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Sample records for syndrome complicating adalimumab

  1. [Peripheral corneal melting syndrome in psoriatic arthritis treated with adalimumab].

    Science.gov (United States)

    Restrepo, Juan Pablo; Medina, Luis Fernando; Molina, María del Pilar

    2015-01-01

    Peripheral corneal melting syndrome is a rare immune condition characterized by marginal corneal thinning and sometimes perforation. It is associated with rheumatic and non-rheumatic diseases. Few cases of peripheral corneal melting have been reported in patients with psoriasis. The pathogenesis is not fully understood but metalloproteinases may play a pathogenic role. Anti-TNF therapy has shown to decrease skin and serum metalloproteinases levels in psoriasis. We report a 61-year-old man with peripheral corneal melting syndrome associated with psoriatic arthritis who received Adalimumab to control skin and ocular inflammation. To our knowledge, this is the first case report of peripheral corneal melting syndrome in psoriatic arthritis treated with Adalimumab showing resolution of skin lesions and complete healing of corneal perforation in three months. Copyright © 2013 Elsevier Editora Ltda. All rights reserved.

  2. A randomized, double-blind, placebo controlled trial of adalimumab for interstitial cystitis/bladder pain syndrome.

    Science.gov (United States)

    Bosch, Philip C

    2014-01-01

    The efficacy of adalimumab for the treatment of interstitial cystitis/bladder pain syndrome was investigated in a phase III, randomized, double-blind, placebo controlled, proof of concept study. Patients with interstitial cystitis/bladder pain syndrome were randomized to receive a loading dose of 80 mg subcutaneous adalimumab followed by 40 mg every 2 weeks or subcutaneous placebo for 12 weeks, and outcome measures were assessed. The incidence of adverse events was also assessed. Of a total of 43 patients 21 received adalimumab and 22 received placebo. Of the patients who received adalimumab, there was a statistically significant improvement demonstrated in the O'Leary-Sant Interstitial Cystitis Symptom and Problem Indexes (p = 0.0002), Interstitial Cystitis Symptom Index (p = 0.0011), Interstitial Cystitis Problem Index (p = 0.0002), and Pelvic Pain, Urgency, Frequency Symptom Scale (p = 0.0017) at 12 weeks compared to baseline. At 12 weeks 11 of 21 (53%) patients in the adalimumab group had a 50% or greater improvement in global response assessment (p ≤ 0.0001). There was not a statistically significant improvement in any outcome measure in patients receiving adalimumab compared to placebo. There were no significant adverse events. Adalimumab treatment resulted in a statistically significant improvement in outcome measures compared to baseline in patients with moderate to severe interstitial cystitis/bladder pain syndrome. Adalimumab failed to demonstrate positive proof of concept compared to placebo due to a significant placebo effect. Copyright © 2014 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.

  3. Steroid-refractory extensive enteritis complicated by ulcerative colitis successfully treated with adalimumab

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    Shinji Okabayashi

    2017-10-01

    Full Text Available Extracolonic involvement of the gastrointestinal tract is extremely uncommon in ulcerative colitis (UC and rarely found in the upper gastrointestinal tract or in postoperative cases since it typically responds to steroids. Here we report a case of UC complicated by extensive ileal inflammation that was refractory to steroids. A 20-year-old man was diagnosed with UC of typical pancolitis without ileal involvement and started treatment with pH-dependent mesalazine and oral prednisolone. Although his symptoms transiently resolved, the condition flared when the steroid dose was tapered down. Computed tomography revealed marked thickening of the ileal wall, and capsule endoscopy and balloon-assisted enteroscopy found diffuse mucosal inflammation with ulcers in the ileum. On the contrary, the inflammation in the colon and rectum was improving. Since the response to the second steroid course was inadequate, treatment with adalimumab and 6-mercaptopurine was initiated and finally achieved clinical and endoscopic remission. The investigation of small intestinal lesions is necessary in patients with UC whose clinical deterioration cannot be explained by colonic lesions.

  4. Complications of nephrotic syndrome

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    Se Jin Park

    2011-08-01

    Full Text Available Nephrotic syndrome (NS is one of the most common glomerular diseases that affect children. Renal histology reveals the presence of minimal change nephrotic syndrome (MCNS in more than 80% of these patients. Most patients with MCNS have favorable outcomes without complications. However, a few of these children have lesions of focal segmental glomerulosclerosis, suffer from severe and prolonged proteinuria, and are at high risk for complications. Complications of NS are divided into two categories: disease-associated and drug-related complications. Disease-associated complications include infections (e.g., peritonitis, sepsis, cellulitis, and chicken pox, thromboembolism (e.g., venous thromboembolism and pulmonary embolism, hypovolemic crisis (e.g., abdominal pain, tachycardia, and hypotension, cardiovascular problems (e.g., hyperlipidemia, acute renal failure, anemia, and others (e.g., hypothyroidism, hypocalcemia, bone disease, and intussusception. The main pathomechanism of disease-associated complications originates from the large loss of plasma proteins in the urine of nephrotic children. The majority of children with MCNS who respond to treatment with corticosteroids or cytotoxic agents have smaller and milder complications than those with steroid-resistant NS. Corticosteroids, alkylating agents, cyclosporin A, and mycophenolate mofetil have often been used to treat NS, and these drugs have treatment-related complications. Early detection and appropriate treatment of these complications will improve outcomes for patients with NS.

  5. Lemierre's Syndrome Complicating Pregnancy

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    M. Thompson

    2007-01-01

    Full Text Available Lemierre's syndrome is an anaerobic suppurative thrombophlebitis involving the internal jugular vein secondary to oropharyngeal infection. There is only one previous case report in pregnancy which was complicated by premature delivery of an infant that suffered significant neurological damage. We present an atypical case diagnosed in the second trimester with a live birth at term. By reporting this case, we hope to increase the awareness of obstetricians to the possibility of Lemierre's syndrome when patients present with signs of unabating oropharyngeal infection and pulmonary symptoms.

  6. [Physiopathology and treatment of nephrotic syndrome complications].

    Science.gov (United States)

    Ducloux, Didier

    2011-02-01

    The nephrotic syndrome is defined by a urinary protein excretion exceeding 3g per day, associated with hypoalbuminaemia (syndrome are multiple, essentially dominated by sodium retention and oedema formation. The oedema physiopathology is related to both increased capillary permeability and primary activation of the Na/K pump in the collect duct. Other complications of the nephrotic syndrome include thromboembolic complications, dyslipidaemia, and infections. The treatment of these complications represents an important part of the general management of the nephritic syndrome. Copyright © 2010 Association Société de néphrologie. Published by Elsevier SAS. All rights reserved.

  7. Respiratory Distress Syndrome and its Complications

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    Eren Kale Cekinmez

    2013-08-01

    Full Text Available Respiratory distress syndrome in premature babies is one of the most common and most important health problems in newborns. Respiratory distress syndrome of newborn is a syndrome in premature infants caused by developmental insufficiency of surfactant production and structural immaturity in the lungs. Respiratory distress syndrome begins shortly after birth and is manifest by tachypnea, tachycardia, chest wall retractions, expiratory grunting, nasal flaring and cyanosis during breathing efforts. Respiratory distress syndrome or complications caused by respiratory distress syndrome are the most important causes of mortality and morbidity in premature infants. This article briefly reviews respiratory distress syndrome and its complications. [Archives Medical Review Journal 2013; 22(4.000: 615-630

  8. Teeth syndrome: diagnosis, complications and management | Sbai ...

    African Journals Online (AJOL)

    Teeth syndrome or fight bite is a specific entity in hand surgery that is little known. It includes infectious complications of the hand following a fist against the teeth. Neglected or misdiagnosed this injury frequently leads to serious complications that could compromise the function of the hand. A retrospective study was ...

  9. Paroxysmal tonic upward gaze complicating Angelman syndrome.

    Science.gov (United States)

    Fukumura, Shinobu; Watanabe, Toshihide; Takayama, Rumiko; Tsutsumi, Hiroyuki

    2015-01-01

    Paroxysmal tonic upward gaze is a childhood oculomotor syndrome characterized by episodes of conjugate upward deviation of the eyes. Its pathogenesis is unknown, and the etiology is heterogeneous. We describe a 2-year-old girl with Angelman syndrome who developed paroxysmal tonic upward gaze at 9 months of age. She presented with developmental delay, blond hair, jerky movements, ataxia, and epilepsy. Genetic testing revealed a maternal deletion of 15q11-13, confirming Angelman syndrome. This is the first report of Angelman syndrome complicated by paroxysmal tonic upward gaze. Both transient paroxysmal tonic upward gaze and Angelman syndrome have been associated with dopaminergic neurons. We speculate that the dopaminergic abnormalities present in Angelman syndrome may cause paroxysmal tonic upward gaze. Copyright © 2015 Elsevier Inc. All rights reserved.

  10. Metabolic complications in the small intestine syndrome

    International Nuclear Information System (INIS)

    Mora, Rafael; Orozco, Reynaldo

    2000-01-01

    Metabolic complications in the syndrome of small intestine is presented in a patient of masculine sex, 27 years old, who consulted for a square of inflammation gingival, migraine, fever, anorexia and adinamia for three days, followed by maculopapular-eritematose eruption for 8 days, coincident with the ampicillin ingestion, and later on severe abdominal pain and diarrhea

  11. Cauda Equina Syndrome Secondary to Complicated Diverticulitis

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    M. ter Horst

    2013-10-01

    Full Text Available A 58-year-old woman presented to the emergency department with cauda equina syndrome and sepsis. The symptoms were attributed to a complicated episode of sigmoid diverticulitis. MRI showed that the diverticulitis had caused an intra-abdominal fistula to a presacrally localized abscess expanding into the spinal canal, compressing the cauda equina nerves. Although Hartmann's procedure was performed, the neurological symptoms persisted, causing the patient to remain partially paraplegic. This case report illustrates that cauda equina syndrome is a condition that can also be caused by intra-abdominal pathology such as diverticulitis.

  12. Idiopathic pulmonary hemosiderosis complicated by Down syndrome.

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    Watanabe, Hirofumi; Ayusawa, Mamoru; Kato, Masataka; Chou, Ami; Komori, Akiko; Abe, Yuriko; Matsumura, Masaharu; Kamiyama, Hiroshi; Izumi, Hiroyuki; Takahashi, Shori

    2015-10-01

    We report the case of a 9-year-old girl with Down syndrome (DS) diagnosed with idiopathic pulmonary hemosiderosis (IPH). Although acute pneumonia complicated by hemolytic anemia was suspected, IPH was finally diagnosed on bronchoscopy. Treatment with prednisolone achieved good clinical response. An association between IPH and DS was not able to be identified, but immunological issues in DS may contribute to the onset of IPH. Recurrent and intractable respiratory symptoms with marked infiltrative shadows in the bilateral lungs and complicated by severe anemia in patients with DS should suggest IPH. © 2015 Japan Pediatric Society.

  13. [Thromboembolic complications of childhood nephrotic syndrome].

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    Gargah, Tahar; Abidi, Kamal; Nourchene, Khelil; Zarrouk, Chokri; Lakhoua, Mohamed R

    2012-02-01

    Vascular thrombosis in the childhood nephrotic syndrome is a rare event. It poses major diagnostic and therapeutic problems. The prognosis depends on early diagnosis and on precocious anticoagulation. The risk of extension of thrombosis and pulmonary embolism is real. To consider the major thrombotic events associated with childhood nephrotic syndrome and to establish an appropriate preventive approach based on objective clinical and laboratory parameters. This is a retrospective study of all cases of patients suffering from idiopathic nephrotic syndrome, during a period of 20 years, starting from January 1990 to December 2009. We selected six patients with vascular thrombosis. The diagnosis was confirmed by appropriate radiological investigation. Six cases of vascular thrombosis were identified among 260 cases of nephrotic syndrome collected during the period study. Patients are divided into five boys and one girl. The mean age was 13.3 years. The localisation of thrombosis is venous in all cases. One patient presented a massively fatal pulmonary embolism. Sinovenous thrombosis has been objectified in 3 patients who were all treated with a good therapeutic response. vascular thrombosis remains one of the most serious complications of nephrotic syndrome in children. Early diagnosis and precocious anticoagulation are essential for preventing the extension of thrombosis.

  14. Complications des syndromes drepanocytaires majeurs a l'hopital ...

    African Journals Online (AJOL)

    Mots clés : Drépanocytose, Complications, Niamey, Niger. ABSTRACT. COMPLICATIONS OF MAJOR SICKLE CELL SYNDROMES AT THE NATIONAL HOSPITAL OF NIAMEY (NIGER). Objective: Study the complications of major sickle cell syndromes in the National Hospital of Niamey (NHN). Method: We conducted a ...

  15. Antiphospholipid Syndrome Complicated by Unilateral Pleural Effusion

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    Yasutaka Mitamura

    2013-07-01

    Full Text Available Antiphospholipid syndrome (APS with pleural effusion is extremely rare. A 75-year-old man was admitted to our hospital for spreading erythema on his trunk and extremities, as well as dyspnea. One year before admission, he had visited us with a 1-year history of erythema and purpura on his legs and occasional fever. Given the diagnosis of APS, we initiated a combination therapy of aspirin and warfarin, but the skin lesions had gradually worsened. A biopsy specimen revealed marked thrombosis in the dermal and subcutaneous small vessels. In addition, chest X-ray and computed tomography demonstrated a large pleural effusion in the left lung. He underwent repeated drainage of the pleural effusion but the effusion recurred. We added oral prednisolone 30 mg daily to his prior anticoagulant therapy. The skin lesions and pleural effusion rapidly improved and disappeared without any complication. Corticosteroids might be a choice of treatment for intractable pleural effusion in APS patients.

  16. Jugular thrombophlebitis complicating bacterial pharyngitis (Lemierre's syndrome)

    International Nuclear Information System (INIS)

    Sena, S.De; Rosenfeld, D.L.; Santos, S.; Keller, I.

    1996-01-01

    Lemierre's syndrome is a rare syndrome caused by Fusobacterium necrophorum, a gram-negative anaerobic organism that normally inhabits the oropharynx. The syndrome follows primary oropharyngeal infection and affects previously healthy adolescents in a characteristic manner with fatal results if left untreated. The authors present two cases seen at their institution and discuss the clinical and radiologic features of the syndrome, along with considerations for patient management. (orig.). With 5 figs

  17. Respiratory Distress Syndrome and its Complications

    OpenAIRE

    Eren Kale Cekinmez; Hacer Yapicioglu Yildizdas; Ferda Ozlu

    2013-01-01

    Respiratory distress syndrome in premature babies is one of the most common and most important health problems in newborns. Respiratory distress syndrome of newborn is a syndrome in premature infants caused by developmental insufficiency of surfactant production and structural immaturity in the lungs. Respiratory distress syndrome begins shortly after birth and is manifest by tachypnea, tachycardia, chest wall retractions, expiratory grunting, nasal flaring and cyanosis during breathing effor...

  18. Grisel's syndrome: A rare complication following traditional uvulectomy

    African Journals Online (AJOL)

    A case is reported of an eight-month-old female patient who had traditional uvulectomy for sore throat complicated by Grisel's syndrome. She was admitted into the hospital one week after uvulectomy with Torticolis. Grisel's syndrome is a nontraumatic atlantoaxial subluxation, usually secondary of an infection or an ...

  19. Fatal Lemierre's syndrome as a complication of chronic otitis media ...

    African Journals Online (AJOL)

    South African Journal of Child Health ... Background. Lemierre's syndrome is septic thrombophlebitis of the internal jugular vein, initiated by an infection of the head and neck region. This septic ... We discuss the case of a 14-year-old male with Lemierre's syndrome as a complication of chronic middle ear infection. Results.

  20. Superior sagittal sinus thrombosis: a rare complication of nephrotic syndrome.

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    Tullu M

    1999-10-01

    Full Text Available A two and half year-old-male child, known case of steroid responsive nephrotic syndrome presented with fever and vomiting of acute onset. He was diagnosed to have superior sagittal sinus thrombosis on a contrast computerised tomographic scan of brain. Recovery was complete without anticoagulant therapy. Superior sagittal sinus thrombosis is an extremely rare complication of nephrotic syndrome.

  1. Thromboembolic complications in childhood nephrotic syndrome: a clinical profile.

    Science.gov (United States)

    Suri, Deepti; Ahluwalia, Jasmina; Saxena, Akshay K; Sodhi, Kushaljit S; Singh, Paramjeet; Mittal, Bhagwant R; Das, Reena; Rawat, Amit; Singh, Surjit

    2014-10-01

    Thromboembolism is a rare life-threatening complication of childhood nephrotic syndrome. We present the clinical profile and outcome of 34 children with 35 events of thromboembolic complications with nephrotic syndrome. Cerebral venous thrombosis (CVT) was the commonest complication seen in 11 (31.4 %) children followed by pulmonary thromboembolism and deep venous thrombosis in 9 (25.7 %) and 6 (16.6 %) children, respectively. Arterial thrombosis resulting in central nervous system infarcts was observed in 7 (20 %) children and 2 children had thrombosis of the peripheral arteries. Episodes were equal in steroid-resistant nephrotic syndrome and steroid-dependent nephrotic syndrome groups. Most of the thromboembolic complications occurred with relapse but 11.4 % of children developed intracranial thrombosis during remission. The most sensitive symptom of CVT was persistent headache while unexplained respiratory distress and hypoxemia pointed towards pulmonary thromboembolism. Hypoalbuminemia was seen in 82.8 % of children, while concurrent infection was seen in 31.4 %. Coexistence of genetic prothrombotic condition was identified and merits evaluation. Early heparin therapy followed by oral anticoagulants resulted in complete recovery in 91.1 % of children. Death occurred in 3 (8.5 %) children and autopsy revealed pulmonary thromboembolism in 2 children. Venous and arterial thrombotic complications can occur in children with nephrotic syndrome. A high index of suspicion is required as the clinical features may be subtle. Neuroimaging and angiographic techniques help in confirming diagnosis. Early aggressive heparin therapy followed by oral anticoagulants is necessary for a favorable outcome.

  2. Higher order structures of Adalimumab, Infliximab and their complexes with TNFα revealed by electron microscopy.

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    Tran, Bich Ngoc; Chan, Siew Leong; Ng, Chloe; Shi, Jian; Correia, Ivan; Radziejewski, Czeslaw; Matsudaira, Paul

    2017-12-01

    Adalimumab and Infliximab are recombinant IgG1 monoclonal antibodies (mAbs) that bind and neutralize human tumor necrosis factor alpha (TNFα). TNFα forms a stable homotrimer with unique surface-exposed sites for Adalimumab, Infliximab, and TNF receptor binding. Here, we report the structures of Adalimumab-TNFα and Infliximab-TNFα complexes modeled from negative stain EM and cryo-EM images. EM images reveal complex structures consisting of 1:1, 1:2, 2:2, and 3:2 complexes of Adalimumab-TNFα and Infliximab-TNFα. The 2:2 complex structures of Adalimumab-TNFα and Infliximab-TNFα show diamond-shaped profiles and the 2D class averages reveal distinct orientations of the Fab domains, indicating different binding modes by Adalimumab and Infliximab to TNFα. After separation by size exclusion chromatography and analysis by negative stain EM, the 3:2 complexes of Adalimumab-TNFα or Infliximab-TNFα complexes are more complicated but retain features recognized in the 2:2 complexes. Preliminary cryo-EM analysis of 3:2 Adalimumab-TNFα complex generated a low-resolution density consistent with a TNFα trimer bound with three Fab domains from three individual antibody molecules, while each antibody molecule binds to two molecules of TNFα trimer. The Fc domains are not visible in the reconstruction. These results show the two mAbs form structurally distinct complexes with TNFα. © 2017 The Protein Society.

