpura nephritis: a case report.

Science.gov (United States)

Kasahara, Katsuaki; Gotoh, Yoshimitsu; Kuroyanagi, Yoshiyuki; Nagano, China

2017-07-14

Toxic epidermal necrolysis (TEN) is a rare life-threatening condition almost exclusively attributed to drugs. The main etiologic factors for TEN are sulphonamides, anticonvulsants, and antibiotics; however, there are no published reports of warfarin causing TEN. We present the case of a 3-year-old patient who developed TEN while receiving treatment for Henoch-Schönlein purpura nephritis (HSPN). With multiple-drug therapy comprising prednisolone, mizoribine, dipyridamole, and warfarin, it is difficult to detect which drug is the causative agent. While in most cases, diagnosis of the causative drug is based on clinical history without a lymphocyte transformation test (LTT), we performed the test three times and identified the causative drug as warfarin at the late phase. We continued HSPN treatment without warfarin, and results showed good renal function without life-threatening complications. To our knowledge, this is the first report about TEN caused by warfarin. Repeated LTTs could be useful for identifying TEN-causative drugs even in the late phase.

Disease: H00225 [KEGG MEDICUS

Lifescience Database Archive (English)

Full Text Available ID:18574040 ... AUTHORS ... Sadler JE ... TITLE ... Von Willebrand factor, ADAMTS13, and thrombotic thrombocytopen...MID:17124092 ... AUTHORS ... Sadler JE ... TITLE ... Thrombotic thrombocytopenic purpura: a moving target. ... JOURN

Low Prevalence of Vitamin D Insufficiency among Nepalese Infants Despite High Prevalence of Vitamin D Insufficiency among Their Mothers

Directory of Open Access Journals (Sweden)

Johanne Haugen

2016-12-01

Full Text Available Background: Describing vitamin D status and its predictors in various populations is important in order to target public health measures. Objectives: To describe the status and predictors of vitamin D status in healthy Nepalese mothers and infants. Methods: 500 randomly selected Nepalese mother and infant pairs were included in a cross-sectional study. Plasma 25(OHD concentrations were measured by LC-MS/MS and multiple linear regression analyses were used to identify predictors of vitamin D status. Results: Among the infants, the prevalence of vitamin D insufficiency (25(OHD <50 nmol/L and deficiency (<30 nmol/L were 3.6% and 0.6%, respectively, in contrast to 59.8% and 14.0% among their mothers. Infant 25(OHD concentrations were negatively associated with infant age and positively associated with maternal vitamin D status and body mass index (BMI, explaining 22% of the variability in 25(OHD concentration. Global solar radiation, maternal age and BMI predicted maternal 25(OHD concentration, explaining 9.7% of its variability. Conclusion: Age and maternal vitamin D status are the main predictors of vitamin D status in infants in Bhaktapur, Nepal, who have adequate vitamin D status despite poor vitamin D status in their mothers.

The Evolution of Juvenile Schönlein-Henoch Purpura

Directory of Open Access Journals (Sweden)

O.E. Chernyshova

2016-10-01

Full Text Available Background. Hemorrhagic vasculitis, or Schönlein-Henoch purpura (SHP, is the most common type of systemic vasculitis in childhood, and peculiarities of the further evolution of the pathological process in adult patients remain unexplored. Objective: to study the evolution of juvenile SHP, comparing the nature of lesions of the skin, joints, heart and kidneys in patients in childhood and adulthood. Material and methods. The study included 92 patients (61 men and 31 women with the average age of 27 years, and the average age of disease onset — 11 years. I degree of the activity of the pathological process is determined in 40 % of cases, II — in 35 %, III — in 25 %. Seropositivity by hyperimmunoglobulinemia A occurred in 27 % of cases, by the presence of rheumatoid factor — in 21 %. At the time of the survey, cutaneous syndrome was diagnosed in 55 % of patients, joint one — in 45 %, kidney one — in 71 %. Renal biopsy was performed in 15 cases. Results. The cutaneous, cutaneous-joint-abdominal and cutaneous-abdominal-renal forms of the disease, lesions of the skin, gastrointestinal tract, wrist, ankle and knee joints become rarer in the process of juvenile SHP evolution, but exceptionally renal variant of the pathological process, changes of skeletal muscles, liver, spleen and the heart are revealed more often, chronic kidney disease with the renal failure progression is developed in 12 % of patients (in 17 % of cases of nephropathy, sacroiliitis, spondylopathy, tendovaginitis, enthesopathies, epiphyseal osteoporosis and meniscitis of the knee joints are arisen, II, III, VI and IV morphological classes of Henoch glomerulonephritis are formed in a ratio of 8 : 4 : 2 : 1 with tubulointerstitial component in all cases, and lymphohistiocytic infiltration of the vascular wall is the unfavorable sign for the prognosis of the disease. Conclusions. In cases of transition of juvenile SHP into the chronic adult form, the disease often obtain

Diagnosis of tricuspid insufficiency by Doppler flowmetry in the inferior vena cava

International Nuclear Information System (INIS)

Smith, H.J.

1986-01-01

Eighty-five patients subjected to routine heart catheterization were examined with duplex scanning of the inferior vena cava. Adequate Doppler recordings and a right ventricular angiography were obtained in 79 of them. Tricuspid insufficiency was found to be present in 34 patients at angiography and in 24 at duplex examination. No false positive Doppler diagnoses of tricuspid insufficiency occurred. The possibility of false positive angiographic diagnoses is discussed. A high correlation was found between percentage reversed flow in the inferior vena cava during ventricular systole and degree of angiographic tricuspid insufficiency. It is concluded that duplex scanning of the inferior vena cava seems to be a good alternative to angiography in the diagnosis and quantification of tricuspid insufficiency. (orig.)

Research on the relativity between gene polymorphism and children cardiac insufficiency.

Science.gov (United States)

He, X-H; Li, C-L; Ling, N; Wang, Q-W; Wang, Z-Z; An, X-J

2017-08-01

We analyzed the relationship between Mink-S27 gene polymorphism and children with cardiac insufficiency. From April 2013 to April 2015, we enrolled 73 cases of children with cardiac insufficiency for this study, and all 73 were placed in the observation group. 76 normal cases were selected for the control group. Restriction fragment length polymorphism (RFLP) was used to make polymorphism analysis of the Mink-S27. Our results showed no significant differences in Mink-S27 genotype and allele distribution in both observation and control groups (p>0.05). In lesion samples collected from children with cardiac insufficiency, we detected significant difference in AA, CC genotype frequency and allele frequency between the observation group and the control group (prelatively high. GNAS2 gene polymorphism was associated with the prevalence of cardiac insufficiency in children. And also the patients' condition was correlated to the frequency of different genotypes and alleles.

Traumatic Tricuspid Insufficiency Requiring Valve Repair in an Acute Setting.

Science.gov (United States)

Enomoto, Yoshinori; Sudo, Yoshio; Sueta, Tomonori

2015-01-01

Tricuspid insufficiency due to penetrating cardiac trauma is rare. Patients with tricuspid insufficiency due to trauma can tolerate this abnormality for months or even years. We report a case of a 66-year-old female with penetrating cardiac trauma on the right side of her heart that required tricuspid valve repair in an acute setting. She sustained cut and stab wounds on her bilateral forearms and in the neck and epigastric region. She had cardiac tamponade and developed pulseless electrical activity, which required emergency surgery. The right ventricle and superior vena cava were dissected approximately 5 cm and 2 cm, respectively. After these wounds had been repaired, the patient's inability to wean from cardiopulmonary bypass suggested rightsided heart failure; transesophageal echocardiography revealed tricuspid insufficiency. Right atriotomy was performed, and a detailed examination revealed that the tricuspid valve septal leaflet was split in two. There was also an atrial septal injury that created a connection with the left atrium; these injuries were not detected from the right ventricular wound. After repair, weaning from cardiopulmonary bypass with mild tricuspid insufficiency was achieved, and she recovered uneventfully. This case emphasized the importance of thoroughly investigating intracardiac injury and transesophageal echocardiography.

Intraoperative Adrenal Insufficiency in a Patient with Prader-Willi Syndrome

OpenAIRE

Barbara, David W.; Hannon, James D.; Hartman, William R.

2012-01-01

Prader-Willi syndrome (PW) is a rare genetic disorder with multi-organ system involvement. These patients present many perioperative challenges including sleep-related breathing disorders, morbid obesity, thick salivary secretions, mental retardation, and difficult intravenous access. PW has been suggested to be associated with central adrenal insufficiency. We report a novel case of persistent severe hypotension from previously undiagnosed and asymptomatic adrenal insufficiency in a pediatri...

von Willebrand factor and its cleaving protease ADAMTS13 balance in coronary artery vessels: Lessons learned from thrombotic thrombocytopenic purpura. A narrative review.

Science.gov (United States)

Morici, Nuccia; Cantoni, Silvia; Panzeri, Francesco; Sacco, Alice; Rusconi, Chiara; Stucchi, Miriam; Oliva, Fabrizio; Cattaneo, Marco

2017-07-01

Deficiency of the von Willebrand factor-cleaving protease ADAMTS13 is central to the pathophysiology of thrombotic thrombocytopenic purpura (TTP), a microangiopathic syndrome that presents as an acute medical emergency. In this review we will explore the evidence of a two-way relationship between TTP and ACS. Moreover, we will review the evidence emerged from epidemiological studies of an inverse relationship between the plasma levels of ADAMTS13 and the risk of ACS. Pubmed, MEDLINE and EMBASE, CINHAL, COCHRANE and Google Scholar databases were searched from inception to January 2017. The search yielded 43 studies representing 23 unique patient cases, 5 case series, 5 cohort studies and 10 case-control studies. Most ACS cases developing in the setting of TTP resolved with standard treatment of the underlying microangiopathy, with only a few requiring coronary invasive management. Antiplatelet therapy was not usually prescribed and all of the currently used P2Y 12 were felt to be a potential trigger for a TTP-like syndrome, although our review revealed that the occurrence of TTP in patients treated with new P2Y 12 antagonists is rare. Most studies confirmed the inverse association among ADAMTS13 levels and ACS. The heart is a definite target organ in TTP. The clinical spectrum of its involvement is probably influenced by local factors that add on to the systemic deficiency characteristic of TTP. It follows that patients with TTP should be carefully monitored for ACS events, especially when multiple risk factors for coronary disease exist. Copyright © 2017 Elsevier Ltd. All rights reserved.

Vitamin D Insufficiency among Free-Living Healthy Young Adults

OpenAIRE

Tangpricha, Vin; Pearce, Elizabeth N.; Chen, Tai C.; Holick, Michael F.

2002-01-01

Long-term vitamin D insufficiency can cause secondary hyperparathyroidism and osteomalacia (1). In addition, there is increasing evidence that vitamin D may protect against common cancers, such as cancer of the colon (2–4), prostate (5), and breast (6). Young adults aged 17 to 35 years drink inadequate amounts of milk (7) and are concerned about exposure to the sun because of the fear of developing skin cancer (8,9), which increases the risk of vitamin D insufficiency (10). We sought to exami...

Autoantibody response against NALP5/MATER in primary ovarian insufficiency and in autoimmune Addison's disease.

Science.gov (United States)

Brozzetti, Annalisa; Alimohammadi, Mohammad; Morelli, Silvia; Minarelli, Viviana; Hallgren, Åsa; Giordano, Roberta; De Bellis, Annamaria; Perniola, Roberto; Kämpe, Olle; Falorni, Alberto

2015-05-01

NACHT leucine-rich-repeat protein 5 (NALP5)/maternal antigen that embryo requires (MATER) is an autoantigen in hypoparathyroidism associated with autoimmune polyendocrine syndrome type 1 (APS1) but is also expressed in the ovary. Mater is an autoantigen in experimental autoimmune oophoritis. The objectives of the study were to determine the frequency of NALP5/MATER autoantibodies (NALP5/MATER-Ab) in women with premature ovarian insufficiency (POI) and in patients with autoimmune Addison's disease (AAD) and to evaluate whether inhibin chains are a target for autoantibodies in POI. Autoantibodies against NALP5/MATER and inhibin chains-α and -βA were determined by radiobinding assays in 172 patients with AAD without clinical signs of gonadal insufficiency, 41 women with both AAD and autoimmune POI [steroidogenic cell autoimmune POI (SCA-POI)], 119 women with idiopathic POI, 19 patients with APS1, and 211 healthy control subjects. NALP5/MATER-Ab were detected in 11 of 19 (58%) sera from APS1 patients, 12 of 172 (7%) AAD sera, 5 of 41 (12%) SCA-POI sera, 0 of 119 idiopathic POI sera and 1 of 211 healthy control sera (P < .001). None of 160 POI sera, including 41 sera from women with SCA-POI and 119 women with idiopathic POI, and none of 211 healthy control sera were positive for inhibin chain-α/βA autoantibodies. NALP5/MATER-Ab are associated with hypoparathyroidism in APS1 but are present also in patients with AAD and in women with SCA-POI without hypoparathyroidism. Inhibin chains do not appear to be likely candidate targets of autoantibodies in human POI.

The immunophenotype of bone marrow lymphocytes in children with immune thrombocytopenic purpura.

Science.gov (United States)

Alavi, Samin; Aryan, Zahra; Ghazizadeh, Farid; Arabi, Nahid; Nikougoftar, Mahin; Ebadi, Maryam

2014-09-01

Primary immune thrombocytopenic purpura (ITP), caused by immune system dysfunction, is recognized as the leading cause of thrombocytopenia in pediatric population. Nonetheless, inadequate studies have been performed on bone marrow immunophenotyping of children with ITP. In this study, we aimed to investigate the immunophenotype of bone marrow lymphocytes in these children. Between 2008 and 2012, 35 children with ITP and 26 age and sex matched healthy controls were recruited. All participants underwent bone marrow aspiration. Appropriate B-cell, T-cell, and myeloid lineage monoclonal antibodies were employed to determine the immunophenotype of these patients. CD10, CD19, and CD20, all indicative of premature B-cell markers, were significantly greater in children with ITP. CD22, mainly expressed on mature B cells was slightly, but not significantly reduced in the patients' group (P = .42). On the other hand, T cell markers including CD2, CD3, CD5, and CD7 were underexpressed. CD33, a specific marker for myeloid lineage, was underexpressed in the patients' group (5.6 ± 4.7 vs. 12.9 ± 7.3, P < .001). Noteworthy, the immunophenotype did not significantly differ between acute and persistent cases. Overall, a phenotype characterized by increased pre-B-cell markers along with decreased T cell immunophenotypic markers was observed in bone marrow lymphocytes of children with ITP in the present study. Further larger scale studies are recommended to confirm our findings, as precise mapping of the immunophenotype of lymphocytes in these patients would pave the road to improved diagnosis and treatment.

Clinical significance of detecting soluble glycocalicin and thrombopoietin in the differential diagnosis of idiopathic thrombocytopenic purpura and aplstic anemia

International Nuclear Information System (INIS)

Zhao Yiming; He Yang; Xu Haiyan; Ruan Changgeng

2010-01-01

Objective: To investigate the clinical significance of detecting soluble platelet glycocalicin (sGC) and thrombopoietin (TPO) in the differential diagnosis of idiopathic thrombocytopenic purpura (ITP) and aplastic anemia (AA). Methods: Plasma sGC and serum TPO in 83 patients with ITP, 47 patients with AA and 50 normal individuals were detected by iminunoradiometric assay (IRMA) and enzymelinked immunosorbent assay (ELISA), respectively. Statistical analysis was performed using Q test and P value of 0.05). But serum TPO level in AA group was significantly higher than that in ITP and normal groups: (857.43 ± 228.43) ng/L vs (90.32 ± 39.43) ng/L and (70.29 ± 25.16) ng/L, and they were considered statistically significant (Q=24.45 and 18.25, both P < 0.01). Conclusion: Detecting plasma sGC and serum TPO might be helpful for differentiating ITP and AA and for understanding the pathophysiology of thrombocytopenia. (authors)

Bedtime procrastination: A self-regulation perspective on sleep insufficiency in the general population.

Science.gov (United States)

Kroese, Floor M; Evers, Catharine; Adriaanse, Marieke A; de Ridder, Denise T D

2016-05-01

Getting insufficient sleep has serious consequences in terms of mental and physical health. The current study is the first to approach insufficient sleep from a self-regulation perspective by investigating the phenomenon of bedtime procrastination: going to bed later than intended, without having external reasons for doing so. Data from a representative sample of Dutch adults (N = 2431) revealed that a large proportion of the general population experiences getting insufficient sleep and regularly goes to bed later than they would like to. Most importantly, a relationship between self-regulation and experienced insufficient sleep was found, which was mediated by bedtime procrastination. © The Author(s) 2014.

Adalimumab treatment in patients with rheumatoid arthritis with renal insufficiency.

Science.gov (United States)

Sumida, Keiichi; Ubara, Yoshifumi; Suwabe, Tatsuya; Hayami, Noriko; Hiramatsu, Rikako; Hasegawa, Eiko; Yamanouchi, Masayuki; Hoshino, Junichi; Sawa, Naoki; Takaichi, Kenmei

2013-03-01

To elucidate the safety of adalimumab for rheumatoid arthritis (RA) patients with renal insufficiency, including those with end-stage renal disease undergoing hemodialysis. Sixty-five RA patients, including 2 patients undergoing hemodialysis, treated with adalimumab in our hospital from December 1, 2008 to June 30, 2011 were retrospectively analyzed. Renal function was evaluated by the estimated glomerular filtration rate (eGFR) calculated from the Cockcroft-Gault formula at the start and end of followup after adalimumab treatment. The proportion of the patients who discontinued or switched adalimumab treatment and the change of the eGFR were compared between patients with (n = 39) and without (n = 26) renal insufficiency, defined as an eGFR patients who discontinued or switched adalimumab treatment (51.3% versus 50.0%; P = 0.53). The mean ± SD changes of eGFR were from 41.6 ± 13.3 to 43.4 ± 17.9 ml/minute/1.73 m(2) in patients with renal insufficiency and from 83.6 ± 17.5 to 83.0 ± 16.8 ml/minute/1.73 m(2) in patients without renal insufficiency, and the differences in each group were not statistically significant (P = 0.92 and P = 0.78, respectively). No severe infections or other severe adverse events were observed in either group during adalimumab treatment. Our data indicate that adalimumab does not worsen renal function and has no serious adverse events even for RA patients with renal insufficiency, including those undergoing hemodialysis, and suggest that it could be a potential therapeutic option for them. Copyright © 2013 by the American College of Rheumatology.

[Perinatal complications in patients with chronic renal insufficiency on hemodialysis].

Science.gov (United States)

Vázquez-Rodríguez, Juan Gustavo; del Angel-García, Guadalupe

2010-09-01

Pregnant patients with chronic renal insufficiency treated with hemodialysis experience adverse perinatal results. To compare perinatal complications of patients with chronic renal insufficiency undergoing hemodialysis who become pregnant vs. the complications of women with chronic renal insufficiency not undergoing dialysis but who then require dialysis during gestation. Transversal and retrospective study that included three patients with chronic renal insufficiency on chronic hemodialysis who became pregnant (group A) and three patients with chronic renal insufficiency without hemodialysis at the time of conception but who required dialysis during gestation (group B). Perinatal results were compared. Statistical analysis was performed with measures of central tendency and dispersion and Student t-test. Group A had 25 sessions vs. group B with 29 hemodialysis sessions (p = 0.88). Maternal complications were anemia 100% (six cases), Cesarean delivery 83.3% (group A 2 cases vs. group B 2 cases), preeclampsia 50% (group A 2 cases vs. group B 1 case), uncontrolled hypertension 50% (group A 2 cases vs. group B 1 case), preterm delivery 50% (group A 2 cases vs. group B 1 case), transfusion 33.3% (group A 2 cases), polyhydramnios 33.3% (group A 1 case vs. group B 1 case) and abortion 16.6% (group A 1 case). Fetal complications included fetal loss 16.6% (group A 1 case), neonatal mortality 33.3% (group A 1 cases vs. group B 1 case), prematurity 50% (group A2 cases vs. group B 1 case), fetal distress 50% (group A 1 case vs. group B 2 cases), respiratory failure 33.3% (group A 2 cases) and fetal growth restriction 16.6% (group A 1 case). Frequency of perinatal complications is elevated in both groups.

Thrombotic Thrombocytopenic Purpura in Black People: Impact of Ethnicity on Survival and Genetic Risk Factors.

Science.gov (United States)

Martino, Suella; Jamme, Mathieu; Deligny, Christophe; Busson, Marc; Loiseau, Pascale; Azoulay, Elie; Galicier, Lionel; Pène, Frédéric; Provôt, François; Dossier, Antoine; Saheb, Samir; Veyradier, Agnès; Coppo, Paul

2016-01-01

Black people are at increased risk of thrombotic thrombocytopenic purpura (TTP). Whether clinical presentation of TTP in Black patients has specific features is unknown. We assessed here differences in TTP presentation and outcome between Black and White patients. Clinical presentation was comparable between both ethnic groups. However, prognosis differed with a lower death rate in Black patients than in White patients (2.7% versus 11.6%, respectively, P = .04). Ethnicity, increasing age and neurologic involvement were retained as risk factors for death in a multivariable model (P Black patients had a better survival than White patients (P = .03). Salvage therapies were similarly performed between both groups, suggesting that disease severity was comparable. The comparison of HLA-DRB1*11, -DRB1*04 and -DQB1*03 allele frequencies between Black patients and healthy Black individuals revealed no significant difference. However, the protective allele against TTP, HLA-DRB1*04, was dramatically decreased in Black individuals in comparison with White individuals. Black people with TTP may have a better survival than White patients despite a comparable disease severity. A low natural frequency of HLA-DRB1*04 in Black ethnicity may account for the greater risk of TTP in this population.

AMH as Predictor of Premature Ovarian Insufficiency

DEFF Research Database (Denmark)

Lunding, Stine Aa; Aksglæde, Lise; Anderson, Richard A

2015-01-01

CONTEXT: The majority of Turner syndrome (TS) patients suffer from accelerated loss of primordial follicles. Low circulating levels of anti-Müllerian hormone (AMH) may predict the lack of spontaneous puberty in prepubertal girls and imminent premature ovarian insufficiency (POI) in TS women...

Impact of placental insufficiency on fetal skeletal muscle growth

Science.gov (United States)

Hay, William W.

2016-01-01

Intrauterine growth restriction (IUGR) caused by placental insufficiency is one of the most common and complex problems in perinatology, with no known cure. In pregnancies affected by placental insufficiency, a poorly functioning placenta restricts nutrient supply to the fetus and prevents normal fetal growth. Among other significant deficits in organ development, the IUGR fetus characteristically has less lean body and skeletal muscle mass than their appropriately-grown counterparts. Reduced skeletal muscle growth is not fully compensated after birth, as individuals who were born small for gestational age (SGA) from IUGR have persistent reductions in muscle mass and strength into adulthood. The consequences of restricted muscle growth and accelerated postnatal “catch-up” growth in the form of adiposity may contribute to the increased later life risk for visceral adiposity, peripheral insulin resistance, diabetes, and cardiovascular disease in individuals who were formerly IUGR. This review will discuss how an insufficient placenta results in impaired fetal skeletal muscle growth and how lifelong reductions in muscle mass might contribute to increased metabolic disease risk in this vulnerable population. PMID:26994511

Drug targeting to tumors: principles, pitfalls and (pre-) clinical progress.

Science.gov (United States)

Lammers, Twan; Kiessling, Fabian; Hennink, Wim E; Storm, Gert

2012-07-20

Many different systems and strategies have been evaluated for drug targeting to tumors over the years. Routinely used systems include liposomes, polymers, micelles, nanoparticles and antibodies, and examples of strategies are passive drug targeting, active drug targeting to cancer cells, active drug targeting to endothelial cells and triggered drug delivery. Significant progress has been made in this area of research both at the preclinical and at the clinical level, and a number of (primarily passively tumor-targeted) nanomedicine formulations have been approved for clinical use. Significant progress has also been made with regard to better understanding the (patho-) physiological principles of drug targeting to tumors. This has led to the identification of several important pitfalls in tumor-targeted drug delivery, including I) overinterpretation of the EPR effect; II) poor tumor and tissue penetration of nanomedicines; III) misunderstanding of the potential usefulness of active drug targeting; IV) irrational formulation design, based on materials which are too complex and not broadly applicable; V) insufficient incorporation of nanomedicine formulations in clinically relevant combination regimens; VI) negligence of the notion that the highest medical need relates to metastasis, and not to solid tumor treatment; VII) insufficient integration of non-invasive imaging techniques and theranostics, which could be used to personalize nanomedicine-based therapeutic interventions; and VIII) lack of (efficacy analyses in) proper animal models, which are physiologically more relevant and more predictive for the clinical situation. These insights strongly suggest that besides making ever more nanomedicine formulations, future efforts should also address some of the conceptual drawbacks of drug targeting to tumors, and that strategies should be developed to overcome these shortcomings. Copyright © 2011 Elsevier B.V. All rights reserved.

Relationship between clinical signs and symptoms of convergence insufficiency.

Science.gov (United States)

Bade, Annette; Boas, Mark; Gallaway, Michael; Mitchell, G Lynn; Scheiman, Mitchell; Kulp, Marjean T; Cotter, Susan A; Rouse, Michael

2013-09-01

The percentage of children who are symptomatic has been shown to increase with the number of signs of convergence insufficiency (CI). Our goal was to investigate whether there is a relationship between the severity of the clinical signs of CI and symptom level reported in children with a three-sign symptomatic CI. The Convergence Insufficiency Treatment Trial enrolled 221 children with symptomatic CI from ages 9 to 17 years. Inclusion criteria included the following three signs of CI: (1) exophoria at near at least 4Δ greater than at distance, (2) insufficient positive fusional vergence (PFV) at near, and (3) a receded near point of convergence (NPC) of 6 cm break or greater. The relationships between the severity of each sign of CI (mild, moderate, and severe) and the level of symptoms as measured by the Convergence Insufficiency Symptom Survey (CISS) at baseline were evaluated. Mean CISS scores were not significantly different between mild, moderate, and severe exophoria (p = 0.60), PFV blur (p = 0.99), Sheard's criterion (p = 0.89), or NPC break (p = 0.84). There was also no difference between the frequency of subjects scoring at mild, moderate, or severe levels on the CISS and the severity of each sign of CI. Correlations between individual clinical signs and the CISS score were very low and not statistically significant. Among symptomatic children with a CISS score of 16 or higher and three clinical signs of CI, there is no further association between the severity of the clinical signs and their level of symptoms.

Early intervention and management of adrenal insufficiency in children.

LENUS (Irish Health Repository)

Moloney, Sinéad

2012-09-01

The endocrine disorder adrenal insufficiency includes inadequate production of the steroid hormone cortisol. This results in poor physiological responses to illness, trauma or other stressors and risk of adrenal crisis. Management is based on administration of hydrocortisone. It is important to avoid under- or over-treatment and increase the dosage during times of physiological stress. To reduce morbidity, hospital admissions and mortality, the education and empowerment of parents and carers, and prompt intervention when necessary are essential. A steroid therapy card for adrenal insufficiency containing personal information on a patient\\'s condition was developed for use by families and their specialist centres.

Novel hypomorphic mutation in IKBKG impairs NEMO-ubiquitylation causing ectodermal dysplasia, immunodeficiency, incontinentia pigmenti, and immune thrombocytopenic purpura.

Science.gov (United States)

Ramírez-Alejo, Noé; Alcántara-Montiel, Julio C; Yamazaki-Nakashimada, Marco; Duran-McKinster, Carola; Valenzuela-León, Paola; Rivas-Larrauri, Francisco; Cedillo-Barrón, Leticia; Hernández-Rivas, Rosaura; Santos-Argumedo, Leopoldo

2015-10-01

NF-κB essential modulator (NEMO) is a component of the IKK complex, which participates in the activation of the NF-κB pathway. Hypomorphic mutations in the IKBKG gene result in different forms of anhidrotic ectodermal dysplasia with immunodeficiency (EDA-ID) in males without affecting carrier females. Here, we describe a hypomorphic and missense mutation, designated c.916G>A (p.D306N), which affects our patient, his mother, and his sister. This mutation did not affect NEMO expression; however, an immunoprecipitation assay revealed reduced ubiquitylation upon CD40-stimulation in the patient's cells. Functional studies have demonstrated reduced phosphorylation and degradation of IκBα, affecting NF-κB recruitment into the nucleus. The patient presented with clinical features of ectodermal dysplasia, immunodeficiency, and immune thrombocytopenic purpura, the latter of which has not been previously reported in a patient with NEMO deficiency. His mother and sister displayed incontinentia pigmenti indicating that, in addition to amorphic mutations, hypomorphic mutations in NEMO can affect females. Copyright © 2015 Elsevier Inc. All rights reserved.

MR appearance of parasymphyseal insufficiency fractures of the os pubis

Energy Technology Data Exchange (ETDEWEB)

Hosono, Mariko; Fujimoto, Ryota; Konishi, Junji [Department of Nuclear Medicine, Faculty of Medicine, Kyoto University, 54 Shogoin-kawahara-cho, Sakyo-ku, Kyoto (Japan); Kobayashi, Hisataka [Nulcear Medicine Department, National Institutes of Health, Building 10, Room 1C-401, Bethesda, MD 20892 (United States); Tsutsui, Kazushige [Department of Radiology, Japanese Red Cross Society Wakayama Medical Center, Wakayama (Japan); Kotoura, Yoshihiko [Department of Orthopaedic Surgery, Nagahama City Hospital, Nagahama, Shiga (Japan); Tsuboyama, Tadao; Nakamura, Takashi [Department of Orthopaedic Surgery, Faculty of Medicine, Kyoto University, Kyoto (Japan); Hayashi, Hikaru [Department of Orthopaedic Surgery, Japanese Red Cross Society Wakayama Medical Center, Wakayama (Japan)

1997-09-01

Objective. To clarify the MRI features of parasymphyseal insufficiency fractures of the os pubis. Design and patients. MRI was performed in four postmenopausal women with parasymphyseal insufficiency fractures. The diagnosis was confirmed with plain films in every patient. T1-weighted and T2-weighted images were obtained in four patients using a 1.5-T unit. Postcontrast T1-weighted imaging was also done in three patients. Results and conclusions. MRI of pubic parasymphyseal insufficiency fracture characteristically demonstrates a hyperintense mass lesion with a hypointense rim on T2-weighted imaging, showing peripheral and septal enhancement after contrast administration. It is important to have this entity in mind in patients with osteoporosis, especially in patients with a history of pelvic irradiation for malignant disease, so as not to misinterpret it as a chondroid tumor or bone metastasis. (orig.). With 2 figs., 2 tabs.

Henoch-Schönlein purpura in children: its relation to oral and to oral and oral and dental health

Directory of Open Access Journals (Sweden)

Arlette Suzy Puspa Pertiwi

2012-09-01

Full Text Available > Background: Henoch-Schönlein purpura (HSP is a rare systemic small vessel vasculitis, which commonly occur in childrenbetween 2 and 10 years of age. The course of the disease is often self-limiting, although may manifest long-term renal morbidity. Theseverity of renal involvement decides about the prognosis of this disease. Many factors can trigger the disease attack, which is the mostcommon is bacterial invasion. Since the oral cavity is often refer as infectious foci to other part of the body, it seemed rationally tobe part that contribute the course of disease, thus management of these infectious foci, if possible, gives rise to an astoundingly goodprognosis. Purpose: This paper will describe a review on HSP and the possible association with oral and dental health since it mightbe related to the prognosis of HSP. reviews: Rashes in children are common; they may develop a rash after prescription of antibiotics.Nevertheless there are some childhood diseases that may manifest a rash presentation, such as HSP. It is important for pediatric dentistto have knowledge about HSP and consider the possibility of dental treatment or disease as potential triggers. Conclusion: Oral anddental condition may be the trigger cause of HSP attack. Therefore, it is important for pediatric dental practitioner to be aware of thecourse of the disease in order to limit the expanding complications.> latar belakang: Henoch-Schönlein purpura (HSP merupakan vaskulitis pembuluh darah kecil sistemik yang jarang terjadi danbiasanya menyerang anak usia 2 hingga 10 tahun. Penyakit tersebut seringkali dapat sembuh sendiri, tetapi pada jangka panjangdapat bermanifestasi dengan morbiditas ginjal. Keparahan keterlibatan ginjal menentukan prognosis penyakit. Banyak faktor yangdapat memicu serangan penyakit, tersering adalah invasi bakteri. Karena rongga mulut sering kali merupakan fokus infeksi terhadapbagian lain dari tubuh, maka mempunyai peluang sebagai faktor pemicu

29 CFR 541.2 - Job titles insufficient.

Science.gov (United States)

2010-07-01

... 29 Labor 3 2010-07-01 2010-07-01 false Job titles insufficient. 541.2 Section 541.2 Labor Regulations Relating to Labor (Continued) WAGE AND HOUR DIVISION, DEPARTMENT OF LABOR REGULATIONS DEFINING AND DELIMITING THE EXEMPTIONS FOR EXECUTIVE, ADMINISTRATIVE, PROFESSIONAL, COMPUTER AND OUTSIDE SALES EMPLOYEES...