  3. Rare Complications of Cervical Spine Surgery: Horner's Syndrome.

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    Traynelis, Vincent C; Malone, Hani R; Smith, Zachary A; Hsu, Wellington K; Kanter, Adam S; Qureshi, Sheeraz A; Cho, Samuel K; Baird, Evan O; Isaacs, Robert E; Rahman, Ra'Kerry K; Polevaya, Galina; Smith, Justin S; Shaffrey, Christopher; Tortolani, P Justin; Stroh, D Alex; Arnold, Paul M; Fehlings, Michael G; Mroz, Thomas E; Riew, K Daniel

    2017-04-01

    A multicenter retrospective case series. Horner's syndrome is a known complication of anterior cervical spinal surgery, but it is rarely encountered in clinical practice. To better understand the incidence, risks, and neurologic outcomes associated with Horner's syndrome, a multicenter study was performed to review a large collective experience with this rare complication. We conducted a retrospective multicenter case series study involving 21 high-volume surgical centers from the AOSpine North America Clinical Research Network. Medical records for 17 625 patients who received subaxial cervical spine surgery from 2005 to 2011 were reviewed to identify occurrence of 21 predefined treatment complications. Descriptive statistics were provided for baseline patient characteristics. Paired t test was used to analyze changes in clinical outcomes at follow-up compared to preoperative status. In total, 8887 patients who underwent anterior cervical spine surgery at the participating institutions were screened. Postoperative Horner's syndrome was identified in 5 (0.06%) patients. All patients experienced the complication following anterior cervical discectomy and fusion. The sympathetic trunk appeared to be more vulnerable when operating on midcervical levels (C5, C6), and most patients experienced at least a partial recovery without further treatment. This collective experience suggests that Horner's syndrome is an exceedingly rare complication following anterior cervical spine surgery. Injury to the sympathetic trunk may be limited by maintaining a midline surgical trajectory when possible, and performing careful dissection and retraction of the longus colli muscle when lateral exposure is necessary, especially at caudal cervical levels.

  4. Maternal and fetal outcome of pregnancy complicated by HELLP syndrome.

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    Gasem, Turki; Al Jama, Fathia E; Burshaid, Sameera; Rahman, Jessica; Al Suleiman, Suleiman A; Rahman, Mohammad S

    2009-12-01

    The study evaluated the maternal and fetal outcome in 64 pregnancies complicated by HELLP syndrome. A retrospective analysis of the medical records was performed of patients with HELLP syndrome managed at this tertiary Obstetric unit between January 1996 and December 2005, who were admitted for preeclamsia/eclampsia and had documented evidence of hemolysis, elevated liver enzymes and low platelet count. Maternal and neonatal complications were recorded and analyzed. The incidence of HELLP syndrome in the study was 8.3%. Mean gestational age at delivery was 32.4 +/- 4.2 weeks and mean birth weight was 1851 +/- 810 g. Forty-two percent of the patients had deliveries <32 weeks and 28% IUGR. Respiratory distress syndrome was the main indication for NICU admissions (33.9%). The PNM rate was 20%. Maternal morbidity rate was 34%. The most common maternal complications were abruptio placentae (36.4%) and DIC (31.8%). There was no maternal death. Once the diagnosis of HELLP syndrome is confirmed, the management depends on several obstetric and maternal variables like gestational age, severity of laboratory abnormalities and fetal status. As soon as the maternal condition is stabilized and fetal assessment is obtained, prompt delivery of the fetus is indicated. It is not yet established whether expectant management in preterm pregnancies with HELLP syndrome would improve perinatal outcome.

  5. Fahr Syndrome Unknown Complication: Overactive Bladder

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    Devrim Tuglu

    2014-01-01

    Full Text Available A 38-year-old male patient was admitted to our outpatient department because of frequency and urgency incontinence. During evaluation it was detected that the patient was suffering from frequency which was progressive for one year, feeling of incontinence, and urgency incontinence. There was no urologic pathology detected in patient’s medical and family history. Neurologic consultation was requested due to his history of boredom, reluctance to do business, balance disorders, and recession for about 3 years. Brain computerized tomography (CT scan revealed that amorphous calcifications were detected in the bilaterally centrum semiovale, basal ganglia, capsula interna, thalami, mesencephalon, pons and bulbus, and the bilateral cerebellar hemispheres. We have detected spontaneous neurogenic detrusor overactivity without sphincter dyssynergia after evaluating the voiding diary, cystometry, and pressure flow study. We consider the detrusor overactivity which occurred one year after the start of the neurological symptoms as the suprapontine inhibition and damage in the axonal pathways in the Fahr syndrome.

  6. Superior Mesenteric Artery Syndrome: An Infrequent Complication of Scoliosis Surgery

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    Metin Keskin

    2014-01-01

    Full Text Available Superior mesenteric artery syndrome is a rare condition that causes a proximal small intestinal obstruction due to contraction of the angle between the superior mesenteric artery and the aorta. Scoliosis surgery is one of the 15 reasons for superior mesenteric artery syndrome, which can present with acute or chronic manifestations. Although conservative treatment is usually possible, surgical treatment is required in certain cases that cannot be treated using conservative methods. In this paper, we describe a patient who developed superior mesenteric artery syndrome after scoliosis surgery and was treated with duodenojejunostomy due to failure and complications of conservative treatment.

  7. Quadrilateral space syndrome: a rare complication of thoracic surgery.

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    Nishimura, Motohiro; Kobayashi, Masao; Hamagashira, Kenichiro; Noumi, Shinpachiro; Ito, Kazuhiro; Kato, Daishiro; Shimada, Junichi

    2008-10-01

    We report a case of quadrilateral space syndrome related to thoracic surgery. A 21-year-old man underwent video-assisted thoracic surgery for a left-sided pneumothorax. After the operation, he presented with difficulties in left arm abduction and paresthesia over the lateral aspect of the shoulder and upper arm. Deltoid muscle atrophy and tenderness over the quadrilateral space were also observed. On further examination, he was diagnosed with isolated paralysis of the axillary nerve, the so-called quadrilateral space syndrome. This is a rare complication, but it interferes with the activities of daily living, and thus one should pay attention to this syndrome.

  8. Spontaneous liver rupture in pregnancy complicating HELLP syndrome: case report

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    Sariyeh Golmahammadlou

    2014-10-01

    Conclusion: Spontaneous liver rupture associated with HELLP syndrome is a rare and life-threatening complication of pregnancy. Unruptured liver hematoma is also a rare condition during pregnancy with a very difficult diagnosis. Using clinical diagnostic tests such as CT scan or MRI would be helpful to improve clinical outcomes.

  9. Eye Complications of Acquired Immune Deficiency Syndrome (AIDS ...

    African Journals Online (AJOL)

    Skoludek_R

    Improved survival as a result of HAART has lead to increase in systemic and ocular complications as well as the appearance of syndromes related to ... activities of daily living. This paper reviews anterior segment and external ocular disorders associated with AIDS in an ... recognised by inspection. Posterior segment and.

  10. HELLP syndrome: a complication or a new autoimmune syndrome?

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    Paola Triggianese

    2014-11-01

    Full Text Available The HELLP (hemolysis, elevated liver enzymes, low platelets syndrome is a pregnancy-specific disease characterized by hemolysis with elevated lactate dehydrogenase, elevated liver enzymes, and decreased platelet count. It is considered a severe variant of the hypertensive disorders that occur during pregnancy together with the pre-eclampsia (PE and the eclampsia giving symptoms in the mother from 20 weeks’ gestation onward. All these conditions are multi-system pregnancy-related diseases associated with an increase in blood pressure and in both the perinatal and the maternal morbidity/mortality. Observational studies suggest that steroid treatment in HELLP syndrome may improve the hematological and biochemical features in the mother and the perinatal outcome. The present review aims to show that the HELLP syndrome may be considered as an autoimmune disorder itself. Biomarkers of the immune system can be a useful tool improving the diagnostic and therapeutic management of women with HELLP by delineating the underlying etiology of this syndrome.

  11. Arterial complications of vascular Ehlers-Danlos syndrome.

    Science.gov (United States)

    Eagleton, Matthew J

    2016-12-01

    Vascular Ehlers-Danlos syndrome (EDS) is a relatively rare genetic syndrome that occurs owing to disorders in the metabolism of fibrillary collagen. These defects affect the soft connective tissues resulting in abnormalities in the skin, joints, hollow organs, and blood vessels. Patients with these defects frequently present at a young age with spontaneous arterial complications involving the medium-sized arteries. Complications involving the hollow organs, such as spontaneous colonic perforation, are observed as well. Given the fragility of the soft tissue, open and endovascular intervention on patients with vascular EDS is fraught with high complication rates. A PubMed search was performed to identify manuscripts published related to vascular EDS. This search included more than 747 articles. These findings were cross-referenced using key terms, including endovascular, embolization, surgery, genetics, pathophysiology, connective tissue disorders, vascular complications, systematic review, type III collagen, and COL3A1. The references in key articles and review articles were evaluated for additional resources not identified in the PubMed search. Care must be taken to balance the risk of intervention vs the risk of continued observation. Life-threatening hemorrhage, however, mandates intervention. With careful, altered approaches to tissue handling, endovascular approaches may provide a safer option for managing the arterial complications observed in patients with vascular EDS. Additional hope may also be found in the use of pharmacologic agents that reduce the incidence and severity of the arterial complications. Copyright © 2016 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.

  12. TOLOSA-HUNT SYNDROME MIMICKING AS ORBITAL COMPLICATION OF SINUSITIS

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    Raveendra . Gadag

    2014-12-01

    Full Text Available The Tolosa – Hunt syndrome is a rare, benign condition characterized by severe unilateral headache with extraocular palsies, orbital pain caused by nonspecific granulomatous inflammation in the cavernous sinus, superior orbital fissure or the orbit. (1-4 The incidence of Tolosa - Hunt syndrome has been estimated as approximately one to two cases per million. The etiology of the syndrome is largely unknown and it can affect people of virtually any age, with no sex predilection. It is usually unilateral, with no predisposition for right or left side; it has been reported as bilateral in 4.1-5 % cases. (2, 4 We report a rare case of Tolosa-Hunt syndrome which was misdiagnosed as sinusitis with orbital complication. The clinical features, diagnostic investigation and the importance of magnetic resonance imaging (MRI studies in the differentiation of the condition are addressed.

  13. Shrinking lung syndrome complicating pediatric systemic lupus erythematosus

    International Nuclear Information System (INIS)

    Burns, Natalie S.; Stevens, Anne M.; Iyer, Ramesh S.

    2014-01-01

    Systemic lupus erythematosis (SLE) can affect the lungs and pleura, usually manifesting with pleural effusions or diffuse parenchymal disease. A rare manifestation of SLE is shrinking lung syndrome, a severe restrictive respiratory disorder. While pleuropulmonary complications of pediatric SLE are common, shrinking lung syndrome is exceedingly rare in children. We present a case of a 13-year-old girl previously diagnosed with lupus, who developed severe dyspnea on exertion and restrictive pulmonary physiology. Her chest radiographs on presentation demonstrated low lung volumes, and CT showed neither pleural nor parenchymal disease. Fluoroscopy demonstrated poor diaphragmatic excursion. While shrinking lung syndrome is described and studied in adults, there is only sparse reference to shrinking lung syndrome in children. (orig.)

  14. Shrinking lung syndrome complicating pediatric systemic lupus erythematosus

    Energy Technology Data Exchange (ETDEWEB)

    Burns, Natalie S. [University of Washington Medical Center, Department of Radiology, Seattle, WA (United States); Stevens, Anne M. [Seattle Children' s Hospital, Division of Rheumatology, Department of Pediatrics, Seattle, WA (United States); Iyer, Ramesh S. [University of Washington School of Medicine, Seattle Children' s Hospital, Department of Radiology, Seattle, WA (United States)

    2014-10-15

    Systemic lupus erythematosis (SLE) can affect the lungs and pleura, usually manifesting with pleural effusions or diffuse parenchymal disease. A rare manifestation of SLE is shrinking lung syndrome, a severe restrictive respiratory disorder. While pleuropulmonary complications of pediatric SLE are common, shrinking lung syndrome is exceedingly rare in children. We present a case of a 13-year-old girl previously diagnosed with lupus, who developed severe dyspnea on exertion and restrictive pulmonary physiology. Her chest radiographs on presentation demonstrated low lung volumes, and CT showed neither pleural nor parenchymal disease. Fluoroscopy demonstrated poor diaphragmatic excursion. While shrinking lung syndrome is described and studied in adults, there is only sparse reference to shrinking lung syndrome in children. (orig.)

  15. Generalized pustular psoriasis complicated by acute respiratory distress syndrome.

    Science.gov (United States)

    Abou-Samra, T; Constantin, J-M; Amarger, S; Mansard, S; Souteyrand, P; Bazin, J-E; D'Incan, M

    2004-02-01

    Psoriasis has a chronic and relatively benign course. However, severe complications are possible. One rare complication is acute interstitial pneumonitis. This entity should be suspected when a patient presents with dyspnoea and high fever. Knowledge of this pathology is crucial, for although it is essential to rule out aetiologies requiring specific management such as microbial infection or drug-related syndromes, diagnosis should not be delayed as its severe clinical course is improved by corticosteroids. We report two patients with an acute respiratory distress syndrome arising during the course of pustular psoriasis. Repeated bacteriological testing in lungs and blood remained negative. In both cases lung involvement was severe, requiring artificial ventilation. Dramatic clinical resolution was obtained by using corticosteroids. Besides infectious causes and drug hypersensitivity to methotrexate or acitretin, acute respiratory distress syndrome, sometimes due to a pulmonary capillary leak syndrome, is a rare cause of pneumonitis in the course of psoriasis, and may be fatal. Its pathogenesis is unknown. However, animal models suggest a role for T-helper (Th) 1 lymphocytes, known to be activated in psoriasis, and a role for tumour necrosis factor-alpha, a major Th1 cytokine, in alveolar damage.

  16. Neurologic complications of cerebral angiography in childhood moyamoya syndrome

    International Nuclear Information System (INIS)

    Robertson, R.L.; Chavali, R.V.; Robson, C.D.; Barnes, P.D.; Burrows, P.E.; Eldredge, E.A.; Scott, R.M.

    1998-01-01

    Purpose. To determine the incidence of neurologic complications of cerebral angiography in children with moyamoya syndrome (MMS) as compared to children without MMS. Materials and methods. One-hundred-ninety consecutive cerebral angiograms obtained in 152 children were evaluated. Sixty of these angiograms were obtained in 40 children with MMS. Patients underwent neurologic evaluation prior to and after the procedure. For this study, a neurologic complication was defined as any new focal neurologic deficit or alteration in mental status occurring during the procedure or within the ensuing 24 hours. Results. There were 2 neurologic complications within 24 hours of angiography, one in the MMS group and one in the non-MMS group. One patient with MMS became mute following angiography. The symptom resolved within 12 hours. One patient without MMS being examined postoperatively for residual arteriovenous malformation developed intracranial hemorrhage requiring reexploration 12 hours after the angiogram. Using a two-tail Fisher's exact test, there was no significant statistical difference in the ischemic (P = 0.3) or hemorrhagic (P = 1.0) complication rates between the group of patients with MMS and the non-MMS groups. Conclusion. The risk of a neurologic complication from cerebral angiography in children with MMS is low and not statistically different from the risk in children with other cerebrovascular disorders. (orig.)

  17. Marfan syndrome and cardiovascular complications: results of a family investigation.

    Science.gov (United States)

    Sarr, Simon Antoine; Djibrilla, Siddikatou; Aw, Fatou; Bodian, Malick; Babaka, Kana; Ngaidé, Aliou Alassane; Dioum, Momar; Ba, Serigne Abdou

    2017-07-19

    Cardiovascular complications in Marfan syndrome (MFS) make all its seriousness. Taking as a basis the Ghent criteria, we conducted a family screening from an index case. The objective was to describe the clinical characteristics of MFS anomalies and to detect cardiovascular complications in our patients. Six subjects were evaluated. Patients had to be in the same uterine siblings of the index case or be a descendant. The objective was to search for MFS based on the diagnostic criteria of Ghent and, subsequently, detecting cardiovascular damage. The average age was 24 years. The examination revealed three cases of sudden death in a context of chest pain. Five subjects had systemic involvement with a score ≥ 7 that allowed to the diagnosis of MFS. Two patients had simultaneously ectopia lentis and myopia. In terms of cardiovascular damage, there were three cases of dilatation of the aortic root, two cases of aortic dissection of Stanford's type A with severe aortic regurgitation in one case and moderate in the other. There were three patients with moderate mitral regurgitation with a case by valve prolapse. The family screening is crucial in Marfan syndrome. It revealed serious cardiovascular complications including sudden death and aortic dissection.

  18. Refeeding Syndrome: An Important Complication Following Obesity Surgery

    Directory of Open Access Journals (Sweden)

    Sonja Chiappetta

    2016-01-01

    Full Text Available Background: Refeeding syndrome (RFS is an important and well-known complication in malnourished patients, but the incidence of RFS after obesity surgery is unknown and the awareness of RFS in obese patients as a postsurgical complication must be raised. We present a case of RFS subsequent to biliopancreatic diversion in a morbidly obese patient. Case Report: A 48-year-old female patient with a BMI of 41.5 kg/m2 was transferred to our hospital due to Wernicke‘s Encephalopathy in a global malabsorptive syndrome after biliopancreatic diversion. Parenteral nutrition, vitamin supplementation and high-dosed intravenous thiamine supplementation were initiated. After 14 days, the patient started to develop acute respiratory failure, and neurological functions were impaired. Blood values showed significant electrolyte disturbances. RFS was diagnosed and managed according to the NICE guidelines. After 14 days, phosphate levels had returned to normal range, and neurological symptoms were improved. Conclusion: Extreme weight loss following obesity surgery has been shown to be associated with undernutrition. These patients are at high risk for evolving RFS, even though they may still be obese. Awareness of RFS as a postsurgical complication, the identification of patients at risk as well as prevention and correct management should be routinely performed at every bariatric center.

  19. [Hemolytic uremic syndrome induced by gemcitabine. A poorly recognized complication?].

    Science.gov (United States)

    Graas, M P; Houbiers, G; Demolin, G; Stultiens, A; Focan, C

    2012-12-01

    This report is concerned with the development of an hemolytic uremic syndrome (HUS) in 6 patients (3 males, 3 females, aged 53 to 73) suffering from an advanced cancer and treated by protracted (>= 4 months) infusions of gemcitabine. Over 4 to 14 months, the patients received 13-34 infusions delivering a cumulative dose oscillating between 9 and 29 g/m2. A progressive alteration of renal function preceeded the acute syndrome. After interruption of gemcitabine and symptomatic treatment, the evolution of haemolytic anemia was generally favourable. This was not the case for renal dysfunction: 2 complete and 1 partial resolution of renal insufficiency were noted, but 1 case required chronic dialysis. Based on the authors experience, the frequency of an HUS complication after protracted gemcitabine treatment could be as high as 2.7 %.

  20. Osteomyelitis: A rare complication of pancreatitis and PPP-syndrome.

    Science.gov (United States)

    Langenhan, Ronny; Reimers, Niklas; Probst, Axel

    2016-03-01

    Pancreatic diseases can be accompanied by periarthritis with bone necrosis and panniculitis (PPP-syndrome). It is postulated that this is caused by systemic activity of pancreatic enzymes leading to microcirculatory disturbances and fat necrosis. The morbidity and mortality of the PPP-syndrome is high. Successful treatment of pancreatitis can lead to resolution of accompanying panniculitis and periarthritis without adverse sequelae, but weeks or months after pancreatitis, asymptomatic necrosis of the bone may become symptomatic by fracturing spontaneously. In this report, we also describe osteomyelitis as a severe septic complication of bone necrosis caused by pancreatitis, in one case as acute tissue necrosis and in another case months after pancreatitis spread haematogenously. Copyright © 2015 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved.

  1. Cellulitis as complication of nephrotic syndrome in a pediatric patient

    Science.gov (United States)

    Siregar, R. S.; Daulay, K. R.; Siregar, B.; Ramayani, O. R.; Eyanoer, P. C.

    2018-03-01

    Nephrotic syndrome is a chronic disease that may act as a risk for other major infection in skin, respiratory and urinary tract, while also increasingthe chance for other diseases, like peritonitis, meningitis, and cellulitis. Cellulitis is often caused by Streptococcus β-hemolytic, Staphylococcus aureus, and Escherichia coli. The clinical features of cellulitis marked with redness rash and well-defined borders, pain pressure and swelling. Hypoalbuminemia which occurs due to proteinuria occurred in this patient acts as a risk factor for cellulitis. It has been reported the case of cellulitis as one of the complications of the nephrotic syndrome in the pediatric patient. The treatment has been given to the patient such as antibiotics and supportive therapy and also planned albumin substitution.