Adrenal Insufficiency as a Cause of Acute Liver Failure: A Case Report

Directory of Open Access Journals (Sweden)

Jamshid Vafaeimanesh

2013-01-01

Full Text Available Introduction. Many diseases and conditions can contribute to elevated liver enzymes. Common causes include viral and autoimmune hepatitis, fatty liver, and bile duct diseases, but, in uncommon cases like liver involvement in endocrine disorders, liver failure is also seen. Adrenal insufficiency is the rarest endocrine disorder complicating the liver. In the previously reported cases of adrenal insufficiency, mild liver enzymes elevation was seen but we report a case with severe elevated liver enzymes and liver failure due to adrenal insufficiency. Based on our knowledge, this is the first report in this field. Case Report. A 39-year-old woman was referred to emergency ward due to drowsiness and severe fatigue. Her laboratory tests revealed prothrombin time: 21 sec, alanine aminotransferase (ALT: 2339 IU/L, aspartate aminotransferase (AST: 2002 IU/L, and ALP: 90 IU/L. No common cause of liver involvement was discovered, and eventually, with diagnosis of adrenal insufficiency and corticosteroid therapy, liver enzymes and function became normal. Finally, the patient was discharged with good general condition. Conclusion. With this report, we emphasize adrenal insufficiency (primary or secondary as a reason of liver involvement in unexplainable cases and recommend that any increase in the liver enzymes, even liver failure, in these patients should be observed.

Thrombotic thrombocytopenic purpura with decreased level of ADAMTS-13 activity and increased level of ADAMTS-13 inhibitor in an adolescent

Directory of Open Access Journals (Sweden)

Eun Mi Yang

2010-03-01

Full Text Available Thrombotic thrombocytopenic purpura (TTP is a thrombotic microangiopathy characterized by endothelial cell damage, resulting in microangiopathic hemolytic anemia, thrombocytopenia, and various degrees of neurological and renal impairment caused by microvascular thrombi. It is rare in children and frequently follows a fatal course. TTP is divided into 2 types: one is inherited and associated with ADAMTS-13 gene mutations and the other is acquired and associated with anti-ADAMTS-13 autoantibodies. The measurement of ADAMTS-13 activity in plasma, identification of ADAMTS-13 circulating inhibitor, anti-ADAMTS-13 IgG, and ADAMTS-13 gene sequencing are crucial to the diagnosis of TTP. Plasma exchanges are the first-line treatment for acquired TTP, combined with steroids and immunosuppressive drugs. Here, we describe the case of an adolescent patient with TTP, confirmed by decreased level of ADAMTS-13 activity and an increased level of ADAMTS-13 inhibitor, who was successfully treated by plasma exchanges.

Radiological features of superomedial iliac insufficiency fractures: a possible mimicker of metastatic disease

International Nuclear Information System (INIS)

Donovan, Andrea; Schweitzer, Mark E.; Rafii, Mahvash; Lax, Allison

2009-01-01

Pelvic insufficiency fractures are common in elderly patients. Because both osteoporosis and metastatic disease occur in similar patient populations, insufficiency fractures may be mistaken for metastatic foci. Although the ilium is not an uncommon location for metastases, insufficiency fractures rarely involve the ilium. The radiological features of insufficiency fractures adjacent to the sacroiliac joint (superomedial ilium) have not been well described. We describe the computed tomography (CT), magnetic resonance (MR), and positron emission tomography (PET) imaging findings of these rare fractures. Six patients (five female, one male; mean age 66 years, age range 47-83 years) with iliac insufficiency fractures adjacent to the sacroiliac joint were identified following retrospective review of a clinical database. Imaging studies, including CT (n = 4), MR (n = 3), and PET (n = 2) were reviewed by two radiologists. Tissue biopsy result was available in one patient. CT demonstrated subtle fracture lucency (n = 2) or linear sclerosis (n = 3) adjacent to the sacroiliac joint; MR marrow changes adjacent to the sacroiliac joint demonstrated a low T1, high T2 signal intensity line (n = 2), or a low T1 and low T2 signal intensity line (n = 1). Fractures were fluorodeoxyglucose avid (n = 2) with average SUV max 2.2. Iliac fractures were bilateral in three patients; additional pelvic insufficiency fractures were present in one patient. In one patient, CT-guided biopsy showed no evidence of malignancy. Recognition of the radiological characteristics of iliac insufficiency fractures is important in order to distinguish them from malignancy. The presence of additional pelvic fractures in characteristic locations in the setting of osteoporosis may help to confirm the diagnosis. (orig.)

Radiological features of superomedial iliac insufficiency fractures: a possible mimicker of metastatic disease

Energy Technology Data Exchange (ETDEWEB)

Donovan, Andrea; Schweitzer, Mark E. [NYU Hospital for Joint Diseases, New York, NY (United States); Rafii, Mahvash [New York, NY (United States); Lax, Allison [Georgetown University Hospital, Washington, DC (United States)

2009-01-15

Pelvic insufficiency fractures are common in elderly patients. Because both osteoporosis and metastatic disease occur in similar patient populations, insufficiency fractures may be mistaken for metastatic foci. Although the ilium is not an uncommon location for metastases, insufficiency fractures rarely involve the ilium. The radiological features of insufficiency fractures adjacent to the sacroiliac joint (superomedial ilium) have not been well described. We describe the computed tomography (CT), magnetic resonance (MR), and positron emission tomography (PET) imaging findings of these rare fractures. Six patients (five female, one male; mean age 66 years, age range 47-83 years) with iliac insufficiency fractures adjacent to the sacroiliac joint were identified following retrospective review of a clinical database. Imaging studies, including CT (n = 4), MR (n = 3), and PET (n = 2) were reviewed by two radiologists. Tissue biopsy result was available in one patient. CT demonstrated subtle fracture lucency (n = 2) or linear sclerosis (n = 3) adjacent to the sacroiliac joint; MR marrow changes adjacent to the sacroiliac joint demonstrated a low T1, high T2 signal intensity line (n = 2), or a low T1 and low T2 signal intensity line (n = 1). Fractures were fluorodeoxyglucose avid (n = 2) with average SUV{sub max} 2.2. Iliac fractures were bilateral in three patients; additional pelvic insufficiency fractures were present in one patient. In one patient, CT-guided biopsy showed no evidence of malignancy. Recognition of the radiological characteristics of iliac insufficiency fractures is important in order to distinguish them from malignancy. The presence of additional pelvic fractures in characteristic locations in the setting of osteoporosis may help to confirm the diagnosis. (orig.)

Endocrine and exocrine pancreatic insufficiency after acute pancreatitis: long-term follow-up study.

Science.gov (United States)

Tu, Jianfeng; Zhang, Jingzhu; Ke, Lu; Yang, Yue; Yang, Qi; Lu, Guotao; Li, Baiqiang; Tong, Zhihui; Li, Weiqin; Li, Jieshou

2017-10-27

Patients could develop endocrine and exocrine pancreatic insufficiency after acute pancreatitis (AP), but the morbidity, risk factors and outcome remain unclear. The aim of the present study was to evaluate the incidence of endocrine and exocrine pancreatic insufficiency after AP and the risk factors of endocrine pancreatic insufficiency through a long-term follow-up investigation. Follow-up assessment of the endocrine and exocrine function was conducted for the discharged patients with AP episodes. Oral Glucose Tolerance Test (OGTT) and faecal elastase-1(FE-1) test were used as primary parameters. Fasting blood-glucose (FBG), fasting insulin (FINS), glycosylated hemoglobin HBA1c, 2-h postprandial blood glucose (2hPG), Homa beta cell function index (HOMA-β), homeostasis model assessment of insulin resistance (HOMA-IR) and FE-1 were collected. Abdominal contrast-enhanced computed tomography (CECT) was performed to investigate the pancreatic morphology and the other related data during hospitalization was also collected. One hundred thirteen patients were included in this study and 34 of whom (30.1%) developed diabetes mellitus (DM), 33 (29.2%) suffered impaired glucose tolerance (IGT). Moreover, 33 patients (29.2%) developed mild to moderate exocrine pancreatic insufficiency with 100μg/gpancreatic insufficiency with FE-1pancreatic necrosis was significant higher than that in the non-pancreatic necrosis group (X 2  = 13.442,P = 0.001). The multiple logistic regression analysis showed that extent of pancreatic necrosisendocrine pancreatic insufficiency. HOMA-IR (P = 0.002, OR = 6.626), Wall-off necrosis (WON) (P = 0.013, OR = 184.772) were the risk factors. The integrated morbidity of DM and IGT after AP was 59.25%, which was higher than exocrine pancreatic insufficiency. 6.2% and 29.2% of patients developed severe and mild to moderate exocrine pancreatic insufficiency, respectively. The extent of pancreatic necrosis>50%, WON and insulin resistance were

Prognostic value of relative adrenal insufficiency after out-of-hospital cardiac arrest.

Science.gov (United States)

Pene, Frédéric; Hyvernat, Hervé; Mallet, Vincent; Cariou, Alain; Carli, Pierre; Spaulding, Christian; Dugue, Marie-Annick; Mira, Jean-Paul

2005-05-01

To assess the prevalence of relative adrenal insufficiency in patients successfully resuscitated after cardiac arrest, and its prognostic role in post-resuscitation disease. A prospective observational single-center study in a medical intensive care unit. 64 patients hospitalised in the intensive care unit after successful resuscitation for out-of-hospital cardiac arrest. A corticotropin-stimulation test was performed between 12 and 24 h following admission: serum cortisol level was measured before and 60 min after administration of tetracosactide 250 microg. Patients with an incremental response less than 9 microg/dl were considered to have relative adrenal insufficiency (non-responders). Variables were expressed as medians and interquartile ranges. 33 patients (52%) had relative adrenal insufficiency. Baseline cortisol level was higher in non-responders than in responders (41 [27.2-55.5] vs. 22.8 [15.7-35.1] microg/dl respectively, P=0.001). A long interval before initiation of cardiopulmonary resuscitation was associated with relative adrenal insufficiency (5 [3-10] vs. 3 [3-5] min, P=0.03). Of the 38 patients with post-resuscitation shock, 13 died of irreversible multiorgan failure. The presence of relative adrenal insufficiency was identified as a poor prognostic factor of shock-related mortality (log-rank P=0.02). A trend towards higher mortality in non-responders was identified in a multivariate logistic regression analysis (odds ratio 6.77, CI 95% 0.94-48.99, P=0.058). Relative adrenal insufficiency occurs frequently after successful resuscitation of out-of-hospital cardiac arrest, and appears to be associated with a poor prognosis in cases of post-resuscitation shock. The role of corticosteroid supplementation should be evaluated in this setting.

[Membranous nephropathy associated to autoimmune thyroiditis, chronic pancreatitis and suprarrenal insufficiency].

Science.gov (United States)

Merino, J L; Fernández Lucas, M; Teruel, J L; Valer, P; Moreira, V; Arambarri, M; Ortuño, J

2004-01-01

A 33 year old female was admitted to the hospital to study aedema and bocio, A nephrotic syndrome was diagnosed and the renal biopsy demonstrated membranous glomerulonephritis, stage II. She was also diagnosed of Hashimoto's autoinmmune thyroiditis: TSH (41.5 uUl/ml), T4 (0.07 ng/dl), antithyroglobuline (1/2560) and antimicrosome (1/6400). Four year latter she was diagnosed of autoinmmune pancreatitis, without evidence of diabetes mellitus or exocrine pancreatic insufficiency. Eight years latter she was diagnosed of primary autoimmune suprarrenal insufficiency: basal cortisol: 2.7 mcg/dl, post ACTH estimulated cortisol: 5.6 mcg/dl, antinuclear antibody (1/160) and antiparietal (1/320). We present a pluriglandular autoimmune syndrome with membranous glomerulonephritis, thyroiditis, pancreatitis and suprarrenal insufficiency. To the best of our knowledge this complex syndrome has not been previously described.

Subchondral insufficiency fracture of the knee: a non-traumatic injury with prolonged recovery time

OpenAIRE

Gourlay, Margaret L; Renner, Jordan B; Spang, Jeffrey T; Rubin, Janet E

2015-01-01

Subchondral insufficiency fractures are non-traumatic fractures that occur immediately below the cartilage of a joint. Although low bone density may be present concurrently, it is not the underlying cause of subchondral insufficiency fractures in the majority of patients. Patients with subchondral insufficiency fracture characteristically have unremarkable plain radiographs, while MRI examination may reveal extensive bone marrow oedema and subchondral bone collapse. This article presents a 51...

Zinc status in chronic pancreatitis and its relationship with exocrine and endocrine insufficiency.

Science.gov (United States)

Girish, Banavara Narasimhamurthy; Rajesh, Gopalakrishna; Vaidyanathan, Kannan; Balakrishnan, Vallath

2009-11-05

A major role of the pancreas in zinc homeostasis has been suggested. To assess erythrocyte zinc status in chronic pancreatitis and to correlate it with pancreatic exocrine and endocrine insufficiency. One hundred and one patients with chronic pancreatitis (34 alcoholic chronic pancreatitis, 67 tropical chronic pancreatitis) were prospectively studied. Disease characteristics and imaging features were recorded. Erythrocyte zinc was estimated by flame atomic absorption spectrophotometry. Exocrine insufficiency was assessed using polyclonal antibody ELISA for pancreatic stool elastase1. Endocrine insufficiency was assessed by serum glucose levels and insulin requirement. Erythrocyte zinc was significantly lower in chronic pancreatitis patients than in the controls (26.5+/-9.5 microg/g Hb vs. 38.0+/-6.6 microg/g Hb; Ppancreatitis than in alcoholic chronic pancreatitis (25.0+/-10.4 microg/g Hb vs. 29.6+/-6.5 microg/g Hb, P=0.001). In chronic pancreatitis patients who had exocrine insufficiency, erythrocyte zinc positively correlated with stool elastase1 (r=0.587, Ppancreatitis patients, and that zinc deficiency correlates with exocrine and endocrine insufficiency. Further studies may clarify the possible benefits of zinc supplementation in chronic pancreatitis.

Does Helicobacter pylori play a role in the pathogenesis of childhood chronic idiopathic thrombocytopenic purpura?

Directory of Open Access Journals (Sweden)

Maryam Maghbool

2009-07-01

Full Text Available Idiopathic thrombocytopenic purpura (ITP is an acute self-limited bleeding disorder that can progress to chronic form in 10-15% of the cases. Helicobacter pylori (H. pylori infection is a possible cause of chronic ITP. We studied 30 children with resistant chronic ITP for H. pylori infection based on the detection of H. pylori fecal antigen. This retrospective study was based on data obtained from medical records of 30 children aged between five and 17 years (median age at ITP diagnosis was ten years. A specially-designed data sheet was used to record information on age, sex, duration of disease, family history of bleeding disorders, previous treatments and median platelet count. In patients with H. pylori infection, antimicrobial treatment consisted of amoxicillin, metronidazol and omeprazol. Response was assessed every month for one year and defined as complete (platelet count >150x109/L or partial (platelet count between 50 and 150x109/L. We detected H. pylori infection in 5 patients. In 4 of them increased platelet count was seen during one year of follow-up and in one patient the platelet count was acceptable during six months. Although the pathological mechanism of H. pylori-induced thrombocytopenia was unclear in our patient sample, the assessment of H. pylori infection and use of eradication therapy should be attempted in chronic and resistant ITP patients.

Thrombotic Thrombocytopenic Purpura in Black People: Impact of Ethnicity on Survival and Genetic Risk Factors.

Directory of Open Access Journals (Sweden)

Suella Martino

Full Text Available Black people are at increased risk of thrombotic thrombocytopenic purpura (TTP. Whether clinical presentation of TTP in Black patients has specific features is unknown. We assessed here differences in TTP presentation and outcome between Black and White patients. Clinical presentation was comparable between both ethnic groups. However, prognosis differed with a lower death rate in Black patients than in White patients (2.7% versus 11.6%, respectively, P = .04. Ethnicity, increasing age and neurologic involvement were retained as risk factors for death in a multivariable model (P < .05 all. Sixty-day overall survival estimated by the Kaplan-Meier curves and compared with the Log-Rank test confirmed that Black patients had a better survival than White patients (P = .03. Salvage therapies were similarly performed between both groups, suggesting that disease severity was comparable. The comparison of HLA-DRB1*11, -DRB1*04 and -DQB1*03 allele frequencies between Black patients and healthy Black individuals revealed no significant difference. However, the protective allele against TTP, HLA-DRB1*04, was dramatically decreased in Black individuals in comparison with White individuals. Black people with TTP may have a better survival than White patients despite a comparable disease severity. A low natural frequency of HLA-DRB1*04 in Black ethnicity may account for the greater risk of TTP in this population.

Prevalence and correlates of insufficient physical activity in school adolescents in Peru.

Science.gov (United States)

Sharma, Bimala; Chavez, Rosemary Cosme; Nam, Eun Woo

2018-01-01

OBJECTIVE To assess the prevalence and correlates of insufficient physical activity in adolescents in Peru. METHODS We used a self-administered questionnaire developed from Global school-based Student Health Survey to collect information from secondary school students in North Lima and Callao in 2015. We carried out Poisson regression with robust variance using generalized linear models to estimate the crude and adjusted prevalence ratios (APR) with 95% confidence intervals (95%CI) of insufficient physical activity for its correlates. RESULTS We have found that 78% of the adolescents did not meet the global recommendation of the World Health Organization on physical activity in the last week before the survey. Female respondents (APR = 1.13, 95%CI 1.04-1.21), respondents who perceived themselves as overweight (APR = 1.10, 95%CI 1.03-1.18), and respondents who consumed insufficient vegetables and fruits [no vegetables (APR = 1.30, 95%CI 1.06-1.59), no fruits (APR = 1.15, 95%CI 1.00-1.31) as compared to those who consumed ≥ 2 servings every day in the last seven days] were more likely to report insufficient physical activity. Adolescents who worked after school (APR = 0.92, 95%CI 0.84-0.99), had physical education classes five times per week (APR = 0.94, 95%CI 0.88-0.99), and had parental supervision (APR = 0.92, 95%CI 0.87-0.98) were less likely to report insufficient physical activity. CONCLUSIONS Sex, work after school, perceived body weight, physical education class, parental support, and healthy dietary behaviors were associated with insufficient physical activity. Attempts to improve physical activity should look for ways to enhance leisure-time physical activity, parental support, physical education classes, healthy dietary behaviors, and normal body weight maintenance in adolescents with integrated efforts from the family and school.

Diabetes mellitus and exocrine pancreatic insufficiency (review of literature

Directory of Open Access Journals (Sweden)

L.T. Daminova

2018-02-01

Full Text Available Currently, an increasing importance is given to the study of the problem of exocrine pancreatic insufficiency, which is observed in a significant number of patients with diabetes mellitus (DM type 1 and 2 and can potentially affect the compensation of DM. The mechanism of reducing the external secretion of the pancreas in DM is associated with an imbalance of inhibitory and stimulating pancreatic secretion of hormones, with fibrosis of the gland as a result of diabetic angiopathy. In type 2 DM, the mechanisms that result from the metabolic syndrome are involved in the pathogenesis of exocrine pancreatic insufficiency. Enzyme replacement the­rapy should be considered as one of the promising methods of treating DM patients.

Post-traumatic tricuspid valve insufficiency. 2 cases of delayed clinical manifestation.

OpenAIRE

Bortolotti, U; Scioti, G; Milano, A; Guglielmi, C; Benedetti, M; Tartarini, G; Balbarini, A

1997-01-01

We present 2 cases of tricuspid insufficiency following blunt chest trauma: 1 was diagnosed 5 months after the trauma and the other, 20 years after the trauma. In both patients, the tricuspid valve was replaced with a porcine bioprosthesis, because valve repair was not considered feasible. These cases emphasize the variability of clinical presentation of post-traumatic tricuspid valve insufficiency and indicate the need for close follow-up of patients after major thoracic trauma.

Central Adrenal Insufficiency and Diabetes Insipidus Misdiagnosed as Severe Depression

Directory of Open Access Journals (Sweden)

Naoki Hiroi

2010-01-01

Full Text Available A 68 year-old Japanese man, who had been suffering from immobilization and disuse syndrome, was admitted to our hospital for evaluation of polyuria with polyposia, hyponatremia and low blood pressure. His plasma osmolality was greater than that of his urine. His endocrinological examination revealed low levels of plasma adrenocorticotropic hormone (ACTH and cortisol, and a normal response of ACTH to the corticotrophin-releasing hormone (CRH challenge. Plasma ACTH did not increase with insulin loading. A low plasma vasopressin (AVP level and no response of AVP to a 5% saline administration were observed. We diagnosed central adrenal insufficiency with central diabetes insipidus. Six months after starting administration of hydrocortisone and 1-deamino-8D-arginine vasopressin, his psychological symptoms had improved, and 1.5 years after starting treatment, he was able to walk. In conclusion, it is not particularly rare for adrenal insufficiency to be misdiagnosed as depression. However, a correct early diagnosis is necessary, because, if adrenal insufficiency is not definitively diagnosed, the patient's quality of life diminishes markedly.

Bedtime procrastination: a self-regulation perspective on sleep insufficiency in the general population

NARCIS (Netherlands)

Kroese, Floor|info:eu-repo/dai/nl/313869871; Evers, Catharine|info:eu-repo/dai/nl/280594232; Adriaanse, Marieke|info:eu-repo/dai/nl/304823023; de Ridder, Denise|info:eu-repo/dai/nl/070706174

2016-01-01

Getting insufficient sleep has serious consequences in terms of mental and physical health. The current study is the first to approach insufficient sleep from a self-regulation perspective by investigating the phenomenon of bedtime procrastination: going to bed later than intended, without having

Association of ACE, VEGF and CCL2 gene polymorphisms with Henoch-Schönlein purpura and an evaluation of the possible interaction effects of these loci in HSP patients.

Science.gov (United States)

Mohammadian, Tahereh; Bonyadi, Mortaza; Nabat, Elahe; Rafeey, Mandana

2017-07-01

Henoch-Schönlein purpura (HSP) is a multisystem, small vessel, leucocytoclastic vasculitis. It is predominantly a childhood vasculitis, rarely reported in adults. Studies have shown that several different genetic factors such as genes involved in inflammatory system and renin-angiotensin system (RAS) are important in the pathogenesis of Henoch-Schönlein purpura. The purpose of this study was to evaluate the independent effect of 3 gene polymorphisms including CCL2-2518 C/T, VEGF-634G/C and ACE(I/D) with HSP disease and their possible joint interactions in developing the disease. In this case-control study 47 HSP cases and 74 unrelated healthy controls were enrolled for evaluation. All individuals were genotyped for CCL2-2518C/T, VEGF-634G/C and ACE(I/D) gene polymorphisms. The possible association of these polymorphisms with susceptibility to develop HSP disease independently and in different joint combinations was evaluated. The frequencies of TT genotype and T allele of CCL2-2518C/T gene polymorphism and CC genotype and C allele of VEGF-634G/C gene polymorphism were significantly high in HSP children (p-values = 0.005 and = 0.007 respectively). Interestingly, studying the joint interaction of these 2 genotypes (CC genotype of VEGF G-634C and TT genotype of CCL2 C-2518T) in this cohort showed a more significant effect in the development of the disease (p gene when combined with II genotype of ACE gene in HSP children was significantly higher (p gene-gene interaction effects of CCL2, VEGF and ACE genes in developing HSP disease.

Adrenal insufficiency in a child with MELAS syndrome.

Science.gov (United States)

Afroze, Bushra; Amjad, Nida; Ibrahim, Shahnaz H; Humayun, Khadija Nuzhat; Yakob, Yusnita

2014-11-01

Mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (MELAS) are established subgroups of mitochondrial encephalomyopathy. m.3243A>G a common point mutation is detected in tRNA in majority of patients with MELAS phenotype whereas m.8344A>G point mutation in tRNA is observed, in MERRF phenotype. Adrenal insufficiency has not been reported in mitochondrial disease, except in Kearns-Sayre Syndrome (KSS), which is a mitochondrial deletion syndrome. We report an unusual presentation in a five year old boy who presented with clinical phenotype of MELAS and was found to have m.8344A>G mutation in tRNA. Addison disease was identified due to hyperpigmentation of lips and gums present from early childhood. This is the first report describing adrenal insufficiency in a child with MELAS phenotype. Copyright © 2014 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

Cocaine/levamisole-induced systemic vasculitis with retiform purpura and pauci-immune glomerulonephritis.

Science.gov (United States)

Veronese, F V; Dode, R S O; Friderichs, M; Thomé, G G; da Silva, D R; Schaefer, P G; Sebben, V C; Nicolella, A R; Barros, E J G

2016-01-01

Levamisole has been increasingly used as an adulterant of cocaine in recent years, emerging as a public health challenge worldwide. Levamisole-associated toxicity manifests clinically as a systemic vasculitis, consisting of cutaneous, hematological, and renal lesions, among others. Purpura retiform, cutaneous necrosis, intravascular thrombosis, neutropenia, and less commonly crescentic nephritis have been described in association with anti-neutrophil cytoplasmic antibodies (ANCAs) and other autoantibodies. Here we report the case of a 49-year-old male who was a chronic cocaine user, and who presented spontaneous weight loss, arthralgia, and 3 weeks before admission purpuric skin lesions in the earlobes and in the anterior thighs. His laboratory tests on admission showed serum creatinine of 4.56 mg/dL, white blood count 3,800/μL, hemoglobin 7.3 g/dL, urinalysis with 51 white blood cells/μL and 960 red blood cells/μL, and urine protein-to-creatinine ratio 1.20. Serum ANCA testing was positive (>1:320), as well as serum anti-myeloperoxidase and anti-proteinase 3 antibodies. Urine toxicology screen was positive for cocaine and levamisole, with 62.8% of cocaine, 32.2% of levamisole, and 5% of an unidentified substance. Skin and renal biopsies were diagnostic for leukocytoclastic vasculitis and pauci-immune crescentic glomerulonephritis, respectively. The patient showed a good clinical response to cocaine abstinence, and use of corticosteroids and intravenous cyclophosphamide. Last serum creatinine was 1.97 mg/dL, white blood cell count 7,420/μL, and hemoglobin level 10.8 g/dL. In levamisole-induced systemic vasculitis, the early institution of cocaine abstinence, concomitant with the use of immunosuppressive drugs in severe cases, may prevent permanent end organ damage and associate with better clinical outcomes.

Cocaine/levamisole-induced systemic vasculitis with retiform purpura and pauci-immune glomerulonephritis

Directory of Open Access Journals (Sweden)

F.V. Veronese

2016-01-01

Full Text Available Levamisole has been increasingly used as an adulterant of cocaine in recent years, emerging as a public health challenge worldwide. Levamisole-associated toxicity manifests clinically as a systemic vasculitis, consisting of cutaneous, hematological, and renal lesions, among others. Purpura retiform, cutaneous necrosis, intravascular thrombosis, neutropenia, and less commonly crescentic nephritis have been described in association with anti-neutrophil cytoplasmic antibodies (ANCAs and other autoantibodies. Here we report the case of a 49-year-old male who was a chronic cocaine user, and who presented spontaneous weight loss, arthralgia, and 3 weeks before admission purpuric skin lesions in the earlobes and in the anterior thighs. His laboratory tests on admission showed serum creatinine of 4.56 mg/dL, white blood count 3,800/μL, hemoglobin 7.3 g/dL, urinalysis with 51 white blood cells/μL and 960 red blood cells/μL, and urine protein-to-creatinine ratio 1.20. Serum ANCA testing was positive (>1:320, as well as serum anti-myeloperoxidase and anti-proteinase 3 antibodies. Urine toxicology screen was positive for cocaine and levamisole, with 62.8% of cocaine, 32.2% of levamisole, and 5% of an unidentified substance. Skin and renal biopsies were diagnostic for leukocytoclastic vasculitis and pauci-immune crescentic glomerulonephritis, respectively. The patient showed a good clinical response to cocaine abstinence, and use of corticosteroids and intravenous cyclophosphamide. Last serum creatinine was 1.97 mg/dL, white blood cell count 7,420/μL, and hemoglobin level 10.8 g/dL. In levamisole-induced systemic vasculitis, the early institution of cocaine abstinence, concomitant with the use of immunosuppressive drugs in severe cases, may prevent permanent end organ damage and associate with better clinical outcomes.

Mild cognitive deficits in patients with primary adrenal insufficiency.

Science.gov (United States)

Tiemensma, Jitske; Andela, Cornelie D; Biermasz, Nienke R; Romijn, Johannes A; Pereira, Alberto M

2016-01-01

The brain is a major target organ for cortisol considering its high density of glucocorticoid receptors. Several states of hypothalamus-pituitary-adrenal dysregulation point towards impairments in cognitive functioning. However, there is a very limited body of research on the effects of hypocortisolism on cognitive functioning. To evaluate cognitive functioning in patients with hypocortisolism (i.e., primary adrenal insufficiency (PAI)) and to examine the possible effect of postponing early-morning hydrocortisone intake on cognitive functioning. Thirty-one patients with PAI on regular morning hydrocortisone intake and 31 healthy matched controls underwent nine neuropsychological tests, evaluating memory and executive functioning. In addition, the effect of normal timing and postponement of morning hydrocortisone intake on neuropsychological tests were assessed in an additional 29 patients with PAI. Compared to controls, patients with PAI performed worse on auditory and visual memory tasks (all P ≤ 0.024) and executive functioning tasks (all P ≤ 0.012). In contrast, patients performed better on a concentration and an attention task (both Paffect the outcomes of neuropsychological tests. Patients on long-term hydrocortisone replacement for PAI show mild cognitive deficits compared to controls. There was no effect of postponement of regular hydrocortisone intake on cognition. Copyright © 2015 Elsevier Ltd. All rights reserved.

Clinical significance of gamma camera renography in chronic renal insufficiency

International Nuclear Information System (INIS)

Dudczak, R.; Frischauf, H.; Kletter, K.

1980-01-01

Gamma camera renography allows, together with renal imaging, an evaluation of individual renal function. For these examinations, I 123 orthoiodohippurate is preferred and most widely used. The results on patients with chronic renal insufficiency, including urologic and posttransplantation patients, are reported. Whereas the method is of clinical significance in evaluating posttransplantation complications and in assessing individual kidney function preoperatively in urology, as well as in monitoring therapeutic effects in the early period of renal disease, it is of limited diagnostic value in chronic renal insufficiency. In this latter regard, clinical and laboratory examinations are of primary importance. (author)

Percutaneous treatment of insufficiency fractures. Principles, technique and review of literature

International Nuclear Information System (INIS)

Beall, Douglas P.; Datir, Abhijit; D'Souza, Sharon L.; D'Souza, Logan S.; Gunda, Divya; Morelli, John; Johnson, Michael Brandon; Nabavizadeh, Nima

2010-01-01

Insufficiency fractures of the pelvis, sacrum, spine, and long bones are painful, debilitating, and are common consequences of osteoporosis. Conventional treatment for these fractures varies from conservative therapy to surgery with plate and screw fixation. The former fails to address the underlying problem of fracture and frequently does not alleviate symptoms, while the latter is invasive and not always possible in older populations with low bone density and numerous co-morbidities. Osseous augmentation with polymethylmethacrylate (PMMA) has been used for over two decades to treat fractures related to osteoporosis, but has not been commonly used to treat fractures outside of the vertebral bodies. Osseous augmentation with PMMA is an image-guided procedure and various techniques have been utilized to treat fracture in different locations. We describe various techniques for image-guided osseous augmentation and treatment of insufficiency fractures with bothPMMA and allograft bone for fractures of the pelvis including sacrum, acetabulum, pubic symphysis, pubic rami ilium; appendicular skeleton including distal radius, proximal femur, and vertebral body. We also describe the potential risks and complications associated with percutaneous treatment of insufficiency fractures and techniques to avoid the pitfalls of the various procedures. We will present the process for patient follow-up and data regarding the pre- and postprocedure pain response in patients undergoing treatment for pelvic insufficiency fractures. (orig.)