  2. Acute Respiratory Distress Syndrome Complicating Strongyloides stercoralis Hyperinfection

    Directory of Open Access Journals (Sweden)

    Ming-Ju Tsai

    2011-03-01

    Full Text Available Strongyloidiasis is endemic in tropic and subtropic areas, but is currently seldom encountered in developed area like Taiwan. We present an elder man with acute respiratory distress syndrome complicating Strongyloides stercoralis hyperinfection. There was no significant clue initially for diagnosing this patient as having S. stercoralis hyperinfection. Neither peripheral eosinophilia nor significant hemoptysis was noted. Bronchoscopy played a critical role to define the unexpected cause of his progressive pulmonary infiltrates. The correct diagnosis was soon made by recognition of the worm in bronchioloalveolar lavage cytology, and specific treatment was initiated promptly. For a septic patient with progressive pulmonary infiltrates, bronchoscopic studies including cytology may be necessary for defining the cause. Hyperinfection strongyloidiasis should be considered as a cause of acute respiratory distress syndrome in immunocompromised patient, especially with the presence of chronic gastrointestinal symptoms.

  3. [Case of lupus nephritis complicated with hemophagocytic syndrome].

    Science.gov (United States)

    Ueno, Risa; Kado, Hiroshi; Shiotsu, Yayoi; Hara, Masayuki; Otani, Mai; Segawa, Hiroyoshi; Sawada, Katsunori; Hatta, Tsuguru

    2012-01-01

    A 27-year-old woman was referred to our hospital because of pancytopenia and nephritic syndrome in November, 2008. The findings of physical and laboratory examinations showed systemic lupus erythematosus (SLE). Diffuse proliferative lupus nephritis(group IV-G(A))was confirmed by renal biopsy. After combined therapy with prednisolone, intravenous cyclophosphamide pulse and mizoribine, proteinuria decreased from 13.0 g/day to 2.0 g/day and the serum complement level recovered to the normal level. However, she visited our hospital again for management of bleeding tendency in July 2009. She was diagnosed as hemophagocytic syndrome (HPS), with pancytopenia, high ferritin, high LDH level and hemophagocytosis in the bone marrow. She was treated effectively with steroid pulse therapy, but relapsed with HPS after two weeks. Although her child caught a cold, the case did not show any sign or symptom of infection, such as the common cold. However, we diagnosed her HPS as infection-associated hemophagocytic syndrome (IAHS) because she was not in the active phase of SLE at the onset of hemophagocytosis and the laboratory findings showed elevation of her serum ferritin and LDH. Therefore, we considered that her infectious sign may have been concealed by immunosuppressive therapy with prednisolone for SLE. It is very difficult to distinguish between IAHS and autoimmune-associated hemophagocytic syndrome (AAHS)in autoimmune diseases, but the differential diagnosis is necessary to treat the HPS. Here, we report an important case of HPS complicated with SLE. This case may attract interest particularly in the management of HPS-complicated autoimmune disease. Therefore, we report it with a review of the literature.

  4. CROHN'S DISEASE: GENERAL CHARACTERISTICS AND TREATMENT WITH ADALIMUMABE BIOPHARMACEUTICAL

    Directory of Open Access Journals (Sweden)

    S. A. Marques

    2018-02-01

    Full Text Available Crohn's Disease (CD is a chronic inflammatory disease that affects the gastrointestinal system. The etiology is not fully understood, however, genetic, immunological, microbiological and environmental factors are related to its genesis. The most common manifestations of this disease are diarrhea, abdominal pain, ulcers and fistulas. In the absence of adequate treatment it can evolve into extra intestinal complications and is also an important risk factor for colon and rectal cancer. The treatment of the disease is palliative and there is no cure for the disease, being considered only the period of remission of symptoms, as a good prognosis. The biopharmaceutical, Adalimumabe, produced by recombinant DNA technology has demonstrated efficacy in the treatment of this disease, as it prevents the action of TNF-α, a cytokine involved in inflammation and is abundant in individuals with CD. Although Adalimumabe has good results, its use leads to side effects that can be mild or fatal, such as the activation of tuberculosis.

  5. Guillain-Barre syndrome complicating chikungunya virus infection.

    Science.gov (United States)

    Agarwal, Ayush; Vibha, Deepti; Srivastava, Achal Kumar; Shukla, Garima; Prasad, Kameshwar

    2017-06-01

    Chikungunya virus (CHIKV) is a mosquito-borne alphavirus which presents with symptoms of fever, rash, arthralgia, and occasional neurologic disease. While outbreaks have been earlier reported from India and other parts of the world, the recent outbreak in India witnessed more than 1000 cases. Various systemic and rarely neurological complications have been reported with CHIKV. We report two cases of Guillain-Barré syndrome (GBS) with CHIKV. GBS is a rare neurological complication which may occur after subsidence of fever and constitutional symptoms by several neurotropic viruses. We describe two cases of severe GBS which presented with rapidly progressive flaccid quadriparesis progressing to difficulty in swallowing and breathing. Both required mechanical ventilation and improved partly with plasmapharesis. The cases emphasize on (1) description of the rare complication in a setting of outbreak with CHIKV, (2) acute axonal as well as demyelinating neuropathy may occur with CHIKV, (3) accurate identification of this entity during outbreaks with dengue, both of which are vector borne and may present with similar complications.

  6. MRI examination of West syndrome complicated by periventricular leukomalacia (PVL)

    Energy Technology Data Exchange (ETDEWEB)

    Fukuda, Kuniaki; Yamashita, Akiko; Endo, Shoichi [National Kagawa Children' s Hospital, Zentuji (Japan); Gouda, Tomoko

    2001-10-01

    The magnetic resonance imaging (MRI) findings of 19 low-birth-weight infants with periventricular leukomalacia (PVL) and cerebral palsy, 6 with West syndrome as a complication (group III) and 13 without it (group II), were compared with those of normal 13 low-birth-weight infants without PVL or cerebral palsy (group I) to investigate the pathogenetic mechanism of West syndrome. All of the subjects were born between 1989 and 1997, and there were no significant differences between the three groups in birth weight or age at the time of the examination. Although the gestation period was significantly longer in the group III (infants with West syndrome) than in group I (control infants), there was no significant difference was seen between group I and group II (infants with PVL and cerebral palsy). PVL was diagnosed by MRI, and the diagnostic standards included periventricular high signal intensity on T2-weighted images, decreased cerebral white matter, and deformity or enlargement of the lateral ventricles. The area of periventricular high signal intensity was significantly larger in group II and group III than in group I, and the occipital lobe white matter was significantly thinner in the former than the latter. The lateral ventricles were significantly larger in group II than in the other two groups. The area of the corpus callosum and the thickness of the splenium and trunk of the corpus callosum were significantly smaller in group II and group III than in group I, but the corpus callosum was significantly shorter in group III than in the other two groups. Findings in the brain stem showed a significantly smaller pons and medulla oblongata in group III than in group I, but there were no significant differences between group II and group I. The results of this study indicate that lower brainstem lesions as well as cerebral lesions are associated with the development of West syndrome. (K.H.)

  7. Epidemiological and sociodemographic factors associated with complicated alcohol withdrawal syndrome.

    Science.gov (United States)

    Monte-Secades, R; Blanco-Soto, M; Díaz-Peromingo, J A; Sanvisens-Bergé, A; Martín-González, M C; Barbosa, A; Rosón-Hernández, B; Tejero-Delgado, M A; Puerta-Louro, R; Rabuñal-Rey, R

    2017-10-01

    To analyse the influence of epidemiological and sociodemographic factors in complicated alcohol withdrawal syndrome (AWS). A multicentre, observational prospective study was conducted on consecutively added patients with AWS hospitalised in internal medicine departments. We recorded sociodemographic, epidemiological, clinical and progression data. Complicated AWS was defined as that which progressed with seizures or delirium tremens. We studied 228 episodes of AWS in 219 patients. The mean age was 54.5 years (SD, 11.5), and 90.8% were men. AWS was the cause for hospitalisation in 39.9% of the patients. Some 27.1% of the cases presented seizures, and 32.4% presented delirium tremens. The daily quantity of alcohol ingested was 17.8 standard drink units (SD, 21.4), with 16.6 years of dependence (SD, 11.3). The pattern of alcohol abuse was regular in 82.8% of the patients. Some 38.4% of the patients were married or had a partner, and 45.6% had children. Some 72.7% of the patients were unemployed or retired. Some 68.5% had only completed primary studies. Some 4.8% consumed cannabis, 5.2% consumed cocaine and 3% consumed opioids. The independent variables related to complicated AWS were consumption of a drug other than alcohol (OR, 5.3; 95% CI 1.5-18.7), low education level (OR, 3.4; 95% CI 1.6-7.3) and hospitalisation for AWS (OR, 2.9; 95% CI 1.5-5.6). The model's receiver operating characteristic area was 0.718 (95% CI 0.643-0.793). Concomitant drug abuse and a low educational level could help identify patients at risk of complicated AWS. Copyright © 2017 Elsevier España, S.L.U. and Sociedad Española de Medicina Interna (SEMI). All rights reserved.

  8. Gastrointestinal variant of Lemierre's syndrome complicating ruptured appendicitis

    Directory of Open Access Journals (Sweden)

    Fadi Al Akhrass

    2015-01-01

    Full Text Available Fusobacterium necrophorum is a non-spore-forming, obligate anaerobic, filamentous, gramnegative bacillus that frequently colonizes the human oral cavity, respiratory tract, and gastrointestinal tract. Fusobacterium species have rarely been implicated in cases of gastrointestinal variant of Lemierre's syndrome. We describe a case of F. necrophorum bacteremia associated with suppurative porto-mesenteric vein thrombosis (PVT following acute ruptured appendicitis. In addition, we list the documented twelve cases of Fusobacterium pylephlebitis. Recanalization of the porto-mesenteric veins and relief of the extrahepatic portal hypertension were achieved with early empiric antibiotic and local thrombolytic therapy. Our patient's case underscores the importance of recognizing Fusobacterium bacteremia as a possible cause of suppurative PVT after disruption of the gastrointestinal mucosa following an acute intraabdominal infectious process. Early treatment of this condition using anticoagulation and endovascular thrombolysis as adjunctive therapies may prevent PVT complications.

  9. A new mouse model of metabolic syndrome and associated complications

    Science.gov (United States)

    Wang, Yun; Zheng, Yue; Nishina, Patsy M; Naggert, Jürgen K.

    2010-01-01

    Metabolic Syndrome (MS) encompasses a clustering of risk factors for cardiovascular disease, including obesity, insulin resistance, and dyslipidemia. We characterized a new mouse model carrying a dominant mutation, C57BL/6J-Nmf15/+ (B6-Nmf15/+), which develops additional complications of MS such as adipose tissue inflammation and cardiomyopathy. A backcross was used to genetically map the Nmf15 locus. Mice were examined in the CLAMS™ animal monitoring system, and dual energy X-ray absorptiometry and blood chemistry analyses were performed. Hypothalamic LepR, SOCS1 and STAT3 phosphorylation were examined. Cardiac function was assessed by Echo- and Electro Cardiography. Adipose tissue inflammation was characterized by in situ hybridization and measurement of Jun kinase activity. The Nmf15 locus mapped to distal mouse chromosome 5 with a LOD score of 13.8. Nmf15 mice developed obesity by 12 weeks of age. Plasma leptin levels were significantly elevated in pre-obese Nmf15 mice at 8 weeks of age and an attenuated STAT3 phosphorylation in the hypothalamus suggests a primary leptin resistance. Adipose tissue from Nmf15 mice showed a remarkable degree of inflammation and macrophage infiltration as indicated by expression of the F4/80 marker and increased phosphorylation of JNK1/2. Lipidosis was observed in tubular epithelial cells and glomeruli of the kidney. Nmf15 mice demonstrate both histological and pathophysiological evidence of cardiomyopathy. The Nmf15 mouse model provides a new entry point into pathways mediating leptin resistance and obesity. It is one of few models that combine many aspects of metabolic syndrome and can be useful for testing new therapeutic approaches for combating obesity complications, particularly cardiomyopathy. PMID:19398498

  10. Dynamic diagnosis of "fishmouthing" syndrome, an overlooked complication of blepharoplasty.

    Science.gov (United States)

    McCord, Clinton D; Miotto, Gabriele C

    2013-05-01

    Dysfunction and/or dehiscence of the lateral canthus is 1 source of symptomatic eyelid closure disorder after blepharoplasty. Because the resulting concentric blinking movement resembles mouth closure in a fish, the name "fishmouthing" syndrome (FS) was given to this condition. Fishmouthing syndrome appears to be an overlooked complication of blepharoplasty. The authors performed dynamic assessments of patients who had eyelid discomfort after blepharoplasty to establish the clinical signs of FS. Preoperative and postoperative videos of 36 patients who presented for secondary blepharoplasty were analyzed retrospectively. All 36 patients experienced symptoms of dry eyes and eye discomfort after their initial blepharoplasty and desired symptomatic and cosmetic improvement. The dynamic signs and diagnostic criteria for FS were established clinically and through video analysis of patients' blinking movements. The most common clinical characteristics of FS included lash deformity ("cow lash" sign), abnormal medial displacement of the lateral canthus during blinking, deformity (rounding/narrowing) of the lateral scleral triangle, and visible eyelid closure deficiency or gapping. Other characteristics were lower-lid retraction and compensatory hypercontraction of the orbicularis oculi adjacent to the inner canthus. Patients with FS present with a combination of clinical symptoms and signs and are best diagnosed through dynamic visualization of the animated tissue during blinking. Evaluation of preoperative videos is an essential tool for surgical planning and for analyzing the results, both before and after corrective surgery, in patients with potential FS. 4.

  11. Complications of bariatric surgery: dumping syndrome, reflux and vitamin deficiencies.

    Science.gov (United States)

    Tack, Jan; Deloose, Eveline

    2014-08-01

    Bariatric surgical procedure are increasingly and successfully applied in the treatment of morbid obesity. Nevertheless, these procedures are not devoid of potential long-term complications. Dumping syndrome may occur after procedures involving at least partial gastric resection or bypass, including Roux-en-Y gastric bypass (RYGB) and sleeve gastrectomy. Diagnosis is based on clinical alertness and glucose tolerance testing. Treatment may involve dietary measures, acarbose and somatostatin analogues, or surgical reintervention for refractory cases. Gastro-esophageal reflux disease (GERD) can be aggravated by vertical banded gastroplasty and sleeve gastrectomy procedures, but pre-existing GERD may improve after RYGB and with adjustable gastric banding. Nutrient deficiencies constitute the most important long-term complications of bariatric interventions, as they may lead to haematological, metabolic and especially neurological disorders which are not always reversible. Malabsorptive procedures, poor postoperative nutrient intake, recurrent vomiting and poor compliance with vitamin supplement intake and regular follow-up are important risk factors. Preoperative nutritional assessment and rigourous postoperative follow-up plan with administration of multi-vitamin supplements and assessment of serum levels is recommended in all patients. Copyright © 2014. Published by Elsevier Ltd.

  12. Neonatal characteristics and perinatal complications in neonates with Down syndrome.

    Science.gov (United States)

    Ergaz-Shaltiel, Zivanit; Engel, Offra; Erlichman, Ira; Naveh, Yaron; Schimmel, Michael S; Tenenbaum, Ariel

    2017-05-01

    The annual rate of Down syndrome (DS) births in Jerusalem is stable, regardless of prenatal screening, and diagnostic measures. We aimed to evaluate our historical cohort for obstetrical characteristics and the neonatal course and complications. We reviewed computerized medical files of neonates with the diagnosis of DS born in the four main hospitals in Jerusalem between the years 2000 and 2010 and evaluated for maternal history and primary neonatal hospitalization. A total of 403 neonates were diagnosed with DS. The average maternal age was 35.6 years, 73% were born via spontaneous vaginal delivery. In all gestational ages, the mean birth weight and head circumference percentiles were significantly lower than the general population (P < 0.001 for both) and at each week the HC percentile was lower than the weight percentile (P < 0.0001), worse among males. Mortality during the primary hospitalization was 3.7%. The most common anomalies were cardiac (79%) with either congenital defects or functional abnormalities, neither influenced the length of hospitalization. The main reasons for prolonged hospitalization were prematurity and anomalies of other (non-cardiac) organs. Common perinatal complications included respiratory failure or need for oxygen supplementation (32%), hyperbilirubinemia (23%), sepsis (6.4%), and feeding difficulties (13%). About 84% were fed by human milk; of those, two thirds were exclusively breast-fed and one third were supplemented with infant formula. In conclusion, infants with DS were small for gestational age with relatively reduced head circumference. Despite the increased rate of congenital anomalies and perinatal complications, most infants were discharged home in good medical condition and were exclusively breastfed. © 2017 Wiley Periodicals, Inc.

  13. Surgical treatment of scoliosis in Marfan syndrome: outcomes and complications.

    Science.gov (United States)

    Qiao, Jun; Xu, Leilei; Liu, Zhen; Zhu, Feng; Qian, Bangping; Sun, Xu; Zhu, Zezhang; Qiu, Yong; Jiang, Qing

    2016-10-01

    To investigate surgical outcomes and complications of scoliosis associated with Marfan syndrome. Inclusion criteria were patients who were 10-20 years of age, had a diagnosis of Marfan syndrome by the Ghent nosology, had scoliosis and had undergone spinal fusion, and had at least 2 years of postoperative follow-up. The medical records of all patients were reviewed for age at the time of surgery, surgical procedures performed, instrumentation type, estimated blood loss (EBL) during surgery, operation time and complications related to surgery. Health-related quality-of-life measures (obtained with the SRS-22 Questionnaire before operation and at the last clinical follow-up) were also recorded. Patients were analyzed as two different groups, Group 1 and Group 2, according to the different approaches employed. Patients receiving combined anterior and posterior surgery were assigned to Group 1 and those who received posterior-only surgery to Group 2. Group 1 consisted of 30 patients (14 males, 16 females) with a mean age at surgery of 16.8 years (range: 10-20 years). Complications in Group 1 included two cases of instrumentation loosening with one removed, one case of instrumentation breakage and one case of chylothorax and hemothorax during video assisted thoracoscopic release. 66 patients (28 males, 38 females) with a mean age at surgery of years 16.4 years (range: 10-20 years) were included in Group 2. Complications in Group 2 included six cases of cerebro-spinal fluid leak, one case of deep wound infection secondary to cerebro-spinal fluid leak, one case of leg weakness and one case of pleural rupture cause by misplacement of pedicle screw. There is no difference of age at surgery, preoperative Cobb angles, and SRS-22 total scores (3.0 vs. 3.1) between the two groups (P > 0.05). Group 1 yielded larger correction rate than Group 2 for both thoracic (62.5 % vs. 56.2 %) and lumbar scoliosis (68.3 % vs. 62.7 %). Loss of correction was similar between the two

  14. Compartment syndrome, rhabdomyolysis and risk of acute renal failure as complications of the lithotomy position.

    NARCIS (Netherlands)

    Bocca, G.; Moorselaar, R.J.A. van; Feitz, W.F.J.; Staak, F.H.J.M. van der; Monnens, L.A.H.

    2002-01-01

    Compartment syndrome, rhabdomyolysis and the risk of acute renal failure are potential complications of the lithotomy position. A six-year-old girl is described who developed a compartment syndrome with rhabdomyolysis after prolonged surgery in the lithotomy position. This complication occurred

  15. Influence of adalimumab treatment on anastomotic strength, degree of inflammation, and collagen formation

    DEFF Research Database (Denmark)

    Ploug, Thomas; Andersen, Kristian; Hansen, Katrine

    2013-01-01

    Adalimumab is a TNF-α inhibitor, which has gained wide use in the treatment of inflammatory bowel diseases. The potential detrimental effect of TNF-α inhibitors on postoperative complications such as anastomotic leakage is unknown. The aim of this study was to investigate the effect of a single...

  16. Complications and challenges associated with polycystic ovary syndrome: current perspectives

    Directory of Open Access Journals (Sweden)

    Palomba S

    2015-07-01

    Full Text Available Stefano Palomba,1 Susanna Santagni,1 Angela Falbo,1 Giovanni Battista La Sala1,21Unit of Obstetrics and Gynecology, Arcispedale Santa Maria Nuova-Scientific Institute of Treatment and Care (IRCCS, Reggio Emilia, 2Department of Obstetrics and Gynecology, University of Modena and Reggio Emilia, Modena, ItalyAbstract: Polycystic ovary syndrome (PCOS represents the most common endocrine dysfunction in fertile women and it is considered a heterogeneous and multifaceted disorder, with multiple reproductive and metabolic phenotypes which differently affect the early- and long-term syndrome’s risks. Women with PCOS present an adverse reproductive profile, including a high risk of pregnancy-induced hypertension, preeclampsia, and gestational diabetes mellitus. Patients with PCOS present not only a higher prevalence of classic cardiovascular risk factors, such as hypertension, dyslipidemia, and type-2 diabetes mellitus, but also of nonclassic cardiovascular risk factors, including mood disorders, such as depression and anxiety. Moreover, at the moment, clinical data on cardiovascular morbidity and mortality in women with PCOS are controversial. Finally, women with PCOS show an increased risk of endometrial cancer compared to non-PCOS healthy women, particularly during premenopausal period. Currently, we are unable to clarify if the increased PCOS early- and long-term risks are totally due to PCOS per se or mostly due to obesity, in particular visceral obesity, that characterized the majority of PCOS patients. In any case, the main endocrine and gynecological scientific societies agree to consider women with PCOS at increased risk of obstetric, cardiometabolic, oncology, and psychological complications throughout life, and it is recommended that these women be accurately assessed with periodic follow-up.Keywords: cardiovascular disease, infertility, polycystic ovary syndrome, PCOS, pregnancy

  17. Surgical complications of hemolytic uremic syndrome: Single center experiences

    Directory of Open Access Journals (Sweden)

    Hooman Nakysa

    2007-01-01

    Full Text Available Purpose: To determine the prevalence, outcome and prognostic factors in children with hemolytic uremic syndrome (HUS who underwent laparotomy. Materials and Methods: The medical records of 104 patients with HUS who presented to our center between 1986 and 2003 were reviewed retrospectively. Data were analyzed using Student′s t test for comparing means, Fisher′s exact test for frequencies and Pearson′s correlation for finding the correlations. Results: 78% of cases presented with vomiting and diarrhea. Seven out of 104 needed surgical exploration. The indications of surgery were acute abdomen, severe abdominal distention and the sign of peritonitis. The findings at laparotomy were intussusceptions, perforation (colon, ileum, gangrene of entire colon, rectosigmoidal tearing, duodenal obstruction and toxic megacolon. Pathological findings were transmural infarction in two cases in which staged surgical management was performed (cecostomy, resection, later anastomosis. Four out of seven patients died because of pulmonary failure, coma and multiple organ failure ( P< 0.05 compared to those who did not need laparotomy. The patients requiring surgery were young (< 3 years, had high leukocyte count (>20000 mm 3 and low albumin level (< 3g/dl ( P< 0.05. Conclusion: Surgical complications of HUS are rare but are assorted with high mortality due to respiratory failure and multiple organ failure. Early decision of laparotomy associated with intensive care, including mechanical ventilation, adequate dialysis and ultrafiltration, are recommended.

  18. A complication of xanthogranulomatous cholecystitis with Mirizzi syndrome.

    Science.gov (United States)

    Zhang, H-Y; Cao, X-D; Chen, J-J; Luo, Y-Q; Wang, X-C

    2015-05-01

    A patient had right upper quadrant pain with sclera was transferred from emergency room to the hospital, she was proposed to have acute cholecystitis, gallstones, obstructive jaundice, and a four-year history of gallbladder stones. The NMR results showed that the gallbladder was significantly enlarged and the gallbladder wall was thickening irregularly. The liver morphology was not abnormal except with extensive intrahepatic bile duct dilatation. The MRCP results demonstrated that the intrahepatic bile ducts were significant expanded. The ERCP results showed that duodenal stenosis and extra-hepatic bile duct stenosis. We placed a plastic stent of 8.5Fr and 12 cm in length in the hepatic duct, and after biliary plastic stent placement, jaundice was rapidly reduced and liver function was improved significantly. A surgery was performed and the final pathologic diagnosis is a complication of Xanthogranulomatous cholecystitis with Mirizzi syndrome. After the surgery of cholecystectomy and a bile duct repair were performed, the patient was recovered well. Conclusively, if a patient was diagnosed as biliary stricture, a biliary metal stent should not be placed until pathological diagnosis of malignancy.

  19. Complications and challenges associated with polycystic ovary syndrome: current perspectives

    Science.gov (United States)

    Palomba, Stefano; Santagni, Susanna; Falbo, Angela; La Sala, Giovanni Battista

    2015-01-01

    Polycystic ovary syndrome (PCOS) represents the most common endocrine dysfunction in fertile women and it is considered a heterogeneous and multifaceted disorder, with multiple reproductive and metabolic phenotypes which differently affect the early- and long-term syndrome’s risks. Women with PCOS present an adverse reproductive profile, including a high risk of pregnancy-induced hypertension, preeclampsia, and gestational diabetes mellitus. Patients with PCOS present not only a higher prevalence of classic cardiovascular risk factors, such as hypertension, dyslipidemia, and type-2 diabetes mellitus, but also of nonclassic cardiovascular risk factors, including mood disorders, such as depression and anxiety. Moreover, at the moment, clinical data on cardiovascular morbidity and mortality in women with PCOS are controversial. Finally, women with PCOS show an increased risk of endometrial cancer compared to non-PCOS healthy women, particularly during premenopausal period. Currently, we are unable to clarify if the increased PCOS early- and long-term risks are totally due to PCOS per se or mostly due to obesity, in particular visceral obesity, that characterized the majority of PCOS patients. In any case, the main endocrine and gynecological scientific societies agree to consider women with PCOS at increased risk of obstetric, cardiometabolic, oncology, and psychological complications throughout life, and it is recommended that these women be accurately assessed with periodic follow-up. PMID:26261426

  20. Infliximab vs. adalimumab in Crohn's disease: results from 327 patients in an Australian and New Zealand observational cohort study.

    Science.gov (United States)

    Doecke, J D; Hartnell, F; Bampton, P; Bell, S; Mahy, G; Grover, Z; Lewindon, P; Jones, L V; Sewell, K; Krishnaprasad, K; Prosser, R; Marr, D; Fischer, J; R Thomas, G; Tehan, J V; Ding, N S; Cooke, S E; Moss, K; Sechi, A; De Cruz, P; Grafton, R; Connor, S J; Lawrance, I C; Gearry, R B; Andrews, J M; Radford-Smith, G L

    2017-02-01

    Maintenance anti-tumour necrosis factor-α (anti-TNFα) treatment for Crohn's disease is the standard of care for patients with an inadequate response to corticosteroids and immunomodulators. To compare the efficacy and safety of infliximab and adalimumab in clinical practice and assess the value of concomitant immunomodulator therapy. We performed an observational cohort study in consecutive patients with Crohn's disease qualifying for anti-TNFα treatment in Australia and New Zealand between 2007 and 2011. Demographic and clinical data were prospectively recorded to identify independent factors associated with induction and maintenance of response to infliximab or adalimumab, or to either anti-TNFα therapy. Three hundred and twenty-seven patients (183 infliximab, 144 adalimumab) successfully applied for treatment. Eighty-nine percent responded in all groups and median maintenance of response was similar for the two agents. Concomitant immunomodulator with infliximab, but not adalimumab, demonstrated a significantly longer response overall (P = 0.002), and significantly fewer disease and treatment-related complications (P = 0.017). Corticosteroids at baseline, and/or in the preceding 12 months, were associated with a 9-13 times greater risk of disease flare during maintenance treatment as compared to no corticosteroids (P infliximab and adalimumab to have similar response characteristics. However, infliximab requires concomitant immunomodulator to achieve optimal maintenance of response comparable to adalimumab monotherapy. The results of this study will assist clinicians in further optimising patient care in their day-to-day clinical practice. © 2016 John Wiley & Sons Ltd.

  1. Budd-Chiari syndrome complicating hydatid liver disease

    International Nuclear Information System (INIS)

    Robotti, G.C.; Meister, F.; Schroeder, R.; Bern Univ.; Bern Univ.

    1985-01-01

    In two female patients a diagnosis of Budd-Chiari syndrome secondary to hepatic echinococcosis was established by CT. One patient developed acute Budd-Chiari syndrome secondary to E. granulosus lesions of the liver. The second patient presented with a picture of chronic Budd-Chiari syndrome secondary to alveolar echinococcosis. CT findings of Budd-Chiari syndrome included ascites, low density areas in the liver parenchyma, hypertrophy of the caudate lobe, non visualisation of the hepatic veins, occlusion of the retrohepatic inferior vena cava and enlarged retroperitoneal veins. (orig.) [de

  2. Sinogenic intracranial complications

    DEFF Research Database (Denmark)

    Kofoed, Mikkel Seremet; Fisker, Niels; Christensen, Anne Estmann

    2018-01-01

    We present two 11-year-old girls with chronic recurrent multifocal osteomyelitis, treated with adalimumab. Both developed severe intracranial complications to sinusitis. Patient 1 had been treated with adalimumab for 15 months when she developed acute sinusitis complicated by an orbital abscess...... and subcortical abscesses in combination with sinusitis. She was treated with endoscopic sinus surgery and intravenous antibiotics. Both patients had developed psoriasis and episodes of infection during treatment. They were non-septic and had low fever on presentation. None of the patients suffered any long...

  3. Pregnancy-related deaths and complications in women with vascular Ehlers-Danlos syndrome.

    Science.gov (United States)

    Murray, Mitzi L; Pepin, Melanie; Peterson, Suzanne; Byers, Peter H

    2014-12-01

    The purpose of this study was to characterize the nature and magnitude of pregnancy risks in women with vascular Ehlers-Danlos syndrome. Pregnancy-related death rate was determined by a review of pedigrees of families with vascular Ehlers-Danlos syndrome. Maternal morbidity was characterized through semistructured interviews with women with vascular Ehlers-Danlos syndrome or their next of kin. Pregnancy-related deaths occurred in 30 of 565 deliveries (5.3%). There was no difference in Kaplan-Meier survival curves between parous versus nulliparous women with vascular Ehlers-Danlos syndrome. Interviews with 39 women indicated that 46% of deliveries were uncomplicated. The most common pregnancy-related complications were third-/fourth-degree lacerations (20%) and preterm delivery (19%). Life-threatening complications occurred in 14.5% of deliveries and included arterial dissection/rupture (9.2%), uterine rupture (2.6%), and surgical complications (2.6%). There were 5 maternal deaths in 76 deliveries (6.5%). The risk of pregnancy-related complications is increased in women with vascular Ehlers-Danlos syndrome compared with the general population; however, survival data indicate that pregnancy does not appear to affect overall mortality compared with nulliparous women with vascular Ehlers-Danlos syndrome. The data were insufficient to determine whether mode or timing of delivery influenced risk of complications. Women with vascular Ehlers-Danlos syndrome should be engaged in a shared decision-making process when contemplating pregnancy and pregnancy management.

  4. Oral manifestations associated with systemic complications of prune belly syndrome.

    Science.gov (United States)

    Pessoa, Larissa; Galvão, Virgilio

    2013-01-01

    Prune belly syndrome (PBS) is a rare congenital malformation of unknown etiology characterized by congenital abnormalities including abdominal wall flaccidity, urinary tract alterations, and bilateral cryptorchidism. The incidence of the syndrome is between 1/35000 and 1/50000 live births and there is little information about the oral findings. The present case describes the oral manifestations of a 15-year-old boy diagnosed with PBS. The findings include enamel hypoplasia associated with generalized hypocalcemic dental lines. In the radiographic exam, pronounced demineralization of the trabecular bone of the jaws, loss of lamina dura in all the teeth, and discontinuity of the mandibular cortical bone were observed. Prune belly syndrome is a rare disease, whose clinical dental aspects are not pathognomonic of the syndrome. The comprehension of the systemic mechanism of PBS and its comorbidities enable an understanding of the systemic etiologic factors associated with oral manifestations. Copyright © 2013 Elsevier Inc. All rights reserved.

  5. Criptosporidiose em paciente com espondilite anquilosante usando adalimumabe Cryptosporidiosis in a patient with ankylosing spondylitis treated with adalimumab

    Directory of Open Access Journals (Sweden)

    Fernando Augusto Chiuchetta

    2010-06-01

    Full Text Available A criptosporidiose é uma doença parasitária causada pelo protozoário Cryptosporidium sp. Observou-se um aumento no número de diagnósticos realizados nos últimos vinte anos, principalmente em pacientes que apresentam imunodeficiências como a síndrome da imunodeficiência humana adquirida e as imunodeficiências induzidas como em pacientes transplantados e nos que necessitam realizar hemodiálise frequentemente. Relata-se o caso de um jovem com espondilite anquilosante que, usando adalimumabe, apresentou diarreia devido à criptosporidiose.Cryptosporidiosis is a parasitic disease caused by a protozoan called Cryptosporidium sp. An increased number of diagnoses were made in the last 20 years, especially in patients with immunodeficiency like the acquired human immunodeficiency syndrome and induced immunodeficiency, such as in transplant patients and those who need frequent hemodialysis, has been observed. We report the case of a young patient with ankylosing spondylitis treated with adalimumab who developed chronic diarrhea secondary to cryptosporidiosis

  6. Le syndrome de Budd-Chiari: une complication rare de la ...

    African Journals Online (AJOL)

    L'atteinte hépatique au cours de la sarcoïdose est une localisation fréquente, habituellement asymptomatique. La cholestase anictérique et l'hypertension portale représentent ses principales complications. Le syndrome de Budd-Chiari est une complication peu connue qui demeure exceptionnelle. Nous rapportons un ...

  7. Increased prevalence of microvascular complications in type 2 diabetes patients with the metabolic syndrome.

    Science.gov (United States)

    Abdul-Ghani, Muhammad; Nawaf, Gamal; Nawaf, Fawaz; Itzhak, Baruch; Minuchin, Oscar; Vardi, Pnina

    2006-06-01

    Microvascular complications of diabetes contribute significantly to the disease morbidity. The metabolic syndrome is common among subjects with diabetes and is a very important risk factor for macrovascular complications. However, its contribution to the microvascular complication has not been assessed. To assess the risk of microvascular complications associated with the metabolic syndrome in diabetes subjects. The study group comprised 415 diabetic subjects attending a primary care clinic. The prevalence of microvascular complications was compared between 270 diabetic subjects with metabolic syndrome (NCEP-III criteria) and 145 diabetic patients without. We found that as a group, diabetic subjects with metabolic syndrome had a significantly higher frequency of microvascular-related complications than diabetic subjects without the syndrome (46.6% and 26.8% respectively, P= 0.0005). These include microalbuminuria (41.5% vs. 23.9%, P= 0.013), neuropathy (10.4% vs. 7.5%, P = 0.38), retinopathy (9.6% vs. 4.1%, P = 0.046) and leg ulcers (7.9% vs. 2.8%, P = 0.044). After adjustment for age, gender, glycemic control, disease duration, lipid profile and blood pressure, metabolic syndrome was associated with a significantly higher risk of microvascular complications: odds ratio (95% confidence interval) for nephropathy 2.27 (1.53-3.34), neuropathy 1.77 (0.79-4.0), retinopathy 3.42 (1.2-9.87), and leg ulcers 3.57 (1.08-11.95). In addition to hyperglycemia and disease duration, the metabolic syndrome is a significant risk factor for the development of microvascular complications in diabetic subjects.

  8. [Computed tomographic features of abdominal compartment syndrome complicated by severe acute pancreatitis].

    Science.gov (United States)

    Wu, Jingtao; Zhu, Qingqiang; Zhu, Wenrong; Chen, Wenxin; Wang, Shouan

    2014-11-25

    To explore the computed tomographic (CT) imaging features of abdominal compartment syndrome (ACS) complicated by severe acute pancreatitis (SAP) to improve the diagnosis of disease. Thirty-six cases of ACS and 61 cases of non-ACS (NACS) complicated by SAP were studied retrospectively. And the meaningful CT features were studied. Among them, the ACS vascular complications of abdominal cavity and gastrointestinal bleeding were found significantly more in ACS than in NACS (P treatment.

  9. Acute brain ischemia as a complication of the Ehlers-Danlos syndrome, the case series.

    Science.gov (United States)

    Pajak, Michal; Majos, Marcin A; Szubert, Wojciech; Stefanczyk, Ludomir; Majos, Agata

    2014-10-01

    Vascular type of Ehlers-Danlos syndrome involves many severe complications leading not only to organ-specific symptoms but often ends in a sudden death. The aim of this paper was to present a diagnostic possibilities and its efficiency rate in patients with vascular complications of Ehlers-Danlos syndrome who suffered from artery dissection resulting in acute brain or limb ischemia. We analysed three patients with diagnosed Ehlers-Danlos syndrome who were referred to radiology department for diagnostic imaging of affected vascular beds, each experienced brain ischemia. The paper also aims at offering some general recommendations for patients suffering from possible complications of type IV Ehlers-Danlos syndrome basing on our own experience and available literature data. © The Author(s) 2013 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

  10. Pharmacological management of metabolic syndrome and its lipid complications

    Directory of Open Access Journals (Sweden)

    T Binesh Marvasti

    2010-09-01

    Full Text Available Obesity epidemic has been spread all over the world in the past few decades and has caused a major public health concern due to its increasing global prevalence. Obese individuals are at higher risks of developing dyslipidemic characteristics resulting in increased triglyceride and LDL-cholesterol content and reduced HDL-cholesterol levels. This disorder has profound implications as afflicted individuals have been demonstrated to be at increased risk of development of hypertension, atherosclerosis, type 2 diabetes and cardiovascular diseases. Today, this phenotype is designated as metabolic syndrome. According to the criteria set by the International Diabetes Federation (IDF, for a patient to be diagnosed with metabolic syndrome, the person must have central obesity plus any two of the following conditions: raised TG, reduced HDL-cholesterol, raised blood pressure, and increased fasting plasma glucose. Current National Cholesterol Education Program Adult Treatment Panel III (NCEP-ATP III guidelines for the treatment of patients with the metabolic syndrome encourage therapies that lower LDL cholesterol and TG and raise HDL-cholesterol. Primary intervention often involves treatment with statins to improve the lipid profiles of these patients. However, recent studies suggest the potential of newly identified drugs including thiazolidinediones, GLP-1 agonists, and DPP-4 inhibitors that seem to be promising in reducing the level of progression of metabolic syndrome related disorders. This review discusses the current pharmacological treatments of the metabolic syndrome with the above mentioned drugs.

  11. Case of polyhydramnios complicated by Opitz G/BBB syndrome.

    Science.gov (United States)

    Tajima, Hiroko; Itoh, Hiroaki; Mochizuki, Ayako; Nakamura, Yuki; Kobayashi, Yukiko; Hirai, Kyuya; Suzuki, Kazunao; Sugihara, Kazuhiro; Ohishi, Akira; Ohzeki, Takehiko; Kanayama, Naohiro

    2010-08-01

    Opitz G/BBB syndrome is a congenital disorder characterized by midline defects, such as hypertelorism, cleft lip and/or palate, hypospadias, and by dysphagia often caused by laryngo-tracheo-esophageal abnormalities. We experienced a case of polyhydramnios in a male dichorionic diamniotic (DD) twin, who was diagnosed with Opitz G/BBB syndrome after birth. It is suggested that severe dysphagia was causatively associated with the development of polyhydramnios. In cases of Opitz G/BBB syndrome, boys are usually more heavily affected than girls, who generally manifest only hypertelorism. In the differential diagnosis of polyhydramnios of unidentified cause in male fetuses, it may be helpful to consider maternal facial characteristics, especially hypertelorism.

  12. Grisel’s Syndrome: A Rare Complication following Adenotonsillectomy

    Directory of Open Access Journals (Sweden)

    Abdulkadir Bucak

    2014-01-01

    Full Text Available Grisel’s syndrome is a nontraumatic atlantoaxial subluxation which is usually secondary of an infection or an inflammation at the head and neck region. It can be observed after surgery of head and neck region. Etiopathogenesis has not been clearly described yet, but increased looseness of paraspinal ligament is thought to be responsible. Patients typically present with painful torticollis. Diagnosis of Grisel’s syndrome is largely based on suspicion of the patient who has recently underwent surgery or history of infection in head and neck region. Physical examination and imaging techniques assist in diagnosis. Therefore, clinicians should be aware of acute nontraumatic torticollis after recently applied the head and neck surgery or undergone upper respiratory tract infection. In this paper, a case of an eight-year-old male patient who had Grisel’s syndrome after adenotonsillectomy is discussed with review of the literature.