Prevalence and Predictors of Functional Vitamin K Insufficiency in Mothers and Newborns in Uganda

Directory of Open Access Journals (Sweden)

Data Santorino

2015-10-01

Full Text Available Vitamin K deficiency bleeding (VKDB in infancy is a serious but preventable cause of mortality or permanent disability. Lack of epidemiologic data for VKDB in sub-Saharan Africa hinders development and implementation of effective prevention strategies. We used convenience sampling to consecutively enroll mothers delivering in a southwestern Uganda Hospital. We collected socio-demographic and dietary information, and paired samples of maternal venous and neonatal cord blood for the immunoassay of undercarboxylated prothrombin (PIVKA-II, a sensitive marker of functional vitamin K (VK insufficiency. We used univariable and multivariable logistic regression models to identify predictors of VK insufficiency. We detected PIVKA-II of ≥0.2 AU (Arbitrary Units per mL/mL (indicative of VK insufficiency in 33.3% (47/141 of mothers and 66% (93/141 of newborns. Importantly, 22% of babies had PIVKA-II concentrations ≥5.0 AU/mL, likely to be associated with abnormal coagulation indices. We found no significant predictors of newborn VK insufficiency, including infant weight (AOR (adjusted odds ratio 1.85, 95% CI (confidence interval 0.15–22.49, gender (AOR 0.54, 95% CI 0.26–1.11, term birth (AOR 0.72, 95% CI 0.20–2.62, maternal VK-rich diet (AOR 1.13, 95% CI 0.55–2.35 or maternal VK insufficiency (AOR 0.99, 95% CI 0.47–2.10. VK insufficiency is common among mothers and newborn babies in southwestern Uganda, which in one fifth of babies nears overt deficiency. Lack of identifiable predictors of newborn VK insufficiency support strategies for universal VK prophylaxis to newborns to prevent VKDB.

Children Receiving Free or Reduced-Price School Lunch Have Higher Food Insufficiency Rates in Summer.

Science.gov (United States)

Huang, Jin; Barnidge, Ellen; Kim, Youngmi

2015-09-01

In 2012, 20% of households in the United States with children lacked consistent access to adequate food. Food insufficiency has significant implications for children, including poor physical and mental health outcomes, behavior problems, and low educational achievements. The National School Lunch Program (NSLP) is one policy solution to reduce food insufficiency among children from low-income families. The objective of this project was to evaluate the association between NSLP participation and household food insufficiency by examining trajectories of food insufficiency over 10 calendar months. The calendar months included both nonsummer months when school is in session and summer months when school is out of session. The study used the data from the Survey of Income and Program Participation and conducted linear growth curve analyses in the multilevel modeling context. Comparisons were made between the trajectories of food insufficiencies among recipients of free or reduced-price lunch and their counterparts who are eligible but choose not to participate in the program. Heads of households that included children receiving free or reduced-price lunch (n = 6867) were more likely to be female, black, unmarried, and unemployed, and have a lower educational attainment than those whose children were eligible but did not receive free or reduced-price lunch (n = 11,396). For households participating in the NSLP, the food insufficiency rate was consistent from January to May at ∼4%, and then increased in June and July to >5%. Meanwhile, food insufficiency among eligible nonrecipients was constant throughout the year at nearly 2%. The NSLP protects households from food insufficiency. Policies should be instituted to make enrollment easier for households. © 2015 American Society for Nutrition.

Spontaneous osteonecrosis of the knee associated with tibial plateau and femoral condyle insufficiency stress fracture

Energy Technology Data Exchange (ETDEWEB)

Narvaez, J.A.; Narvaez, J.; Lama, E.De; Sanchez, A. [Department of Magnetic Resonance Imaging, IDI Hospital Duran i Reynals, Ciutat Sanitaria i Universitaria de Bellvitge, Gran Via s/n, 08907, L' Hospitalet de Llobregat (Barcelona) (Spain)

2003-08-01

The purpose of this article is to describe the association between spontaneous osteonecrosis and insufficiency stress fractures of the knee. To determine whether insufficiency stress fracture is associated with spontaneous osteonecrosis of the knee, we retrospectively reviewed the medical charts and imaging studies of all patients with spontaneous osteonecrosis of the knee, studied by MR imaging, seen in a tertiary hospital over an 8-year period. Four women (age range 66-84 years) presented spontaneous osteonecrosis of the knee associated with insufficiency stress fracture of the medial tibial plateau. One of these patients also presented a concomitant insufficiency stress fracture of the medial femoral condyle. Radiographs were diagnostic of spontaneous osteonecrosis of the medial femoral condyle in three cases, and insufficiency stress fracture of the medial tibial plateau was detected in one case. Magnetic resonance imaging allows the diagnosis of both conditions in all four cases. Spontaneous osteonecrosis of the knee may be associated with insufficiency stress fracture of the medial femoral condyle and the medial tibial plateau. This association provides additional arguments in favor of the traumatic etiology of spontaneous osteonecrosis of knee. (orig.)

Spontaneous osteonecrosis of the knee associated with tibial plateau and femoral condyle insufficiency stress fracture

International Nuclear Information System (INIS)

Narvaez, J.A.; Narvaez, J.; Lama, E.De; Sanchez, A.

2003-01-01

The purpose of this article is to describe the association between spontaneous osteonecrosis and insufficiency stress fractures of the knee. To determine whether insufficiency stress fracture is associated with spontaneous osteonecrosis of the knee, we retrospectively reviewed the medical charts and imaging studies of all patients with spontaneous osteonecrosis of the knee, studied by MR imaging, seen in a tertiary hospital over an 8-year period. Four women (age range 66-84 years) presented spontaneous osteonecrosis of the knee associated with insufficiency stress fracture of the medial tibial plateau. One of these patients also presented a concomitant insufficiency stress fracture of the medial femoral condyle. Radiographs were diagnostic of spontaneous osteonecrosis of the medial femoral condyle in three cases, and insufficiency stress fracture of the medial tibial plateau was detected in one case. Magnetic resonance imaging allows the diagnosis of both conditions in all four cases. Spontaneous osteonecrosis of the knee may be associated with insufficiency stress fracture of the medial femoral condyle and the medial tibial plateau. This association provides additional arguments in favor of the traumatic etiology of spontaneous osteonecrosis of knee. (orig.)

Pentoxifylline in the treatment of radiation-related pelvic insufficiency fractures of bone

International Nuclear Information System (INIS)

Bese, N.S.; Oezgueroglu, M.; Kamberoglu, K.; Karahasanoglu, T.; Oeber, A.

2003-01-01

The reported incidence of bone complications after radiation therapy is quite low. The most commonly seen bone complication is insufficiency fractures of the pubis and sacrum. Treatment of insufficiency fractures consists of conservative care, and mineral replacement may be useful. The resolution of symptoms takes at least one year with these treatments. Vascular damage has an important role in the etiology of late radiation injury in normal tissues. Progressive ischemic changes further weaken the bone structure, which can cause fractures, and healing is also delayed. Pentoxifylline is a methylxanthine derivative that is shown to increase tissue blood flow. Here, we present a 63-year-old male patient with pelvic insufficiency fractures due to postoperative pelvic irradiation for rectal adenocarcinoma. The patient received pelvic radiotherapy to a total dose of 50.4 Gy with concomitant 5-FU. Six months after the completion of radiotherapy, the patient presented with severe pelvic pain. Pelvic magnetic resonance imaging (MRI) demonstrated abnormal signal intensity with insufficiency fractures at the sacrum and bone marrow edema near the fractures, but not an abnormal intensity that revealed bone metastases. Neither distant nor locoregional recurrence was observed at his work-up. The final diagnosis was insufficiency fractures of the pelvic bones owing to irradiation, and pentoxifylline (400 mg, 3 times daily, peroral, 1,200 mg/day) was used for eight months as treatment. Dramatic clinical improvement was obtained in six months, and objective healing was revealed with MRI. We concluded that pentoxifylline is a cost-effective drug with minimal adverse effects in treating radiation damage of bone. (author)

Female androgen insufficiency: the Princeton consensus statement on definition, classification, and assessment.

Science.gov (United States)

Bachmann, Gloria; Bancroft, John; Braunstein, Glenn; Burger, Henry; Davis, Susan; Dennerstein, Lorraine; Goldstein, Irwin; Guay, Andre; Leiblum, Sandra; Lobo, Rogerio; Notelovitz, Morris; Rosen, Raymond; Sarrel, Philip; Sherwin, Barbara; Simon, James; Simpson, Evan; Shifren, Jan; Spark, Richard; Traish, Abdul

2002-04-01

To evaluate the evidence for and against androgen insufficiency as a cause of sexual and other health-related problems in women and to make recommendations regarding definition, diagnosis, and assessment of androgen deficiency states in women. Evaluation of peer-review literature and consensus conference of international experts. Multinational conference in the United States. Premenopausal and postmenopausal women with androgen deficiency. Evaluation of peer-review literature and development of consensus panel guidelines. The term "female androgen insufficiency" was defined as consisting of a pattern of clinical symptoms in the presence of decreased bioavailable T and normal estrogen status. Currently available assays were found to be lacking in sensitivity and reliability at the lower ranges, and the need for an equilibrium dialysis measure was strongly emphasized. Causes of androgen insufficiency in women were classified as ovarian, adrenal, hypothalamic-pituitary, drug-related, and idiopathic. A simplified management algorithm and clinical guidelines were proposed to assist clinicians in diagnosis and assessment. Androgen replacement is currently available in several forms, although none has been approved for treatment of sexual dysfunction or other common symptoms of female androgen insufficiency. Potential risks associated with treatment were identified, and the need for informed consent and careful monitoring was noted. Finally, the panel identified key goals and priorities for future research. A new definition of androgen insufficiency in women has been proposed along with consensus-based guidelines for clinical assessment and diagnosis. A simplified management algorithm for women with low androgen in the presence of clinical symptoms and normal estrogen status has also been proposed.

Many Routes to an Antibody Heavy-Chain CDR3: Necessary, Yet Insufficient, for Specific Binding

Science.gov (United States)

D’Angelo, Sara; Ferrara, Fortunato; Naranjo, Leslie; Erasmus, M. Frank; Hraber, Peter; Bradbury, Andrew R. M.

2018-01-01

Because of its great potential for diversity, the immunoglobulin heavy-chain complementarity-determining region 3 (HCDR3) is taken as an antibody molecule’s most important component in conferring binding activity and specificity. For this reason, HCDR3s have been used as unique identifiers to investigate adaptive immune responses in vivo and to characterize in vitro selection outputs where display systems were employed. Here, we show that many different HCDR3s can be identified within a target-specific antibody population after in vitro selection. For each identified HCDR3, a number of different antibodies bearing differences elsewhere can be found. In such selected populations, all antibodies with the same HCDR3 recognize the target, albeit at different affinities. In contrast, within unselected populations, the majority of antibodies with the same HCDR3 sequence do not bind the target. In one HCDR3 examined in depth, all target-specific antibodies were derived from the same VDJ rearrangement, while non-binding antibodies with the same HCDR3 were derived from many different V and D gene rearrangements. Careful examination of previously published in vivo datasets reveals that HCDR3s shared between, and within, different individuals can also originate from rearrangements of different V and D genes, with up to 26 different rearrangements yielding the same identical HCDR3 sequence. On the basis of these observations, we conclude that the same HCDR3 can be generated by many different rearrangements, but that specific target binding is an outcome of unique rearrangements and VL pairing: the HCDR3 is necessary, albeit insufficient, for specific antibody binding. PMID:29568296

Pelvic insufficiency fractures associated with radiation atrophy: clinical recognition and diagnostic evaluation

International Nuclear Information System (INIS)

Mumber, M.P.; Greven, K.M.; Haygood, T.M.

1997-01-01

Pelvic bone injuries are infrequent complications of radiotherapy. However, insufficiency fractures in irradiated pelvic bones may be underdetected, particularly in postmenopausal women. We describe the clinical presentation, radiologic evaluation, and course of disease in three patients with postradiation pelvic insufficiency fractures. Differential diagnosis included metastatic disease, tumor recurrence, and second malignancy. Recognition of radiographic features may prevent unnecessary, possibly morbid treatments. (orig.). With 6 figs

Vitamin D insufficiency in the elderly orthopaedic patient: an epidemic phenomenon.

Science.gov (United States)

Maier, Gerrit Steffen; Horas, Konstantin; Seeger, Jörn Bengt; Roth, Klaus Edgar; Kurth, Andreas Alois; Maus, Uwe

2015-04-01

The purpose of this observational study was to evaluate serum levels of 25-OH-D of elderly patients presenting with orthopaedic illness. Furthermore, we enquired about potential confounders and risk factors of hypovitaminosis D in comorbidities and daily medication of the elderly. Vitamin D levels in 1,083 patients aged >70 years and admitted to an orthopaedic surgery department were measured. Univariate and multivariate analyses were used to assess risk factors for insufficient vitamin D levels. Overall, 86 % of patients had insufficient serum levels of 25-OH-D and >60 % were vitamin D deficient. Serum vitamin D levels were lower during winter and months with fewer sunshine hours. Patients presenting with obesity, hypertension and osteoporosis were more likely to have low vitamin D levels. We found a high prevalence of hypovitaminosis D in elderly, nonhospitalized orthopaedic patients. Given the well-known effects of vitamin D on bone metabolism and muscle health, as well as its nonskeletal effects, vitamin D insufficiency may have a negative impact.

Prevalence and effects of functional vitamin K insufficiency

NARCIS (Netherlands)

Riphagen, Ineke J.; Keyzer, Charlotte A.; Drummen, Nadja E.A.; Borst, de Martin H.; Beulens, Joline W.J.; Gansevoort, Ron T.; Geleijnse, Johanna M.; Muskiet, Frits A.J.; Navis, Gerjan; Visser, Sipke T.; Vermeer, Cees; Kema, Ido P.; Bakker, Stephan J.L.

2017-01-01

Matrix Gla Protein (MGP) is a strong vitamin K-dependent inhibitor of soft tissue calcification. We assessed the prevalence of functional vitamin K insufficiency, as derived from plasma desphospho-uncarboxylated MGP (dp-ucMGP), and investigated whether plasma dp-ucMGP is associated with all-cause

Health consequences of shift work and insufficient sleep

NARCIS (Netherlands)

Kecklund, L.G.; Axelsson, J.

2016-01-01

This review summarises the literature on shift work and its relation to insufficient sleep, chronic diseases, and accidents. It is based on 38 meta-analyses and 24 systematic reviews, with additional narrative reviews and articles used for outlining possible mechanisms by which shift work may cause

Vitamin D deficiency and insufficiency among patients with prostate cancer.

Science.gov (United States)

Trump, Donald L; Chadha, Manpreet K; Sunga, Annette Y; Fakih, Marwan G; Ashraf, Umeer; Silliman, Carrie G; Hollis, Bruce W; Nesline, Mary K; Tian, Lili; Tan, Wei; Johnson, Candace S

2009-10-01

To assess the frequency of vitamin D deficiency among men with prostate cancer, as considerable epidemiological, in vitro, in vivo and clinical data support an association between vitamin D deficiency and prostate cancer outcome. The study included 120 ambulatory men with recurrent prostate cancer and 50 with clinically localized prostate cancer who were evaluated and serum samples assayed for 25-OH vitamin D levels. Then 100 controls (both sexes), matched for age and season of serum sample, were chosen from a prospective serum banking protocol. The relationship between age, body mass index, disease stage, Eastern Cooperative Oncology Group performance status, season and previous therapy on vitamin D status were evaluated using univariate and multivariate analyses. The mean 25-OH vitamin D level was 25.9 ng/mL in those with recurrent disease, 27.5 ng/mL in men with clinically localized prostate cancer and 24.5 ng/mL in controls. The frequency of vitamin D deficiency (<20 ng/mL) and insufficiency (20-31 ng/mL) was 40% and 32% in men with recurrent prostate; 28% had vitamin D levels that were normal (32-100 ng/mL). Among men with localized prostate cancer, 18% were deficient, 50% were insufficient and 32% were normal. Among controls, 31% were deficient, 40% were insufficient and 29% were normal. Metastatic disease (P = 0.005) and season of blood sampling (winter/spring; P = 0.01) were associated with vitamin D deficiency in patients with prostate cancer, while age, race, performance status and body mass index were not. Vitamin D deficiency and insufficiency were common among men with prostate cancer and apparently normal controls in the western New York region.

Obstructed pancreaticojejunostomy partly explains exocrine insufficiency after pancreatic head resection.

Science.gov (United States)

Nordback, Isto; Parviainen, Mickael; Piironen, Anneli; Räty, Sari; Sand, Juhani

2007-02-01

The majority of patients with long-term survival after pancreatic head resection suffer from pancreatic exocrine insufficiency. The objective of this study was to investigate whether this is due to glandular malfunction or obstructed pancreaticojejunal anastomosis. Twenty-six patients (10 M, 16 F, mean age 61 years, range 34-81 years) were re-examined a median of 52 months (range 3-76 months) after pancreatic head resection and end-to-end invaginated pancreaticojejunostomy. Pancreatic exocrine function was measured by fecal elastase-1 assay. The size of the pancreatic remnant, glandular secretion and the flow through the anastomosis were analyzed with secretin-stimulated dynamic magnetic resonance pancreatography (D-MRP). All patients had pancreatic exocrine insufficiency, 24 (92%) of them having severe insufficiency. Eighteen patients (69%) reported moderate to severe diarrhea. Lowest fecal elastase-1 concentrations were associated with the initial diagnosis of chronic pancreatitis or ductal adenocarcinoma, suggesting preoperative primary or secondary chronic pancreatitis as important determinants. The size of the remnant gland did not correlate with the fecal elastase-1 concentrations. D-MRP failed in three patients. Severe glandular malfunctions were found in 7 (30%) of the 23 successful D-MRP examinations. The anastomosis was totally obstructed in 5 patients (22%) or partially obstructed in 6 (26%) but remained perfectly open in 5 patients (22%). The five patients with perfect anastomoses had the highest measured median fecal elastase-1 activity. Although late diarrhea and pancreatic exocrine insufficiency may be partly induced already by the disease treated with resection, at least half may be explained by obstructed anastomosis. To obtain better late functional results, improvements may be required in the surgical techniques.

Relative adrenal insufficiency in post-transplant lymphoproliferative disorder.

Directory of Open Access Journals (Sweden)

Cinclair R

2003-01-01

Full Text Available Post-transplant lymphoproliferative disorder is treated with rapid decrement of immunosuppressive therapy. This cannot be achieved with ease in patients on long-term glucocorticoid therapy, as chronically suppressed adrenal glands may not be capable of mounting adequate response to stress. A 52-year-old Caucasian male presented with fever, orthostatic hypotension, lymphadenopathy and hyponatraemia. Serum cortisol levels were within normal levels with a sub optimal response to stimulation by ACTH. Hyponatraemia and orthostasis responded poorly to fluid restriction, saline and salt repletion but corrected after increasing the steroid dose. The normal baseline cortisol levels represented a stimulated adrenal gland, however, the ACTH stimulation had inadequate response. This sub optimal stimulation and a good response to increased steroids suggest the presence of relative or occult adrenal insufficiency. Relative adrenal insufficiency must be considered in patients who have received prolonged glucocorticoid therapy and have symptoms such as hypotension and/or hyponatraemia.

Recurrence rates after abdominal and vaginal cerclages in women with cervical insufficiency

DEFF Research Database (Denmark)

Sneider, Kirstine; Christiansen, Ole Bjarne; Sundtoft, Iben Blaabjerg

2017-01-01

PURPOSE: To estimate the incidence of cervical insufficiency and compare recurrence rates of second trimester miscarriage/delivery in second and third pregnancies after prophylactic vaginal cerclage, abdominal cerclage, or no cerclage. METHODS: Retrospective chart review of a representative...... register-based sample of 621 women with a spontaneous second trimester miscarriage/delivery in the first pregnancy in Denmark (1997-2012). We used strict criteria for the diagnosis of cervical insufficiency. The outcome of subsequent pregnancies was described by treatment with prophylactic vaginal cerclage......, abdominal cerclage, or no prophylactic cerclage. Women were followed until June 2015. RESULTS: Of 621 women, 149 (24%) fulfilled the strict criteria of cervical insufficiency. Prophylactic treatment with abdominal cerclage (n = 20), vaginal cerclage (n = 59), and no prophylactic cerclage (n = 61) resulted...

Mathematical models for the study of the dynamics of indium-111-labelled platelets in idiopathic thrombocytopenic purpura

International Nuclear Information System (INIS)

Savolainen, S.

1992-01-01

Platelet kinetics in patients with idiopathic thrombocytopenic purpura (ITP) was investigated by applying various models (compartmental and open models, and functional and uptake analyses) to data on indium-111 labelled platelets monitored with a gamma camera following intravenous injection of labelled platelets. The usefulness of the selected models was tested by relating kinetic data to pathophysiological phenomena. A comparison of the results of platelet and colloid kinetics showed that the splenic platelet kinetics in ITP patients does not seem to be primarily dependent on the reticuloendothelial system. Although closed three-compartmental analysis seemed to be superior to the other models applied, none of the methods of analysis tested in this study appears to provide a complete description of short-lived platelet dynamics, as for every model certain assumptions that are not entirely relevant have to be made; this stresses the importance of combining various methods for a comprehensive analysis of a complex phenomenon. Conclusions concerning the function of biological systems should be based on more than one dynamic model or calculation method, since applying only one model (or calculation method) may give artifactual results due to poor statistics of observed data or to inexactness of the assumptions concerning the model

VENOUS INSUFFICIENCY AND THROMBOEMBOLIC DISEASE IN BARIATRIC SURGERY PATIENTS

Directory of Open Access Journals (Sweden)

Bonno van BELLEN

2013-09-01

Full Text Available Context Morbid obesity is associated with various co-morbidities, including chronic venous insufficiency. Bariatric surgery is the only effective treatment for morbid obesity, but with potential risks and possible complications, including venous thromboembolism. Objective To determine the prevalence of clinical and ultrasonographic signs of chronic venous insufficiency in morbid obese patients in preparation for bariatric surgery and the incidence of post-operative venous thromboembolic disease. Methods Patients on work-up for bariatric surgery of Centro Terapêutico Especializado em Fígado (CETEFI and Pro-Gastro surgical teams of the Hospital Beneficência Portuguesa de São Paulo were included. The analysed data were pre-operative findings for venous insufficiency (CEAP - clinical, etiological, anatomical, physiopathologic - classification and venous ultrassonographic findings, type of surgery (open or laparoscopic, abdominal circumference, body mass index (BMI and post-operative ultrassonography search for venous insufficiency and deep venous thrombosis. Results Between March 2007 and December 2009, 95 patients candidates for bariatric surgery had clinical and duplex scan evaluation of the lower limbs venous system. Of the 95 patients, 53 were submitted to the surgical procedure. There was a predominance of women (77.9%, the average age was 38.5 years, average preoperative weight 124.6 kg and average BMI of 45.5 kg/m2. Regarding obesity, 16.8% were obese, and 83.1% were morbidly obese. In relation to the venous findings, 86.3% of the patients did fit CEAP classification less than 3 and 13.7% greater than or equal to 3. Among the post-operative complications, there were four cases of wound infection. Three patients developed post-operative distal venous thrombosis (7.5%, but no one had clinically manifested pulmonary embolism. Conclusion No relation between BMI, CEAP classification and venous ultrassonographic findings were found. Although

Acute disseminated melioidosis giving rise to pneumonia and renal abscesses complicated with thrombotic thrombocytopenic purpura in a post partum woman: a case report.

Science.gov (United States)

Wijewickrama, Piyumi Sachindra Alwis; Weerakoon, Rohini

2017-11-29

Melioidosis is an established endemic infection in Sri Lanka, caused by Burkholderia pseudomallei, a gram negative bacterium distributed in saprophytes in soil and surface water. Main mode of transmission is via percutaneous inoculation. Pneumonia is the most common presentation in acute disease. We report a 33 year old previously healthy Sinhalese female with an occupational exposure to surface water in paddy fields, who was on postpartum day 6 following an uncomplicated pregnancy and delivery via an elective caesarian section. She presented with a 1 day history of breathlessness, preceded by a brief episode of fever. She had occasional right side coarse crackles and pitting oedema of both lower limbs. Shortly after admission, she developed type one respiratory failure needing invasive mechanical ventilation. Initial chest x-ray revealed slight obliteration of right medial diaphragmatic border while echocardiogram revealed moderate pulmonary hypertension. Computed tomography pulmonary angiogram excluded a pulmonary embolism, but revealed bilateral multi-lobar consolidation. Abdominal computed tomography demonstrated bilateral pyelonephritis with renal abscesses. As initial cultures were inconclusive, melioidosis antibody levels were done due to high degree of suspicion, which was found to be positive with a titer of 1:2560. A diagnosis of melioidosis was made based on the suggestive clinical picture, exposure history and the highly positive antibody level. She developed left side focal seizures together with thrombocytopenia and microangiopathic haemolytic anemia, suggestive of thrombotic thrombocytopenic purpura. Magnetic resonance imaging of brain was negative for cerebral abscesses but revealed extensive minute haemorrhagic foci throughout the cerebrum. Thus, the final diagnosis was acute melioidosis causing pneumonia and renal abscesses, complicated with thrombotic thrombocytopenic purpura and sepsis. She demonstrated dramatic response to high dose meropenem

Insufficient free-time physical activity and occupational factors in Brazilian public school teachers

Directory of Open Access Journals (Sweden)

Douglas Fernando Dias

Full Text Available ABSTRACT OBJECTIVE To evaluate if perceived occupational factors are associated with insufficient free-time physical activity in Brazilian public school teachers. METHODS The relationship between insufficient physical activity (< 150 minutes/week and variables related to work was analyzed in 978 elementary and high school teachers calculating the prevalence ratio (PR and 95% confidence interval (95%CI in Poisson regression models, adjusted for sociodemographic and health variables. RESULTS The prevalence of insufficient physical activity was 71.9%, and this condition was associated independently with the perception of bad or regular balance between personal and professional life (PR = 1.09; 95%CI 1.01–1.18, perception that standing time affects the work (PR = 1.16; 95%CI 1.01–1.34, low or very low perception of current ability for the physical requirements of work (PR = 1.21; 95%CI 1.08–1.35, and temporary employment contract (PR = 1.13; 95%CI 1.03–1.25. The teaching of physical education was associated with lower prevalence of insufficient physical activity (PR = 0.78; 95%CI 0.64–0.95. CONCLUSIONS The perception of adverse working conditions is associated with increased prevalence of insufficient physical activity in teachers and should be considered for the promotion of physical activity in this population.

Ticlopidine-, clopidogrel-, and prasugrel-associated thrombotic thrombocytopenic purpura: a 20-year review from the Southern Network on Adverse Reactions (SONAR).

Science.gov (United States)

Jacob, Sony; Dunn, Brianne L; Qureshi, Zaina P; Bandarenko, Nicholas; Kwaan, Hau C; Pandey, Dilip K; McKoy, June M; Barnato, Sara E; Winters, Jeffrey L; Cursio, John F; Weiss, Ivy; Raife, Thomas J; Carey, Patricia M; Sarode, Ravindra; Kiss, Joseph E; Danielson, Constance; Ortel, Thomas L; Clark, William F; Rock, Gail; Matsumoto, Masanori; Fujimura, Yoshihiro; Zheng, X Long; Chen, Hao; Chen, Fei; Armstrong, John M; Raisch, Dennis W; Bennett, Charles L

2012-11-01

Thienopyridine-derivatives (ticlopidine, clopidogrel, and prasugrel) are the primary antiplatelet agents. Thrombotic thrombocytopenic purpura (TTP) is a rare drug-associated syndrome, with the thienopyridines being the most common drugs implicated in this syndrome. We reviewed 20 years of information on clinical, epidemiologic, and laboratory findings for thienopyridine-associated TTP. Four, 11, and 11 cases of thienopyridine-associated TTP were reported in the first year of marketing of ticlopidine (1989), clopidogrel (1998), and prasugrel (2010), respectively. As of 2011, the FDA received reports of 97 ticlopidine-, 197 clopidogrel-, and 14 prasugrel-associated TTP cases. Severe deficiency of ADAMTS-13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) was present in 80% and antibodies to 100% of these TTP patients on ticlopidine, 0% of the patients with clopidogrel-associated TTP (p < 0.05), and an unknown percentage of patients with prasugrel-associated TTP. TTP is associated with use of each of the three thienopyridines, although the mechanistic pathways may differ. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

News about the genetics of congenital primary adrenal insufficiency.

Science.gov (United States)

Roucher-Boulez, Florence; Mallet-Motak, Delphine; Tardy-Guidollet, Véronique; Menassa, Rita; Goursaud, Claire; Plotton, Ingrid; Morel, Yves

2018-04-13

Primary adrenal insufficiency (PAI) is characterized by impaired production of steroid hormones due to an adrenal cortex defect. This condition incurs a risk of acute insufficiency which may be life-threatening. Today, 80% of pediatric forms of PAI have a genetic origin but 5% have no clear genetic support. Recently discovered mutations in genes relating to oxidative stress have opened the way to research on genes unrelated to the adrenal gland. Identification of causal mutations in a gene responsible for PAI allows genetic counseling, guidance of follow-up and prevention of complications. This is particularly true for stress oxidative anomalies, as extra-adrenal manifestations may occur due to the sensitivity to oxidative stress of other organs such as the heart, thyroid, liver, kidney and pancreas. Copyright © 2018 Elsevier Masson SAS. All rights reserved.

Population-Level Incidence and Predictors of Surgically Induced Diabetes and Exocrine Insufficiency after Partial Pancreatic Resection.

Science.gov (United States)

Elliott, Irmina A; Epelboym, Irene; Winner, Megan; Allendorf, John D; Haigh, Philip I

2017-01-01

Endocrine and exocrine insufficiency after partial pancreatectomy affect quality of life, cardiovascular health, and nutritional status. However, their incidence and predictors are unknown. To identify the incidence and predictors of new-onset diabetes and exocrine insufficiency after partial pancreatectomy. We retrospectively reviewed 1165 cases of partial pancreatectomy, performed from 1998 to 2010, from a large population-based database. Incidence of new onset diabetes and exocrine insufficiency RESULTS: Of 1165 patients undergoing partial pancreatectomy, 41.8% had preexisting diabetes. In the remaining 678 patients, at a median 3.6 months, diabetes developed in 274 (40.4%) and pancreatic insufficiency developed in 235 (34.7%) patients. Independent predictors of new-onset diabetes were higher Charlson Comorbidity Index (CCI; hazard ratio [HR] = 1.62 for CCI of 1, p = 0.02; HR = 1.95 for CCI ≥ 2, p pancreatitis (HR = 1.51, p = 0.03). There was no difference in diabetes after Whipple procedure vs distal pancreatic resections, or malignant vs benign pathologic findings. Independent predictors of exocrine insufficiency were female sex (HR = 1.32, p = 0.002) and higher CCI (HR = 1.85 for CCI of 1, p insufficiency (HR = 0.35, p endocrine and exocrine insufficiency were 40% and 35%, respectively. These data are critical for informing patients' and physicians' expectations.

Premature ovarian insufficiency in young girls: repercussions on uterine volume and bone mineral density.