  13. Down Syndrome Increases the Risk of Short-Term Complications After Total Hip Arthroplasty.

    Science.gov (United States)

    Boylan, Matthew R; Kapadia, Bhaveen H; Issa, Kimona; Perfetti, Dean C; Maheshwari, Aditya V; Mont, Michael A

    2016-02-01

    Down syndrome is the most common chromosomal abnormality and is associated with degenerative hip disease. Because of the recent increase in life expectancy for patients with this syndrome, orthopaedic surgeons are likely to see an increasing number of these patients who are candidates for total hip arthroplasty (THA). Using Nationwide Inpatient Sample (NIS) data from 1998 to 2010, we compared the short-term adverse outcomes of THA among 241 patients with Down syndrome and a matched 723-patient cohort. Specifically, we assessed: (1) incidence of THA; (2) perioperative medical and surgical complications during the primary hospitalization; (3) length of stay; and (4) hospital charges. The annual mean number of patients with Down syndrome undergoing THA was 19. Compared to matched controls, Down syndrome patients had an increased risk of perioperative (OR, 4.33; PDown syndrome patients had significantly higher incidence rates of pneumonia (P=.001), urinary tract infection (PDown syndrome patients were 26% longer (PDown syndrome who is a candidate for THA, orthopaedic surgeons should educate the patient, family and their clinical decision makers about the increased risk of medical complications (pneumonia and urinary tract infections), surgical complications (wound hemorrhage), and lengths of stay compared to the general population. Copyright © 2016 Elsevier Inc. All rights reserved.

  14. Lupus-Negative Libman-Sacks Endocarditis Complicated by Catastrophic Antiphospholipid Syndrome.

    Science.gov (United States)

    Murtaza, Ghulam; Iskandar, Joy; Humphrey, Tara; Adhikari, Sujeen; Kuruvilla, Aneesh

    2017-04-01

    Libman-Sacks endocarditis is characterized by sterile and verrucous lesions that predominantly affect the aortic and mitral valves. In most cases, patients do not have significant valvular dysfunction. However, patients with significant valvular dysfunction may present with serious complications such as cardiac failure, arrhythmias, and thromboembolic events. Recently, association of Libman-Sacks endocarditis with antiphospholipid antibody syndrome (APS) has been made. APS is most commonly defined by venous and arterial thrombosis, recurrent pregnancy loss, and thrombocytopenia. While the syndrome can be a primary syndrome, it is usually secondary to systemic lupus erythematosus. Catastrophic antiphospholipid syndrome (CAPS) can be a life-threatening presentation of APS and can occur in 1% of patients with antiphospholipid syndrome. We present a very rare case of a young female patient with lupus-negative Libman-Sacks endocarditis complicated by CAPS.

  15. Broken Heart Syndrome – An intra operative complication

    Directory of Open Access Journals (Sweden)

    Zara Wani

    2018-03-01

    Full Text Available We report a case of Broken Heart Syndrome in a 56 year old Postmenopausal woman suffered while undergoing simple biopsy procedure for vocal cord polyp that lead to physical, mental and financial burden both for the patient as well as the doctors. A team of cardiologists based on clinical and echocardiographic findings made the diagnosis of this case.

  16. Locus coeruleus syndrome as a complication of tectal surgery

    NARCIS (Netherlands)

    Kronenburg, Annick; Spliet, Wim G.; Broekman, Marieke; Robe, Pierre A.

    2015-01-01

    We describe a case of a 48-year-old woman who underwent a resection of a tectal pilocytic astrocytoma complicated by a sequence of fluctuating consciousness, psychosis with complex hallucinations and lasting sleeping disturbances in which she vividly acts out her dreams. Based on the clinical and

  17. An unusual complication after craniofacial surgery for Apert syndrome

    Directory of Open Access Journals (Sweden)

    Abhay A Lune

    2014-01-01

    A case of Apert syndrome who had undergone craniofacial surgery elsewhere 4 years back presented to us with purulent discharge near the lateral orbital margin of right orbit, watering and redness of the right eye. He had telltale signs of this syndrome in the form of skull deformities such as brachycephaly, frontal bony prominence, mid-face hypoplasia, proptosis and syndactyly of both hands and feet. There was a surgical scar of previous craniofacial surgery over the bi-coronal region. He had a discharging granuloma over the lateral orbital margin and the adjacent lower eyelid had developed cicatricial ectropion. X-ray and computed tomography scan orbit confirmed the clinical suspicion of osteomyelitis of the underlying zygomatic bone at the site of miniplate and screw fixation of the earlier surgery. He was treated with excision of granuloma and extraction of loose screw and infected miniplate while ectropion was corrected by rotation advancement of temporal skin flap.

  18. Pneumomediastinum Complicating Diabetic Ketoacidosis and Boerhaave’s Syndrome

    Directory of Open Access Journals (Sweden)

    Samer Alkhuja

    2013-01-01

    Full Text Available An 18-year-old man presented with altered mental status. He was found to have diabetic ketoacidosis. Chest X-ray showed pneumomediastinum. After intubation for air-way protection, an oral-gastric tube was placed. A chest computed tomography scan showed the tip of the oral-gastric tube to be in the right hemithorax. The patient underwent a thoracotomy and was managed in the intensive care unit. Both diabetic ketoacidosis and Boerhaave's syndrome should be considered as possible causes of pneumomediastinum in a patient with similar presentation. Boerhaave's syndrome should be ruled out prior to the insertion of an oral-gastric tube to avoid further morbidities.

  19. [Sudden death and cardiovascular complications in Marfan syndrome: impact of surgical intervention].

    Science.gov (United States)

    Ohtsubo, Satoshi; Itoh, Tsuyoshi

    2005-07-01

    Marfan syndrome is an autosomal dominant disorder of connective tissue characterized by abnormalities involving the skeletal, ocular, and cardiovascular systems. The cardiovascular complications of the syndrome lead to a reduced life expectancy for affected individuals if left untreated. Major cause of death include acute aortic dissection, aortic rupture, and sudden death, which resulted from congenital vascular fragility. Such life-threatening complications in Marfan syndrome can be managed effectively, by routine aortic imaging, beta-adrenergic blockade, and prophylactic replacement of the aortic root before the diameter exceeds 5.0 to 5.5 mm. Valve preserving aortic root reconstruction yielded improved postoperative quality of life compared with Bentall operation, by reducing late complications related to anticoagulants. It should be carried out before onset of aortic regurgitation for long-term native valve durability.

  20. Nephrotic syndrome complicated with portal, splenic, and superior mesenteric vein thrombosis

    Directory of Open Access Journals (Sweden)

    Bong Soo Park

    2014-09-01

    Full Text Available Thromboembolism is a major complication of nephrotic syndrome. Renal vein thrombosis and deep vein thrombosis are relatively common, especially in membranous nephropathy. However, the incidence of portal vein and superior mesenteric vein (SMV thrombosis in patients with nephrotic syndrome is very rare. To date, several cases of portal vein thrombosis treated by anticoagulation therapy, not by thrombolytic therapy, have been reported as a complication of nephrotic syndrome. Here, we report a case of portal, splenic, and SMV thrombosis in a patient with a relapsed steroid dependent minimal change disease who was treated successfully with anticoagulation and thrombolytic therapy using urokinase. Radiologic findings and his clinical conditions gradually improved. Six months later, a complete remission of the nephrotic syndrome was observed and the follow-up computed tomography scan showed the disappearance of all portal vein, splenic vein, and SMV thrombi.

  1. [Catastrophic antiphospholipid syndrome complicated by cardiogenic shock - a case report].

    Science.gov (United States)

    Zieba, Bozena; Wegrzyn, Agnieszka; Mital, Andrzej; Szczepińska-Nowak, Maria; Lewicki, Lukasz; Chmielecki, Michał; Puchalski, Wiesław; Rynkiewicz, Andrzej

    2009-07-01

    A case of a 29-year-old woman 18 days after delivery with catastrophic antiphospholipid syndrome secondary (CAPS) due to undiagnosed systemic lupus erythematosus, leading to cardiogenic shock is reported. Laboratory evaluation revealed increased anticardiolipin antibodies, lupus anticoagulant, antinuclear antibody and thrombocytopenia. Left ventricular ejection fraction was 20%, neurologic deficit and acute renal failure were also present. Cardiac involvement is common in CAPS, but cardiomyopathy due to microvascular thrombosis is rare. CAPS should be considered as a cause of acute heart failure in a women with systemic lupus erythematosus. In the presented case early therapy with anticoagulants, steroids, immunoglobulins and plasmaferesis was beneficial.

  2. Superior sagittal sinus thrombosis: a rare complication in a child with nephrotic syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Pirogovsky, A.; Adi, M.; Barzilai, N. [Dept. of Radiology, Kaplan Medical Center, Rehovot (Israel); Dagan, A.; Sinai, L.; Sthoeger, D. [Div. of Paediatrics, Kaplan Medical Center, Rehovot (Israel); Tabachnik, E. [Div. of Paediatrics, Kaplan Medical Center, Rehovot (Israel); Paediatric ICU, Kaplan Hospital, Rehovot (Israel)

    2001-10-01

    A 2-year-old boy with new-onset nephrotic syndrome developed recurrent vomiting, apathy and papilloedema. Superior sagittal sinus thrombosis was diagnosed on cranial CT and MRI. He gradually recovered after treatment with heparin, fresh frozen plasma and warfarin with complete resolution of the thrombosis after 1 month. Superior sagittal sinus thrombosis is an extremely rare complication of nephrotic syndrome in children. Early diagnosis is essential for institution of anticoagulation therapy and a successful outcome. (orig.)

  3. Superior sagittal sinus thrombosis: a rare complication in a child with nephrotic syndrome

    International Nuclear Information System (INIS)

    Pirogovsky, A.; Adi, M.; Barzilai, N.; Dagan, A.; Sinai, L.; Sthoeger, D.; Tabachnik, E.

    2001-01-01

    A 2-year-old boy with new-onset nephrotic syndrome developed recurrent vomiting, apathy and papilloedema. Superior sagittal sinus thrombosis was diagnosed on cranial CT and MRI. He gradually recovered after treatment with heparin, fresh frozen plasma and warfarin with complete resolution of the thrombosis after 1 month. Superior sagittal sinus thrombosis is an extremely rare complication of nephrotic syndrome in children. Early diagnosis is essential for institution of anticoagulation therapy and a successful outcome. (orig.)

  4. Hypothalamic-Pituitary-Adrenal Suppression and Iatrogenic Cushing's Syndrome as a Complication of Epidural Steroid Injections

    OpenAIRE

    Leary, Joyce; Swislocki, Arthur

    2013-01-01

    Epidural steroid injections are well accepted as a treatment for radicular back pain in appropriate candidates. While overall incidence of systemic side effects has not been well established, at least five biochemically proven cases of iatrogenic Cushing's Syndrome have been reported as complications of epidural steroid treatment. We present an additional case of iatrogenic Cushing's Syndrome and adrenal suppression in a middle-aged woman who received three epidural steroid injections over a...

  5. [Acute pseudo-obstruction of the colon (Ogilvie syndrome) after cesarean section--a rare complication].

    Science.gov (United States)

    Kolben, M; Loos, W

    1993-08-01

    Despite the fact, that acute pseudo-obstruction of the colon (Ogilvie's syndrome) is a rare entity, many of the reported cases have occurred after gynaecologic and obstetric surgical procedures. In order to avoid life-threatening complications, such as caecal perforation, early detection and initiation of conservative treatment is necessary. We report on three cases with Ogilvie's syndrome after caesarean section. Symptoms and treatment are discussed.

  6. [Vitamin D deficiency rickets complicating Dorfman-Chanarin syndrome].

    Science.gov (United States)

    Barraud, C; Cano, A; Boulay, C; Milh, M; Bollini, G; Chabrol, B

    2015-04-01

    Vitamin D deficiency rickets remains a public health issue in many parts of the world. In France, this diagnosis has almost disappeared since 1992 with routine vitamin D supplementation for children. Therefore, it is more difficult for doctors to identify risk factors and early signs of this disease. In this article, we report a rickets diagnosis acquired by vitamin D deficiency in a child who presented with the onset of a genu valgum and difficulty walking at the age of 9½ years. This patient was a Comorian child followed up from his birth for Dorfman-Chanarin syndrome. Dorfman-Chanarin syndrome is a rare disease, with about 80 cases reported in the literature. It belongs to the group of neutral lipid storage diseases (NLSD) characterized especially on the skin by ichthyosis. This child presented risk factors for vitamin D deficiency (dark skin color, prolonged and exclusive breastfeeding, premature end of supplementation, and particularly severe ichthyosis) that should have alerted us to the risk of vitamin D deficiency and the need for supplementation. This case highlights the importance of vitamin D, especially if there are risk factors such as ichthyosis, and the need to remain watchful in monitoring all chronic diseases. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

  7. The economics of adalimumab for ulcerative colitis.

    Science.gov (United States)

    Xie, Feng

    2015-06-01

    Ulcerative colitis is a chronic inflammatory disease, characterized by diffuse mucosal inflammation in the colon. Adalimumab, as a TNF-α blocker, offers a safe and efficacious treatment option for patients with moderate to severe ulcerative colitis and refractory or intolerant to conventional medications; however, its cost-effectiveness profile has not yet been well established. Future economic evaluations should choose appropriate comparators in the context of target-reimbursement decision making and focus on cost-effectiveness over a long time horizon.

  8. Hereditary multiple exostoses: from genetics to clinical syndrome and complications

    Energy Technology Data Exchange (ETDEWEB)

    Vanhoenacker, Filip M.; Hul, Wim van; Wuyts, Wim; Willems, P.J.; Schepper, Arthur M. de

    2001-12-01

    Objective: To give an overview of genetic, clinical and radiological aspects in two families over four generations with known hereditary multiple exostoses (HME). Methods and material: After linkage analysis in both families to localize the defective gene, mutation analysis was performed in these genes to identify the underlying mutation. In the 31 affected individuals, location, number and morphology and evolution of exostosis, evolution of remodeling defects at the metaphysis, and the extent of possible complications were evaluated on clinical and imaging (plain radiography, computed tomography (CT), and magnetic resonance imaging (MRI)) data over a lifetime period. Results and conclusions: Both families demonstrate the gene defect in the same EXT-2 gene locus on chromosome 11p. Exostoses are preferentially located in the lower extremity (hip, knee and lower leg), humerus, and forearm. Any other bone may be involved, except for the calvaria of the skull and the mandible. Exostoses are rather sessile than pedunculated. Exostosis is rarely present at birth but develops gradually and may persist to grow slowly after closure of the growth plates. Preferential expression of the remodeling defect was seen in the hip, distal femur (trumpet-shaped metaphysis) and forearm (shortening of the ulna with secondary bowing of the radius and development of a pseudo-Madelung deformity). These radiological manifestations start at the age of 4-5 years and become more obvious as the enchondral bone formation progresses with age. Reported complications in these families consist of local entrapment phenomenons (vessel, tendon, nerve), frictional bursitis, and sarcomatous transformation. MRI was able to suggest these complications and is the imaging technique of choice in the evaluation of symptomatic exostoses.

  9. Hereditary multiple exostoses: from genetics to clinical syndrome and complications

    International Nuclear Information System (INIS)

    Vanhoenacker, Filip M.; Hul, Wim van; Wuyts, Wim; Willems, P.J.; Schepper, Arthur M. de

    2001-01-01

    Objective: To give an overview of genetic, clinical and radiological aspects in two families over four generations with known hereditary multiple exostoses (HME). Methods and material: After linkage analysis in both families to localize the defective gene, mutation analysis was performed in these genes to identify the underlying mutation. In the 31 affected individuals, location, number and morphology and evolution of exostosis, evolution of remodeling defects at the metaphysis, and the extent of possible complications were evaluated on clinical and imaging (plain radiography, computed tomography (CT), and magnetic resonance imaging (MRI)) data over a lifetime period. Results and conclusions: Both families demonstrate the gene defect in the same EXT-2 gene locus on chromosome 11p. Exostoses are preferentially located in the lower extremity (hip, knee and lower leg), humerus, and forearm. Any other bone may be involved, except for the calvaria of the skull and the mandible. Exostoses are rather sessile than pedunculated. Exostosis is rarely present at birth but develops gradually and may persist to grow slowly after closure of the growth plates. Preferential expression of the remodeling defect was seen in the hip, distal femur (trumpet-shaped metaphysis) and forearm (shortening of the ulna with secondary bowing of the radius and development of a pseudo-Madelung deformity). These radiological manifestations start at the age of 4-5 years and become more obvious as the enchondral bone formation progresses with age. Reported complications in these families consist of local entrapment phenomenons (vessel, tendon, nerve), frictional bursitis, and sarcomatous transformation. MRI was able to suggest these complications and is the imaging technique of choice in the evaluation of symptomatic exostoses

  10. Premature Birth with Complicated Perinatal Course Delaying Diagnosis of Prader-Willi Syndrome

    Directory of Open Access Journals (Sweden)

    G. Ciana

    2011-01-01

    Full Text Available Prader-Willi syndrome in the newborn is essentially characterized by marked hypotonia, feeding difficulties, hypogonadism, and possible characteristic facial features. However, diagnosis at this age may be particularly difficult, and dysmorphic features may be subtle or absent. Prematurity can furthermore delay clinical features recognition and typical complications due to preterm birth may contribute to divert the diagnosis. We describe a preterm baby with a complicated perinatal course later diagnosed as PWS.

  11. Cerebellar Mutism Syndrome and Other Complications After Surgery in the Posterior Fossa in Adults

    DEFF Research Database (Denmark)

    Wibroe, Morten; Rochat, Per; Juhler, Marianne

    2018-01-01

    BACKGROUND: Cerebellar mutism syndrome (CMS) is rarely described in adults; however, data on self-assessed linguistic complications after posterior fossa surgery do not exist. METHODS: Through a prospective single-center study, data on 59 tumor operations in the posterior fossa were collected pre...... of the few known risk factors for CMS in children. Thus, the cerebellar midline seems to be a vulnerable region for speech and language complications also in adults....

  12. Premature birth with complicated perinatal course delaying diagnosis of prader-willi syndrome.

    Science.gov (United States)

    Ciana, G; Fertz, M C; Pecile, V; Demarini, S

    2011-01-01

    Prader-Willi syndrome in the newborn is essentially characterized by marked hypotonia, feeding difficulties, hypogonadism, and possible characteristic facial features. However, diagnosis at this age may be particularly difficult, and dysmorphic features may be subtle or absent. Prematurity can furthermore delay clinical features recognition and typical complications due to preterm birth may contribute to divert the diagnosis. We describe a preterm baby with a complicated perinatal course later diagnosed as PWS.

  13. Posterior reversible encephalopathy syndrome complicating diabetic ketoacidosis; an important treatable complication.

    Science.gov (United States)

    Jones, Rachel; Redler, Kasey; Witherick, Jonathan; Fuller, Geraint; Mahajan, Tripti; Wakerley, Benjamin R

    2017-03-01

    Development of acute neurological symptoms secondary to cerebral oedema is well described in diabetic ketoacidosis (DKA) and often has a poor prognosis. We present the clinical and radiological data of a 17-yr-old girl who developed cortical blindness, progressive encephalopathy, and seizures caused by posterior reversible encephalopathy syndrome (PRES) that developed after her DKA had resolved. Vasogenic oedema in PRES resolves if the underlying trigger is identified and eliminated. In this case, hypertension was identified as the likely precipitating factor and following treatment her vision and neurological symptoms rapidly improved. We suggest how recent DKA may have contributed to the development of PRES in this patient. © 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  14. Posterior reversible encephalopathy syndrome: An atypical postpartum complication

    Directory of Open Access Journals (Sweden)

    Debashish Paul

    2016-01-01

    Full Text Available Posterior reversible encephalopathy syndrome (PRES is presented by headache, altered mental status, blurring of vision, vomiting and seizure in conjunction with radiological finding of posterior cerebral white matter edema. Data suggest that most cases occur in young middle-aged with marked female preponderance, hypertension being the most common cause. In this case, it was diagnosed in a normotensive patient in the postnatal period that underwent cesarean section. The initial symptoms had misled toward a diagnosis of postdural puncture headache. Symptomatic treatment was started immediately in the ICU. This is an interesting case as the patient was a normotensive one without any other contributory factors and there was unanticipated delay in diagnosing the case until the time we could get a magnetic resonance imaging report.

  15. [A rare complication of ERCP: Mallory-Weiss syndrome].

    Science.gov (United States)

    Sciumè, Carmelo; Geraci, Girolamo; Pisello, Franco; Li Volsi, Francesco; Facella, Tiziana; Frazzetta, Michele; Raimondo, Dario; Passariello, Paolo; Modica, Giuseppe

    2005-01-01

    To describe the management and outcome after endoscopic treatment of hematemesis by Mallory-Weiss Syndrome (MWS) occurred after CPRE (suspected choledocolithiasis). Although cough and retching is common during EGD or CPRE, MWS resulting from endoscopy seems to be uncommon (0.0001-0.04%) and always self-limiting. The patient was submitted to CPRE with the suspicion of choledocholithiasis. Eight hours after CPRE the patient presented with hematemesis and hypotension. With emergency EGD, the AA identified a small bleeding mucosal tear (visible vessel with spurting) just proximal to the esophagogastric junction. The patient was safely treated with endoscopic hemoclipping after the failure of sclerotherapy. The usefulness of hemoclipping in MWS is emphasized: although always self-limiting, endoscopic hemostasis is mandatory in high risk patients. The hemoclips are effective and safe in hemostasis in the case of bleeding visible vessel (spurting or oozing), even with or after sclerotherapy. The hemoclips not obstacles the healing.

  16. SMART Syndrome: Case report of a rare complication after cerebral radiation therapy

    International Nuclear Information System (INIS)

    Truntzer, P.; Salze, P.; Monjour, A.; Gaultier, C.; Ahle, G.; Guillerme, F.; Boutenbat, G.; Atlani, D.; Stilhart, B

    2012-01-01

    The authors report a 71-year-old woman case who developed, 7 years after a cerebral radiation therapy for a parieto-occipital glioblastoma, a stroke-like migraine attacks after radiotherapy syndrome (SMART syndrome), a rare complication characterized by reversible neurologic deficits with migraine described after cerebral irradiation. Transient gyri-form reversible enhancement is found on MRI during crises. This case report allows discussing the clinical, iconographic presentation and the clinical outcome of this syndrome at the light of the literature publication. (authors)

  17. PP085. Hypertensive complication in pregnancy - HELLP syndrome - One year study (2012).

    Science.gov (United States)

    Miranda, Mariana; Ambrosio, Bruna; Gomes, Rui; Santos, Isabel; Matos, Teresa; Nazare, Antonia

    2013-04-01

    HELLP syndrome is characterized by hemolysis with a microangiopathic blood smear, elevated liver enzymes, and low platelet count. It develops in 10-20% of women with severe preeclampsia/eclampsia. The syndrome is associated with maternal morbidities - DIC, renal failure, pulmonary edema and hepatic hematoma/rupture. Some need transfusions and others require laparotomies for intraabdominal bleeding. Study the incidence and related risk factors of HELLP syndrome in Pregnancy, as well as the maternal/fetal outcome. A retrospective study of admissions for HELLP syndrome in 2012.The statistical analysis was based on Excel 2007. In 55 admissions for hypertensive complications in pregnancy, 3 women had HELLP syndrome - 2 were black race and 1 was caucasian. The average maternal age was 29. None had relevant medical history. 2 of the women developed HELLP syndrome after severe preeclampsia. The fetal/neonatal outcomes were prematurity in 2 cases, birth weight average was 1798g and none had apgar-index below 7. There was no fetal death. Premature delivery occurred in 2 cases and all were by cesarean. Maternal complications that determined Intensive Care Unit Admission was recorded in one case - laparotomy for internal bleeding and transfusions were needed. No maternal death occured. HELLP syndrome is associated with many morbidities which risk increases with severity of symptoms/lab results. We had no aggresive/fatal outcomes. Copyright © 2013. Published by Elsevier B.V.

  18. Cochlear implantation in Pendred syndrome and non-syndromic enlarged vestibular aqueduct - clinical challenges, surgical results, and complications.

    Science.gov (United States)

    Mey, Kristianna; Bille, Michael; Cayé-Thomasen, Per

    2016-10-01

    To explore specific clinical issues, surgical results, and complications of 80 cochlear implantations (CI) in 55 patients with Pendred syndrome (PS) or non-syndromic enlarged vestibular aqueduct (NSEVA). Previous studies have focused either on unselected case series or on populations with mixed cochlear malformations. PS/NSEVA accounts for up to 10% of congenital SNHL, rendering this a large group of cochlear implant candidates. The abnormal inner ear anatomy of these patients may be associated with a lower surgical success rate and a higher rate of complications. Retrospective review of patients' medical records and CT/MRI. Tertiary referral center. The medical records and CT/MRI images of 55 PS/NSEVA patients receiving 80 cochlear implantations from 1982-2014 were reviewed. Demographic data, surgical results, intra-operative incidents, and post-operative complications were retrieved. Complications occurred in 36% of implantations; 5% hereof major complications. Gushing/oozing from the cochleostoma occurred in 10% of implantations and was related to transient, but not prolonged post-operative vertigo. Intra-operative risks of gushing/oozing and post-operative vertigo are the primary clinical issues in PS/NSEVA patients regarding CI. Nonetheless, the surgical success rate is high and the major complication rate is low; similar to studies of unselected series of CI recipients.

  19. Osteomalacia complicating renal tubular acidosis in association with Sjogren's syndrome.

    Science.gov (United States)

    El Ati, Zohra; Fatma, Lilia Ben; Boulahya, Ghada; Rais, Lamia; Krid, Madiha; Smaoui, Wided; Maiz, Hedi Ben; Beji, Soumaya; Zouaghi, Karim; Moussa, Fatma Ben

    2014-09-01

    Renal involvement in Sjogren's syndrome (SS) is not uncommon and may precede other complaints. Tubulointerstitial nephritis is the most common renal disease in SS and may lead to renal tubular acidosis (RTA), which in turn may cause osteomalacia. Nevertheless, osteomalacia rarely occurs as the first manifestation of a renal tubule disorder due to SS. We herewith describe a 43-year-old woman who was admitted to our hospital for weakness, lumbago and inability to walk. X-ray of the long bones showed extensive demineralization of the bones. Laboratory investigations revealed chronic kidney disease with serum creatinine of 2.3 mg/dL and creatinine clearance of 40 mL/min, hypokalemia (3.2 mmol/L), hypophosphatemia (0.4 mmol/L), hypocalcemia (2.14 mmol/L) and hyperchloremic metabolic acidosis (chlorine: 114 mmol/L; alkaline reserve: 14 mmol/L). The serum alkaline phosphatase levels were elevated. The serum levels of 25-hydroxyvitamin D and 1,25-dihydroxy vitamin D were low and borderline low, respectively, and the parathyroid hormone level was 70 pg/L. Urinalysis showed inappropriate alkaline urine (urinary PH: 7), glycosuria with normal blood glucose, phosphaturia and uricosuria. These values indicated the presence of both distal and proximal RTA. Our patient reported dryness of the mouth and eyes and Schirmer's test showed xerophthalmia. An accessory salivary gland biopsy showed changes corresponding to stage IV of Chisholm and Masson score. Kidney biopsy showed diffuse and severe tubulo-interstitial nephritis with dense lymphoplasmocyte infiltrates. Sicca syndrome and renal interstitial infiltrates indicated SS as the underlying cause of the RTA and osteomalacia. The patient received alkalinization, vitamin D (Sterogyl ®), calcium supplements and steroids in an initial dose of 1 mg/kg/day, tapered to 10 mg daily. The prognosis was favorable and the serum creatinine level was 1.7 mg/dL, calcium was 2.2 mmol/L and serum phosphate was 0.9 mmol/L.

  20. Cauda equina syndrome: A rare complication in intensive care

    Directory of Open Access Journals (Sweden)

    Jagatsinh Yogendrasinh

    2009-01-01

    Full Text Available A 73-year-old married retired woman with a history of myocardial infarction and primary biliary cirrhosis was admitted to intensive care unit with complaints of chest pain. She was suspected to have pulmonary embolism (PE and was treated with low-molecular-weight heparin (LMWH and aspirin. She had computerized tomographic pulmonary angiography on next day, which ruled out any evidence of PE, until she was continued on LMWH. Three days later, she developed progressive right leg weakness and loss of sphincter control and patchy loss of sensation from T10 and below. She was seen by neurologist and had an MRI scan, which showed extensive subdural clot compressing the conus and lower half of the thoracic cord. She underwent T9-L1, L3, L5-S1 laminectomies, and evacuation and decompression of the clot. She showed very slight recovery following the surgery and left with residual paraparesis. This case is reported to raise awareness among intensivists to be cautious in establishing the diagnosis before prescribing the LMWH and be vigilant to diagnose cauda equina syndrome and treat promptly to avoid residual neurological problems.

  1. Adalimumab-induced acute interstitial lung disease in a patient with rheumatoid arthritis

    Directory of Open Access Journals (Sweden)

    Olivia Meira Dias

    2014-01-01

    Full Text Available The use of immunobiological agents for the treatment of autoimmune diseases is increasing in medical practice. Anti-TNF therapies have been increasingly used in refractory autoimmune diseases, especially rheumatoid arthritis, with promising results. However, the use of such therapies has been associated with an increased risk of developing other autoimmune diseases. In addition, the use of anti-TNF agents can cause pulmonary complications, such as reactivation of mycobacterial and fungal infections, as well as sarcoidosis and other interstitial lung diseases (ILDs. There is evidence of an association between ILD and the use of anti-TNF agents, etanercept and infliximab in particular. Adalimumab is the newest drug in this class, and some authors have suggested that its use might induce or exacerbate preexisting ILDs. In this study, we report the first case of acute ILD secondary to the use of adalimumab in Brazil, in a patient with rheumatoid arthritis and without a history of ILD.

  2. Wish to conceive and concerns to develop cardiovascular complications during pregnancy in patients with Turner syndrome

    NARCIS (Netherlands)

    I.M. van Hagen (Iris); A.L. Duijnhouwer (Anthonie L.); M.J. ten Kate-Booij (Marianne); R.H.M. Dykgraaf (Ramon); J.J. Duvekot (Hans); E.M.W.J. Utens (Elisabeth); J.W. Roos-Hesselink (Jolien)

    2017-01-01

    textabstractIntroduction: Turner syndrome (TS) is associated with subfertility and infertility. Nevertheless, an increasing number of women become pregnant through oocyte donation. The wish to conceive may be negatively influenced by the fear of cardiovascular complications. The aim was to

  3. Survival and complication free survival in Marfan's syndrome: implications of current guidelines

    NARCIS (Netherlands)

    Groenink, M.; Lohuis, T. A.; Tijssen, J. G.; Naeff, M. S.; Hennekam, R. C.; van der Wall, E. E.; Mulder, B. J.

    1999-01-01

    OBJECTIVE: To evaluate survival and complication free survival in patients with Marfan's syndrome and to assess the possible influence of recently revised guidelines for prophylactic aortic root replacement in these patients. METHODS: 130 patients who had been attending one institution over 14 years

  4. Management of xerostomia and other complications of Sjögren's syndrome.

    Science.gov (United States)

    Pinto, Andres

    2014-02-01

    This article provides an overview of the published literature in English in the past 63 years involving the management of xerostomia and other oral complications of Sjögren's syndrome. The evidence supporting studied interventions was evaluated using published criteria. Copyright © 2014 Elsevier Inc. All rights reserved.

  5. [A postpartum woman with toxic shock syndrome: group A streptococcal infection, a much feared postpartum complication.

    NARCIS (Netherlands)

    Abbink, K.; Kortekaas, J.C.; Buise, M.P.; Dokter, J.; Kuppens, S.M.; Hasaart, T.H.M.

    2016-01-01

    BACKGROUND: The development of toxic shock syndrome (TSS) after an invasive group A streptococcal (GAS) infection in the postpartum period is a much feared complication. The mortality rate of TSS with necrotizing fasciitis is 30 to 50%. CASE DESCRIPTION: We present the case of a woman with atypical

  6. Association of Trabecular Bone Score with Inflammation and Adiposity in Patients with Psoriasis: Effect of Adalimumab Therapy

    Directory of Open Access Journals (Sweden)

    José L. Hernández

    2016-01-01

    Full Text Available Studies on trabecular bone score (TBS in psoriasis are lacking. We aim to assess the association between TBS and inflammation, metabolic syndrome features, and serum adipokines in 29 nondiabetic patients with psoriasis without arthritis, before and after 6-month adalimumab therapy. For that purpose, adjusted partial correlations and stepwise multivariable linear regression analysis were performed. No correlation was found between TBS and disease severity. TBS was negatively associated with weight, BMI, waist perimeter, fat percentage, and systolic and diastolic blood pressure before and after adalimumab. After 6 months of therapy, a negative correlation between TBS and insulin resistance (p=0.02 and leptin (p=0.01 and a positive correlation with adiponectin were found (p=0.01. The best set of predictors for TBS values at baseline were female sex (p=0.015, age (p=0.05, and BMI (p=0.001. The best set of predictors for TBS following 6 months of biologic therapy were age (p=0.001, BMI (p<0.0001, and serum adiponectin levels (p=0.027. In conclusion, in nondiabetic patients with moderate-to-severe psoriasis, TBS correlates with metabolic syndrome features and inflammation. This association is still present after 6 months of adalimumab therapy. Moreover, serum adiponectin levels seem to be an independent variable related to TBS values, after adalimumab therapy.

  7. The clinical value of pulmonary perfusion imaging complicated with pulmonary embolism in children of nephrotic syndrome

    International Nuclear Information System (INIS)

    Lin Jun; Chen Ning; Miao Weibing; Peng Jiequan; Jiang Zhihong; Wu Jing

    2001-01-01

    To investigate the clinical features of complicated with pulmonary embolism nephrotic syndrome in children. 99m Tc-MAA pulmonary perfusion imaging was performed on 30 nephrotic syndrome in children with elevated plasma D-dimer. Results shown that 14 of 30 patients were found to have pulmonary embolism (46.7%). Pulmonary perfusion imaging showed an involvement of 1 pulmonary segment in 3 cases, 2 segments in 2 cases and over 3 segments in other 9 cases. Among them, there were 7 segments involved in one case. After two weeks of heparin anti-coagulative therapy, most cases showed a recovery. The result of this study suggested that pulmonary embolism is a common complication of nephrotic syndrome. Pulmonary perfusion imaging is simple, effective and accurate method for the diagnosis of pulmonary embolism, and it also can help to assess the value of clinical therapy

  8. Fatal Lemierre’s syndrome as a complication of chronic otitis media with cholesteatoma

    Directory of Open Access Journals (Sweden)

    Tashneem Harris

    2016-12-01

    Full Text Available Background. Lemierre’s syndrome is septic thrombophlebitis of the internal jugular vein, initiated by an infection of the head and neck region. This septic thrombophlebitis gives rise to septic microemboli that can disseminate throughout the body to form septic infarctions and abscesses, with the most frequent site being pulmonary. Methods. We discuss the case of a 14-year-old male with Lemierre’s syndrome as a complication of chronic middle ear infection. Results. The patient developed septic shock and microemboli, and subsequently died. Conclusion. This case report illustrates that untreated chronic middle ear infection can lead to potentially fatal complications such as Lemierre’s syndrome, and emphasises the importance of timeous treatment of chronic middle ear pathology.

  9. A case of Lemierre's syndrome with septic shock and complicated parapneumonic effusions requiring intrapleural fibrinolysis

    Directory of Open Access Journals (Sweden)

    Daniel P. Croft

    2015-01-01

    Full Text Available Lemierre's syndrome is a septic thrombophlebitis of the internal jugular vein, which can lead to severe systemic illness. We report a case of an otherwise healthy 26-year-old man who suffered from pharyngitis followed by septic shock requiring intubation and vasopressor support from Fusobacterium necrophorum bacteremia. The septic emboli to his lungs caused complicated bilateral parapneumonic effusions, which recurred after initial drainage. He required bilateral chest tubes and intrapleural tPA to successfully drain his effusions. His fever curve and overall condition improved with the resolution of his effusions and after a 33-day hospitalization, he recovered without significant disability. The severity of his illness and difficult to manage complicated parapneumonic effusions were the unique facets of this case. Using an evidence-based approach of tPA and DNase for complicated parapneumonic effusions in Lemierre's syndrome can be safe and effective.

  10. Effectiveness of infliximab after adalimumab failure in Crohn's disease.

    Science.gov (United States)

    Chaparro, María; Andreu, Montserrat; Barreiro-de Acosta, Manuel; García-Planella, Esther; Ricart, Elena; Domènech, Eugeni; Esteve, María; Merino, Olga; Nos, Pilar; Peñalva, Mireia; Gisbert, Javier P

    2012-10-07

    To evaluate the effectiveness of infliximab as a second-line therapy in Crohn's disease patients after adalimumab failure. A historical cohort study in a community-based gastroenterology practice evaluated Crohn's disease patients treated with infliximab (induction plus maintenance) after adalimumab failure. Patients were identified using a large Spanish database (ENEIDA). We included 15 Crohn's disease patients who received infliximab after adalimumab failure. Five patients discontinued adalimumab due to loss of response, 3 due to adverse events and 7 due to partial response. After infliximab therapy was started, all patients who had interrupted adalimumab due to loss of efficacy regained response. All patients who discontinued adalimumab due to adverse events responded to infliximab and maintained this response; one of these patients had an uneventful course on infliximab, but 2 developed adverse events. None of the 7 patients who interrupted adalimumab due to partial response reached remission with infliximab. Switching from adalimumab to infliximab may be useful in patients who develop adverse effects or loss of response, however, the benefit of infliximab in primary nonresponders was not established.

  11. Management of Crohn's disease in poor responders to adalimumab

    Directory of Open Access Journals (Sweden)

    de Boer NKH

    2014-04-01

    Full Text Available Nanne KH de Boer,1 Mark Löwenberg,2 Frank Hoentjen3 1Department of Gastroenterology and Hepatology, VU University Medical Center, Amsterdam, the Netherlands; 2Department of Gastroenterology and Hepatology, Academic Medical Center, Amsterdam, the Netherlands; 3Department of Gastroenterology and Hepatology, Radboud University Medical Center, Nijmegen, the Netherlands Abstract: Anti-tumor necrosis factor therapy with adalimumab is an effective therapy for the induction and maintenance of remission in moderate to severe Crohn's disease. Although a large proportion of patients show a favorable clinical response to adalimumab, therapy failure is common. In this review, we provide a practical overview of adalimumab therapy in patients with Crohn's disease, with a specific focus on the clinical management of adalimumab failure. In the case of inadequate efficacy, a thorough assessment is required to confirm inflammatory disease activity and rule out noninflammatory causes. Evaluation may include biomarkers (fecal calprotectin and serum C-reactive protein, colonoscopy, and/or magnetic resonance enterography/enteroclysis. Furthermore, adalimumab trough levels and antibodies to adalimumab are informational after the confirmation of active inflammation. In the case of low or undetectable adalimumab trough levels, dose escalation to 40 mg weekly is recommended, whereas high antibody titers or adverse events frequently require switching to an alternative anti-TNF agent such as infliximab. Active inflammation despite therapeutic adalimumab trough levels requires alternative strategies such as switching to drugs with a different mode of action or surgical intervention. Keywords: anti-TNF, biological, inflammatory bowel disease, loss of response, infliximab

  12. Spinal fusion for scoliosis in Rett syndrome with an emphasis on early postoperative complications.

    Science.gov (United States)

    Gabos, Peter G; Inan, Muharrem; Thacker, Mihir; Borkhu, Buttugs

    2012-01-15

    Retrospective case-control study. To examine the postoperative complications of posterior spinal fusion in a population of patients with Rett syndrome (RS). Scoliosis is a common feature of RS, a progressive neurologic disorder affecting almost exclusively females. Despite this, there is little published information regarding the surgical treatment of scoliosis in this disorder. Sixteen consecutive female patients with RS treated by posterior spinal fusion and unit rod instrumentation for progressive scoliosis between 1995 and 2003 were evaluated. Only patients with a minimum of 2-year follow-up were included. Preoperative medical conditions and postoperative complications were recorded. As a control group, we randomly selected 32 spastic quadriplegic patients who underwent the identical procedure during the same time period, selected from our database and matched according to age, level of neurologic impairment, and medical complexity. There was a high rate of early medical complications in the RS patients, with 28 major and 37 minor complications. Only 1 patient did not have a major medical complication, and every patient had at least 1 minor gastrointestinal and/or respiratory complication. Major respiratory complications occurred in 10 patients (63%) and comprised 61% of all major complications. Major gastrointestinal complications occurred in 6 patients (37%) and comprised 21% of all major complications. Other major complications included disseminated intravascular coagulopathy (1 patient), subacute bacterial endocarditis (1 patient), sacral decubiti requiring surgical debridement (2 patients), and extensive bilateral heterotopic ossification of the hips (1 patient). There were no cases of instrumentation failure, pseudarthrosis, deep infection, or need for rod revision. Postoperative complication scores were similar to those in patients with spastic quadriplegic pattern cerebral palsy. Spinal fusion for scoliosis in RS can give a satisfactory technical result

  13. A case report of minimal change nephrotic syndrome complicated with portal, splenic and superior mesenteric vein thrombosis.