Science.gov (United States)

Bakhsh, Hanadi; Dei, Metella; Bucciantini, Sandra; Balzi, Daniela; Bruni, Vincenzina

2015-01-01

To evaluate biological differences among young subjects with premature ovarian insufficiency (POI) commencing at different stages of life. Retrospective observational study. Careggi University Hospital Participants: One hundred sixty-two females aged between 15 and 29 years with premature ovarian insufficiency. Data were collected as a retrospective chart review of baseline evaluation at diagnosis of premature ovarian insufficiency (POI). About 162 participants were divided into four groups based on gynecological age. Two primary outcome variables (uterine development and bone mineral density (BMD)) were analyzed in terms of differences among groups and in a multivariate logistic regression analysis. Uterine development was clearly jeopardized when estrogen insufficiency started at a very young age. Total body BMD showed significant differences among the four groups studied, clearly corresponding to the duration of ovarian function. Data were discussed in relation to the choice of hormone replacement therapy regimens.

Altered Cyclosporine Absorption in a Patient with Ulcerative Colitis, Sclerosing Cholangitis and Pancreatic Insufficiency

Directory of Open Access Journals (Sweden)

Mark G Swain

1991-01-01

Full Text Available Pancreatic insufficiency leading to altered cyclosporine absorption is reported in a 37-year-old man with ulcerative colitis and sclerosing cholangitis. Asymptomatic chronic pancreatitis occurs frequently in patients with ulcerative colitis, and even more commonly when there is coexistent sclerosing cholangitis. However, pancreatic insufficiency has been documented in only one patient previously with ulcerative colitis and sclerosing cholangitis. Pancreatic function testing can help to identify the complex etiology of malabsorption in these patients and is recommended in patients when liver transplantation is contemplated, as pancreatic insufficiency may alter the absorption of cyclosporine.

[Acquired thrombotic thrombocytopenic purpura after vascular prosthesis implantation for impending rupture of an abdominal aortic aneurysm].

Science.gov (United States)

Naito, Chiaki; Ogawa, Yoshiyuki; Yanagisawa, Kunio; Ishizaki, Takuma; Mihara, Masahiro; Handa, Hiroshi; Isonishi, Ayami; Hayakawa, Masaki; Matsumoto, Masanori; Nojima, Yoshihisa

2016-03-01

Acquired thrombotic thrombocytopenic purpura (TTP) is caused by autoantibodies against ADAMTS13. TTP patients run a rapidly fatal course unless immediate plasma exchange (PEX) is initiated upon diagnosis. Herein, we report a 72-year-old man with TTP, which developed after he underwent artificial blood vessel replacement surgery for an abdominal aneurysm with impending rupture. In the perioperative period, the patient received several platelet transfusions for severe thrombocytopenia (minimum platelet count: 0.6×10(4)/μl). Thereafter, he was admitted to our department for rapidly progressing coma with multiple cerebral infarctions, and was transferred to the ICU. Based on the tentative diagnosis of TTP, we immediately began PEX and steroid pulse therapy. The diagnosis was confirmed thereafter by markedly reduced ADAMTS13 activity (<0.5%) and his being positive for the ADAMTS13 inhibitor. We performed PEX for five consecutive days and administered high-dose prednisolone (PSL). On the second hospital day (HD), his platelet count rose along with improvement of his consciousness level. The ADAMTS13 inhibitor was not detected on the 10th HD. TTP did not relapse and his general condition improved despite tapering of PSL. In this case, by closely monitoring ADAMTS13-related parameters and minimizing the number of plasma exchanges, the patient was able to achieve a remission without the use of boosting inhibitors.

Successful Prednisolone Therapy in Elderly Patients with Severe Forms of Henoch–Schönlein Purpura Nephritis

Directory of Open Access Journals (Sweden)

Saiko Kato-Okada

2015-01-01

Full Text Available Introduction Recently, Henoch–Schönlein purpura (HSP has been observed in elderly people, although it was believed to be uncommon in these subjects. The increased risks of developing end-stage renal disease (ESRD in adults in comparison with children were highlighted by different studies; however, limited data are available on the treatment of HSP nephritis in adults. Methods Between 2002 and 2008, five elderly Japanese patients (>65 years old (mean age, 68 years, ranging from 65 to 72 with severe forms of HSP nephritis were entered into a prospective study to evaluate prednisolone therapy on the outcome of nephropathy in terms of clinical symptoms and histopathological changes. The patients were considered at risk of developing chronic renal failure when they presented with a nephrotic syndrome and crescentic glomeruli. Results At the last follow-up, 4–10 years after initiation of the therapy, four patients had clinically recovered and one died of lung cancer. No patients developed ESRD. The clinical outcome seemed to be correlated with glomerular activity (massive proteinuria and crescent formation. In spite of a relatively large dose of prednisolone, a few adverse effects, such as insomnia and skin lesions, were observed. Discussion Our preliminary small study suggests that renal outcome as well as survival of elderly patients with severe forms of HSP might be altered by aggressive prednisolone therapy.

Splenectomy vs. rituximab as a second-line therapy in immune thrombocytopenic purpura: a single center experience.

Science.gov (United States)

Al Askar, Ahmed S; Shaheen, Naila A; Al Zahrani, Mohsen; Al Otaibi, Mohammed G; Al Qahtani, Bader S; Ahmed, Faris; Al Zughaibi, Mohand; Kamran, Ismat; Mendoza, May Anne; Khan, Altaf

2018-01-01

Immune thrombocytopenic purpura (ITP) is a common hematological disease treated primarily by corticosteroids. The aim of the present study was to compare response rate between patients, underwent splenectomy vs. rituximab as second-line therapy. Adult patients diagnosed with ITP who did not respond to corticosteroids or relapsed during the period 1990-2014 were included in a quasi-experimental study. Categorical variables were compared using Fisher exact test. Response to treatment was compared using logistic regression. Data were analyzed using SAS V9.2. One-hundred and forty-three patients with ITP were identified through medical records. Of 62 patients treated, 30 (48.38%) required second-line therapy. 19 (63%) patients received rituximab, and 11 (37%) underwent splenectomy. Platelets at diagnosis were not different between study groups (p = 0.062). Splenectomy group patients were younger (p = 0.011). Response to second-line therapy showed no significant difference between two groups (OR 2.03, 95% CI (0.21-22.09), p = 0.549). Results did not show a statistically significant difference in platelet counts over time between treatment groups (p = 0.101). When used exclusively as a second-line therapy for steroid-refractory ITP, the response rate was not statistically different between rituximab and splenectomy. However, further large studies are needed to assess the response rates for these treatment modalities as a second-line therapy.

Synthesis of neutron-rich transuranic nuclei in fissile spallation targets

Energy Technology Data Exchange (ETDEWEB)

Mishustin, Igor, E-mail: mishustin@fias.uni-frankfurt.de [Frankfurt Institute for Advanced Studies, J.-W. Goethe University, 60438 Frankfurt am Main (Germany); “Kurchatov Institute”, National Research Center, 123182 Moscow (Russian Federation); Malyshkin, Yury, E-mail: malyshkin@fias.uni-frankfurt.de [Frankfurt Institute for Advanced Studies, J.-W. Goethe University, 60438 Frankfurt am Main (Germany); Institute for Nuclear Research, Russian Academy of Sciences, 117312 Moscow (Russian Federation); Pshenichnov, Igor, E-mail: pshenich@fias.uni-frankfurt.de [Frankfurt Institute for Advanced Studies, J.-W. Goethe University, 60438 Frankfurt am Main (Germany); Institute for Nuclear Research, Russian Academy of Sciences, 117312 Moscow (Russian Federation); Greiner, Walter [Frankfurt Institute for Advanced Studies, J.-W. Goethe University, 60438 Frankfurt am Main (Germany)

2015-04-15

A possibility of synthesizing neutron-rich superheavy elements in spallation targets of Accelerator Driven Systems (ADS) is considered. A dedicated software called Nuclide Composition Dynamics (NuCoD) was developed to model the evolution of isotope composition in the targets during a long-time irradiation by intense proton and deuteron beams. Simulation results show that transuranic elements up to {sup 249}Bk can be produced in multiple neutron capture reactions in macroscopic quantities. However, the neutron flux achievable in a spallation target is still insufficient to overcome the so-called fermium gap. Further optimization of the target design, in particular, by including moderating material and covering it by a reflector could turn ADS into an alternative source of transuranic elements in addition to nuclear fission reactors.

Is there any correlation between vitamin D insufficiency and diabetic retinopathy?

Institute of Scientific and Technical Information of China (English)

Shokoufeh; Bonakdaran; Nasser; Shoeibi

2015-01-01

AIM: To determine a relation between vitamin D level,which is an inhibitor of angiogenesis, and diabetic retinopathy and its risk factorsMETHODS: In a clinic-based cross sectional study two hundred and thirty-five type 2 diabetic patients older than 20 y were selected. Patients were classified according to ophthalmologic examination as following:no diabetic retinopathy(NDR)(n =153), non-proliferative diabetic retinopathy(NPDR)(n =64) and proliferative diabetic retinopathy(PDR)(n =18). Study subjects were tested for fasting blood glucose, glycated hemoglobin A1C(Hb A1C), lipid profile, microalbuminuria, Hs CRP,IGF1, insulin(in patients without history of insulin taking)and 25 hydroxy vitamin D [25(OH) D] levels. Vitamin D insufficiency was defined according to 25(OH) D level less than 30 ng/m L. The relationship between diabetic retinopathy and serum 25(OH) D insufficiency was evaluated.RESULTS: The prevalence of diabetic retinopathy was34.8% in our patients. Long duration of diabetes,hypertension, poor glycemic control, diabetic nephropathy, hyperinsulinemia and insulin resistance were risk factors for diabetic retinopathy but 25(OH) D level was not significant different between NDR, NPDR and PDR groups. Correlation between 25(OH) D level and other known risk factors of diabetic retinopathy was not significant.CONCLUSION: This study did not find any association between diabetic retinopathy and its severity and vitamin D insufficiency. Vitamin D insufficiency is not related to risk factors of diabetic retinopathy.

Paraoxonase activity in patients with chronic renal failure and hepatic insufficiency

International Nuclear Information System (INIS)

Jamal, S.; Ishaq, M.; Hussain, S.M.W.; Alam, J.A.; Hussain, S.

2010-01-01

Paraoxonase (PON), a high density lipoprotein (HDL) associated enzyme, is believed to protect against the oxidation of low density lipoprotein (LDL) and hence affects the risk of vascular disease. PON is sensitive to oxidants and is inactivated by oxidized lipids, and thus it can be postulated that increased oxidative stress may decrease plasma PON activity in patients with chronic renal failure (CRF) and hepatic insufficiency (HI). Moreover, in CRF and HI patients, in contrast to normal individuals, higher levels of plasma biochemical parameters and liver enzymes had an inverse correlation with PON activity. In this study we aimed to investigate PON activity, total bilirubin, creatinine, urea and liver enzymes alanine aminotransferase and alkaline phosphatase that are the index of renal and hepatic insufficiency. We have analyzed plasma from pre-dialysis patients and compared the results with the normal individuals. We observed a positive association of PON activity with that of the disease state i.e. the activity of this enzyme was significantly lower in the patients (p < 0.001). Furthermore, the indicators of renal and hepatic insufficiency were significantly elevated as compared to the normal subjects. Based on our results we conclude that in CRF and HI, in contrast to normal individuals, higher levels of plasma biochemical parameters and liver enzymes had inverse correlation with PON activity. Collectively, these findings may add details to the understanding of the role that PON plays in chronic renal failure and hepatic insufficiency. (author)

Combined metabolomic and correlation networks analyses reveal fumarase insufficiency altered amino acid metabolism.

Science.gov (United States)

Hou, Entai; Li, Xian; Liu, Zerong; Zhang, Fuchang; Tian, Zhongmin

2018-04-01

Fumarase catalyzes the interconversion of fumarate and l-malate in the tricarboxylic acid cycle. Fumarase insufficiencies were associated with increased levels of fumarate, decreased levels of malate and exacerbated salt-induced hypertension. To gain insights into the metabolism profiles induced by fumarase insufficiency and identify key regulatory metabolites, we applied a GC-MS based metabolomics platform coupled with a network approach to analyze fumarase insufficient human umbilical vein endothelial cells (HUVEC) and negative controls. A total of 24 altered metabolites involved in seven metabolic pathways were identified as significantly altered, and enriched for the biological module of amino acids metabolism. In addition, Pearson correlation network analysis revealed that fumaric acid, l-malic acid, l-aspartic acid, glycine and l-glutamic acid were hub metabolites according to Pagerank based on their three centrality indices. Alanine aminotransferase and glutamate dehydrogenase activities increased significantly in fumarase deficiency HUVEC. These results confirmed that fumarase insufficiency altered amino acid metabolism. The combination of metabolomics and network methods would provide another perspective on expounding the molecular mechanism at metabolomics level. Copyright © 2017 John Wiley & Sons, Ltd.

Response of geomorphic and geological processes to insufficient ...

Indian Academy of Sciences (India)

Depositional models have been constructed for each segment: a constant shelf break model with insufficient sediment supply in the east, and a migration shelf break model with plenty sediment supply in the west. This case study contributes to the understanding of the upper slope sedimentary process and stratigraphic style ...

Prevalence of insufficient, borderline, and optimal hours of sleep among high school students - United States, 2007.

Science.gov (United States)

Eaton, Danice K; McKnight-Eily, Lela R; Lowry, Richard; Perry, Geraldine S; Presley-Cantrell, Letitia; Croft, Janet B

2010-04-01

We describe the prevalence of insufficient, borderline, and optimal sleep hours among U.S. high school students on an average school night. Most students (68.9%) reported insufficient sleep, whereas few (7.6%) reported optimal sleep. The prevalence of insufficient sleep was highest among female and black students, and students in grades 11 and 12. Published by Elsevier Inc.

Subchondral insufficiency fracture of the femoral head in a patient with alkaptonuria

Energy Technology Data Exchange (ETDEWEB)

Hamada, Takahiro; Shida, Jun-ichi; Inokuchi, Akihiko; Arizono, Takeshi [Kyushu Central Hospital, Department of Orthopaedic Surgery, Fukuoka-city (Japan); Yamamoto, Takuaki [Kyushu University, Department of Orthopaedic Surgery, Graduate School of Medical Sciences, Fukuoka-city (Japan)

2014-06-15

We report a patient with alkaptonuria accompanied by bilateral rapidly destructive arthrosis of the hip. The destruction of the left hip joint with its severe functional impairment necessitated total hip arthroplasty (THA). The outcome was satisfactory. Both magnetic resonance imaging (MRI) and pathologic findings were compatible with a subchondral insufficiency fracture. A year and half later, during a follow-up visit, the patient complained of right coxalgia. Radiography showed that the right femoral head had already disappeared, requiring THA of the right hip. Although there have been a few reports of rapid destructive hip osteoarthritis associated with ochronotic arthropathy, the pathogenesis of the destructive change is not clear. Subchondral insufficiency fracture was diagnosed on MR imaging and pathologically confirmed in our patient with alkaptonuria, suggesting that subchondral insufficiency fracture is one of the causes of ochronotic hip destruction. (orig.)

Subchondral insufficiency fracture of the femoral head in a patient with alkaptonuria

International Nuclear Information System (INIS)

Hamada, Takahiro; Shida, Jun-ichi; Inokuchi, Akihiko; Arizono, Takeshi; Yamamoto, Takuaki

2014-01-01

We report a patient with alkaptonuria accompanied by bilateral rapidly destructive arthrosis of the hip. The destruction of the left hip joint with its severe functional impairment necessitated total hip arthroplasty (THA). The outcome was satisfactory. Both magnetic resonance imaging (MRI) and pathologic findings were compatible with a subchondral insufficiency fracture. A year and half later, during a follow-up visit, the patient complained of right coxalgia. Radiography showed that the right femoral head had already disappeared, requiring THA of the right hip. Although there have been a few reports of rapid destructive hip osteoarthritis associated with ochronotic arthropathy, the pathogenesis of the destructive change is not clear. Subchondral insufficiency fracture was diagnosed on MR imaging and pathologically confirmed in our patient with alkaptonuria, suggesting that subchondral insufficiency fracture is one of the causes of ochronotic hip destruction. (orig.)

Subchondral insufficiency fracture of the femoral head in a patient with alkaptonuria.

Science.gov (United States)

Hamada, Takahiro; Yamamoto, Takuaki; Shida, Jun-ichi; Inokuchi, Akihiko; Arizono, Takeshi

2014-06-01

We report a patient with alkaptonuria accompanied by bilateral rapidly destructive arthrosis of the hip. The destruction of the left hip joint with its severe functional impairment necessitated total hip arthroplasty (THA). The outcome was satisfactory. Both magnetic resonance imaging (MRI) and pathologic findings were compatible with a subchondral insufficiency fracture. A year and half later, during a follow-up visit, the patient complained of right coxalgia. Radiography showed that the right femoral head had already disappeared, requiring THA of the right hip. Although there have been a few reports of rapid destructive hip osteoarthritis associated with ochronotic arthropathy, the pathogenesis of the destructive change is not clear. Subchondral insufficiency fracture was diagnosed on MR imaging and pathologically confirmed in our patient with alkaptonuria, suggesting that subchondral insufficiency fracture is one of the causes of ochronotic hip destruction.

Inflammatory manifestations of experimental lymphatic insufficiency.

Directory of Open Access Journals (Sweden)

Raymond Tabibiazar

2006-07-01

Full Text Available BACKGROUND: Sustained lymph stagnation engenders a pathological response that is complex and not well characterized. Tissue inflammation in lymphedema may reflect either an active or passive consequence of impaired immune traffic. METHODS AND FINDINGS: We studied an experimental model of acute post-surgical lymphedema in the tails of female hairless, immunocompetent SKH-1 mice. We performed in vivo imaging of impaired immune traffic in experimental, murine acquired lymphatic insufficiency. We demonstrated impaired mobilization of immunocompetent cells from the lymphedematous region. These findings correlated with histopathological alterations and large-scale transcriptional profiling results. We found intense inflammatory changes in the dermis and the subdermis. The molecular pattern in the RNA extracted from the whole tissue was dominated by the upregulation of genes related to acute inflammation, immune response, complement activation, wound healing, fibrosis, and oxidative stress response. CONCLUSIONS: We have characterized a mouse model of acute, acquired lymphedema using in vivo functional imaging and histopathological correlation. The model closely simulates the volume response, histopathology, and lymphoscintigraphic characteristics of human acquired lymphedema, and the response is accompanied by an increase in the number and size of microlymphatic structures in the lymphedematous cutaneous tissues. Molecular characterization through clustering of genes with known functions provides insights into processes and signaling pathways that compose the acute tissue response to lymph stagnation. Further study of genes identified through this effort will continue to elucidate the molecular mechanisms and lead to potential therapeutic strategies for lymphatic vascular insufficiency.

[Clinical features of 32 patients with cutaneous small vessel vasculitis].

Science.gov (United States)

López de Maturana, Donaldo; Amaro, Patricio; Segovia, Laura; Balestrini, Claudia

2004-02-01

The skin is a common target of small vessel vasculitis, with a wide assortment of pathological changes. This condition is usually associated to systemic diseases. To report the clinical and pathological features of patients with cutaneous small vessel vasculitis. A retrospective review of 32 patients with a pathological diagnosis of cutaneous vasculitis. Seventy two percent of patients were women. Cutaneous lesions were mainly located in the lower limbs (94%). The most common lesion was palpable purpura (62%). Connective tissue diseases and systemic vasculitis were the most commonly associated systemic diseases. Palpable purpura is the most common manifestation of cutaneous small vessel vasculitis, that is usually associated to connective tissue diseases or systemic vasculitis.

Maternal thyroid hormone insufficiency during pregnancy and risk of neurodevelopmental disorders in offspring: A systematic review and meta-analysis.

Science.gov (United States)

Thompson, William; Russell, Ginny; Baragwanath, Genevieve; Matthews, Justin; Vaidya, Bijay; Thompson-Coon, Jo

2018-04-01

In the last 2 decades, several studies have examined the association between maternal thyroid hormone insufficiency during pregnancy and neurodevelopmental disorders in children and shown conflicting results. This systematic review aimed to assess the evidence for an association between maternal thyroid hormone insufficiency during pregnancy and neurodevelopmental disorders in children. We also sought to assess whether levothyroxine treatment for maternal thyroid hormone insufficiency improves child neurodevelopment outcomes. We performed systematic literature searches in MEDLINE, EMBASE, PSYCinfo, CINAHL, AMED, BNI, Cochrane, Scopus, Web of Science, GreyLit, Grey Source and Open Grey (latest search: March 2017). We also conducted targeted web searching and performed forwards and backwards citation chasing. Meta-analyses of eligible studies were carried out using the random-effects model. We identified 39 eligible articles (37 observational studies and 2 randomized controlled trials [RCT]). Meta-analysis showed that maternal subclinical hypothyroidism and hypothyroxinaemia are associated with indicators of intellectual disability in offspring (odds ratio [OR] 2.14, 95% confidence interval [CI] 1.20 to 3.83, P = .01, and OR 1.63, 95% CI 1.03 to 2.56, P = .04, respectively). Maternal subclinical hypothyroidism and hypothyroxinaemia were not associated with attention deficit hyperactivity disorder, and their effect on the risk of autism in offspring was unclear. Meta-analysis of RCTs showed no evidence that levothyroxine treatment for maternal hypothyroxinaemia or subclinical hypothyroidism reduces the incidence of low intelligence quotient in offspring. Although studies were generally of good quality, there was evidence of heterogeneity between the included observational studies (I 2 72%-79%). Maternal hypothyroxinaemia and subclinical hypothyroidism may be associated with intellectual disability in offspring. Currently, there is no evidence that levothyroxine

Higher visceral fat area increases the risk of vitamin D insufficiency and deficiency in Chinese adults.

Science.gov (United States)

Zhang, Meilin; Li, Ping; Zhu, Yufeng; Chang, Hong; Wang, Xuan; Liu, Weiqiao; Zhang, Yuwen; Huang, Guowei

2015-01-01

Visceral fat area (VFA), a novel sex-specific index for visceral fat obesity (VFO) might play a major role in the development of vitamin D deficiency. However, the association between VFA and vitamin D insufficiency and deficiency in Chinese population is less clear. The aim of this study was to explore the population-level association between VFA and vitamin D insufficiency and deficiency among Chinese men and women. This cross-sectional study involved 1105 adults aged 20-70 years living in Tianjin who were randomly selected and medically examined. All subjects underwent the bioelectrical impedance analysis (BIA) method to estimate the VFA. Serum 25-hydroxyvitamin D3 (25(OH) D3) level was assayed by the high-performance liquid chromatography (HPLC) method and defined insufficiency and deficiency following recommended cutoffs. The association between VFA and vitamin D insufficiency and deficiency was estimated using binary regression analysis. The total prevalence of vitamin D insufficiency (25(OH) D3: 20-29 μg/L) and deficiency (25(OH) D3  0.05). Moreover, increased VFA was observed to be associated with higher vitamin D insufficiency or deficiency risk with a positive dose-response trend (P for trend convenience surrogate marker for visceral adipose measurement and could be used in identifying the risk of vitamin D insufficiency and deficiency in routine health examination.

Rectal hydrocortisone during vomiting in children with adrenal insufficiency.

Science.gov (United States)

Ní Chróinín, M; Fallon, M; Kenny, D; Moriarty, S; Hoey, H; Costigan, C

2003-01-01

To evaluate rectal hydrocortisone as an emergency glucocorticoid replacement therapy in adrenal insufficient children. A parental questionnaire evaluated preferred treatment, problems or benefits of i.m. and rectal hydrocortisone, frequency and indications for administration and who administered treatment. Admissions of children with adrenal insufficiency were monitored. There were 39/52 families who responded to the questionnaire. 93% (26/28) preferred rectal hydrocortisone. Parents or children who previously received emergency treatment from a doctor now self-administered rectal hydrocortisone. The cost of suppositories and i.m. hydrocortisone is similar; however, storage of suppositories was inconvenient. One girl presented with pneumonia and collapse despite rectal hydrocortisone and a hydrocortisone level at admission of >2000 nmol/l with normal electrolytes. Rectal hydrocortisone is an acceptable and safe emergency therapy. We still advise i.m. hydrocortisone if rectal administration is not possible or with suppository extrusion.

Lethal Progressive Thoracic Insufficiency in a Neonate Due to Jarcho Levin Syndrome

Science.gov (United States)

Bhutia, Euden; Maria, Arti; Verma, Arushi; Sethi, Sidharth Kumar

2014-01-01

A rare case of Jarcho Levin syndrome (JLS) presenting as a lethal progressive respiratory insufficiency in early neonatal period is reported. The neonate had classical features of this syndrome including vertebral segmentation defects, typical costo-vertebral fusion defects and scoliosis resulting in small thoracic volume and limited chest expansion; all consistent with a clinical diagnosis of JLS with thoracic insufficiency. In addition, our case had a rare association of dextrocardia and acyanotic congenital heart disease. PMID:24741543

Gastroenterological surgery for patients with chronic respiratory insufficiency.

Science.gov (United States)

Shimada, M; Kano, T; Matsuzaki, Y; Miyazaki, N; Ninomiya, K

1998-01-01

The aim of this study was to clarify the surgical indications for patients with chronic respiratory insufficiency. Fourteen patients with chronic respiratory insufficiency who underwent abdominal surgical procedures, were retrospectively studied. The surgical indications were carefully determined based primarily on the performance status (PS) of each patient and cardiopulmonary function tests. A PS of equal to or less than 3, which meant the patient's status required bed rest > 50% of the time, and the need for assistance in performing normal activities were all factors considered for surgical indications. During the period studied, two patients were excluded from the surgical indications due to the fact that one was at a terminal stage of pulmonary disease and was completely bedridden (PS = 4), while the other demonstrated active pneumonia with a considerable amount of purulent sputa. Regarding the pulmonary function tests for patients who underwent surgery, the lowest limits of those examinations were as follows: 810 ml of vital capacity (VC), 23.8% of predicted VC, 610 ml of forced expiratory volume in one second (FEV1.0), 38.6% of predicted FEV1.0, 50.5 mmHg of PaO2 while inhaling 4 liters of oxygen and 73.8 mmHg of PaCO2. No surgery related mortality or hospital death within 30 days after operation was observed. Only two patients had cardiopulmonary complications (consisting of pulmonary edema with atrial fibrillation in one patient, and acute myocardial infarction in another patient). However, neither pneumonia, prolonged ventilatory support for more than 2 days, nor the need for a tracheostomy after surgery was observed. Gastroenterological surgery is thus considered to be indicated even for patients with chronic respiratory insufficiency, as long as the PS can be maintained (PS of equal to or less than 3) and no active pneumonia with a considerable amount of purulent sputa is present.

Traumatic tricuspid valve insufficiency. Experience in thirteen patients.

Science.gov (United States)

van Son, J A; Danielson, G K; Schaff, H V; Miller, F A

1994-11-01

From 1964 through June 1993, thirteen patients with traumatic tricuspid insufficiency were treated surgically; all were male, and the ages ranged from 17 to 64 years (median 39 years). The condition was associated with blunt chest trauma in all patients: motor vehicle accidents in twelve and an explosion of a tank of compressed air in one. The median duration between trauma and operation was 17 years (range 1 month to 37 years). Preoperatively, six patients were in sinus rhythm and seven were in atrial fibrillation. At operation, the right ventricular function appeared moderately to severely depressed in twelve patients. In twelve patients, the anterior leaflet was flail because of chordal rupture (n = 9), rupture of anterior papillary muscle (n = 3), or tear in the anterior leaflet (n = 1). In one patient, the septal leaflet was missing and in another it was retracted and adherent to the ventricular septum. In five patients the tricuspid valve was repaired and in eight it was replaced. In seven patients in the latter group, the chordae, papillary muscles, and/or tricuspid valve leaflet(s) were found to be in a contracted and atrophic state, precluding repair. No early or late deaths occurred. At follow-up extending to 26 years (median 12 years), 12 patients are in New York Heart Association class I and one patient is in class II. Nine patients were in sinus rhythm and four were in atrial fibrillation. Although our experience indicates that good functional results can still be achieved many years after the onset of traumatic tricuspid valve insufficiency, earlier diagnosis and surgical treatment should increase the feasibility of tricuspid valve insufficiency, earlier diagnosis and surgical treatment should increase the feasibility of tricuspid valve repair, prevent progressive deterioration of right ventricular function, and increase the possibility of maintaining late sinus rhythm in a greater number of patients.

Hydrocortisone dose in adrenal insufficiency : Balancing harms and benefits

NARCIS (Netherlands)

Werumeus Buning, Jorien

2017-01-01

Patients with secondary adrenal insufficiency do not produce cortisol and are therefore treated with hydrocortisone tablets. The optimal substitution dose for hydrocortisone is unknown. We therefore performed this study, in which two different doses of hydrocortisone and its effect on cognition

Association between vitamin D insufficiency and elevated serum uric acid among middle-aged and elderly Chinese Han women.

Directory of Open Access Journals (Sweden)

Hao Peng

Full Text Available BACKGROUND: Association between vitamin D insufficiency and hyperuricemia has not been reported so far. We aimed to study the association of vitamin D insufficiency with elevated serum uric acid among middle-aged and elderly Chinese Han women. METHODS: We collected data from participants residing in Jinchang district of Suzhou from January to May, 2010. Serum uric acid, 25-hydroxy vitamin D and other traditional biomarkers including fasting plasma glucose and blood lipids were determined in 1726 women aged above 30 years. Association between vitamin D insufficiency and elevated uric acid was analyzed in premenopausal and postmenopausal women, respectively. RESULTS: Among postmenopausal women, 25-hydroxy vitamin D level of participants with elevated uric acid was lower than that of those with normal uric acid (median [interquartile range]: 35[28-57] vs 40[32-58], µg/L; P = 0.006. Elevated uric acid was more prevalent in participants with vitamin D insufficiency compared to those without vitamin D insufficiency (16.50% vs 8.08%; P<0.001. Association between vitamin D insufficiency and elevated uric acid was not significant among premenopausal women. However, participants with vitamin D insufficiency were more likely to have elevated uric acid compared with those without vitamin D insufficiency among postmenopausal women (OR, 95% CI: 2.38, 1.47-3.87. Moreover, after excluding individuals with diabetes and/or hypertension, the association of vitamin D insufficiency with elevated uric acid was still significant (OR, 95% CI: 2.48, 1.17-5.44. CONCLUSIONS: Vitamin D insufficiency was significantly associated with elevated uric acid among postmenopausal Chinese Han women. This study suggested that a clinical trial should be conducted to confirm the association of vitamin D insufficiency with hyperuricemia.

Subchondral insufficiency fracture of the knee: a non-traumatic injury with prolonged recovery time.

Science.gov (United States)

Gourlay, Margaret L; Renner, Jordan B; Spang, Jeffrey T; Rubin, Janet E

2015-06-08

Subchondral insufficiency fractures are non-traumatic fractures that occur immediately below the cartilage of a joint. Although low bone density may be present concurrently, it is not the underlying cause of subchondral insufficiency fractures in the majority of patients. Patients with subchondral insufficiency fracture characteristically have unremarkable plain radiographs, while MRI examination may reveal extensive bone marrow oedema and subchondral bone collapse. This article presents a 51-year-old postmenopausal woman, a physician, who had subchondral insufficiency fractures of the knee associated with prolonged standing during clinical work. She was treated with partial weight bearing on crutches until 14 months after the injury, viscosupplementation at 4 months to treat osteoarthritis and teriparatide treatment to improve bone healing at 7 months. By 26 months after the injury, she tolerated independent walking with a fabric knee support but still experienced mild posterolateral knee pain and numbness on prolonged standing. 2015 BMJ Publishing Group Ltd.

Predictive factors of adrenal insufficiency in patients admitted to acute medical wards: a case control study

Directory of Open Access Journals (Sweden)

Oboni Jean-Baptiste

2013-01-01

Full Text Available Abstract Background Adrenal insufficiency is a rare and potentially lethal disease if untreated. Several clinical signs and biological markers are associated with glucocorticoid failure but the importance of these factors for diagnosing adrenal insufficiency is not known. In this study, we aimed to assess the prevalence of and the factors associated with adrenal insufficiency among patients admitted to an acute internal medicine ward. Methods Retrospective, case-control study including all patients with high-dose (250 μg ACTH-stimulation tests for suspected adrenal insufficiency performed between 2008 and 2010 in an acute internal medicine ward (n = 281. Cortisol values Results 32 patients (11.4% presented adrenal insufficiency; the others served as controls. Among all clinical and biological parameters studied, history of glucocorticoid withdrawal was the only independent factor significantly associated with patients with adrenal insufficiency (Odds Ratio: 6.71, 95% CI: 3.08 –14.62. Using a logistic regression, a model with four significant and independent variable was obtained, regrouping history of glucocorticoid withdrawal (OR 7.38, 95% CI [3.18 ; 17.11], p-value p-value 0.044, eosinophilia (OR 17.6, 95% CI [1.02; 302.3], p-value 0.048 and hyperkalemia (OR 2.41, 95% CI [0.87; 6.69], p-value 0.092. The AROC (95% CI was 0.75 (0.70; 0.80 for this model, with 6.3 (0.8 – 20.8 for sensitivity and 99.2 (97.1 – 99.9 for specificity. Conclusions 11.4% of patients with suspected adrenal insufficient admitted to acute medical ward actually do present with adrenal insufficiency, defined by an abnormal response to high-dose (250 μg ACTH-stimulation test. A history of glucocorticoid withdrawal was the strongest factor predicting the potential adrenal failure. The combination of a history of glucocorticoid withdrawal, nausea, eosinophilia and hyperkaliemia might be of interest to suspect adrenal insufficiency.

THE METHOD OF MANAGEMENT WOMEN WITH ISTHMIC-CERVICAL INSUFFICIENCY

Directory of Open Access Journals (Sweden)

Мария Сергеевна Воронцова

2018-01-01

Full Text Available Isthmic-cervical insufficiency (ICI is one of the most common causes of spontaneous premature births. In II trimester of pregnancy in the structure of miscarriages, the share of ICI accounts for about 40 % of cases. The purpose – evaluate the effectiveness of the complex method of correction of isthmic-cervical insufficiency. Materials and methods. A simple, open, prospective, randomized study was performed on the basis of the City Clinical Perinatal Center in Omsk. Under observation, there were 60 patients aged 18-40 years in gestation period of 13-33 weeks 6 days with a proven diagnosis of «isthmic-cervical insufficiency». The main group included 30 patients who received treatment with a new combined method: the obstetric pessary «CVK», which differs from the analogues by a more anatomical ellipsoidal form and an increased number of perforations to improve the outflow of vaginal secretions in combination with intravaginal injection of micronized progesterone in a dosage of 100 mg 2 times a day and a drug normalizing the biocenosis of the vagina, as a lyophilized culture of lactobacilli L. casei rhamnosus Doderleini in a dosage of 340 mg, 1 capsule intravaginally at night 2 times a week. The comparison group consisted of 30 patients who received treatment: obstetric silicone pessary + micronized progesterone at a dosage of 200 mg per day intravaginally. The outcomes of pregnancy and childbirth were evaluated. Results. The total number of premature births in the main group was 4 (13.3 % compared to 12 (40 % in the comparison group (p = 0,023. Based on the results of the study, a statistically significant reduction in patients with recurrent vaginal flora (p = 0,0001 was obtained and, due to a decrease in this indicator, a general decrease in the number of women with complications of pregnancy was registered (p = 0,019. The conclusion. The proposed method of correction of isthmic-cervical insufficiency is more effective

A NANOS3 mutation linked to protein degradation causes premature ovarian insufficiency

OpenAIRE

Wu, X; Wang, B; Dong, Z; Zhou, S; Liu, Z; Shi, G; Cao, Y; Xu, Y

2013-01-01

Primary ovarian insufficiency (POI), or premature ovarian failure, is defined as the cessation of ovarian function before the age of 40. An insufficient ovarian follicle pool derived from primordial germ cells (PGCs) is an important cause of POI. Although the Nanos gene family is known to be required for PGC development and maintenance in diverse model organisms, the relevance of this information to human biology is not yet clear. In this study, we screened the coding regions of the NANOS1, N...

THE MODERN APPROACH OF CHILDREN’S TREATMENT WITH VELOPHARYNGEAL INSUFFICIENCY IN THE POSTOPERATIVE PERIOD

Directory of Open Access Journals (Sweden)

O. V. Nelyubina

2014-01-01

Full Text Available Velopharyngeal insufficiency is one of the main causes of speech disorders in children with congenital cleft palate, leading to social exclusion and disability of patients. This article describes an integrated approach to the treatment of velopharyngeal insufficiency, aimed in improving the anatomical and physiological function of velopharyngeal ring. Velopharyngeal ring is considered as the nervemuscle complex in the treatment of patients with this pathology. It is shown that a one-time complex treatment of velopharyngeal insufficiency, estimated with the program «Compare» in the below-described clinical example, increased velopharyngeal closure ring by 25%, thus eliminating nasality and significantly improve the quality of speech.

Readability of online patient education materials for velopharyngeal insufficiency.

Science.gov (United States)

Xie, Deborah X; Wang, Ray Y; Chinnadurai, Sivakumar

2018-01-01

Evaluate the readability of online and mobile application health information about velopharyngeal insufficiency (VPI). Top website and mobile application results for search terms "velopharyngeal insufficiency", "velopharyngeal dysfunction", "VPI", and "VPD" were analyzed. Readability was determined using 10 algorithms with Readability Studio Professional Edition (Oleander Software Ltd; Vandalia, OH). Subgroup analysis was performed based on search term and article source - academic hospital, general online resource, peer-reviewed journal, or professional organization. 18 unique articles were identified. Overall mean reading grade level was a 12.89 ± 2.9. The highest reading level among these articles was 15.47-approximately the level of a college senior. Articles from "velopharyngeal dysfunction" had the highest mean reading level (13.73 ± 2.11), above "velopharyngeal insufficiency" (12.30 ± 1.56) and "VPI" (11.66 ± 1.70). Articles from peer-reviewed journals had the highest mean reading level (15.35 ± 2.79), while articles from academic hospitals had the lowest (12.81 ± 1.66). There were statistically significant differences in reading levels between the different search terms (P reading level guidelines, online patient education materials for VPI are disseminated with language too complex for most readers. There is also a lack of VPI-related mobile application data available for patients. Patients will benefit if future updates to websites and disseminated patient information are undertaken with health literacy in mind. Future studies will investigate patient comprehension of these materials. Copyright © 2017 Elsevier B.V. All rights reserved.

Long-term results of external valvuloplasty in adult patients with isolated great saphenous vein insufficiency

Directory of Open Access Journals (Sweden)

Sarac A

2014-04-01

Full Text Available Atilla Sarac,1 Artan Jahollari,1 Sureyya Talay,1 Sevket Ozkaya,2 Ertugrul Ozal1 1Department of Cardiovascular Medicine, Samsun Medical Park Hospital, Samsun, Turkey; 2Department of Pulmonary Medicine, Faculty of Medicine, Bahcesehir University, Istanbul, Turkey Objective: The aim of this study is to present our 7-year results of external valvuloplasty for isolated great saphenous vein (GSV insufficiency. Methods: External valvuloplasty was applied in 83 patients with isolated GSV insufficiency. Follow-up consisted of venous color duplex scanning performed on the first postoperative day, the first postoperative month, and then annually. Valvular insufficiency, venous reflux, and venous thrombosis formation in the saphenofemoral junction were the main outcomes. Results: A complete clinical and radiological healing was observed in 50 patients (60%. In 13 cases (15.6%, a secondary surgical treatment was performed consisting of vena saphena magna high ligation/stripping and varicose vein excisions, mainly due to severe and progressive vena saphena magna valvular insufficiency and clinical persistence of symptoms. Eight patients (9.6% developed superficial vein thrombosis, and only one patient (1.2% developed deep vein thrombosis. Contact was lost from 32 patients (38.5% for different reasons. Conclusion: External valvuloplasty is an effective surgical technique for selected cases of isolated GSV insufficiency without extensive varicose dilatations. This alternative method can be safely administered as an alternative to high ligation and conventional GSV stripping. Keyword: vein, saphenous, insufficiency, external valv, valvuloplasty, varicosis

Fatigue and insufficiency fractures

International Nuclear Information System (INIS)

Lodwick, G.S.; Rosenthal, D.I.; Kattapuram, S.V.; Hudson, T.M.

1987-01-01

The incidence of stress fracture is increasing. In our younger society this is due largely to a preocupation with physical conditioning, but in our elderly population it is due to improved recognition and better methods of detection and diagnosis. Stress fracture of the elderly is an insufficiency fracture which occurs in the spine, the pelvis, the sacrum and other bones afflicted with disorders which cause osteopenia. Stress fracture is frequently misdiagnosed as a malignant lesion of bone resulting in biopsy. Scintiscanning provides the greatest frequency of detection, while computed tomography often provides the definitive diagnosis. With increased interest and experience a better insight into the disease has been achieved, and what was once thought of as a simple manifestation of mechanical stress is now known to be an orderly, complex pattern of physiological changes in bone which conform to a model by Frost. The diffuse nature of these changes can be recognized by scintigraphy, radiography and magnetic resonance imaging. 27 refs.; 8 figs

Clinicoroentgenological diagnosis of chronic venous ovarian insufficiency

International Nuclear Information System (INIS)

Grakova, L.S.; Galkin, E.V.; Naumova, E.B.

1989-01-01

The paper is devoted to clinicoroentgenological correlations of venous renogonadal hemodynamics in 168 women of reproductive age (151 women with infertility, habitual abortion, disordered menstrual function and 17 patients without disorders of the reproductive tract). Clinicolaboratory investigation was followed by visceral and parietal flebography for the detection of a pathological venous reflux into the ovarian pampiniform plexus. Clinicoroentgenological semiotics of chronic venous ovarian insufficiency was analyzed. Infertility was shown to be the main clinical manifestation of ovarian varicocele

Structural basis for small molecule targeting of the programmed death ligand 1 (PD-L1)

NARCIS (Netherlands)

Zak, Krzysztof M.; Grudnik, Przemyslaw; Guzik, Katarzyna; Zieba, Bartosz J.; Musielak, Bogdan; Dömling, Alexander; Dubin, Grzegorz; Holak, Tad A.

2016-01-01

Targeting the PD-1/PD-L1 immunologic checkpoint with monoclonal antibodies has provided unprecedented results in cancer treatment in the recent years. Development of chemical inhibitors for this pathway lags the antibody development because of insufficient structural information. The first

Respiratory insufficiency with preserved diaphragmatic function in amyotrophic lateral sclerosis.

Science.gov (United States)

Yamauchi, Rika; Imai, Tomihiro; Tsuda, Emiko; Hozuki, Takayoshi; Yamamoto, Daisuke; Shimohama, Shun

2014-01-01

We performed a longitudinal study to elucidate the correlation between respiratory insufficiency and respiratory biomarkers, including diaphragmatic compound muscle action potential (DCMAP), at the initiation of noninvasive ventilation (NIV) in patients with amyotrophic lateral sclerosis (ALS). The patients were assessed at least every six months. Additional assessments were performed at the start of respiratory therapy when the patients met the criteria for the initiation of NIV. Each assessment consisted of a full neurological examination, a phrenic nerve conduction study, respiratory function tests, and nocturnal pulsed oximetry. We enrolled 43 patients with either definite or probable ALS as defined by the revised El Escorial criteria. The patients were divided into two groups according to the timing of the initiation of respiratory therapy. Seventeen patients (group A) met the criteria for NIV initiation when their DCMAP remained normal. Twenty-six patients (group B) met the criteria when their DCMAP decreased below normal limits. Although respiratory function parameters were significantly worse in group B compared with group A at NIV initiation, more than 80% of the patients in both groups developed nocturnal desaturation during sleep. DCMAP is not always a reliable indicator for determining the optimal timing for NIV initiation during the progression of respiratory insufficiency in ALS. Physicians should be aware of the risk of respiratory insufficiency during sleep in patients with ALS.

Assessing adrenal insufficiency of corticosteroid secretion using free versus total cortisol levels in critical illness

NARCIS (Netherlands)

Molenaar, N.; Groeneveld, A.B.J.; Dijstelbloem, H.M.; de Jong, M.F.C.; Girbes, A.R.J.; Heijboer, A.C.; Beishuizen, A.

2011-01-01

Purpose: To study the value of free versus total cortisol levels in assessing relative adrenal insufficiency during critical illness-related corticosteroid insufficiency. Methods: A prospective study in a mixed intensive care unit from 2004 to 2007. We consecutively included 49 septic and 63

A doppler-based evaluation of peripheral lower limb arterial insufficiency in diabetes mellitus

International Nuclear Information System (INIS)

Shaheen, R.; Sohail, S.

2010-01-01

To determine the frequency, level and flow patterns of lower limb arterial insufficiency in diabetic patients on Doppler ultrasound study. Study Design: Cross-sectional study. Place and Duration of Study: Radiology Department, Civil Hospital, Karachi, from February 2007 to September 2008. Methodology: One hundred adult diabetic patients with suspected peripheral vascular insufficiency irrespective of gender were included. Demographic data, presenting complaints, treatment history, and level of HbA1c were recorded. Doppler evaluated arterial status and ankle brachial index (ABI) were recorded on proforma. Statistical analysis were done on SPSS version 12. Results: The mean HbA1c was 8.4 +- 1.4 gm/dl, a majority of 77% having a controlled level of < 10 mg/dl. Arterial insufficiency on Doppler ultrasound was documented in 62% (p=0.016) and the dorsalis paedis artery was the predominant site of stenosis (24%). Spectral broadening and biphasic flow were salient features. The mean value of resistive index in stenotic cases was 0.563 +- 0.16 with a mean velocity difference of 0.37 +- 0.29 m/s (p < 0.001) at the site of stenosis. Conclusion: Peripheral vascular insufficiency was a significant finding in patients having diabetes for an average of 9.8 years, even in the presence of controlled HbA1c. The dorsalis paedis was the commonest site of involvement. The insufficiency was moderate with a biphasic flow pattern in a majority of cases. Difference in resistive index and flow velocities at and above the site of stenosis provided an important clue to the diagnosis of level of stenosis that helps in planning limb salvage management. (author)

Hospitals: Soft Target for Terrorism?

Science.gov (United States)

De Cauwer, Harald; Somville, Francis; Sabbe, Marc; Mortelmans, Luc J

2017-02-01

In recent years, the world has been rocked repeatedly by terrorist attacks. Arguably, the most remarkable were: the series of four coordinated suicide plane attacks on September 11, 2001 on buildings in New York, Virginia, and Pennsylvania, USA; and the recent series of two coordinated attacks in Brussels (Belgium), on March 22, 2016, involving two bombings at the departure hall of Brussels International Airport and a bombing at Maalbeek Metro Station located near the European Commission headquarters in the center of Brussels. This statement paper deals with different aspects of hospital policy and disaster response planning that interface with terrorism. Research shows that the availability of necessary equipment and facilities (eg, personal protective clothing, decontamination rooms, antidotes, and anti-viral drugs) in hospitals clearly is insufficient. Emergency teams are insufficiently prepared: adequate and repetitive training remain necessary. Unfortunately, there are many examples of health care workers and physicians or hospitals being targeted in both political or religious conflicts and wars. Many health workers were kidnapped and/or killed by insurgents of various ideology. Attacks on hospitals also could cause long-term effects: hospital units could be unavailable for a long time and replacing staff could take several months, further compounding hospital operations. Both physical and psychological (eg, posttraumatic stress disorder [PTSD]) after-effects of a terrorist attack can be detrimental to health care services. On the other hand, physicians and other hospital employees have shown to be involved in terrorism. As data show that some offenders had a previous history with the location of the terror incident, the possibility of hospitals or other health care services being targeted by insiders is discussed. The purpose of this report was to consider how past terrorist incidents can inform current hospital preparedness and disaster response planning

[Experimental validation of the efficacy of laser-magnetic therapy for chronic placental insufficiency].

Science.gov (United States)

Ordzhonikidze, N V; Filimonov, V G; Klimenko, P A; Kondrikov, N I; Akin'shina, V S; Berlin, Iu V

1994-01-01

A new pathogenetically based non-medicamentous method for correction of uteroplacental bloodflow disturbances has been developed on the model of chronic placental insufficiency in rats. A single 5 min laser-magnetic exposure on day 21 of normal pregnancy resulted in a vasodilating effect with reduction of the peripheral resistance in the uterine horn vessels and with improvement of their blood supply. A new LAMA laser magneto-therapeutic device was employed. Daily 5 min sessions of laser magnetic therapy administered to rats with chronic placental insufficiency from pregnancy days 15-16 to 21 normalized uterine horn contractility and resulted in positive morphofunctional changes in the components of the uterine horns and placenta, being associated with a noticeable improvement of fetal functions. Hence, laser magnetic therapy may be regarded as an effective non-drug method for therapy of chronic placental insufficiency.

Reversible adrenal insufficiency and heterophile antibodies in a case of autoimmune polyendocrinopathy syndrome.

Science.gov (United States)

Kharb, Sandeep; Gundgurthi, Abhay; Dutta, Manoj K; Garg, M K

2013-12-01

A 27-year-old male was admitted with diabetic ketoacidosis and altered sensorium with slurring of speech and ataxia. He was managed with intravenous insulin and fluids and later shifted to basal bolus insulin regimen and during further evaluation was diagnosed to be suffering from primary hypothyroidism and adrenal insufficiency. He was started on thyroxin replacement and steroids only during stress. After three months of follow up he was clinically euthyroid. His glycemic control was adequate on oral anti-hyperglycemic drugs and adrenal insufficiency recovered. However, his thyrotropin levels were persistently elevated on adequate replacement doses of thyroxin. His repeat TSH was estimated after precipitating serum with polyethylene glycol which revealed normal TSH. Here we report reversible adrenal insufficiency with hypothyroidism with falsely raised TSH because of presence of heterophile antibodies in a case of poly glandular endocrinopathy syndrome.

[Management of childhood idiopathic thrombocytopenic purpura: a survey of members of the French Society of Pediatric Hematology and Immunology (SHIP)].

Science.gov (United States)

Lutz, P; Villega, F; Ducassou, S; Entz-Werle, N; Michel, G

2007-05-01

The purpose of this study was to report in acute childhood idiopathic thrombocytopenic purpura (ITP) the current practices of French paediatric hematologists and to compare them to recent publications of American and British teams. A questionnaire was sent online to the members of the French Society of Pediatric Hematology/Immunology (SHIP). This questionnaire, adapted from a similar american study conducted in 2001, asked 16 questions based on the clinical presentation of a 5-year-old boy referred for an acute ITP. 59/123 SHIP members responded to the survey. In response to question regarding initial treatment, 86% of physicians would be given active treatments and only 9% would rarely or never administer any drug. When asked which agent would be used in case of treatment, 68% would choose to prescribe intravenous immunoglobulins and 32% corticosteroids, nobody recommended the use of anti-D immunoglobulins. Furthermore, 83% would usually hospitalize such a child. Finally, this study allowed us to update the current French management of treating pediatric ITP which is almost comparable among this subset of pediatric hematologists, but showed some discrepancies comparatively to the American and British studies.

Photosensitivity and Acute Liver Insufficiency in Late-Onset Erythropoietic Protoporphyria with a Chromosome 18q Abnormality

Directory of Open Access Journals (Sweden)

Yuka Oshikawa

2012-07-01

Full Text Available Late-onset erythropoietic protoporphyria (EPP is rare, and it is usually associated with an acquired somatic mutation of the ferrochelatase gene secondary to hematological malignancy such as myelodysplastic syndrome or myeloproliferative disorder. In 0.5–1% of patients with EPP, deposition of protoporphyrin in the liver leads to progressive liver insufficiency. Herein, we report the case of a 67-year-old female who developed EPP with typical photosensitivity and hemolytic anemia. Six months later, she was admitted with acute liver damage with a rapidly progressing course, and developed liver insufficiency. She recovered from the liver insufficiency after undergoing plasmapheresis and red blood cell exchange transfusion. A bone marrow examination revealed normal features; however, a cytogenetic analysis identified an abnormal clone of cells with a translocation between chromosomes 13q12 and 18q21.1. This is the first report of a patient who recovered from liver insufficiency. The results of this report suggest that plasmapheresis and red blood cell exchange transfusion are effective for treating liver insufficiency in patients with late-onset EPP.

Chronic Heroin Dependence Leading to Adrenal Insufficiency

Directory of Open Access Journals (Sweden)

Gautam Das

2014-01-01

Full Text Available Opioids have been the mainstay for pain relief and palliation over a long period of time. They are commonly abused by drug addicts and such dependence usually imparts severe physiologic effects on multiple organ systems. The negative impact of opioids on the endocrine system is poorly understood and often underestimated. We describe a patient who developed severe suppression of the hypothalamic-pituitary adrenal (HPA axis leading to secondary adrenal insufficiency due to long standing abuse of opioids.

Assessing adrenal insufficiency of corticosteroid secretion using free versus total cortisol levels in critical illness

NARCIS (Netherlands)

Molenaar, Nienke; Johan Groeneveld, A. B.; Dijstelbloem, Hilde M.; de Jong, Margriet F. C.; Girbes, Armand R. J.; Heijboer, Annemieke C.; Beishuizen, Albertus

2011-01-01

To study the value of free versus total cortisol levels in assessing relative adrenal insufficiency during critical illness-related corticosteroid insufficiency. A prospective study in a mixed intensive care unit from 2004 to 2007. We consecutively included 49 septic and 63 non-septic patients with

Why Sleep Matters-The Economic Costs of Insufficient Sleep: A Cross-Country Comparative Analysis.

Science.gov (United States)

Hafner, Marco; Stepanek, Martin; Taylor, Jirka; Troxel, Wendy M; van Stolk, Christian

2017-01-01

The Centers for Disease Control and Prevention (CDC) in the United States has declared insufficient sleep a "public health problem." Indeed, according to a recent CDC study, more than a third of American adults are not getting enough sleep on a regular basis. However, insufficient sleep is not exclusively a US problem, and equally concerns other industrialised countries such as the United Kingdom, Japan, Germany, or Canada. According to some evidence, the proportion of people sleeping less than the recommended hours of sleep is rising and associated with lifestyle factors related to a modern 24/7 society, such as psychosocial stress, alcohol consumption, smoking, lack of physical activity and excessive electronic media use, among others. This is alarming as insufficient sleep has been found to be associated with a range of negative health and social outcomes, including success at school and in the labour market. Over the last few decades, for example, there has been growing evidence suggesting a strong association between short sleep duration and elevated mortality risks. Given the potential adverse effects of insufficient sleep on health, well-being and productivity, the consequences of sleep-deprivation have far-reaching economic consequences. Hence, in order to raise awareness of the scale of insufficient sleep as a public-health issue, comparative quantitative figures need to be provided for policy- and decision-makers, as well as recommendations and potential solutions that can help tackling the problem.

Nocturnal foot blood flow in patients with arterial insufficiency

DEFF Research Database (Denmark)

Jelnes, Rolf; Tønnesen, K H

1984-01-01

Twenty-four hour continuous recording of xenon (133Xe) wash-out from the forefoot was performed on patients with normal circulations (n = 10) and on patients with different degrees of arterial insufficiency (n = 36). During day hours the calculated subcutaneous blood flow in the forefoot was on a...

Renal and suprarenal insufficiency secondary to familial Mediterranean fever associated with amyloidosis: a case report

Directory of Open Access Journals (Sweden)

Sari Nagehan

2011-08-01

Full Text Available Abstract Introduction Familial Mediterranean fever is an autosomal recessive disease that predominantly affects people of the Mediterranean coast. One of the most frequent complications of the disease is amyloidosis. This clinical entity is known as secondary (also called AA amyloidosis. Case presentation In this report, we describe the case of a 33-year-old Turkish man with familial Mediterranean fever and chronic renal insufficiency. He was admitted to our clinic with symptoms of suprarenal insufficiency. The patient died three months later as a result of cardiac arrest. Conclusion Our aim is to make a contribution to the literature by reporting a case of combined insufficiency due to the accumulation of renal and adrenal amyloid in a patient with familial Mediterranean fever, which has very rarely been described in the literature. We hope that adrenal insufficiency, which becomes fatal if not diagnosed and treated rapidly, will come to mind as easily as chronic renal failure in clinical practice.

Rozrolimupab, symphobodies against rhesus D, for the potential prevention of hemolytic disease of the newborn and the treatment of idiopathic thrombocytopenic purpura.

Science.gov (United States)

Stasi, Roberto

2010-12-01

Currently under codevelopment by Symphogen and Swedish Orphan Biovitrum, rozrolimupab is the first in a new class of recombinant polyclonal antibodies, known as symphobodies, produced using a proprietary technology from Symphogen. Rozrolimupab is being investigated for the prevention of hemolytic disease of the fetus and newborn (HDFN) and for the treatment of idiopathic thrombocytopenic purpura (ITP). Rozrolimupab comprises 25 genetically unique IgG1 antibodies, all of which are specific for the rhesus D (RhD) erythrocyte protein. In preclinical studies, rozrolimupab demonstrated binding to erythrocytes that was comparable with that of two plasma-derived anti-D Ig preparations. In a phase I clinical trial in healthy male volunteers, treatment with rozrolimupab was not associated with serious adverse events. In a phase II clinical trial of rozrolimupab in healthy, male, RhD-negative volunteers, rozrolimupab dose-dependently cleared RhD-positive erythrocytes from the circulation. Phase II clinical trials in ITP and HDFN are currently ongoing. Phase III clinical trials are necessary to establish the efficacy and safety profile of rozrolimupab compared with standard plasma-derived anti-D Ig preparations.

Recurrent Thrombotic Thrombocytopenic Purpura-Like Syndrome as a Paraneoplastic Phenomenon in Malignant Peritoneal Mesothelioma: A Case Report and Review of the Literature

Directory of Open Access Journals (Sweden)

Francisco Socola

2012-01-01

Full Text Available We report the case of an African American male with no significant past medical history presenting with recurrent, rapidly relapsing episodes of thrombotic thrombocytopenic purpura (TTP despite aggressive treatment with several lines of treatment. Incidentally, these episodes were associated with severe abdominal pain which eventually developed into acute abdomen and prompted exploratory laparotomy, revealing diffuse carcinomatosis with a tumor located on the left pelvis that was encasing the distal sigmoid colon. Pathology made a final diagnosis of peritoneal mesothelioma. TTP-like syndrome (TTP-LS has been described as a paraneoplastic phenomenon in several malignancies but never before in the setting of malignant mesothelioma. Paraneoplastic TTP-like syndrome has historically been associated with a dismal prognosis and particular clinical and laboratory abnormalities described in this paper. It is of utmost importance to make a prompt determination whether TTP is idiopathic or secondary to an underlying condition because of significant differences in their prognosis, treatment, and response. This paper also reviews the current literature regarding this challenging condition.

Functional pancreatic insufficiency after surgical treatment in the light of the latest international recommendations

Directory of Open Access Journals (Sweden)

D. S. Bordin

2017-01-01

Full Text Available Exocrine  and  endocrine   insufficiencies  are  frequent complications of surgical treatment for pancreatic diseases. The presence  and  extent  of the insufficiency depend on the underlying  disorder, type of surgical procedure, extent of pancreatic resection, and anatomical reconstruction. Increased surgical  activity  determines  the  importance  of the  evidence-based guidelines  for management of patients  after  pancreatic  surgery. The article presents  an  overview of international Evidence-based Guidelines for the Management of Exocrine Pancreatic  Insufficiency after  Pancreatic  Surgery (2016 and United European Gastroenterology evidence-based guidelines  for the  diagnosis  and therapy of chronic pancreatitis (HaPanEU, 2017.

Retrospective study of effect of therapy on computer vision syndrome patients having convergence insufficiency

Directory of Open Access Journals (Sweden)

Nidhi Tiwari

2017-01-01

Full Text Available Aim: To study effect of therapy among computer users having convergence insufficiency. Materials and Methods: Hundred people between age group of 20-35 and who worked in IT companies were enrolled in the study. They were subjected to orthoptic evaluation to establish diagnosis of convergence insufficiency. They were then called for in-office therapy for 12 days daily. They underwent brock string exercises and at the end of therapy re-evaluation was done. Results: All the patients who underwent the therapy showed improvement in Near point of convergence at the end of therapy. They were also symptomatically better. Conclusions: Convergence insufficiency is a common occupation hazard among IT professionals. But it can be treated with appropriate therapy.

Nasopharyngeal pushback in treatment of velopharyngeal insufficiency.

Science.gov (United States)

Smith, H W; Lee, K J

1976-02-01

We describe a new technique for extensive retropositioning of the soft palate for the treatment of velopharyngeal insufficiency. This technique is identified as a nasopharyngeal pushback, and has been used repeatedly in conjunction with both a Cronin nasal flap and a superiorly based pharyngeal flap when maximum retropositioning was needed. This procedure has been used for over ten years, each time obtaining an additional pushback distance equal to or greater than the distance achieved by freeling the soft palate from the posterior border of the hard palate.

[An update on the treatment of venous insufficiency in pregnancy].

Science.gov (United States)

Rodríguez-Nora, B; Álvarez-Silvares, E

Chronic venous insufficiency is a long-term pathological condition resulting from anatomical or functional alterations of the venous system. This leads to the appearance of symptoms and physical signs that affect a large part of the population and particularly pregnant women, due to the physiology of pregnancy. The few published studies on the use of pharmacological treatments of venous insufficiency in this group of the population, often makes the management of this condition difficult in routine clinical practice. A review is presented in this article, with all the latest updates in the treatment of this condition during pregnancy. There are numerous general, and some pharmacological, recommendations, that we can safely offer the pregnant patient. Copyright © 2017 Sociedad Española de Médicos de Atención Primaria (SEMERGEN). Publicado por Elsevier España, S.L.U. All rights reserved.

Chemotherapy-Induced Regression of an Adrenocorticotropin-Secreting Pituitary Carcinoma Accompanied by Secondary Adrenal Insufficiency

Directory of Open Access Journals (Sweden)

Robert Frank Cornell

2013-01-01

Full Text Available Purpose. Adrenocorticotropin- (ACTH- secreting pituitary carcinomas are rare and require multimodality treatment. The aim of this study was to report the response to various therapies and discuss the potential development of secondary adrenal insufficiency with cytotoxic chemotherapy. Methods. This report describes a man with a large silent corticotroph adenoma progressing to endogenous hypercortisolism and metastatic ACTH-secreting pituitary carcinoma over a period of 14 years. Results. Seven years after initial presentation, progressive tumor enlargement associated with the development of hypercortisolism mandated multiple pituitary tumor debulking procedures and radiotherapy. Testing of the Ki-67 proliferation index was markedly high and he developed a hepatic metastasis. Combination therapy with cisplatin and etoposide resulted in a substantial reduction in tumor size, near-complete regression of his liver metastasis, and dramatic decrease in ACTH secretion. This unexpectedly resulted in symptomatic secondary adrenal insufficiency. Conclusions. This is the first reported case of secondary adrenal insufficiency after use of cytotoxic chemotherapy for metastatic ACTH-secreting pituitary carcinoma. High proliferative indices may be predictive of dramatic responses to chemotherapy. Given the potential for such responses, the development of secondary adrenal insufficiency may occur and patients should be monitored accordingly.

Suppression of spleen pathological function by roentgenoendovascular occlussion in some blood systemic diseases

International Nuclear Information System (INIS)

Rabkin, I.Kh.; Matevosov, A.L.; Gotman, L.N.