    Science.gov (United States)

    Wang, Jun; Fan, QiuLing; Chen, Ying; Dong, Xuezhu; Zhang, YuXia; Feng, JiangMin; Ma, JianFei; Wang, LiNing

    2012-06-01

    Venous thrombosis is common in nephrotic syndrome, but portal vein thrombosis has a relatively low incidence in patients with nephrotic syndrome. We describe here a case of an 18-year old male student with newly diagnosed nephrotic syndrome that was complicated with portal, splenic and superior mesenteric vein thrombosis. In the presence of newly diagnosed nephrotic syndrome of minimal change disease, thrombus formation can occur and should be noted, particularly when it occurs, in rare sites. The recognition in nephrotic syndrome complicated with portal, splenic and superior mesenteric vein thrombosis should be emphasized.

  14. Embryonal natural orifice transluminal endoscopic surgery treating severe acute pancreatitis complicated by abdominal compartment syndrome.

    Science.gov (United States)

    Zhu, Huiming; Liao, Xiumin; Guo, Shaoqing; Xuong, Gaofei; Jiang, Di; Liu, Yujie

    2014-10-01

    This study aims to estimate the value of embryonal natural orifice transluminal endoscopic surgery (ENOTES) as a treatment for severe acute pancreatitis (SAP) complicated by abdominal compartment syndrome (ACS). The patients who were randomized into ENOTES group and surgery group underwent ENOTES and laparotomy, respectively. The Efficacy and complications of these two treatments were compared. Enterocinesia was observed earlier in patients of ENOTES group than that of surgery group. Acute Physiology and Chronic Health Evaluation II (APACHE II) score of patients in ENOTES group was superior to that of surgery group on the 1st, 3rd and 5th day after treatment (P complications and mortality were observed between two groups (P complications.

  15. Nontraumatic atlantoaxial subluxation (Grisel syndrome): a rare complication of otolaryngological procedures.

    Science.gov (United States)

    Yu, Kathy K; White, David R; Weissler, Mark C; Pillsbury, Harold C

    2003-06-01

    At the conclusion of the report, the readers should be able to recognize the rare complication of adenotonsillectomy of Grisel syndrome, discuss its pathogenesis, and provide early, effective treatment. Analysis of the clinical presentation, plain radiographs, computed tomography scans, and magnetic resonance imaging scans of a child with Grisel syndrome after adenotonsillectomy. Retrospective study of the case record and a review of the literature regarding the pathogenesis, treatment modalities, outcome, and prognosis after early and delayed treatment of Grisel syndrome. Pathophysiology of atlantoaxial subluxation revolves around the periodontoid vascular plexus that drains the posterosuperior pharyngeal region. Infectious and inflammatory emboli cause synovial engorgement, weakening paraspinal ligaments. Radiological studies play an important role in diagnosis. Treatment consists of cervical immobilization, muscle relaxants, analgesics, and antibiotics. Full resolution is expected with early conservative management. Failure to recognize the syndrome can lead to catastrophic results. Recognition of Grisel syndrome in addition to other rare complications of adenotonsillectomy requires a high index of suspicion. Early intervention is the critical factor for a positive outcome. However, delayed diagnosis is common and can result in catastrophic consequences, including neurological deficits, cosmetic deformity and, in rare instances, paralysis and death in the short term.

  16. HELLP Syndrome Complicated with Postpartum Subcapsular Ruptured Liver Hematoma and Purtscher-Like Retinopathy

    Directory of Open Access Journals (Sweden)

    Daniela Cernea

    2012-01-01

    Full Text Available Purtscher's retinopathy is usually associated with trauma, acute pancreatitis, vasculitis, lupus, and bone fractures. It was rarely described postpartum in patients with preeclampsia as well as associated with HELLP syndrome. We present a case of a multiparous patient aged 44 with severe preeclampsia and postpartum HELLP syndrome complicated with Purtscher-like retinopathy and large ruptured subcapsular liver hematoma that required emergency abdominal surgery after premature delivery of a dead fetus. Postsurgical outcome was favorable regarding both liver function and visual acuity.

  17. Hypothalamic-Pituitary-Adrenal Suppression and Iatrogenic Cushing's Syndrome as a Complication of Epidural Steroid Injections

    Directory of Open Access Journals (Sweden)

    Joyce Leary

    2013-01-01

    Full Text Available Epidural steroid injections are well accepted as a treatment for radicular back pain in appropriate candidates. While overall incidence of systemic side effects has not been well established, at least five biochemically proven cases of iatrogenic Cushing's Syndrome have been reported as complications of epidural steroid treatment. We present an additional case of iatrogenic Cushing's Syndrome and adrenal suppression in a middle-aged woman who received three epidural steroid injections over a four-month period. We review this case in the context of previous cases and discuss diagnostic and management issues.

  18. Hypothalamic-Pituitary-Adrenal Suppression and Iatrogenic Cushing's Syndrome as a Complication of Epidural Steroid Injections.

    Science.gov (United States)

    Leary, Joyce; Swislocki, Arthur

    2013-01-01

    Epidural steroid injections are well accepted as a treatment for radicular back pain in appropriate candidates. While overall incidence of systemic side effects has not been well established, at least five biochemically proven cases of iatrogenic Cushing's Syndrome have been reported as complications of epidural steroid treatment. We present an additional case of iatrogenic Cushing's Syndrome and adrenal suppression in a middle-aged woman who received three epidural steroid injections over a four-month period. We review this case in the context of previous cases and discuss diagnostic and management issues.

  19. Ectopic ACTH syndrome complicated by multiple opportunistic infections treated with percutaneous ablation of the adrenal glands.

    Science.gov (United States)

    Chan, Chrystal; Roberts, James Mark

    2017-11-14

    Ectopic adrenocorticotropic hormone (ACTH)-related Cushing's syndrome can lead to multiple complications including severe immunosuppression. If the ACTH-secreting tumour cannot be found, definitive treatment is surgical adrenalectomy, typically followed by glucocorticoid replacement. Here, we present a case of fulminant respiratory failure secondary to coinfection with Pneumocystis jirovecii and cytomegalovirus in a patient with ectopic ACTH-dependent Cushing's syndrome with occult primary. Due to significant deconditioning, she was unable to undergo definitive adrenalectomy and instead underwent percutaneous microwave ablation of the adrenal glands. © 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.

  20. Preventing the aortic complications of Marfan syndrome: a case-example of translational genomic medicine

    Science.gov (United States)

    Li-Wan-Po, Alain; Loeys, Bart; Farndon, Peter; Latham, David; Bradley, Caroline

    2011-01-01

    The translational path from pharmacological insight to effective therapy can be a long one. We aim to describe the management of Marfan syndrome as a case-example of how pharmacological and genomic insights can contribute to improved therapy. We undertook a literature search for studies of Marfan syndrome, to identify milestones in description, understanding and therapy of the syndrome. From the studies retrieved we then weaved an evidence-based description of progress. Marfan syndrome shows considerable heterogeneity in clinical presentation. It relies on defined clinical criteria with confirmation based on FBN1 mutation testing. Surgical advances have prolonged life in Marfan syndrome. First-line prophylaxis of complications with β-adrenoceptor blockers became established on the basis that reduction of aortic pressure and heart rate would help. Over-activity of proteinases, first suggested in 1980, has since been confirmed by evidence of over-expression of matrix metalloproteinases (MMP), notably MMP-2 and MMP-9. The search for MMP inhibitors led to the evaluation of doxycycline, and both animal studies and small trials, provided early evidence that this widely used antimicrobial agent was useful. Identification of the importance of TGF-β led to evaluation of angiotensin II type I receptor (AT1R) blockers with highly promising results. Combination prophylactic therapy would appear rational. Pharmacological and genomic research has provided good evidence that therapy with losartan and doxycycline would prevent the aortic complications of Marfan syndrome. If on-going well designed trials confirm their efficacy, the outlook for Marfan syndrome patients would be improved considerably. PMID:21276043

  1. Sickle cell disease complicated by post-streptococcal glomerulonephritis, cerebral hemorrhage and reversible posterior leucoencephalopathy syndrome.

    Science.gov (United States)

    Pashankar, Farzana D; Ment, Laura R; Pearson, Howard A

    2008-04-01

    A patient with homozygous hemoglobin SS disease presented with an intracerebral hemorrhage complicating reversible posterior leucoencephalopathy syndrome (RPLS), secondary to hypertension associated with acute post-streptococcal glomerulonephritis (APSGN). Distinguishing potentially reversible causes of central nervous system events from primary cerebral infarction or hemorrhage in patients with sickle cell disease is important because the management and prognosis of these complications is very different. Similarly, because of the difference in prognosis between APSGN and other forms of sickle cell nephropathy, it is also important to differentiate these conditions. (c) 2008 Wiley-Liss, Inc.

  2. Difficult-to-treat-pediatric Crohn's disease: focus on adalimumab

    Directory of Open Access Journals (Sweden)

    Zeisler B

    2015-04-01

    Full Text Available Bella Zeisler, Jeffrey S Hyams Division of Digestive Diseases, Hepatology, and Nutrition, Connecticut Children's Medical Center Hartford, University of Connecticut School of Medicine, Department of Pediatrics, Farmington, CT, USA Abstract: Adalimumab is a fully humanized anti-tumor necrosis factor alpha monoclonal antibody that was recently granted regulatory approval in the USA for the treatment of moderate to severe Crohn’s disease (CD in children. Like infliximab, the first biologic agent used to treat pediatric CD, regulatory approval was secured many years following approval for adults. The long delay between adult and pediatric approval has led to many years of off-label use of adalimumab, although it is anticipated that the use of adalimumab may further increase with official regulatory approval. To date, pediatric literature on the use of adalimumab for treatment of CD is limited, and pediatric practitioners have mostly extrapolated from research and experience provided by the adult literature. The aim of this paper is to review the literature regarding adalimumab for the treatment of pediatric CD, and includes a review of landmark adult studies as well as the pivotal pediatric study that facilitated regulatory approval. We also discuss the role of anti-tumor necrosis factor alpha agents including adalimumab in the current treatment paradigm for pediatric CD. Keywords: pediatrics, Crohn's disease, adalimumab, biologic agent

  3. Generalized tetanus could be complicated with Guillain–Barré syndrome

    Directory of Open Access Journals (Sweden)

    Jae Hoon Lee

    2016-07-01

    Full Text Available A retrospective analysis of patients diagnosed with tetanus was conducted to evaluate the occurrence of Guillain–Barré syndrome (GBS. Two of 13 tetanus cases were complicated with GBS. Their symptoms and signs related to GBS improved markedly after a 5-day infusion of intravenous immunoglobulin. Physicians should keep in mind that GBS can be an important cause of muscle weakness in patients with tetanus.

  4. [Tumor lysis syndrome in a pregnancy complicated with acute lymphoblastic leukemia].

    Science.gov (United States)

    Álvarez-Goris, M P; Sánchez-Zamora, R; Torres-Aguilar, A A; Briones Garduño, J C

    2016-04-01

    Acute leukemia is rare during pregnancy, affects about 1 in 75,000 pregnancies, of all leukemias diagnosed only 28% are acute lymphoblastic leukemia, this is a risk factor to develop spontaneous tumor lysis syndrome, it's a oncologic complication potentially deadly if the prophylactic treatment its avoided. Cases of acute lymphoblastic leukemia associated with pregnancy has been poorly documented in the literature the association of these two entities to pregnancy is the first report published worldwide, so the information is limited.

  5. Generalized tetanus could be complicated with Guillain-Barré syndrome.

    Science.gov (United States)

    Lee, Jae Hoon; Hwang, Yun Su; Cho, Ji Hyun

    2016-07-01

    A retrospective analysis of patients diagnosed with tetanus was conducted to evaluate the occurrence of Guillain-Barré syndrome (GBS). Two of 13 tetanus cases were complicated with GBS. Their symptoms and signs related to GBS improved markedly after a 5-day infusion of intravenous immunoglobulin. Physicians should keep in mind that GBS can be an important cause of muscle weakness in patients with tetanus. Copyright © 2016 The Author(s). Published by Elsevier Ltd.. All rights reserved.

  6. Severe Postoperative Complications may be Related to Mesenteric Traction Syndrome during Open Esophagectomy

    DEFF Research Database (Denmark)

    Ambrus, R; Svendsen, L B; Secher, N H

    2017-01-01

    syndrome during esophagectomy. METHODS: Flushing, hemodynamic variables, and plasma 6-keto-PGF1α were recorded during the abdominal part of open ( n = 25) and robotically assisted ( n = 25) esophagectomy. Postoperative complications were also registered, according to the Clavien-Dindo classification.......036). For patients who developed flushing, heart rate and plasma 6-keto-PGF1α also increased ( p = 0.001 and p 

  7. Case Report: Cervical chondrocalcinosis as a complication of Gitelman syndrome [version 1; referees: 2 approved

    Directory of Open Access Journals (Sweden)

    Zahra Iqbal

    2016-05-01

    Full Text Available Gitelman syndrome is an inherited tubulopathy leading to a hypokalaemic metabolic alkalosis with hypomagnesaemia and hypocalciuria. Most cases are due to mutations in SLC12A3, encoding the apical thiazide sensitive co-transporter in the distal convoluted tubule. Musculoskeletal effects of Gitelman syndrome are common, including muscle weakness, tetany and cramps. Chronic hypomagnesaemia can lead to chondrocalcinosis, which often affects knees but can affect other joints. Here we present a case of Gitelman syndrome complicated by cervical chondrocalcinosis leading to neck pain and numbness of the fingers. Treatments directed at correcting both hypokalaemia and hypomagnesaemia were initiated and allowed conservative non-surgical management of the neck pain. Recognition of chondrocalcinosis is important and treatments must be individualised to correct the underlying hypomagnesaemia.

  8. [Approaches to the treatment of patients with climacteric disorders complicated with menopausal metabolic syndrome with cholestasis].

    Science.gov (United States)

    Gavrilova, N P; Seliverstov, P V; Tatarova, N A; Radchenko, V G

    2014-01-01

    Development of the individual comprehensive program of follow treatment of patients with climacteric disorders complicated MMS (menopausal metabolic syndrome) with cholestasis; on the basis of application of low-dose hormone replacement therapy in combination with ursodeoxycholic acid, to improve the quality of life. We observed 101 woman with climacteric syndrome, obesity and cholestasis; conducted a comprehensive clinical and laboratory examination, ultrasound of the hepatobiliary system, measurement modified menopausal index (MMI), the measurement of the quality of life on questionnaire SF-36 before treatment and after 6 and 12 months. Positive and statistically significant changes in lipid spectrum, the activity of transaminases, bilirubin and its fractions, improvement of MMI and quality of life, the indices of coagulation remained virtually unchanged. Low-dose hormone replacement therapies in combination with ursodeoxycholic acid are highly effective drugs for the treatment of menopausal syndrome, which normalize lipid profile of patients and the performance of the hepatobiliary system.

  9. [Quality of life of patients at late terms after surgical treatment of complicated forms of diabetic foot syndrome].

    Science.gov (United States)

    Grachev, T V; Levchik, E Iu

    2010-01-01

    Quality of life were explored in an open prospective controlled study in 116 patients with complicated diabetic foot syndrome (experimental group) and 30 diabetics without this syndrome (control group) by questioning of quality of life (questionnaires SF-36, Functional Scale of Lower Extremity) in long terms (6-18 months) after surgical treatment. Advantages of foot "saving" resections in surgical treatment of complicated diabetic boot syndrome were detected: patients without amputations had the physical wellbeing score and the Functional Scale of Lower Extremity number, comparable with the group of diabetics without diabetic foot syndrome. The integral mental wellbeing scores were low in all observed groups of patients.

  10. Complications of Stevens-Johnson syndrome beyond the eye and skin.

    Science.gov (United States)

    Saeed, Hajirah; Mantagos, Iason S; Chodosh, James

    2016-02-01

    Ocular and cutaneous disease are common chronic sequelae of Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) and have been well described in the literature. Long-term complications affecting other organ systems have not been so well described. The purpose of this review article is to highlight non-ocular and non-cutaneous chronic complications of SJS/TEN. The PubMed database was searched for the keywords "Stevens-Johnson syndrome" and "toxic epidermal necrolysis" through September 1, 2014. Relevant articles were then reviewed in full. 138 articles in the English language were found that described chronic sequelae of SJS/TEN. Our search revealed six affected organ systems other than the eyes and integument, with chronic sequelae from SJS/TEN: respiratory, gastrointestinal/hepatic, oral, otorhinolaryngologic, gynecologic/genitourinary, and renal. Complications involving these organs systems appeared likely to reduce the quality of life for SJS/TEN survivors. SJS/TEN is a multi-organ disease requiring multidisciplinary care from a variety of specialists. Affected patients have complex hospital stays, and their quality of life may be severely impacted by multiple long-term complications. We believe that preventative care in the acute setting might limit the development and progression of many of the sequelae described above. Copyright © 2015 Elsevier Ltd and ISBI. All rights reserved.

  11. Cavernous sinus thrombosis syndrome and brainstem involvement in patient with leptospirosis: Two rare complications of leptospirosis

    Directory of Open Access Journals (Sweden)

    Shahriyar Alian

    2014-01-01

    Full Text Available Leptospirosis is a bacterial disease that is caused by pathogenic spirochetes of the genus Leptospira. It can affect humans and animals. In humans, it can lead to a wide spectrum of symptoms. It is known as the most common zoonosis in the world. The typical presentation of the disease is an acute biphasic febrile illness with or without jaundice. Less common clinical manifestations may result from involvement of different human body systems. In many places, this disease may be under-diagnosed, especially when associated with neurological complications. Moreover, without treatment, leptospirosis can lead to organ damages, and even death. Neurological complications are uncommon and are reported in a few cases. Cavernous sinus thrombosis syndrome and brainstem involvement are rare complications of leptospirosis and are associated with a high mortality risk. To our knowledge, no such cases have been reported in the literature.

  12. Incidence and Spectrum of Renal Complications and Extrarenal Diseases and Syndromes in 380 Children and Young Adults With Horseshoe Kidney.