1987-01-01

It is shown that REO of splenic artery in thrombocytopenic purpura, hypoplastic and autoimmune hemolytic anemia manifests the same clinical effect as splenectomy. This treatment procedure may substitute splenectomy in a definite group of patients with above-mentioned blood systemic diseases or precede it as a preparation and prognosis stage. Stage-by-stage total embolization of splenic artery is a necessary technical method permitting to attain the necessary medical effect without risk of developing acute spleen infarction. At present REO is used in some blood diseases in patients with high risk of operation, particularly with active hemorrhagic syndrome of adrenal insufficiency, concomitant inflammatory processes

Introduction of a Successful Pregnancy in a Patient with Advanced Chronic Renal Insufficiency

Directory of Open Access Journals (Sweden)

H. Saghafi

2008-04-01

Full Text Available Background and ObjectiveIn the women with chronic renal insufficiency ovulation is suppressed therefore they rarely become pregnant. If pregnancy occurs, they might encounter many conflictions. It may lead to death (fetus or mother. The aim of this study was reporting a successful pregnancy in a patient with advanced chronic renal insufficiency.Case reportThe patient was a 32 years old woman with long period of infertility (8 years. The first main clinical symptom was abdominal pain especially in hypogastric area as well as hyperuremia, elevated levels of creatinine (2.9 mg/dl, mild proteinuria and hematuria. The urine specific gravity was 1010. Sonography data showed asymmetrical small kidneys. Other complaints were pruritus and flank pain during urination. The primary diagnosis was chronic renal failure due to probable chronic pyelonephritis. After an interval she returned with positive pregnancy test. She decided to continue the pregnancy in despite of obstetrician belief for aborting. During pregnancy, proteinuria reached to two plus, hemoglobin fell to 9.7, creatinine levels reached to 3.7 mg/dl and blood pressure was fluctuating between 110/80 and 130/85 mmHg. She admitted in the hospital in third trimester of pregnancy because of preterm labor. However the pain was suppressed after starting magnesium sulfate infusion. Keywords: Renal Insufficiency, Chronic Renal Insufficiency, Pregnancy

The Proteomic Analysis of Pancreatic Exocrine Insufficiency Protein Marker in Type 2 Diabetes Mellitus Patients

Science.gov (United States)

Srihardyastutie, Arie; Soeatmadji, DW; Fatchiyah; Aulanni'am

2018-01-01

Type 2 Diabetes Mellitus (T2D) is the vast majority case of diabetes. Patient with T2D is at higher risk for developing acute or chronic pancreatitis. Prolonged hyperglycemia results in damages to tissue, which also causes dysfunctions of some organ systems, including enzyme or hormone secretions. Commonly, dysfunction or insufficiency of pancreatic exocrine is evaluated by increasing activity of serum pancreatic enzyme, such as amylase and lipase. Although incidence of pancreatitis was found in Indonesian T2D, the pathogenic mechanism still unclear. The aim of this study was to characterize the marker protein that indicated the correlation of pancreatic exocrine insufficiency with progression of T2D. Proteomic analysis using LC-MS/MS was used in identification and characterization of protein marker which indicates insufficiency pancreatic exocrine. First step, protein profile was analyzed by SDS-PAGE methods using serum sample of T2D compared with normal or healthy control, as negative control, and pancreatitis patients, as positive control. Protein with 18 kDa was found as a candidate protein marker which indicated the pancreatic exocrine insufficiency in T2D. The further identification of that protein using LC-MS/MS showed 4 peptide fragments. In silico analysis of the peptide fragment indicated the correlation of pancreatic exocrine insufficiency with progression of T2D was METTL10 - methyltransferase like protein-10.

Dealing with Insufficient Location Fingerprints in Wi-Fi Based Indoor Location Fingerprinting

Directory of Open Access Journals (Sweden)

Kai Dong

2017-01-01

Full Text Available The development of the Internet of Things has accelerated research in the indoor location fingerprinting technique, which provides value-added localization services for existing WLAN infrastructures without the need for any specialized hardware. The deployment of a fingerprinting based localization system requires an extremely large amount of measurements on received signal strength information to generate a location fingerprint database. Nonetheless, this requirement can rarely be satisfied in most indoor environments. In this paper, we target one but common situation when the collected measurements on received signal strength information are insufficient, and show limitations of existing location fingerprinting methods in dealing with inadequate location fingerprints. We also introduce a novel method to reduce noise in measuring the received signal strength based on the maximum likelihood estimation, and compute locations from inadequate location fingerprints by using the stochastic gradient descent algorithm. Our experiment results show that our proposed method can achieve better localization performance even when only a small quantity of RSS measurements is available. Especially when the number of observations at each location is small, our proposed method has evident superiority in localization accuracy.

Adrenal hypoplasia congenita: a rare cause of primary adrenal insufficiency and hypogonadotropic hypogonadism

Directory of Open Access Journals (Sweden)

Marta Loureiro

2015-09-01

Full Text Available Primary adrenal insufficiency is defined by the impaired synthesis of adrenocortical hormones due to an intrinsic disease of the adrenal cortex. Determining its etiology is crucial to allow adequate long-term management and genetic counseling. We report the case of a male adolescent that presented in the neonatal period with adrenal crisis and received replacement therapy for primary adrenal insufficiency. During follow-up, adrenal hypoplasia congenita (AHC was suspected given his persistently raised adrenocorticotropic hormone levels, with markedly low 17-OH progesterone and androstenedione levels. DNA sequence analysis revealed a mutation in NR0B1 gene (c.1292delG, confirming the diagnosis. Delayed puberty and persistent low levels of gonadotropins led to testosterone replacement therapy. X-linked AHC is a rare cause of primary adrenal insufficiency and hypogonadotropic hypogonadism, related to mutations in NR0B1 gene. Despite its rarity, AHC should be considered in patients who present with primary adrenal failure, low levels of 17-OH progesterone and hypogonadotropic hypogonadism.

The physical mechanism of successful treatment for cervical insufficiency

Science.gov (United States)

Baumer, Alexa; Amechi, Alexis; Codrington, Paige; Leftwich, Megan C.

2017-11-01

Cervical insufficiency is a medical condition during pregnancy in which the uterine cervix softens and begins to dilate before reaching full term, usually between 18 and 22 weeks gestation. It is the most common cause of second trimester pregnancy loss. One clinical technique used to treat cervical insufficiency is the cervical cerclage, a procedure to close the cervix with a purse-string stitch. There are conflicting findings on the efficacy of a cerclage, with most studies relying on statistical evidence. The purpose of this investigation is to examine the mechanical limitations of a cervical cerclage by pressurizing a stitched, synthetic cervix until rupture. A synthetic model of the cervix is generated using ultrasound images collected by clinical collaborators and fabricated with silicon to imitate physiological properties. Medical residents from The George Washington University Hospital stitch the synthetic cervixes using clinical techniques. Pressure transducers record the maximum force on the stitch before rupture. The results of this study will provide insight into the most effective clinical interventions and the mechanism of their success.

Retrospective analysis of rituximab therapy and splenectomy in childhood chronic and refractory immune thrombocytopenic purpura.

Science.gov (United States)

Ay, Yilmaz; Karapinar, Tuba H; Oymak, Yesim; Toret, Ersin; Demirag, Bengu; Ince, Dilek; Ozcan, Esin; Moueminoglou, Nergial; Koker, Sultan A; Vergin, Canan

2016-06-01

Immune thrombocytopenic purpura (ITP) results from accelerated platelet destruction mediated by autoantibodies to platelet glycoproteins. Some patients with chronic ITP are refractory to all therapies [steroids, intravenous immunoglobulin (IVIG), anti-D and immunosuppresive drugs] and have chronic low platelet counts and episodic bleeding. We retrospectively evaluated the efficacy and safety of rituximab treatment and splenectomy in paediatric patients diagnosed with chronic and refractory ITP who were unresponsive to steroids, IVIG, cyclosporine and mycophenolate mofetil. Records of patients with chronic and refractory ITP in 459 patients with primary ITP who were followed up in our hospital from January 2005 to December 2014 were reviewed. Fifteen of patients received rituximab and/or applied splenectomy. Fifteen chronic ITP patients (10 boys, five girls) with a mean age of 10 years were enrolled in the study. Two of these patients were suffering from Evans syndrome. The median time since diagnosis of ITP was 10 years. The median follow-up duration after starting Rituximab and splenectomy were 13 and 9.5 months, respectively.None of the seven patients who were treated with rituximab achieved a response. A splenectomy was performed in six of the seven patients who had been treated with rituximab. Complete and partial responses were achieved in 67 and 33% of the patients, respectively. We evaluated the clinical characteristics and responses of chronic ITP patients who did not receive rituximab therapy and underwent a splenectomy. The success rate was 100% in the eight patients with chronic and refractory ITP. Rituximab therapy might not be beneficial for some children with severe chronic ITP who are refractory to standard agents. A splenectomy might be useful and preferable to rituximab.

Standardization of terminology, definitions and outcome criteria in immune thrombocytopenic purpura of adults and children: report from an international working group.

Science.gov (United States)

Rodeghiero, Francesco; Stasi, Roberto; Gernsheimer, Terry; Michel, Marc; Provan, Drew; Arnold, Donald M; Bussel, James B; Cines, Douglas B; Chong, Beng H; Cooper, Nichola; Godeau, Bertrand; Lechner, Klaus; Mazzucconi, Maria Gabriella; McMillan, Robert; Sanz, Miguel A; Imbach, Paul; Blanchette, Victor; Kühne, Thomas; Ruggeri, Marco; George, James N

2009-03-12

Diagnosis and management of immune thrombocytopenic purpura (ITP) remain largely dependent on clinical expertise and observations more than on evidence derived from clinical trials of high scientific quality. One major obstacle to the implementation of such studies and in producing reliable meta-analyses of existing data is a lack of consensus on standardized critical definitions, outcome criteria, and terminology. Moreover, the demand for comparative clinical trials has dramatically increased since the introduction of new classes of therapeutic agents, such as thrombopoietin receptor agonists, and innovative treatment modalities, such as anti-CD 20 antibodies. To overcome the present heterogeneity, an International Working Group of recognized expert clinicians convened a 2-day structured meeting (the Vicenza Consensus Conference) to define standard terminology and definitions for primary ITP and its different phases and criteria for the grading of severity, and clinically meaningful outcomes and response. These consensus criteria and definitions could be used by investigational clinical trials or cohort studies. Adoption of these recommendations would serve to improve communication among investigators, to enhance comparability among clinical trials, to facilitate meta-analyses and development of therapeutic guidelines, and to provide a standardized framework for regulatory agencies.

Reviewing Hit Discovery Literature for Difficult Targets: Glutathione Transferase Omega-1 as an Example.

Science.gov (United States)

Xie, Yiyue; Dahlin, Jayme L; Oakley, Aaron J; Casarotto, Marco G; Board, Philip G; Baell, Jonathan B

2018-05-10

Early stage drug discovery reporting on relatively new or difficult targets is often associated with insufficient hit triage. Literature reviews of such targets seldom delve into the detail required to critically analyze the associated screening hits reported. Here we take the enzyme glutathione transferase omega-1 (GSTO1-1) as an example of a relatively difficult target and review the associated literature involving small-molecule inhibitors. As part of this process we deliberately pay closer-than-usual attention to assay interference and hit quality aspects. We believe this Perspective will be a useful guide for future development of GSTO1-1 inhibitors, as well serving as a template for future review formats of new or difficult targets.

Health-related quality of life of immune thrombocytopenic purpura patients: results from a web-based survey.

Science.gov (United States)

Snyder, Claire F; Mathias, Susan D; Cella, David; Isitt, John J; Wu, Albert W; Young, Joan

2008-10-01

To assess the health-related quality of life (HRQOL) of immune thrombocytopenic purpura (ITP) patients. This was a cross-sectional, descriptive study comparing ITP patients' HRQOL to age and gender matched controls. ITP patients from the Platelet Disorder Support Association were recruited until 1000 surveys had been completed. Controls were randomly sampled from the Harris Interactive Online Panel. ITP patients and controls completed a one-time web-based survey, including a comprehensive HRQOL assessment. ITP patients completed the SF-36, the EQ-5D, and the ITP-Patient Assessment Questionnaire (ITP-PAQ). Controls completed the SF-36 and EQ-5D only. ITP patients' SF-36 and EQ-5D scores were compared to controls in unadjusted and adjusted analyses. Associations between splenectomy status, duration of illness, and platelet count with ITP patients' HRQOL scores were also examined. This analysis included 1002 ITP patients and 1031 controls. ITP patients scored worse on seven of eight SF-36 domains and the Physical and Mental Summary scores (all p PAQ scales: Bother, Psychological, Fear, Social Activity, and Work (all p PAQ scales. Lower platelet count was consistently associated with worse ITP-PAQ scores and had weaker associations with SF-36 and EQ-5D scores. ITP was associated with consistent and statistically significant deficits on generic HRQOL measures. The ITP-PAQ demonstrated differences based on disease severity and treatments. The self-selection bias in the two samples limits the generalizability of the results to all patients with ITP. Further research is needed in more generalizable samples.

Cervical spondylosis: a rare and curable cause of vertebrobasilar insufficiency.

Science.gov (United States)

Denis, Daniel J; Shedid, Daniel; Shehadeh, Mohammad; Weil, Alexander G; Lanthier, Sylvain

2014-05-01

Spondylotic vertebral artery (VA) compression is a rare cause of vertebrobasilar insufficiency and stroke. A 53-year-old man experienced multiple brief vertebrobasilar transient ischemic attacks (TIAs) and strokes, not apparently triggered by neck movements. Brain magnetic resonance imaging (MRI) documented consecutive infarcts, first in the left then right medial posterior inferior cerebellar artery (PICA) territories. Angiography showed two extracranial right vertebral artery (VA) stenoses, left VA hypoplasia, absence of left PICA and a dominant right PICA. Computed tomography angiography revealed right VA compression by osteophytes at C5-C6 and C6-C7 levels. No further vertebrobasilar insufficiency symptoms occurred in the 65 months following VA surgical decompression. Our literature review found 49 published surgical cases with vertebrobasilar symptoms caused by cervical spondylosis. Forty cases had one or more brief TIAs frequently triggered by neck movements. Three cases presented with stroke without prior TIA, with symptoms suggesting a top of the basilar artery embolic infarcts (one combined with a PICA infarct). Six cases had both TIAs and minor stroke. VA compression by uncovertebral osteophytes at the C5-C6 level was common. Dynamic angiography done in 38 cases systematically revealed worsening of VA stenosis or complete occlusion with either neck extension or rotation (ipsilateral when specified). Contralateral VA incompetence was found in 14 patients. Spondylotic VA stenosis can cause hemodynamic TIAs and watershed strokes, especially when contralateral VA insufficiency is combined to specific neck movements. Low-amplitude neck movement may suffice in severe cases. Embolic vertebrobasilar events are less frequent. VA decompression from spondylosis may prevent recurrent ischemic episodes.

Predictive factors of endocrine and exocrine insufficiency after resection of a benign tumour of the pancreas.

Science.gov (United States)

Neophytou, Hélène; Wangermez, Marc; Gand, Elise; Carretier, Michel; Danion, Jérôme; Richer, Jean-Pierre

2018-04-01

The aim of the present study is to evaluate the risk factors of endocrine and exocrine insufficiency occurring few years after pancreatic resections in a consecutive series of patients who underwent pancreatoduodenectomy (PD), left pancreatectomy (LP) or enucleation for benign neoplasms at a referral centre. Pancreatic exocrine insufficiency (PEI) was defined by the onset of steatorrhea associated with weight loss, and endocrine insufficiency was determinate by fasting plasma glucose. Association between pancreatic insufficiency and clinical, pathological, and perioperative features was studied using univariate and multivariate Cox regression analysis. A prospective cohort of 92 patients underwent PD (48%), LP (44%) or enucleation (8%) for benign tumours, from 2005 to 2016 in the University Hospital in Poitiers (France). The median follow-up was 68.6±42.4months. During the following, 54 patients developed exocrine insufficiency whereas 32 patients presented endocrine insufficiency. In the Cox model, a BMI>28kg/m 2 , being a man and presenting a metabolic syndrome were significantly associated with a higher risk to develop postoperative diabetes. The risks factors for the occurrence of PEI were preoperative chronic pancreatitis, a BMIpancreatic head, biological markers of chronic obstruction and fibrotic pancreas. Undergoing LP or enucleation were protective factors of PEI. Histological categories such as neuroendocrine tumours and cystadenomas were also associated with a decreased incidence of PEI. Men with metabolic syndrome and obesity should be closely followed-up for diabetes, and patients with obstructive tumours, pancreatic fibrosis or chronic pancreatitis require a vigilant follow up on their pancreatic exocrine function. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

Ideal Timing of Starting Weight-Bearing After Calcaneal Insufficiency Fracture: A Case Report and Review of the Literature

OpenAIRE

Imamura; Mochizuki; Kawakami; Momohara

2016-01-01

Introduction Criteria for starting weight-bearing on the heel with a symptomatic calcaneal insufficiency fracture have not yet been reported. Case Presentation We describe a rare case of a 52-year-old woman with a calcaneal insufficiency fracture who sustained a second ipsilateral calcaneal insufficiency fracture within a short time span. The initial fracture was not evident radiographically, but was detected using magnetic resona...

Adverse childhood experiences and frequent insufficient sleep in 5 U.S. States, 2009: a retrospective cohort study.

Science.gov (United States)

Chapman, Daniel P; Liu, Yong; Presley-Cantrell, Letitia R; Edwards, Valerie J; Wheaton, Anne G; Perry, Geraldine S; Croft, Janet B

2013-01-03

Although adverse childhood experiences (ACEs) have previously been demonstrated to be adversely associated with a variety of health outcomes in adulthood, their specific association with sleep among adults has not been examined. To better address this issue, this study examines the relationship between eight self-reported ACEs and frequent insufficient sleep among community-dwelling adults residing in 5 U.S. states in 2009. To assess whether ACEs were associated with frequent insufficient sleep (respondent did not get sufficient rest or sleep ≥ 14 days in past 30 days) in adulthood, we analyzed ACE data collected in the 2009 Behavioral Risk Factor Surveillance System, a random-digit-dialed telephone survey in Arkansas, Louisiana, New Mexico, Tennessee, and Washington. ACEs included physical abuse, sexual abuse, verbal abuse, household mental illness, incarcerated household members, household substance abuse, parental separation/divorce, and witnessing domestic violence before age 18. Smoking status and frequent mental distress (FMD) (≥ 14 days in past 30 days when self-perceived mental health was not good) were assessed as potential mediators in multivariate logistic regression analyses of frequent insufficient sleep by ACEs adjusted for race/ethnicity, gender, education, and body mass index. Overall, 28.8% of 25,810 respondents reported frequent insufficient sleep, 18.8% were current smokers, 10.8% reported frequent mental distress, 59.5% percent reported ≥ 1 ACE, and 8.7% reported ≥ 5 ACEs. Each ACE was associated with frequent insufficient sleep in multivariate analyses. Odds of frequent insufficient sleep were 2.5 (95% CI, 2.1-3.1) times higher in persons with ≥ 5 ACEs compared to those with no ACEs. Most relationships were modestly attenuated by smoking and FMD, but remained significant. Childhood exposures to eight indicators of child maltreatment and household dysfunction were significantly associated with frequent insufficient sleep during

Adverse childhood experiences and frequent insufficient sleep in 5 U.S. States, 2009: a retrospective cohort study

Directory of Open Access Journals (Sweden)

Chapman Daniel P

2013-01-01

Full Text Available Abstract Background Although adverse childhood experiences (ACEs have previously been demonstrated to be adversely associated with a variety of health outcomes in adulthood, their specific association with sleep among adults has not been examined. To better address this issue, this study examines the relationship between eight self-reported ACEs and frequent insufficient sleep among community-dwelling adults residing in 5 U.S. states in 2009. Methods To assess whether ACEs were associated with frequent insufficient sleep (respondent did not get sufficient rest or sleep ≥14 days in past 30 days in adulthood, we analyzed ACE data collected in the 2009 Behavioral Risk Factor Surveillance System, a random-digit-dialed telephone survey in Arkansas, Louisiana, New Mexico, Tennessee, and Washington. ACEs included physical abuse, sexual abuse, verbal abuse, household mental illness, incarcerated household members, household substance abuse, parental separation/divorce, and witnessing domestic violence before age 18. Smoking status and frequent mental distress (FMD (≥14 days in past 30 days when self-perceived mental health was not good were assessed as potential mediators in multivariate logistic regression analyses of frequent insufficient sleep by ACEs adjusted for race/ethnicity, gender, education, and body mass index. Results Overall, 28.8% of 25,810 respondents reported frequent insufficient sleep, 18.8% were current smokers, 10.8% reported frequent mental distress, 59.5% percent reported ≥1 ACE, and 8.7% reported ≥ 5 ACEs. Each ACE was associated with frequent insufficient sleep in multivariate analyses. Odds of frequent insufficient sleep were 2.5 (95% CI, 2.1-3.1 times higher in persons with ≥5 ACEs compared to those with no ACEs. Most relationships were modestly attenuated by smoking and FMD, but remained significant. Conclusions Childhood exposures to eight indicators of child maltreatment and household dysfunction were significantly

Relationship of renal insufficiency and clinical features or comorbidities with clinical outcome in patients hospitalised for acute heart failure syndromes.

Science.gov (United States)

Kajimoto, Katsuya; Sato, Naoki; Takano, Teruo

2017-12-01

Renal insufficiency is a well-known predictor of adverse events in patients with acute heart failure syndromes (AHFS). However, it remains unclear whether there are subgroups of AHFS patients in whom renal insufficiency is related to a higher risk of adverse events because of the heterogeneity of this patient population. Therefore, we investigated the relationship between renal insufficiency, clinical features or comorbidities, and the risk of adverse events in patients with AHFS. Of 4842 patients enrolled in the Acute Decompensated Heart Failure Syndromes (ATTEND) registry, 4628 patients (95.6%) were evaluated in the present study in order to assess the relationship of renal insufficiency and clinical features or comorbidities with all-cause mortality after admission. Renal insufficiency was defined as an estimated creatinine clearance of ⩽40 mL/min (calculated by the Cockcroft-Gault formula) at admission. The median follow-up period after admission was 524 (391-789) days. The all-cause mortality rate after admission was significantly higher in patients with renal insufficiency (36.7%) than in patients without renal insufficiency (14.4%). Stratified analysis was performed in order to explore the heterogeneity of the influence of renal insufficiency on all-cause mortality. This analysis revealed that an ischaemic aetiology and a history of diabetes, atrial fibrillation, serum sodium, and anaemia at admission had significant influences on the relationship between renal insufficiency and all-cause mortality. The present study demonstrated that the relationship between renal insufficiency and all-cause mortality of AHFS patients varies markedly with clinical features or comorbidities and the mode of presentation due to the heterogeneity of this patient population.

Imaging findings in systemic childhood diseases presenting with dermatologic manifestations.

Science.gov (United States)

Fink, Adam Z; Gittler, Julia K; Nakrani, Radhika N; Alis, Jonathan; Blumfield, Einat; Levin, Terry L

Many childhood diseases often present with skin abnormalities with which radiologists are largely unfamiliar. Knowledge of associated dermatologic manifestations may aid the radiologist in confirming the diagnosis and recommending targeted imaging of affected organs. We review the imaging findings in childhood diseases associated with dermatologic manifestations. Diseases include dermatologic findings which herald underlying malignancy (Neuroblastoma, leukemia/lymphoma, Langerhans cell histiocytosis),are associated with risk of malignancy (Epidermolysis Bullosa, basal cell nevus syndrome, Cowden's syndrome, Tuberous Sclerosis),or indicate a systemic inflammatory/immune disorder (Kawasaki's disease, Henoch Schonlein Purpura, systemic lupus erythematosus, scleroderma, sarcoidosis, dermatomyositis and immune thrombocytopenic purpura). Familiarity with pertinent findings in childhood diseases presenting with dermatologic manifestations in childhood diseases aids the radiologist in confirming the diagnosis and guiding imaging workup. Copyright © 2017 Elsevier Inc. All rights reserved.

A new peroxisomal disorder with fetal and neonatal adrenal insufficiency

NARCIS (Netherlands)

Vanhole, C.; de Zegher, F.; Casaer, P.; Devlieger, H.; Wanders, R. J.; Vanhove, G.; Jaeken, J.

1994-01-01

A boy with a new type of adrenoleukodystrophy is described. This was characterised by fetal and neonatal adrenal insufficiency, a neurological picture as seen in neonatal adrenoleukodystrophy, but with a normal number of peroxisomes in the liver and a peroxisomal dysfunction limited to the very long

Polymorphisms in Genes Coding for Cytokines, Mannose-Binding Lectin, Collagen Metabolism and Thrombophilia in Women with Cervical Insufficiency

DEFF Research Database (Denmark)

Sundtoft, Iben; Uldbjerg, Niels; Steffensen, Rudi

2015-01-01

OBJECTIVE: To study the association between cervical insufficiency and single nucleotide polymorphisms in seven genes coding for pro- and anti-inflammatory cytokine-related factors, mannose-binding lectin 2 (MBL2), collagen1α1 (COL1A1), factor II and factor V Leiden genes. METHODS: In a case......-control study, potential maternal biomarkers for cervical insufficiency were investigated in 30 women with a history of second-trimester miscarriage or preterm birth due to cervical insufficiency and in 70 control women. RESULTS: Homozygous carriers of the interleukin 6 (IL6) -174 genotype GG had an odds ratio...... (OR) of 3.1 [95% confidence interval (95% CI) 1.3-7.4, p = 0.01] and MBL2 genotypes coding for low or intermediate levels of plasma MBL had an OR of 3.3 (95% CI 1.2-9.0, p = 0.01) for cervical insufficiency compared with controls. Serum MBL levels were lower in women with cervical insufficiency than...

Effective antihypertensive treatment postpones renal insufficiency in diabetic nephropathy

DEFF Research Database (Denmark)

Parving, H H; Smidt, U M; Hommel, E

1993-01-01

The effect of long-term, aggressive, antihypertensive treatment on kidney function in diabetic nephropathy was studied prospectively in 11 insulin-dependent diabetic patients (mean age, 30 years). Renal function was assessed every 4 months by measurement of glomerular filtration rate (GFR) (single...... infarction (GFR, 46 mL/min/1.73 m2). Effective antihypertensive treatment postpones renal insufficiency in diabetic nephropathy....

Bilateral Avascular Necrosis and Pelvic Insufficiency Fractures Developing after Pelvic Radiotherapy in a Patient with Prostate Cancer: A Case Report

Directory of Open Access Journals (Sweden)

Hidayet Sarı

2016-12-01

Full Text Available Prostate cancer is one of the most common malignancies in men. Pelvic radiotherapy is commonly used in both radical and palliative treatment for prostate cancer. Radiation-induced adverse effects might be seen on adjacent healthy tissues (such as vessels, bones and soft tissues with the exception of targeted area. Particularly several years after radiotherapy, low back and hip pain may occur due to bone edema, necrosis or fractures. In these cases, whether complaints due to the degenerative, metastatic or radiotherapy complications must be examined and appropriate treatment should be arranged. For this purpose, we present our elderly patient who received radiotherapy for prostate cancer, and thereafter, developed bilateral avascular hip necrosis and pelvic insufficiency fractures.

Diagnosis of tricuspid insufficiency by Doppler flowmetry in the inferior vena cava. A comparison with right ventricular angiography

Energy Technology Data Exchange (ETDEWEB)

Smith, H.J.

Eighty-five patients subjected to routine heart catheterization were examined with duplex scanning of the inferior vena cava. Adequate Doppler recordings and a right ventricular angiography were obtained in 79 of them. Tricuspid insufficiency was found to be present in 34 patients at angiography and in 24 at duplex examination. No false positive Doppler diagnoses of tricuspid insufficiency occurred. The possibility of false positive angiographic diagnoses is discussed. A high correlation was found between percentage reversed flow in the inferior vena cava during ventricular systole and degree of angiographic tricuspid insufficiency. It is concluded that duplex scanning of the inferior vena cava seems to be a good alternative to angiography in the diagnosis and quantification of tricuspid insufficiency.

Various musculoskeletal manifestations of chronic renal insufficiency

International Nuclear Information System (INIS)

Lim, C.Y.; Ong, K.O.

2013-01-01

Musculoskeletal manifestations in chronic renal insufficiency are caused by complex bone metabolism alterations, now described under the umbrella term of chronic kidney disease mineral- and bone-related disorder (CKD-MBD), as well as iatrogenic processes related to renal replacement treatment. Radiological imaging remains the mainstay of disease assessment. This review aims to illustrate the radiological features of CKD-MBD, such as secondary hyperparathyroidism, osteomalacia, adynamic bone disease, soft-tissue calcifications; as well as features associated with renal replacement therapy, such as aluminium toxicity, secondary amyloidosis, destructive spondyloarthropathy, haemodialysis-related erosive arthropathy, tendon rupture, osteonecrosis, and infection

Vitamin D Insufficiency and Cognitive Function Trajectories in Older Adults: The Rancho Bernardo Study.

Science.gov (United States)

Laughlin, Gail A; Kritz-Silverstein, Donna; Bergstrom, Jaclyn; Reas, Emilie T; Jassal, Simerjot K; Barrett-Connor, Elizabeth; McEvoy, Linda K

2017-01-01

Evidence of a role for vitamin D (VitD) in cognitive aging is mixed and based primarily on extreme VitD deficiency. We evaluated the association of VitD insufficiency with cognitive function in older, community-dwelling adults living in a temperate climate with year-round sunshine. A population-based longitudinal study of 1,058 adults (median age 75; 62% women) who had cognitive function assessed and serum levels of 25-hydroxyvitaminD (25OHD) measured in 1997-99 and were followed for up to three additional cognitive function assessments over a 12-year period. Overall, 14% (n = 145) of participants had VitD insufficiency defined as 25OHD age, sex, education, and season, VitD insufficiency was associated with poorer baseline performance on the Mini-Mental Status Exam (MMSE) (p = 0.013), Trails Making Test B (Trails B) (p = 0.015), Category Fluency (p = 0.006), and Long Term Retrieval (p = 0.019); differences were equivalent to 5 years of age. For those with VitD insufficiency, the odds of mildly impaired performance at baseline were 38% higher for MMSE (p = 0.08), 78% higher for Trails B (p = 0.017), and 2-fold higher for Category Fluency and Long Term Retrieval (both p = 0.001). VitD insufficiency was not related to the rate of cognitive decline on any test or the risk of developing impaired performance during follow-up. In this population with little VitD deficiency, even moderately low VitD was associated with poorer performance on multiple domains of cognitive function. Low VitD did not predict 12-year cognitive decline. Clinical trials are essential to establish a causal link between VitD and cognitive well-being.

Food insufficiency, housing and health-related quality of life: results from the Positive Spaces, Healthy Places study.

Science.gov (United States)

Choi, Stephanie K Y; Fielden, Sarah; Globerman, Jason; Koornstra, J J Jay; Hambly, Keith; Walker, Glen; Sobota, Michael; O'Brien-Teengs, Doe; Watson, James; Bekele, Tsegaye; Greene, Saara; Tucker, Ruthann; Hwang, Stephen W; Rourke, Sean B; Healthy Places Team, The Positive Spaces

2015-01-01

Studies of people living with HIV who are homeless or unstably housed show a high prevalence of food insufficiency (>50%) and associated poor health outcomes; however, most evidence is in the form of cross-sectional studies. To better understand this issue, we conducted a longitudinal study to examine the impact of food insufficiency and housing instability on overall physical and mental health-related quality of life (HRQoL) among people living with HIV in Ontario. Six hundred and two adults living with HIV were enrolled in the Positive Spaces, Healthy Places study and followed from 2006 to 2009. Interviewer-administered questionnaires were used, and generalized linear mixed-effects models constructed to examine longitudinal associations between food insufficiency, housing instability and physical and mental HRQoL. At baseline, 57% of participants were classified as food insufficient. After adjusting for potential confounders, longitudinal analyses revealed a significant, negative association between food insufficiency and physical and mental HRQoL outcomes, respectively [effect size (ES) with 95% confidence interval (CI): (ES = -2.1, CI = -3.9,-0.3); (ES = -3.5, CI = -6.1,-1.5)]. Furthermore, difficulties meeting housing costs were shown to have additional negative impacts on mental HRQoL. Food insufficiency is highly prevalent among people living with HIV in Ontario, particularly for those with unstable housing. This vulnerable group of individuals is in urgent need of changes to current housing programmes, services and policies, as well as careful consideration of their unmet nutritional needs.

The role of the anterolateral ligament in ACL insufficient and reconstructed knees on rotary stability

DEFF Research Database (Denmark)

Tavlo, Mette; Eljaja, S; Tranum-Jensen, Jørgen

2016-01-01

Studies suggest that the anterolateral ligament (ALL) is important for knee stability. The purpose was to clarify ALL's effect on rotatory and anterior-posterior stability in the anterior cruciate ligament (ACL)-insufficient and reconstructed knees and the effect of reconstruction of an insuffici......Studies suggest that the anterolateral ligament (ALL) is important for knee stability. The purpose was to clarify ALL's effect on rotatory and anterior-posterior stability in the anterior cruciate ligament (ACL)-insufficient and reconstructed knees and the effect of reconstruction...... of an insufficient ALL. Eighteen cadaveric knees were included. Stability was tested for intact (+ALL), detached (-ALL) and reconstructed (+ reALL) ALL, with ACL removed (-ACL) and reconstructed (+ACL) in six combinations. All were tested in 0, 30, 60, and 90 °C flexion. Anterior-posterior stability was measured...... with a rolimeter. Rotation with a torque of 8.85 Nm was measured photographically. The ALL was well defined in 78% of knees. ACL reconstruction had a significant effect on anterior-posterior stability. Detaching the ALL had a significant effect on internal rotatory stability and on anterior-posterior stability...

Adrenal insufficiency in critically ill septic patients at Dr George ...

African Journals Online (AJOL)

Adele

eosinophillia and hypoglycemia. Hyponatremia and hyperkalemia are uncommon. There has been much controversy regarding the criteria for the diagnosis of adrenal insufficiency.10,11,12 The diagnosis would be best made using an end-organ marker of adrenal steroid action. No such marker is available at present. Free.

24 CFR 982.454 - Termination of HAP contract: Insufficient funding.

Science.gov (United States)

2010-04-01

... 24 Housing and Urban Development 4 2010-04-01 2010-04-01 false Termination of HAP contract... Assistance Payments Contract and Owner Responsibility § 982.454 Termination of HAP contract: Insufficient funding. The PHA may terminate the HAP contract if the PHA determines, in accordance with HUD requirements...

A Case of Rathke’s Cleft Cyst Associated with Transient Central Adrenal Insufficiency and Masked Diabetes Insipidus

Directory of Open Access Journals (Sweden)

Masahiro Asakawa

2014-01-01

Full Text Available A 73-year-old woman admitted to our hospital because of headache, poor appetite, malaise, weight loss, and vomiting was found to have central adrenal insufficiency and thyrotoxicosis due to silent thyroiditis. Polyuria developed after replacement with glucocorticoid (masked diabetes insipidus, which was controlled with nasal administration of desmopressin. Magnetic resonance imaging of the brain showed a large cystic pituitary mass (18 × 18 × 12 mm extending suprasellarly to the optic chiasm. Transsphenoidal surgery revealed that the pituitary tumor was Rathke’s cleft cyst. Following surgery, replacement with neither glucocorticoid nor desmopressin was needed any more. Therefore, it is suggested that Rathke’s cleft cyst is responsible for the masked diabetes insipidus and the central insufficiency. Furthermore, it is speculated that thyrotoxicosis with painless thyroiditis might induce changes from subclinical adrenal insufficiency to transiently overt insufficiency.

The Soft Palate Friendly Speech Bulb for Velopharyngeal Insufficiency.

Science.gov (United States)

Kahlon, Sukhdeep Singh; Kahlon, Monaliza; Gupta, Shilpa; Dhingra, Parvinder Singh

2016-09-01

Velopharyngeal insufficiency is an anatomic defect of the soft palate making palatopharyngeal sphincter incomplete. It is an important concern to address in patients with bilateral cleft lip and palate. Speech aid prosthesis or speech bulbs are best choice in cases where surgically repaired soft palate is too short to contact pharyngeal walls during function but these prosthesis have been associated with inadequate marginal closure, ulcerations and patient discomfort. Here is a case report of untreated bilateral cleft lip and palate associated with palatal insufficiency treated by means of palate friendly innovative speech bulb. This modified speech bulb is a combination of hard acrylic and soft lining material. The hard self-curing acrylic resin covers only the hard palate area and a permanent soft silicone lining material covering the soft palate area. A claw-shaped wire component was extended backwards from acrylic and was embedded in soft silicone to aid in retention and approximation of two materials. The advantage of adding the soft lining material in posterior area helped in covering the adequate superior extension and margins for maximal pharyngeal activity. This also improved the hypernasality, speech, comfort and overall patient acceptance.

First experience using navigation-guided radiofrequency kyphoplasty for sacroplasty in sacral insufficiency fractures

Energy Technology Data Exchange (ETDEWEB)

Klingler, J.H.; Kluge, P.; Sircar, R.; Kogias, E.; Scholz, C.; Krueger, M.T.; Scheiwe, C.; Hubbe, U. [Freiburg Univ. Medical Center, Freiburg (Germany). Dept. of Neurosurgery

2013-08-15

Purpose: To evaluate the efficacy and safety of navigation-guided radiofrequency kyphoplasty for sacroplasty in patients with sacral insufficiency fractures. Methods: In this single-center retrospective observational study, four consecutive patients with sacral insufficiency fractures were treated with navigation-guided radiofrequency kyphoplasty for sacroplasty between April 2010 and May 2012. Symptom characteristics, pain duration and pain intensity were recorded for each patient. Cement extravasation was evaluated in thin-sliced and triplanar reconstructed CT scans of the sacrum. Results: Four female patients with painful sacral insufficiency fractures and extensive osteopenic areas significantly improved from an average pre-treatment VAS score of 8.3 {+-} 0.5 to 2.3 {+-} 1.0 (p < 0.001) on the first postoperative day and to 1.3 {+-} 1.9 (p < 0.004) at follow-up (mean, 20.1 weeks). Slight cement extravasations were observed without evidence of being symptomatic. No major complications or procedure-related morbidity were noted. Conclusion: From the limited experience in four patients, navigation-guided radiofrequency kyphoplasty appears to be a safe and effective treatment option for sacral insufficiency fractures even though asymptomatic cement extravasation was noted. The use of navigation based on intraoperative 3 D images simplifies the positioning of the navigated bone needles via the long axis approach. The radiofrequency kyphoplasty system provides the possibility to administer a sufficient amount of bone cement with a well-defined viscosity over the entire period of the procedure leading to high security and low cement extravasation. Sacroplasty provides rapid and enduring pain relief and facilitates prompt mobilization. (orig.)

MRI of congenital pituitary insufficiency

International Nuclear Information System (INIS)

Almeida Magalhaes, Alvaro C. de; Uehara, Karla C.; Iezzi, Denise

1995-01-01

We compare 1,5 T magnetic resonance (MR) image findings in 193 patients with congenital pituitary congenital insufficiency. One hundred and thirty nine of the MR studies were obtained in patients who had isolated growth hormone deficiency. Other fifth - four patients had multiple pituitary hormone deficiency. On MR images, normal anterior and posterior lobes of the pituitary glands can be clearly differentiated because the posterior lobe has a characteristic high intensity on TI-weighted images. In fifty-four patients, the high- intensity of the posterior lobe was not seen, but a similar high signal intensity was observed at the proximal stump in fifty-one patients. this high- intensity area is the newly formed ectopic posterior lobe, which also secrets anti-diuretic hormone just as the posterior lobe would. MR imaging can demonstrate the transection of the pituitary stalk and the formation of the ectopic lobe, revealing to be a useful diagnostic tool in the definition of the type of alteration in growth defects of endocrine origin. (author)

The Use of Canine Stifle Orthotics for Cranial Cruciate Ligament Insufficiency

Directory of Open Access Journals (Sweden)

Brittany Jean Carr

2016-01-01

Full Text Available Objective: To assess weight bearing of dogs treated for unilateral cranial cruciate ligament insufficiency with a custom stifle orthotic.Background: Cranial cruciate ligament (CCL insufficiency is the most common cause of hind limb lameness in dogs. While there are numerous options for surgical management, surgery is not always an option. Recently, the use of canine stifle orthotics has also emerged as a means to non-surgically manage patients with cranial cruciate ligament insufficiency.  Evidentiary value:  This is a retrospective study of ten dogs treated for unilateral cranial cruciate ligament rupture with a stifle orthotic.Methods: Medical records (January 2005- December 2012 of ten dogs treated for unilateral cranial cruciate ligament rupture with a stifle orthotic were reviewed.  Temporospatial gait analysis was performed using a pressure sensing walkway at baseline and 90 days or greater post orthotic placement to identify weight bearing with total pressure index % (TPI%.Results: TPI% improved significantly by 5.1% in the affected limb when compared to baseline (p = 0.0020. At final gait analysis, TPI% significantly improved by 3% in the affected limb with the orthotic off when compared to the unaffected limb (p = 0.0020.Conclusion: Custom canine stifle orthotics allow for improved weight bearing in the affected limb.Application: Custom canine stifle orthotics should be considered for cases with concurrent medical conditions or financial constraints that do not allow for surgical intervention.

21 CFR 516.36 - Insufficient quantities of MUMS-designated drugs.

Science.gov (United States)

2010-04-01

... SERVICES (CONTINUED) ANIMAL DRUGS, FEEDS, AND RELATED PRODUCTS NEW ANIMAL DRUGS FOR MINOR USE AND MINOR SPECIES Designation of a Minor Use or Minor Species New Animal Drug § 516.36 Insufficient quantities of... the 7-year period of exclusive marketing rights. (b) If, within the time that FDA specifies, the...

Treatment of insufficient lactation is often not evidence-based

DEFF Research Database (Denmark)

Axelsson, Paul Bryde; Bjerrum, Flemming; Løkkegaard, Ellen Christine Leth

2014-01-01

INTRODUCTION: Breast milk has many advantages over formula for infants in developed and developing countries alike. Despite intentions of breastfeeding, some women develop insufficient lactation. Treatment options traditionally include breastfeeding education and pharmacotherapy. MATERIAL AND MET...... be initiated by counselling and education. Only when these treatment options are exhausted should pharmacotherapy with a suitable medication be considered. FUNDING: not relevant. TRIAL REGISTRATION: not relevant....

Population-based estimates of the prevalence of FMR1 expansion mutations in women with early menopause and primary ovarian insufficiency.

Science.gov (United States)

Murray, Anna; Schoemaker, Minouk J; Bennett, Claire E; Ennis, Sarah; Macpherson, James N; Jones, Michael; Morris, Danielle H; Orr, Nick; Ashworth, Alan; Jacobs, Patricia A; Swerdlow, Anthony J

2014-01-01

Primary ovarian insufficiency before the age of 40 years affects 1% of the female population and is characterized by permanent cessation of menstruation. Genetic causes include FMR1 expansion mutations. Previous studies have estimated mutation prevalence in clinical referrals for primary ovarian insufficiency, but these are likely to be biased as compared with cases in the general population. The prevalence of FMR1 expansion mutations in early menopause (between the ages of 40 and 45 years) has not been published. We studied FMR1 CGG repeat number in more than 2,000 women from the Breakthrough Generations Study who underwent menopause before the age of 46 years. We determined the prevalence of premutation (55-200 CGG repeats) and intermediate (45-54 CGG repeats) alleles in women with primary ovarian insufficiency (n = 254) and early menopause (n = 1,881). The prevalence of the premutation was 2.0% in primary ovarian insufficiency, 0.7% in early menopause, and 0.4% in controls, corresponding to odds ratios of 5.4 (95% confidence interval = 1.7-17.4; P = 0.004) for primary ovarian insufficiency and 2.0 (95% confidence interval = 0.8-5.1; P = 0.12) for early menopause. Combining primary ovarian insufficiency and early menopause gave an odds ratio of 2.4 (95% confidence interval = 1.02-5.8; P = 0.04). Intermediate alleles were not significant risk factors for either early menopause or primary ovarian insufficiency. FMR1 premutations are not as prevalent in women with ovarian insufficiency as previous estimates have suggested, but they still represent a substantial cause of primary ovarian insufficiency and early menopause.

Quality of life and cognitive function in patients with pituitary insufficiency

NARCIS (Netherlands)

Burman, P.; Deijen, J.B.

1998-01-01

This review is concerned with the psychosocial functioning and the quality of life in patients with pituitary insufficiency who are receiving conventional hormone replacement therapy. The possible negative effects of pituitary surgery, treatment with irradiation, and suboptimal replacement regimens

Platelet destruction in autoimmune thrombocytopenic purpura: kinetics and clearance of indium-111-labeled autologous platelets

International Nuclear Information System (INIS)

Stratton, J.R.; Ballem, P.J.; Gernsheimer, T.; Cerqueira, M.; Slichter, S.J.

1989-01-01

Using autologous 111 In-labeled platelets, platelet kinetics and the sites of platelet destruction were assessed in 16 normal subjects (13 with and three without spleens), in 17 studies of patients with primary autoimmune thrombocytopenic purpura (AITP), in six studies of patients with secondary AITP, in ten studies of patients with AITP following splenectomy, and in five thrombocytopenic patients with myelodysplastic syndromes. In normal subjects, the spleen accounted for 24 +/- 4% of platelet destruction and the liver for 15 +/- 2%. Untreated patients with primary AITP had increased splenic destruction (40 +/- 14%, p less than 0.001) but not hepatic destruction (13 +/- 5%). Compared with untreated patients, prednisone treated patients did not have significantly different spleen and liver platelet sequestration. Patients with secondary AITP had similar platelet counts, platelet survivals, and increases in splenic destruction of platelets as did patients with primary AITP. In contrast, patients with myelodysplastic syndromes had a normal pattern of platelet destruction. In AITP patients following splenectomy, the five nonresponders all had a marked increase (greater than 45%) in liver destruction compared to five responders (all less than 40%). Among all patients with primary or secondary AITP, there was an inverse relationship between the percent of platelets destroyed in the liver plus spleen and both the platelet count (r = 0.75, p less than 0.001) and the platelet survival (r = 0.86, p less than 0.001). In a stepwise multiple linear regression analysis, total liver plus spleen platelet destruction, the platelet survival and the platelet turnover were all significant independent predictors of the platelet count. Thus platelet destruction is shifted to the spleen in primary and secondary AITP. Failure of splenectomy is associated with a marked elevation in liver destruction

Insufficient time for leisure and perceived health and stress in working parents with small children.

Science.gov (United States)

Håkansson, Carita; Axmon, Anna; Eek, Frida

2016-10-17

More knowledge about how recovery may promote health among parents with small children is needed. To explore whether insufficient time for leisure was associated with poorer perceived health and higher stress in working parents. A further aim was to explore potential gender differences in the association between insufficient time for leisure and poor perceived health. A postal survey including the perceived stress scale and three measures of subjective health - self-rated health (SF-36), work-related fatigue (Swedish occupational fatigue questionnaire), and Lund subjective health complaints - as well as questions about time for leisure was completed by 965 women and 597 men. Risk ratios for poor perceived health and stress were estimated using Poisson regression, in which also gender interaction was analysed. The results showed higher risk for perceived stress among parents reporting insufficient time for relaxation, and more subjective health complaints among those reporting insufficient time to spend with their children. Overall, effects were larger among women than among men. A good balance between work and leisure seems to be of importance for working parents' perceived health and stress.

Diuretics, calciuria and secondary hyperparathyroidism in the Chronic Renal Insufficiency Cohort.

Science.gov (United States)

Isakova, Tamara; Anderson, Cheryl A M; Leonard, Mary B; Xie, Dawei; Gutiérrez, Orlando M; Rosen, Leigh K; Theurer, Jacquie; Bellovich, Keith; Steigerwalt, Susan P; Tang, Ignatius; Anderson, Amanda Hyre; Townsend, Raymond R; He, Jiang; Feldman, Harold I; Wolf, Myles

2011-04-01

Secondary hyperparathyroidism is a common complication of chronic kidney disease (CKD) that is associated with bone disease, cardiovascular disease and death. Pathophysiological factors that maintain secondary hyperparathyroidism in advanced CKD are well-known, but early mechanisms of the disease that can be targeted for its primary prevention are poorly understood. Diuretics are widely used to control volume status and blood pressure in CKD patients but are also known to have important effects on renal calcium handling, which we hypothesized could alter the risk of secondary hyperparathyroidism. We examined the relationship of diuretic treatment with urinary calcium excretion, parathyroid hormone (PTH) levels and prevalence of secondary hyperparathyroidism (PTH ≥ 65 pg/mL) in a cross-sectional study of 3616 CKD patients in the Chronic Renal Insufficiency Cohort. Compared with no diuretics, treatment with loop diuretics was independently associated with higher adjusted urinary calcium (55.0 versus 39.6 mg/day; P diuretics. However, coadministration of thiazide and loop diuretics was associated with blunted urinary calcium (30.3 versus 55.0 mg/day; P diuretics alone. Loop diuretic use was associated with greater calciuria, PTH levels and odds of secondary hyperparathyroidism compared to no treatment. These associations were attenuated in patients who were coadministered thiazides. Diuretic choice is a potentially modifiable determinant of secondary hyperparathyroidism in CKD.

Telomere Elongation and Naive Pluripotent Stem Cells Achieved from Telomerase Haplo-Insufficient Cells by Somatic Cell Nuclear Transfer

Directory of Open Access Journals (Sweden)

Li-Ying Sung

2014-12-01

Full Text Available Summary: Haplo-insufficiency of telomerase genes in humans leads to telomere syndromes such as dyskeratosis congenital and idiopathic pulmonary fibrosis. Generation of pluripotent stem cells from telomerase haplo-insufficient donor cells would provide unique opportunities toward the realization of patient-specific stem cell therapies. Recently, pluripotent human embryonic stem cells (ntESCs have been efficiently achieved by somatic cell nuclear transfer (SCNT. We tested the hypothesis that SCNT could effectively elongate shortening telomeres of telomerase haplo-insufficient cells in the ntESCs with relevant mouse models. Indeed, telomeres of telomerase haplo-insufficient (Terc+/− mouse cells are elongated in ntESCs. Moreover, ntESCs derived from Terc+/− cells exhibit naive pluripotency as evidenced by generation of Terc+/− ntESC clone pups by tetraploid embryo complementation, the most stringent test of naive pluripotency. These data suggest that SCNT could offer a powerful tool to reprogram telomeres and to discover the factors for robust restoration of telomeres and pluripotency of telomerase haplo-insufficient somatic cells. : Sung et al. demonstrate in a mouse model that telomeres of telomerase haplo-insufficient cells can be elongated by somatic cell nuclear transfer. Moreover, ntESCs derived from Terc+/− cells exhibit pluripotency evidenced by generation of Terc+/−ntESC clone pups by tetraploid embryo complementation, the most stringent test of naive pluripotency.

Duration of adrenal insufficiency during treatment for childhood acute lymphoblastic leukemia

DEFF Research Database (Denmark)

Vestergaard, Therese Risom; Juul, Anders; Lausten-Thomsen, Ulrik

2011-01-01

Children with acute lymphoblastic leukemia (ALL) recive high doses of glucocorticosteroid as part of their treatment. This may lead to suppression of the hypothalamic-pituitary-adrenal axis, acute adrenal insufficiency, and ultimately to life-threatening conditions. This study explores the adrena...

Helicobacter pylori Eradication in Patients with Immune Thrombocytopenic Purpura: A Review and the Role of Biogeography.

Science.gov (United States)

Frydman, Galit H; Davis, Nick; Beck, Paul L; Fox, James G

2015-08-01

Idiopathic thrombocytopenic purpura (ITP) is typically a diagnosis of exclusion, assigned by clinicians after ruling out other identifiable etiologies. Since a report by Gasbarrini et al. in 1998, an accumulating body of evidence has proposed a pathophysiological link between ITP and chronic Helicobacter pylori (H. pylori) infection. Clinical reports have described a spontaneous resolution of ITP symptoms in about 50% of chronic ITP patients following empirical treatment of H. pylori infection, but response appears to be geography dependent. Studies have also documented that ITP patients in East Asian countries are more likely to express positive antibody titers against H. pylori-specific cytotoxic-associated gene A (CagA), a virulence factor that is associated with an increased risk for gastric diseases including carcinoma. While a definitive mechanism by which H. pylori may induce thrombocytopenia remains elusive, proposed pathways include molecular mimicry of CagA by host autoantibodies against platelet surface glycoproteins, as well as perturbations in the phagocytic activity of monocytes. Traditional treatments of ITP have been largely empirical, involving the use of immunosuppressive agents and immunoglobulin therapy. However, based on the findings of clinical reports emerging over the past 20 years, health organizations around the world increasingly suggest the detection and eradication of H. pylori as a treatment for ITP. Elucidating the exact molecular mechanisms of platelet activation in H. pylori-positive ITP patients, while considering biogeographical differences in response rates, could offer insight into how best to use clinical H. pylori eradication to treat ITP, but will require well-designed studies to confirm the suggested causative relationship between bacterial infection and an autoimmune disease state. © 2015 John Wiley & Sons Ltd.

Helicobacter pylori Eradication in Patients with Immune Thrombocytopenic Purpura: A Review and the Role of Biogeography

Science.gov (United States)

Frydman, Galit H.; Davis, Nick; Beck, Paul L.; Fox, James G.

2015-01-01

Idiopathic thrombocytopenic purpura (ITP) is typically a diagnosis of exclusion, assigned by clinicians after ruling out other identifiable etiologies. Since a report by Gasbarrini et al. in 1998, an accumulating body of evidence has proposed a pathophysiological link between ITP and chronic Helicobacter pylori (H. pylori) infection. Clinical reports have described a spontaneous resolution of ITP symptoms in about 50% of chronic ITP patients following empirical treatment of H. pylori infection, but response appears to be geography dependent. Studies have also documented that ITP patients in East Asian countries are more likely to express positive antibody titers against H. pylori-specific cytotoxic-associated gene A (CagA), a virulence factor that is associated with an increased risk for gastric diseases including carcinoma. While a definitive mechanism by which H. pylori may induce thrombocytopenia remains elusive, proposed pathways include molecular mimicry of CagA by host autoantibodies against platelet surface glycoproteins, as well as perturbations in the phagocytic activity of monocytes. Traditional treatments of ITP have been largely empirical, involving the use of immunosuppressive agents and immunoglobulin therapy. However, based on the findings of clinical reports emerging over the past 20 years, health organizations around the world increasingly suggest the detection and eradication of H. pylori as a treatment for ITP. Elucidating the exact molecular mechanisms of platelet activation in H. pylori-positive ITP patients, while considering biogeographical differences in response rates, could offer insight into how best to use clinical H. pylori eradication to treat ITP, but will require well-designed studies to confirm the suggested causative relationship between bacterial infection and an autoimmune disease state. PMID:25728540

Tricuspid insufficiency detected by equilibrium gated radionuclide study

International Nuclear Information System (INIS)

Handler, B.; Pavel, D.G.; Lam, W.; Byrom, E.; Swiryn, S.; Pietras, R.; Rosen, K.M.

1981-01-01

The results of a gated radionuclide cardiac study are reported in a patient with biventricular failure and tricuspid insufficiency demonstrated by clinical evaluation, M-mode and 2-D sector echocardiography, and cardia catheterization. The processed gated radionuclide cardiac study showed a left ventricular/right ventricular stroke volume ratio of 0.5; expansion of the hepatic blood pool demonstrated by hepatic time activity curve and calculation of an '''expansion fraction''; and synchronous changes of count rate of the atrial and hepatic regions detected by phase analysis

The efficacy of supplemental intraosseous anesthesia after insufficient mandibular block

Directory of Open Access Journals (Sweden)

Samir Prohić

2005-02-01

Full Text Available It is a well-known scientific fact that only a small percentage of infiltration of inferior alveolar nerve is clinically proven to be efficient. The objective of this study was to determine the anesthetic efficacy of supplemental intraosseous injection, used after the insufficient classical mandibular block that didn't provide deep pulp anesthesia of mandibular molar planed for extraction. The experimental teeth consisted of 98 mandibular molars with clinical indication for extraction. Based on the history of disease, we indicated the extraction of the tooth. After that each tooth was tested with a electric pulp tester P1. We tested the pulp vitality and precisely determined the level of vitality. After that, each patient received classical mandibular block, and the pulp vitality was tested again. If the pulp tester indicated negative vitality for the certain mandibular molar, and the patient didn't complain about pain or discomfort during the extraction, the molar was extracted and the result was added to anesthetic success rate for the classical mandibular block. If, five minutes after receiving the mandibular block, the pulp tester indicated positive vitality (parameters of vitality or the patient complained about pain or discomfort (parameters of pain and discomfort, we used the Stabident intraosseous anesthesia system. Three minutes after the application of supplemental intraosseous injection the molar was tested with the pulp tester again. The anesthetic solution used in both anesthetic techniques is lidocaine with 1:100.000 epinephrine. The results of this study indicate that the anesthetic efficacy of the mandibular block is 74.5%, and that supplemental intraosseous anesthesia, applied after the insufficient mandibular block, provides pulpal anesthesia in 94.9% of mandibular molars. The difference between anesthetic efficacy of the classical mandibular block and anesthetic efficacy of the supplemental intraosseous anesthesia, applied

The efficacy of supplemental intraosseous anesthesia after insufficient mandibular block.

Science.gov (United States)

Prohić, Samir; Sulejmanagić, Halid; Secić, Sadeta

2005-02-01

It is a well-known scientific fact that only a small percentage of infiltration of inferior alveolar nerve is clinically proven to be efficient. The objective of this study was to determine the anesthetic efficacy of supplemental intraosseous injection, used after the insufficient classical mandibular block that didn't provide deep pulp anesthesia of mandibular molar planed for extraction. The experimental teeth consisted of 98 mandibular molars with clinical indication for extraction. Based on the history of disease, we indicated the extraction of the tooth. After that each tooth was tested with a electric pulp tester P1. We tested the pulp vitality and precisely determined the level of vitality. After that, each patient received classical mandibular block, and the pulp vitality was tested again. If the pulp tester indicated negative vitality for the certain mandibular molar, and the patient didn't complain about pain or discomfort during the extraction, the molar was extracted and the result was added to anesthetic success rate for the classical mandibular block. If, five minutes after receiving the mandibular block, the pulp tester indicated positive vitality (parameters of vitality) or the patient complained about pain or discomfort (parameters of pain and discomfort), we used the Stabident intraosseous anesthesia system. Three minutes after the application of supplemental intraosseous injection the molar was tested with the pulp tester again. The anesthetic solution used in both anesthetic techniques is lidocaine with 1:100.000 epinephrine. The results of this study indicate that the anesthetic efficacy of the mandibular block is 74.5%, and that supplemental intraosseous anesthesia, applied after the insufficient mandibular block, provides pulpal anesthesia in 94.9% of mandibular molars. The difference between anesthetic efficacy of the classical mandibular block and anesthetic efficacy of the supplemental intraosseous anesthesia, applied after the

Community nephrology: audit of screening for renal insufficiency in a high risk population.

Science.gov (United States)

Kissmeyer, L; Kong, C; Cohen, J; Unwin, R J; Woolfson, R G; Neild, G H

1999-09-01

The rate of acceptance onto dialysis programmes has doubled in the past 10 years and is steadily increasing. Early detection and treatment of renal failure slows the rate of progression. Is it feasible to screen for patients who are at increased risk of developing renal failure? We have audited primary care records of patients aged 50-75 years who have either hypertension or diabetes, and are therefore considered to be at high risk of developing renal insufficiency. Our aim was to see whether patients had had their blood pressure measured and urine tested for protein within 12 months, and plasma creatinine measured within 24 months. This was a retrospective study of case notes and computer records in 12 general practices from inner and greater London. A total of 16,855 patients were aged 50-75 years. From this age group, 2693 (15.5%) patients were identified as being either hypertensive or diabetic, or both. Of the 2561 records audited, 1359 (53.1%) contained a plasma creatinine measured within 24 months, and 11% of these (150) had a value > 125 micromol/l. This equates to a prevalence of renal insufficiency of > 110,000 patients per million in this group. Forty two patients (28%) had been referred to a nephrologist. Of records audited, 73% contained a blood pressure measurement and 29% contained a test for proteinuria within 12 months. There is a high prevalence of chronic renal insufficiency in hypertensive and diabetic patients. It is feasible to detect renal insufficiency at a primary care level, but an effective system will require computerized databases that code for age, ethnicity, measurement of blood pressure and renal function, as well as diagnoses.

Elevated hair cortisol concentrations in children with adrenal insufficiency on hydrocortisone replacement therapy.

Science.gov (United States)

Noppe, G; van Rossum, E F C; Vliegenthart, J; Koper, J W; van den Akker, E L T

2014-12-01

Glucocorticoid replacement therapy in patients with adrenal insufficiency needs to be tailored to the individual patient based on body composition and clinical signs and symptoms as no objective method for assessment of treatment adequacy is available. Current treatment regimens are often not satisfactory, which is shown by the adverse metabolic profile and doubled mortality rates in treated adrenal insufficiency patients. Measurement of cortisol concentrations in hair reflect the long-term systemic cortisol exposure and may be of use in refinement of hydrocortisone treatment. We aimed to study whether long-term cortisol (hydrocortisone) levels, as measured in scalp hair, are similar in children with adrenal insufficiency and healthy children. We set up a case control study, measuring anthropometric characteristics and hair cortisol concentrations (HCC) in 54 hydrocortisone substituted children with adrenal insufficiency (AI patients) in the age of 4-18 years and 54 healthy children matched for gender and age. Mean HCC were significantly higher in AI patients compared with healthy controls (mean 13·3 vs 8·2 pg/mg, P = 0·02). AI patients also had a higher BMI (P < 0·001) and waist circumference (WC) (P = 0·02). HCC was significantly associated with BMI (P = 0·002) and WC (P = 0·002). HCC explained 13% of the difference in BMI and 29% of the difference in WC between AI patients and controls. Hydrocortisone-treated AI patients have increased HCC and adverse anthropometric characteristics compared with healthy controls. HCC measurement may be of value in identifying overtreatment and thereby improve hydrocortisone replacement therapy. © 2014 John Wiley & Sons Ltd.

An evaluation of clinical treatment of convergence insufficiency for children with reading difficulties

Directory of Open Access Journals (Sweden)

Dusek Wolfgang A

2011-08-01

Full Text Available Abstract Background The present study investigates two different treatment options for convergence insufficiency CI for a group of children with reading difficulties referred by educational institutes to a specialist eye clinic in Vienna. Methods One hundred and thirty four subjects (aged 7-14 years with reading difficulties were referred from an educational institute in Vienna, Austria for visual assessment. Each child was given either 8Δ base-in reading spectacles (n = 51 or computerised home vision therapy (HTS (n = 51. Thirty two participants refused all treatment offered (clinical control group. A full visual assessment including reading speed and accuracy were conducted pre- and post-treatment. Results Factorial analyses demonstrated statistically significant changes between results obtained for visits 1 and 2 for total reading time, reading error score, amplitude of accommodation and binocular accommodative facility (within subjects effects (p Conclusions Reading difficulties with no apparent intellectual or psychological foundation may be due to a binocular vision anomaly such as convergence insufficiency. Both the HTS and prismatic correction are highly effective treatment options for convergence insufficiency. Prismatic correction can be considered an effective alternative to HTS.

Why do patients with severe arterial insufficiency get pain during sleep?

DEFF Research Database (Denmark)

Jelnes, Rolf; Bülow, J; Tønnesen, K H

1987-01-01

Simultaneous measurement during 24 h of mean arterial blood pressure (MABP) and forefoot subcutaneous adipose tissue blood flow (SBF) was undertaken in eight patients (15 feet) with different degrees of arterial insufficiency. The recordings were undertaken with the patients in the supine position...

Autoimmune hyperthyroidism due to secondary adrenal insufficiency: resolution with glucocorticoids.

Science.gov (United States)

Skamagas, Maria; Geer, Eliza B

2011-01-01

To describe the course of autoimmune hyperthyroid disease in a patient with corticotropin (ACTH) deficiency treated with glucocorticoids. We report the clinical presentation, laboratory data, imaging studies, and management of a patient with weight loss, fatigue, apathy, hallucinations, and arthritis. Autoimmune hyperthyroidism (positive thyroperoxidase and thyroglobulin antibodies and borderline positive thyrotropin receptor antibody) was diagnosed in a 71-year-old woman. New psychotic symptoms prompted brain magnetic resonance imaging, which revealed a partially empty sella. Undetectable morning cortisol, undetectable ACTH, and failure to stimulate cortisol with synthetic ACTH (cosyntropin 250 mcg) secured the diagnosis of long-standing secondary adrenal insufficiency. Hydrocortisone replacement improved the patient's symptoms, resolved the thyroid disease, and decreased thyroid antibody titers. In retrospect, the patient recalled severe postpartum hemorrhage requiring blood transfusion at age 38 years. A Sheehan event probably occurred 33 years before the patient presented with corticotropin deficiency. Hyperthyroidism accelerated cortisol metabolism and provoked symptoms of adrenal insufficiency. The hypocortisolemic state may precipitate hyperimmunity and autoimmune thyroid disease. Rapid resolution of hyperthyroidism and decreased thyroid antibody titers with glucocorticoid treatment support this hypothesis.

Two-stage triolein breath test differentiates pancreatic insufficiency from other causes of malabsorption

International Nuclear Information System (INIS)

Goff, J.S.

1982-01-01

In 24 patients with malabsorption, [ 14 C]triolein breath tests were conducted before and together with the administration of pancreatic enzymes (Pancrease, Johnson and Johnson, Skillman, N.J.). Eleven patients with pancreatic insufficiency had a significant rise in peak percent dose per hour 14 CO 2 excretion after Pancrease, whereas 13 patients with other causes of malabsorption had no increase in 14 CO 2 excretion (2.61 +/- 0.96 vs. 0.15 +/- 0.45, p less than 0.001). The two-stage [ 14 C]triolein breath test appears to be an accurate and simple noninvasive test of fat malabsorption that differentiates steatorrhea secondary to pancreatic insufficiency from other causes of steatorrhea

Impact of chronic Immune Thrombocytopenic Purpura (ITP on health-related quality of life: a conceptual model starting with the patient perspective

Directory of Open Access Journals (Sweden)

George James N

2008-02-01

Full Text Available Abstract Background Immune thrombocytopenic purpura (ITP, a condition characterized by autoimmune-mediated platelet destruction and suboptimal platelet production, is associated with symptoms such as bruising, epistaxis, menorrhagia, mucosal bleeding from the gastrointestinal and urinary tracts and, rarely central nervous system bleeding. The aim of this research is to develop a conceptual model to describe the impact of ITP and its treatment on patients' health-related quality of life (HRQoL. Methods A literature search and focus groups with adult ITP patients were conducted to identify areas of HRQoL affected by ITP. Published literature was reviewed to identify key HRQoL issues and existing questionnaires used to assess HRQoL. Focus group transcripts were reviewed, and common themes were extracted by grouping conceptual categories that described the impact on HRQoL. Results The literature synthesis and themes from the focus group data suggest that decreased platelet counts, disease symptoms, and treatment side effects influence multiple domains of HRQoL for ITP patients. Key areas affected by ITP and its treatments include emotional and functional health, work life, social and leisure activities, and reproductive health. Conclusion ITP affects various areas of HRQoL. This conceptual model will help inform the evaluation of therapeutic strategies for ITP.

Urinary hemosiderin: role in evaluation of chronic venous insufficiency

Directory of Open Access Journals (Sweden)

Ashish Lal Shrestha

2012-08-01

Full Text Available Chronic venous insufficiency (CVI leads to skin changes with dermal hemosiderin deposition. We studied the presence of hemosiderin in the urine to assess if this could be used as a biochemical marker for CVI. Hereby we present a case control study conducted in a tertiary care centre in South India. There were 100 cases with evidence of advanced CVI (the Clinical-Etiology-Anatomy-Pathophysiology classification: C5, C6 confirmed by duplex scanning. Controls were 50 patients with leg ulcers due to other etiologies. All patients were subjected to urinary hemosiderin testing. In all 100 patients with CVI (C5 and C6 disease axial venous reflux was confirmed by duplex ultrasound. Superficial venous reflux was noted in 71% of patients and deep venous reflux in 54.%. Primary venous insufficiency was the etiology in 81% of patients. Only 4/100 patients had detectable amounts of hemosiderin in the urine. Urine hemosiderin testing to determine presence or absence of CVI yielded the following values: positive predictive value-80%; negative predictive value-33%; sensitivity-4% and specificity-98%. The test could not be recommended as a marker of CVI. In Indian patients urinary hemosiderin is not a useful screening test in CVI.

History of cervical insufficiency increases the risk of pelvic organ prolapse and stress urinary incontinence in parous women.

Science.gov (United States)

Sheyn, David; Addae-Konaedu, Kateena L; Bauer, Alison M; Dawodu, Konyinsola I; Hackney, David N; El-Nashar, Sherif A

2018-01-01

A likely contributor to pelvic floor disorders is injury and degradation of connective tissue components such as collagen and elastin, leading to weakening of the pelvic floor. Prior studies have found similar connective tissue component changes in women with cervical insufficiency (CI). However, the connection between pelvic floor disorders and cervical insufficiency has not previously been evaluated. Our objective was to determine whether a history of cervical insufficiency is associated with an increased risk of pelvic organ prolapse and stress urinary incontinence after controlling for confounders. The study used de-identified clinical data from a large multi-institution electronic health records HIPAA-compliant data web application, Explorys Inc. (Cleveland, Ohio, USA). Women with a history of at least one prior delivery after at least 20 weeks' gestation between the years 1999 and 2016 were identified. Logistic regression models were used to identify risk factors and adjust for confounders. The primary outcome was subsequent development of either stress incontinence or pelvic organ prolapse. A total of 1,182,650 women were identified, of whom 30,890 (2.6%) had a history of cervical cerclage or insufficiency. A history of cervical insufficiency was associated with an increased risk of either pelvic organ prolapse or stress urinary incontinence (aOR=1.93, 95%CI: 1.84-2.02). A history of cervical insufficiency was more strongly associated with an increased risk of pelvic organ prolapse (aOR=2.06, 95%CI: 1.91-2.21) than with stress urinary incontinence (aOR=1.91, 95%CI: 1.80-2.02). A history of cervical insufficiency is associated with an increased risk of development of pelvic organ prolapse and stress urinary incontinence. Copyright © 2017 Elsevier B.V. All rights reserved.

Autoimmune polyglandular syndrome type 3 (APS-3) among patients with premature ovarian insufficiency (POI).

Science.gov (United States)

Szlendak-Sauer, Katarzyna; Jakubik, Daniel; Kunicki, Michał; Skórska, Jolanta; Smolarczyk, Roman

2016-08-01

Autoimmune polyglandular syndrome type 3 - (APS-3), is defined as the coexistence of autoimmune thyroiditis with other non-ovarian autoimmune diseases without primary adrenal insufficiency. Additionally the definition of APS-3 also includes primary ovarian insufficiency (POI) coexistence with autoimmune thyroiditis. The main goal of that study is to assess the prevalence of APS-3 defined as coexistence of autoimmune thyroiditis with POI in population of 46 XX karyotype women with primary ovarian insufficiency (POI). The second goal is to investigate hormonal profile and insulin sensitivity in women with POI and subgroups of women with APS-3 - POI/APS-3(+) and without APS 3 - POI/APS-3(-). Anthropometric measurements, coexistence of autoimmune diseases, androgens, fasting glucose and insulin, glucose and insulin at 60' and 120' of oral glucose tolerance test (OGTT) and homeostasis model for insulin resistance (HOMA-IR), were determine in 98 patients aged between 18 and 39 with spontaneous 46 XX primary ovarian insufficiency (POI), in 33 POI/APS-3(+), 65 POI/APS-3(-), and 75 healthy controls. Continuous data were summarized by the mean±standard deviation (SD), and categorical data by number (percentages). Data were checked for normality using Shapiro-Wilk test, the comparison between groups were performed using non-parametric Mann-Whitney or Kruskall-Wallis test. Pearson's correlation coefficient was used to assess the relationships between parameters. Statistical significance was defined as p values APS-3(+) and POI/APS-3(-) showed significantly lower serum androgens in comparison to controls. Additionally women with POI/APS-3(+) showed hyperinsulinemia after 1h of OGTT; No significant differences in serum fasting glucose, insulin and during 2h OGTT between groups were observed. The prevalence of APS-3 is 33.7% in patients with spontaneous 46 XX primary ovarian insufficiency. Women with POI, POI/APS-3(+) and POI/APS-3(-) feature lower testosterone, androstendione

Distal vertebral artery reconstruction when managing vertebrobasilar insufficiency

Directory of Open Access Journals (Sweden)

D. M. Galaktionov

2017-11-01

Full Text Available This article presents a literature review devoted to the reconstruction of the distal vertebral artery and a clinical case of successful surgical treatment of a patient suffering from vertebrobasilar insufficiency caused by occlusion of the vertebral artery in a proximal segment. The external carotid artery-distal vertebral artery bypass was performed by using the radial artery.Received 27 February 2017. Revised 25 July 2017. Accepted 3 August 2017.Funding: The study did not have sponsorship.Conflict of interest: The authors declare no conflict of interest. 

Cervical collagen is reduced in non-pregnant women with a history of cervical insufficiency and a short cervix

DEFF Research Database (Denmark)

Sundtoft, Iben; Langhoff-Roos, Jens; Sandager, Puk

2017-01-01

INTRODUCTION: Preterm cervical shortening and cervical insufficiency may be caused by a constitutional weakness of the cervix. The aim of this study was to assess the cervical collagen concentration in non-pregnant women with a history of cervical insufficiency or of a short cervix in the second...... trimester of pregnancy. MATERIAL AND METHODS: In this case-control study we included non-pregnant women one year or more after pregnancy: 55 controls with a history of normal delivery; 27 women with a history of cervical insufficiency; and 10 women with a history of a short cervix (... women with a history of a long cervix (>95th percentile) at gestational weeks 18-20. We obtained biopsies (3 × 3-4 mm) from the ectocervix and determined the collagen concentration by measuring the hydroxyproline concentration. RESULTS: Women with cervical insufficiency had lower collagen concentrations...

PEGylated anticancer-carbon nanotubes complex targeting mitochondria of lung cancer cells

Science.gov (United States)

Kim, Sang-Woo; Lee, Yeon Kyung; Lee, Jong Yeon; Hong, Jeong Hee; Khang, Dongwoo

2017-11-01

Although activating apoptosis in cancer cells by targeting the mitochondria is an effective strategy for cancer therapy, insufficient targeting of the mitochondria in cancer cells restricts the availability in clinical treatment. Here, we report on a polyethylene glycol-coated carbon nanotube (CNT)-ABT737 nanodrug that improves the mitochondrial targeting of lung cancer cells. The polyethylene glycol-coated CNT-ABT737 nanodrug internalized into the early endosomes via macropinocytosis and clathrin-mediated endocytosis in advance of early endosomal escape and delivered into the mitochondria. Cytosol release of the nanodrug led to apoptosis of lung cancer cells by abruption of the mitochondrial membrane potential, inducing Bcl-2-mediated apoptosis and generating intracellular reactive oxygen species. As such, this study provides an effective strategy for increasing the anti-lung cancer efficacy by increasing mitochondria accumulation rate of cytosol released anticancer nanodrugs.

The Soft Palate Friendly Speech Bulb for Velopharyngeal Insufficiency

OpenAIRE

Kahlon, Sukhdeep Singh; Kahlon, Monaliza; Gupta, Shilpa; Dhingra, Parvinder Singh

2016-01-01

Velopharyngeal insufficiency is an anatomic defect of the soft palate making palatopharyngeal sphincter incomplete. It is an important concern to address in patients with bilateral cleft lip and palate. Speech aid prosthesis or speech bulbs are best choice in cases where surgically repaired soft palate is too short to contact pharyngeal walls during function but these prosthesis have been associated with inadequate marginal closure, ulcerations and patient discomfort. Here is a case report of...

Low incidence of adrenal insufficiency after transsphenoidal surgery in patients with acromegaly: a long-term follow-up study

NARCIS (Netherlands)

Burgers, Anne Marij G.; Kokshoorn, Nieke E.; Pereira, Alberto M.; Roelfsema, Ferdinand; Smit, Johannes W. A.; Biermasz, Nienke R.; Romijn, Johannes A.

2011-01-01

The long-term prevalence of adrenal insufficiency after transsphenoidal surgery for GH-secreting pituitary adenomas is unknown. However, recently a single study reported a high prevalence of adrenal insufficiency in acromegalic patients after surgical and/or medical treatment without postoperative

Changes of serum aldosterone levels in patients with different stages of chronic renal insufficiency

International Nuclear Information System (INIS)

Zou Jun; Du Xueliang; Jiang Gengru

2005-01-01

Objective: To study the correlationship between the serum aldosterone levels and different stages of chronic renal insufficiency. Methods: Plasma renin activity (PRA), serum angiotensin II (Ang II) contents and serum aldosterone concentration (SACs) were determined with RIA in 42 patients with chronic renal insufficiency from various causes. The patients were divided into three groups according to their endogenous creatinine clearance rate: Group 1, (n=14) Ccr≥60ml/(min·1.73m 2 ); Group 2, (n =13) 20ml/(min·1.73m 2 ) ≤Ccr 2 ); Group 3, (n=15) Ccr 2 ). Results: The SACs values in Group 3 patients were significantly higher than those in Group 1 and Group 2 patients (P<0.01). The SACs values in Group 2 patients were also significantly higher than those in Group 1 patients (P<0.05). Ccr values were higher negatively correlated with the SACs values (r= -0.685, P<0.001). Conclusion: As the creatine clearance rate gradually deteriorated, the SACs values increased correspondingly in patients with chronic renal insufficiency from various causes. (authors)

Prevalence and Effects of Functional Vitamin K Insufficiency : The PREVEND Study

NARCIS (Netherlands)

Riphagen, Ineke J; Keyzer, Charlotte A; Drummen, Nadja E A; de Borst, Martin H; Beulens, Joline W J; Gansevoort, Ron T; Geleijnse, Johanna M; Muskiet, Frits A J; Navis, Gerjan; Visser, Sipke T; Vermeer, Cees; Kema, Ido P; Bakker, Stephan J L

2017-01-01

Matrix Gla Protein (MGP) is a strong vitamin K-dependent inhibitor of soft tissue calcification. We assessed the prevalence of functional vitamin K insufficiency, as derived from plasma desphospho-uncarboxylated MGP (dp-ucMGP), and investigated whether plasma dp-ucMGP is associated with all-cause

Factors predicting the duration of adrenal insufficiency in patients successfully treated for Cushing disease and nonmalignant primary adrenal Cushing syndrome.

Science.gov (United States)

Prete, Alessandro; Paragliola, Rosa Maria; Bottiglieri, Filomena; Rota, Carlo Antonio; Pontecorvi, Alfredo; Salvatori, Roberto; Corsello, Salvatore Maria

2017-03-01

Successful treatment of Cushing syndrome causes transient or permanent adrenal insufficiency deriving from endogenous hypercortisolism-induced hypothalamus-pituitary-adrenal-axis suppression. We analyzed pre-treatment factors potentially affecting the duration of adrenal insufficiency. We conducted a retrospective analysis on patients successfully treated for Cushing disease (15 patients) who underwent transsphenoidal surgery, and nonmalignant primary adrenal Cushing syndrome (31 patients) who underwent unilateral adrenalectomy, divided into patients with overt primary adrenal Cushing syndrome (14 patients) and subclinical primary adrenal Cushing syndrome (17 patients). Epidemiological data, medical history, and hormonal parameters depending on the etiology of hypercortisolism were collected and compared to the duration of adrenal insufficiency. The median duration of follow-up after surgery for Cushing disease and primary adrenal Cushing syndrome was 70 and 48 months, respectively. In the Cushing disease group, the median duration of adrenal insufficiency after transsphenoidal surgery was 15 months: younger age at diagnosis and longer duration of signs and symptoms of hypercortisolism before diagnosis and surgery were associated with longer duration of adrenal insufficiency. The median duration of adrenal insufficiency was 6 months for subclinical primary adrenal Cushing syndrome and 18.5 months for overt primary adrenal Cushing syndrome. The biochemical severity of hypercortisolism, the grade of hypothalamus-pituitary-adrenal-axis suppression, and treatment with ketoconazole before surgery accounted for longer duration of adrenal insufficiency. In patients with Cushing disease, younger age and delayed diagnosis and treatment predict longer need for glucocorticoid replacement therapy after successful transsphenoidal surgery. In patients with primary adrenal Cushing syndrome, the severity of hypercortisolism plays a primary role in influencing the duration of

Bilateral visual loss and cerebral infarction after spleen embolization in a trauma patient with idiopathic thrombocytopenic purpura: A case report.

Science.gov (United States)

Wang, Wei-Ting; Li, Yu-Yu; Lin, Wan-Ching; Chen, Jen-Yin; Lan, Kuo-Mao; Sun, Cheuk-Kwan; Hung, Kuo-Chuan

2018-04-01

Splenic artery embolization (SAE) is a common procedure in trauma patients with blunt splenic injuries. We report a case of acute ischemic stroke following orthopedic surgery in a patient with post-SAE reactive thrombocytosis. A 37-year-old woman with idiopathic thrombocytopenic purpura (ITP) suffered from multiple trauma scheduled for open reduction and internal fixation for right tibial and left radius fracture five days after SAE. The patient did not have any thromboembolic complications, although the platelet counts increased from 43 × 10/L to 568 × 10/L within two days after SAE. Surgery was completed under general anesthesia with tracheal intubation without complications. The patient complained of visual loss followed by limb weakness on the fourth and eighth hour postoperatively. Magnetic resonance imaging (MRI) of head demonstrated ischemic change over bilateral basal ganglia, and occipital areas, suggesting the diagnosis of cortical blindness. To suppress platelet count and avoid platelet hyper-aggregation, anti-platelet drug (i.e., oral aspirin 100 mg daily), hydration, and hydroxyurea (i.e., 20 mg/kg daily) were used for the treatment of reactive thrombocytosis. Although right-sided hemiparesis persisted, the patient reported mild visual recovery. She was discharged four months after SAE with active rehabilitation. Our report highlights an increased risk of acute arterial thromboembolic events in patients with reactive thrombocytosis, especially those undergoing surgery.

Púrpura de Henoch-Schönlein com acometimento incomum de face Unusual face involvement in Henoch-Schönlein purpura

Directory of Open Access Journals (Sweden)

Carolina Bassoli de Azevedo

2009-12-01

Full Text Available A Púrpura de Henoch-Shönlein é uma vasculite de causa idiopática, que se caracteriza pelo depósito predominante de IgA na parede dos pequenos vasos, envolvendo tipicamente pele, intestino, articulações e glomérulo renal. O acometimento cutâneo ocorre principalmente em membros inferiores e região glútea, sendo raramente encontrado em face e membros superiores. Relatamos o caso de uma paciente do sexo feminino, com seis anos de idade, que apresentava lesões púrpuricas em região auricular, periorbital, malar, mentoniana e membros superiores, além de artralgia e volvo intestinal. Após tratamento cirúrgico e pulsoterapia com glicocorticoide, houve regressão dos sintomas, sem maiores complicações.Henoch-Schönlein Purpura is an idiopathic vasculitis characterized by deposits of immunoglobulin, mainly IgA, on the walls of small vessels, typically involving the skin, gut, joints, and renal glomeruli. Cutaneous involvement affects specially the lower limbs and buttocks, and it is seldom found on the face and upper limbs. We report the case of a 6-year old girl with purpuric lesions over the auricular, periorbital, malar, and mentonian regions and the upper limbs, arthralgia, and intestinal torsion. After surgical treatment and pulse therapy with glucocorticoids, her symptoms subsided without further complications.

Initial clinical experience with a novel biodegradable ring in patients with functional tricuspid insufficiency: Kalangos Biodegradable Tricuspid Ring.

Science.gov (United States)

Burma, O; Ustunsoy, H; Davutoglu, V; Celkan, M A; Kazaz, H; Pektok, E

2007-08-01

Tricuspid annuloplasty procedures have been widely performed in clinics for many years. The Kalangos Biodegradable Tricuspid Ring (Kalangos Biodegradable Tricuspid Ring, Bioring SA, Lonay, Switzerland) is a novel prosthesis for the treatment of tricuspid insufficiency. The aim of this study was to evaluate the clinical and echocardiographic results of this novel prosthesis for functional tricuspid insufficiency. Between October 2005 and May 2006, 15 patients with the diagnosis of moderate or severe functional tricuspid insufficiency were treated by implantation of a Kalangos Biodegradable Tricuspid Ring. All patients were evaluated clinically and by echocardiography preoperatively, and control tests were performed at the end of the 1st and 6th month following surgery. Moderate and severe insufficiency was documented in 11 and 4 patients, respectively, in the preoperative tests. 1 and 6 months after surgery, 4 patients had trace and 1 patient had mild tricuspid insufficiency, while 10 patients had none. At the 1st and 6th month follow-up, systolic pulmonary arterial pressure, right atrial dimension and right ventricular diastolic diameter were found to be significantly lower than the preoperative values ( P tricuspid valve area had decreased significantly at the end of the 1st month; however, no significant difference was found between the 1st and 6th month tests ( P > 0.05). Three-quarters of the annuloplasty ring had degraded at 6 months. No complications related to the prosthesis or the procedure occurred within this period. Kalangos Biodegradable Ring is a promising prosthesis in patients with functional tricuspid insufficiency, with encouraging initial results.

Cytogenetic Analysis of 65 Women with Premature Ovarian Insufficiency

OpenAIRE

Seda Ates

2016-01-01

Aim: Premature ovarian insufficiency (POI) is characterized as amenorrhea for more than 6 months, occurring before the age of 40, with an increased follicle-stimulating hormone and low estrogen concentrations. The aim of our study is to determine the types and distribution of cytogenetic abnormalities among women with POI. Material and Method: The study is based on the retrospective karyotype analysis of 65 women with idiopathic POI referred to the Medical Genetics Department at the Bezmialem...

Osteoporotic compression fracture of the thoracolumbar spine and sacral insufficiency fracture: incidence and analysis of the relationship according to the clinical factors

International Nuclear Information System (INIS)

Kong, Jeong Hwa; Park, Ji Sun; Ryu, Kyung Nam

2006-01-01

To evaluate the incidence of sacral insufficiency fracture in osteoporotic patient with compression fracture of the thoracolumbar (T-L) spine on magnetic resonance image (MRI), and to analyze the correlation of variable clinical factors and the incidence of sacral insufficiency fracture. We retrospectively reviewed 160 patients (27 men, 133 women; age range of 50 to 89 years) who underwent spinal MRI and had compression fracture of the T-L spine. Compression fractures due to trauma or tumor were excluded. We evaluated the incidence of sacral insufficiency fracture according to the patients' age, sex, number of compression fractures, and the existence of bone marrow edema pattern of compression fracture. During the same period, we evaluated the incidence of spinal compression fracture in the patients of pelvic insufficiency fracture. Out of the 160 patients who had compression fracture in the T-L spine, 17 (10.6%) had insufficiency fracture of the sacrum. Compression fracture occurred almost 5 times more frequently in women (27:133), but the incidence of sacral insufficiency fracture was 2/27 for men (7.4%) and 15/133 for women (11.3%), with no statistically significant difference (ρ = 0.80). According to age, the ratio of insufficiency fracture to compression fracture was 0% (0/23) in the 50's, 10.6% (7/66) in the 60's, 12.5% (7/56) in the 70's, and 20.0% (3/15) in the 80's. In respect of single and multiple compression fracture, the incidence of sacral insufficiency fracture was 8/65 for men (12.3%) and 9/95 for women (9.5%), showing no significant difference (ρ = 0.37). In the patients with and without compression fracture with bone marrow edema, insufficiency fracture occurred in 5/76 (6.6%) and 12/84 (14.3%), respectively. On the other hand, of the 67 patients who had pelvic insufficiency fracture, 27 (40.3%) also had spinal compression fracture. About 10% of the patients with osteoporotic compression fracture in the T/L spine also had pelvic sacral

Comparative analysis of intraoral radiographs with variation of tube angulation to detect insufficient crown margins.

Science.gov (United States)

Sailer, Benjamin F; Geibel, Margrit-Ann

2013-01-01

Variations in angulation of the x-ray tube affect the appearance of insufficient approximal crown margins on intraoral radiographs. This study examines the impact of such angular variation on the assessment of digital radiographs using three different X-ray tubes--Heliodent DS (Sirona), Gendex Expert DC (KaVo Dental) and Focus (KaVo Dental)--as well as the Gendex Visualix eHD CCD sensor (KaVo Dental). The test specimens, crowned teeth 46 from two mandibles provided by the Institute of Anatomy and Cell Biology, were examined with each tube. The results indicate great differences in the angles indicative of insufficient crown margins on X-ray images. Because of beam divergence and the crown marginal gap, the length and width of which frequently varies, it is difficult to infer any optimum angle from the data. This leads to the conclusion that at present, it is not possible to establish ideal angles for visualization of insufficient approximal crown margins.

A case report of neonatal alloimmune thrombocytopenic purpura: the importance of correct diagnosis for future pregnancies

Directory of Open Access Journals (Sweden)

Rita Fontão-Wendel

Full Text Available CONTEXT: Neonatal alloimmune thrombocytopenic purpura (NAITP is a neonatal disorder characterized by maternal alloimmunization against fetal platelet antigens inherited from the father. Intracranial hemorrhage leading to death or permanent neurological disability may occur in the fetus. CASE REPORT: A healthy 30-year-old woman gave birth to her first baby by cesarean after an uneventful 36-week pregnancy. Ten hours after birth, the infant presented severe petechiae, with platelet count of 8 x 10³/µl. The mother’s platelet count was normal (180 x 10³/µl. The infant received intravenous immunoglobulin and was discharged 18 days later, with platelet count of 100 x 10³/µl. The cause of thrombocytopenia was not elucidated at that time. One year later, the infant died of neuroblastoma. Since the parents wanted another child, they were referred for investigation of this thrombocytopenia. Platelet genotyping and platelet antibody screening were performed, showing total HPA-1 system mismatch between mother (HPA-1b1b and father (HPA-1a1a, with anti-HPA-1a antibodies in the mother’s serum. We concluded that the first baby was born with NAITP. Thus, in the second pregnancy, the mother was treated with several infusions of intravenous immunoglobulin. Careful ultrasound monitoring was performed, with normal results for mother and fetus throughout the pregnancy. The second baby was born by cesarean at 39 weeks, presenting 92x10³ platelets/µl six hours after birth. The baby’s platelets were genotyped as HPA-1a1b and the mother’s serum again showed anti-HPA-1a antibodies. No clinical bleeding was observed. Intravenous immunoglobulin therapy was an effective treatment for preventing NAITP in the second baby.

Diuretics, calciuria and secondary hyperparathyroidism in the Chronic Renal Insufficiency Cohort

Science.gov (United States)

Isakova, Tamara; Anderson, Cheryl A. M.; Leonard, Mary B.; Xie, Dawei; Gutiérrez, Orlando M.; Rosen, Leigh K.; Theurer, Jacquie; Bellovich, Keith; Steigerwalt, Susan P.; Tang, Ignatius; Anderson, Amanda Hyre; Townsend, Raymond R.; He, Jiang; Feldman, Harold I.; Wolf, Myles

2011-01-01

Background. Secondary hyperparathyroidism is a common complication of chronic kidney disease (CKD) that is associated with bone disease, cardiovascular disease and death. Pathophysiological factors that maintain secondary hyperparathyroidism in advanced CKD are well-known, but early mechanisms of the disease that can be targeted for its primary prevention are poorly understood. Diuretics are widely used to control volume status and blood pressure in CKD patients but are also known to have important effects on renal calcium handling, which we hypothesized could alter the risk of secondary hyperparathyroidism. Methods. We examined the relationship of diuretic treatment with urinary calcium excretion, parathyroid hormone (PTH) levels and prevalence of secondary hyperparathyroidism (PTH ≥ 65 pg/mL) in a cross-sectional study of 3616 CKD patients in the Chronic Renal Insufficiency Cohort. Results. Compared with no diuretics, treatment with loop diuretics was independently associated with higher adjusted urinary calcium (55.0 versus 39.6 mg/day; P secondary hyperparathyroidism (odds ratio 2.1; 95% CI 1.7–2.6). Thiazide monotherapy was associated with lower calciuria (25.5 versus 39.6 mg/day; P hyperparathyroidism (odds ratio 1.3 versus 2.1; P for interaction = 0.05) compared with loop diuretics alone. Conclusions. Loop diuretic use was associated with greater calciuria, PTH levels and odds of secondary hyperparathyroidism compared to no treatment. These associations were attenuated in patients who were coadministered thiazides. Diuretic choice is a potentially modifiable determinant of secondary hyperparathyroidism in CKD. PMID:21382989

A prospective evaluation of pancreatic exocrine function in patients with acute pancreatitis: correlation with extent of necrosis and pancreatic endocrine insufficiency.

Science.gov (United States)

Boreham, B; Ammori, B J

2003-01-01

The aim of this prospective study was to assess pancreatic exocrine function in patients recovering from a first attack of acute pancreatitis, and to evaluate its relationship to severity of attack, extent of pancreatic necrosis and severity of pancreatic endocrine insufficiency. Between December 2000 and November 2001, 23 patients were prospectively evaluated. Pancreatic exocrine function was measured by the faecal elastase-1 test and insufficiency was classified as moderately impaired or severely impaired. Pancreatic necrosis was determined by contrast-enhanced CT scan, and its extent was categorised according to Balthazar's classification. The severity of pancreatic endocrine insufficiency was categorised according to insulin dependence. Attacks were classified as mild (n = 16) or severe (n = 7) according to the Atlanta criteria. Pancreatic exocrine insufficiency was significantly more frequent in patients recovering from severe attacks than mild (n = 6, 86% vs. n = 2, 13%; p = 0.002), and in those who developed pancreatic necrosis or pseudocyst than those who did not (6 of 7 patients vs. 2 of 16 patients, and 5 of 5 patients vs. 3 of 18 patients respectively; p = 0.002). The development of exocrine insufficiency correlated strongly with the extent of pancreatic necrosis (r = -0.754, p pancreatic endocrine insufficiency (n = 4, r = -0.453, p = 0.03). Pancreatic exocrine insufficiency is a common occurrence in patients recovering from severe acute pancreatitis, and its severity correlates with the extent of pancreatic necrosis and the severity of concomitant pancreatic endocrine insufficiency. Copyright 2003 S. Karger AG, Basel and IAP

Food insufficiency and food insecurity as risk factors for physical disability among Palestinian refugees in Lebanon: Evidence from an observational study.

Science.gov (United States)

Salti, Nisreen; Ghattas, Hala

2016-10-01

Potential interactions between malnutrition and disability are increasingly recognized, and both are important global health issues. Causal effects working from nutrition to disability and from disability back to nutrition present an empirical challenge to measuring either of these effects. However, disability affects nutrition whatever the cause of disability, whereas nutrition is likelier to affect disease-related disability than war- or work-related disability. This paper investigates the association of food insufficiency with the risk of physical disability. Data on disability by cause allow us to address the difficulty of reverse causality. Multinomial logit regressions of disability by cause on food insufficiency are run using survey data from 2010 on 2575 Palestinian refugee households in Lebanon. Controls include household sociodemographic, health and economic characteristics. Regressions of food insufficiency on disability by cause are also run. Disability has a significant coefficient in regressions of food insufficiency, whatever the cause of disability; but in regressions of disability on food insufficiency, food insufficiency is significant only for disease-related disability (log odds of disease-related disability .78 higher, p = .008). The difference in the results by cause of disability is evidence of a significant association between food insufficiency and disease-related disability, net of any reverse effect from disability to food access. The association between disease-related disability and food insufficiency is statistically significant suggesting that even taking into account feedback from disability to nutrition, nutrition is an effective level of intervention to avert the poverty-disability trap resulting from the impoverishing effect of disability. Copyright © 2016 Elsevier Inc. All rights reserved.