    Science.gov (United States)

    Je, Bo-Kyung; Kim, Hee Kyung; Horn, Paul S

    2015-12-01

    The objective of our study was to evaluate the incidence and spectrum of renal complications and of extrarenal diseases and syndromes in children with horseshoe kidney (HSK). The clinical data and imaging studies of 380 subjects (median age, 2.8 years) diagnosed with HSK over a 24-year period were reviewed: 366 children and young adults and 14 fetuses, one of whom died in utero. All renal complications and extrarenal diseases and syndromes were documented. Patient age, initial imaging modality, follow-up imaging modalities, and imaging modality at diagnosis were recorded. One hundred seventy-five patients (46.1%) had renal complications. Pelvocaliectasis without an identifiable cause was the most common complication (n = 83, 21.8%) followed by vesicoureteral reflux (n = 37, 9.7%). Less common renal complications included duplex kidney, multicystic dysplastic kidney, renal stones, asymmetric renal size, ureteropelvic junction obstruction, ureteral stones, acute pyelonephritis, trauma, and renal rhabdomyosarcoma. One hundred ninety patients (50%) had extrarenal diseases or syndromes. Gastrointestinal tract (n = 62) and vertebral (n = 54) anomalies were the most common. Forty-nine patients had syndromes. Turner syndrome was the most common (n = 16) followed by caudal regression syndrome (n = 9). HSK was missed on initial imaging in 42 of 364 postnatal renal examinations and was diagnosed later. The patient groups with renal complications and with extrarenal diseases or syndromes and a missed diagnosis of HSK were significantly younger than the groups without them (p children with HSK have renal complications or extrarenal diseases or syndromes. The incidence is higher in younger children, and the spectrum of complications, diseases, and syndromes differs from those reported in previous studies of adults with HSK. Children with HSK, especially young children, therefore require follow-up renal examinations and extensive extrarenal workup.

  13. Neurovascular complications of marfan syndrome: a retrospective, hospital-based study.

    Science.gov (United States)

    Wityk, Robert J; Zanferrari, Carla; Oppenheimer, Stephen

    2002-03-01

    Small case series have associated Marfan syndrome with cerebral and spinal ischemia or hemorrhage. However, there has been no investigation of the frequency and etiology of neurovascular disorders in a large series of Marfan patients. We conducted a retrospective, hospital-based study of all Marfan syndrome patients seen in an 8-year period. Records were reviewed in detail, and clinical characteristics of those with and without a neurovascular diagnosis compared. Of 513 patients, 18 (3.5%) had a neurovascular diagnosis, as follows: transient ischemic attack (11), cerebral infarction (2), spinal cord infarction (2), subdural hematoma (2), and spinal subarachnoid hemorrhage (1). A cardioembolic source was identified in 12 of 13 patients with cerebral ischemia, as follows: prosthetic heart valves (9), mitral valve prolapse (2), and atrial fibrillation (1). Chronic anticoagulant therapy was a likely cause in 2 of 3 patients with hemorrhagic events. Compared with other Marfan syndrome patients, those with neurovascular events were older (39.6 versus 31.7 years, P=0.04) and more likely to be in atrial fibrillation (22.2% versus 3.2%, P=heart valves (61.1% versus 7.7%, P=0.001), and to be taking anticoagulant therapy (72.2% versus 16.1%, P<0.001). Aortic disease, a putative factor in the etiology of neurovascular complications, was present in equal measure in Marfan patients with and without neurovascular complications (78% versus 65%, P=NS). Neurovascular complications of Marfan syndrome are rare during 8 years of follow-up, and generally are ischemic in nature. A high-risk cardiac source was identified in the majority. A significant association with vascular dissection was not established.

  14. Infectious complications in patients with crush syndrome following the Wenchuan earthquake

    Directory of Open Access Journals (Sweden)

    ZHANG Hui

    2013-02-01

    Full Text Available 【Abstract】 Objective: To analyze the results of clini-cal and bacteriological examinations in patients with crush syndrome who suffered infectious complications after an earthquake in Sichuan, China. Methods: A total of 313 bacteriological samples among 147 patients with crush syndrome were collected. Infectious complications, results of microbiological examinations, po-tential risk factors of infection and mortality were analyzed statistically. Results: In the obtained database, 112 out of the 147 (75.7% patients had infectious complications, in which, wound infection, pulmonary infection, and sepsis were most common. The time under the rubble and the time from injury to treatment were related to the occurrence of wound infec-tion (P=0.013, odds ratio 2.25; P=0.017, odds ratio 2.31. Sep-sis and wound infection were more common in patients who underwent fasciotomy or amputation than in those who did not (P=0.001. Conclusion: Quick rescue and injury treatment can decrease the infection risk in crush syndrome patients. It is better to obtain microorganic proofs before applying antibiotics, and bacteriological and drug sensitivity data should be taken into account, especially considering that most of these infections are hospital-acquired and drug resistance. Emphasizing the accuracy and efficiency of wound management in emergency situations, cautiously assessing the indications for fasciotomy to avoid open wounds from unnecessary osteofascial compartment de-compression incisions may decrease the incidence of in-fection and ameliorate the prognosis. Key words: Earthquakes; Crush syndrome; Infection; Microbial sensitivity tests

  15. Infusion reactions associated with rituximab treatment for childhood-onset complicated nephrotic syndrome.

    Science.gov (United States)

    Kamei, Koichi; Ogura, Masao; Sato, Mai; Ito, Shuichi; Ishikura, Kenji

    2018-02-09

    Infusion reaction (IR) is defined as an adverse event within 24 h after monoclonal antibody infusion. In non-Hodgkin lymphoma, IR incidence following rituximab treatment is high (77-80%), but there are no data in complicated nephrotic syndrome. Records of rituximab infusions in patients with complicated nephrotic syndrome between February 2006 and December 2014 at the National Center for Child Health and Development were reviewed. Rituximab was administered at doses of 375 mg/m 2 . The severity of IR was evaluated using the Common Terminology Criteria for Adverse Events ver. 4.0. For 309 rituximab infusions in 159 patients (male, 110; median age, 12 years), IR was observed in 165 infusions (53.4%). Respiratory symptoms were most common (66% of all events). Ninety-five percent of the IR was observed within 3 h after rituximab infusion initiation. Sixty-eight percent of the events were classified as grade 1 and others classified as grade 2. Only 18% required medical intervention. CD20 cell count in patients with IR was significantly higher than in patients without IR. Incidence of IR was similar in subsequent rituximab treatment after B-cell recovery. Patients who experienced IR at first rituximab treatment were more likely to experience recurrent IR with subsequent treatments compared to those not having IR at initial treatment (odds ratio 3.64; p nephrotic syndrome, respiratory symptoms were the major type of IR, mostly observed within 3 h of infusion. Incidence of IR was lower and its severity milder in patients with complicated nephrotic syndrome than those with lymphoma.

  16. Calcinosis Cutis Complicated by Compartment Syndrome Following Extravasation of Calcium Gluconate in a Neonate: A Case Report

    Directory of Open Access Journals (Sweden)

    Tuo-Kang Chen

    2010-08-01

    Full Text Available Hypocalcemia most frequently occurs in premature neonates. It is usually treated by intravenous (iv calcium supplementation. However, complications caused by extravasation of iv calcium gluconate include localized soft tissue calcification, necrosis, cellulitis, osteomyelitis, and even compartment syndrome. We present a rare case of iatrogenic calcinosis cutis complicated by compartment syndrome secondary to extravasation of iv calcium gluconate in a neonate. Emergent fasciotomy was performed twice for decompression of compartment syndrome. Histologic findings revealed necrosis and calcification. Appropriate antibiotics were administered to control secondary infection. To the best of our knowledge, there were no previous case reports of calcinosis cutis with compartment syndrome in infants. Although iatrogenic calcinosis cutis is generally a benign entity, the early recognition of the presentation of extravasation of calcium gluconate is important to avoid severe complications and possible medical malpractice disputes. This report aims to raise doctors' awareness of the presentation, course, and management of this relatively rare iatrogenic complication.

  17. A Case of Compartment Syndrome in the Scrotum and Inguinal Area Complicating Idiopathic Nephrotic Syndrome of Childhood.

    Science.gov (United States)

    Saqan, Rola S; Alazab, Rami S; Allouh, Mohammed

    2017-11-01

    Idiopathic nephrotic syndrome (INS) is a kidney disease characterized by massive proteinuria. Protein loss leads to decreased oncotic pressure shifting the fluids into the interstitial space causing edema, complications such as infections and thromboembolism occur. We report a 7-year-old, diagnosed with NS presenting with a relapse. He developed ascites and scrotal edema followed by severe scrotal pain and redness, progressing rapidly to ecchymosis in the inguinal areas not in continuity with the scrotum. Ultrasound with color Doppler was inconclusive, scrotal exploration was done along with skin incision in the inguinal areas and scrotum, which appeared to relieve the condition rapidly.

  18. Orbital Cellulitis in a Psoriatic Patient Treated With Adalimumab.

    Science.gov (United States)

    Katana, Vienna G; Carpenter, Robert J; Trafeli, John P; Kwan, Julia M

    2017-03-01

    Psoriatic patients on tumor necrosis factor alpha inhibitors (TNFi) may be at increased risk for upper respiratory tract infections, including chronic rhino-sinusitis (CRS). Rarely, CRS can progress to orbital cellulitis (OC), an emergent ophthalmic complication wherein respiratory flora from paranasal sinus disease intrude the retro-orbital space. Single case report. We report the first case, to our knowledge, of an invasive sinusitis that rapidly evolved into OC in a patient receiving adalimumab treatment for plaque psoriasis and psoriatic arthritis. After TNFi withdrawal and appropriate medical and surgical intervention, the patient fully recovered. However, on resumption of TNFi therapy, symptoms of recalcitrant CRS returned. More investigation is needed to explore how TNFi might predispose to chronic, refractory rhino-sinusitis and subsequent progression to OC. Military physicians and other medical providers should be aware of this proposed new disease entity and the potential for rapidly evolving and invasive infections in immunocompromised patients. Screening and monitoring for chronic infectious disease, such as CRS before initiating and during TNFi therapy is warranted. Reprint & Copyright © 2017 Association of Military Surgeons of the U.S.

  19. Adalimumab treatment for severe recalcitrant chronic plaque psoriasis.

    LENUS (Irish Health Repository)

    Ryan, C

    2012-02-01

    AIM: To assess the efficacy and safety profile of adalimumab in patients with severe, recalcitrant chronic plaque psoriasis, and to assess short-term overlapping of other systemic treatment with adalimumab to prevent flaring of disease. METHODS: This was a retrospective study comprising 39 patients with chronic plaque psoriasis treated with adalimumab between October 2005 and January 2008. All had failed treatment with other systemic agents, including biological therapies in 59% of patients. Patients were started on adalimumab 40 mg weekly or fortnightly, as clinically indicated. Severity of psoriasis was assessed by the Psoriasis Area and Severity Index (PASI). Therapeutic response was assessed by 75% improvement on PASI (PASI 75). All adverse events were recorded. RESULTS: Results were analysed separately for those treated with adalimumab only and those on combination treatment. PASI 75 was achieved in 38% (8 of 21 patients at week 16), 62% (13 of 21 patients) at week 24, 69% (9 of 13 patients) at week 48% and 71% (5 of 7 patients) at week 72 in the adalimumab-only group, compared with 56% (5 of 9 patients) at week 16, 50% (4 of 8 patients) at week 24, 80% (4 of 5 patients) at week 48% and 67% (2 of 3 patients) at week 72 in the combined group. Of the 39 patients, 15 (38%) achieved a PASI of 0 at some point in their treatment. Adalimumab was well tolerated; 38% of patients experienced side-effects, which were generally mild. CONCLUSION: Adalimumab was effective in a group of patients with psoriasis refractory to other systemic therapies, including biological treatments, and was well tolerated.

  20. Complicated Cholelithiasis: An Unusual Combination of Acute Pancreatitis and Bouveret Syndrome

    Directory of Open Access Journals (Sweden)

    Ioannis Baloyiannis

    2012-07-01

    Full Text Available Bouveret syndrome is a rare form of gallstone ileus. The purpose of the present study was to present the unusual case of a female patient with complicated cholelithiasis manifested as a combination of acute pancreatitis and concomitant Bouveret syndrome. A 61-year-old female patient was admitted to the emergency department complaining of mid-epigastric and right upper quadrant abdominal pain radiating band-like in the thoracic region of the back as well as repeated episodes of vomiting over the last 24 h. The initial correct diagnosis of pancreatitis was subsequently combined with the diagnosis of Bouveret syndrome as a computed tomography scan revealed the presence of a gallstone within the duodenum causing luminal obstruction. After failure of endoscopic gallstone removal, a surgical approach was undertaken where gallstone removal was followed by cholecystectomy and restoration of the anatomy by eliminating the fistula. The concomitant pancreatitis complicated the postoperative period and prolonged the length of hospital stay. However, the patient was discharge on the 45th postoperative day. Attempts for endoscopic removal of the impacted stone should be the initial therapeutic step. Surgery should be reserved for cases refractory to endoscopic intervention and when definite treatment is the actual challenge.

  1. A rare complication of Ramsey Hunt Syndrome: Sınus vein thrombosis

    Directory of Open Access Journals (Sweden)

    Ramiz Ahmedov

    2011-03-01

    Full Text Available Ramsay-Hunt Syndrome (RHS is a rare affection characterized by peripheral facial paralysis (PFP, skin eruption in the auricular canal and cochleovestibular symptoms. It is produced by varicella-zoster virus(VZV reactivation at the geniculate ganglia. In elderly and immunocompromised individuals, the virus may reactivate to produce shingles (zoster. After zoster resolves, many elderly patients experience postherpetic neuralgia. Uncommonly, VZV can spread to large cerebral arteries to cause a spectrum of large-vessel vascular damage, ranging from vasculopathy to vasculitis, with stroke. In immunocompromised individuals, especially those with cancer or acquired immunodeficiency syndrome, deeper tissue penetration of the virus may occur (as compared with immunocompetent individuals, with resultant myelitis, small-vessel vasculopathy, ventriculitis, and meningoencephalitis. The polymerase chain reaction (PCR analysis of cerebrospinal fluid remains the mainstay for diagnosing the neurologic complications of VZV during life. We report a case of Ramsay Hunt syndrome complicated with cerebral venous thrombosis. Patient received treatment with acyclovir and anticoagulation. Early treatment with acyclovir therapy and anticoagulation could improve the recovery rate of facial nerve palsy and sinus vein thrombosis

  2. Complicated cholelithiasis: an unusual combination of acute pancreatitis and bouveret syndrome.

    Science.gov (United States)

    Baloyiannis, Ioannis; Symeonidis, Dimitrios; Koukoulis, Georgios; Zachari, Eleni; Potamianos, Spyros; Tzovaras, George

    2012-05-01

    Bouveret syndrome is a rare form of gallstone ileus. The purpose of the present study was to present the unusual case of a female patient with complicated cholelithiasis manifested as a combination of acute pancreatitis and concomitant Bouveret syndrome. A 61-year-old female patient was admitted to the emergency department complaining of mid-epigastric and right upper quadrant abdominal pain radiating band-like in the thoracic region of the back as well as repeated episodes of vomiting over the last 24 h. The initial correct diagnosis of pancreatitis was subsequently combined with the diagnosis of Bouveret syndrome as a computed tomography scan revealed the presence of a gallstone within the duodenum causing luminal obstruction. After failure of endoscopic gallstone removal, a surgical approach was undertaken where gallstone removal was followed by cholecystectomy and restoration of the anatomy by eliminating the fistula. The concomitant pancreatitis complicated the postoperative period and prolonged the length of hospital stay. However, the patient was discharge on the 45th postoperative day. Attempts for endoscopic removal of the impacted stone should be the initial therapeutic step. Surgery should be reserved for cases refractory to endoscopic intervention and when definite treatment is the actual challenge.

  3. Adalimumab for treating childhood plaque psoriasis: a clinical trial evaluation.

    Science.gov (United States)

    Di Lernia, Vito

    2017-12-01

    Most systemic therapies have not been systematically investigated in moderate to severe childhood plaque psoriasis. Evidence on the efficacy and safety of systemic treatments is limited and therapeutic guidelines are lacking. Recently adalimumab, a fully human monoclonal antibody that binds tumor necrosis factor (TNF)- alpha, was investigated in childhood psoriasis. Adalimumab is licensed for many inflammatory conditions including chronic plaque psoriasis in adults. Areas covered: A randomized phase III study published provided favourable efficacy and safety data of adalimumab in childhood psoriasis. The active comparator was methotrexate. After 16 weeks of treatment, a PASI 75 score was achieved in 58% of patients within the adalimumab 0.8 mg/kg group compared with 32% of patients within the methotrexate group. Safety data gave no evidence of drug-related serious adverse events and no organ toxicity. This is the first randomised controlled study of either adalimumab or methotrexate in children and adolescents with psoriasis. Expert opinion: The aforementioned trial was the first to provide clinical data on adalimumab's efficacy and safety in the short term when treating children and adolescents with psoriasis. Through the use of an active comparator, this study has opened the way for the future assessment of systemic therapies in children and adolescent with this condition.

  4. Ophthalmic complications including retinal detachment in hyperimmunoglobulinemia E (Job′s syndrome: Case report and review of literature

    Directory of Open Access Journals (Sweden)

    Arora Vipul

    2009-01-01

    Full Text Available Hyperimmunoglobulinemia E (Job′s syndrome is characterized by markedly increased levels of immunoglobulin E, recurrent cutaneous and systemic pyogenic infections, atopic dermatitis, and peripheral eosinophilia. Although ocular involvement in Job′s syndrome is rare, there are reports of keratoconus, staphylococcal chalazia with blepharitis, and endophthalmitis by various authors. We present the first case report of retinal detachment with complicated cataract in Job′s syndrome.

  5. Gastrointestinal Surgery and Related Complications in Patients with Ehlers-Danlos Syndrome

    DEFF Research Database (Denmark)

    Burcharth, Jakob; Rosenberg, Jacob

    2012-01-01

    Introduction: Ehlers-Danlos syndrome (EDS) is a rare inherited group of connective tissue diseases characterized by joint hypermobility, skin hyperextensibility and bruising tendency. Common features of patients with EDS include vascular and gastrointestinal perforations. The purpose of this syst......Introduction: Ehlers-Danlos syndrome (EDS) is a rare inherited group of connective tissue diseases characterized by joint hypermobility, skin hyperextensibility and bruising tendency. Common features of patients with EDS include vascular and gastrointestinal perforations. The purpose...... of this systematic review is to address gastrointestinal diseases and the complications associated with surgical treatment of diseases relating to the gastrointestinal system in patients with EDS. Methods: PubMed search including the Medical Subject Heading (MeSH) terms 'Ehlers-Danlos Syndrome' and 'Gastrointestinal...... Diseases', and an Embase search including the Map Term to Subject Heading 'Ehlers-Danlos Syndrome' with 'AND' function of the keyword 'Gastrointestinal'. Results: The literature search resulted in inclusion of 53 articles after application of eligibility criteria. The primary results drawn from...

  6. [Extracranial vertebral artery aneurysm complicating Klippel-Feil syndrome: case report].

    Science.gov (United States)

    Shimizu, S; Kojima, T; Morooka, Y; Tanaka, K; Nakagawa, Y; Kuroki, M

    1996-10-01

    The Klippel-Feil syndrome is one of the spinal malformations characterized by fusion of the cervical vertebrae. It is well known that the malformation can cause some neurologic disorders. However, an aneurysm in the vertebral artery associated with the Klippel-Feil syndrome is extremely rare, with only one case having been reported in the literature. We present a 39-year-old female with a sudden onset of disturbed consciousness. Lateral cervical x-ray films showed the Klippel-Feil syndrome and hypermobility between C1 and C2. MRI showed multiple infarctions in the posterior circulation, including bilateral thalami. Right vertebral angiogram identified the extracranial vertebral artery aneurysm as the source of the emboli. The patient gradually recovered with conservative therapy. The aneurysm was thought to be produced by chronic arterial trauma secondary to excessive movement between C1 and C2. We conclude that an extracranial vertebral artery aneurysm is a serious complication in a patient with the Klippel-Feil syndrome.

  7. Capgras syndrome: a clinical manifestation of watershed cerebral infarct complicating the use of extracorporeal membrane oxygenation.

    Science.gov (United States)

    Dejode, J M; Antonini, F; Lagier, P; Martin, C

    2001-08-01

    Ischaemic cerebral accidents are frequent following extracorporeal membrane oxygenation (ECMO), especially after fixing the reinjection cannula in the right primitive carotid artery, which leads to an interruption in downstream flow. We describe a rare and unusual symptom of cerebral ischaemic accident that is known as Capgras syndrome. This feature is interesting because it may be documented by computed tomography (CT) scan and particular electroencephalography signals. It appears that our observation represents the first documented case of Capgras syndrome complicating ECMO. This incident emphasizes the potential hazards associated with right common artery ligature for venoarterial extracorporeal membrane oxygenation (VAECMO). In addition, it shows that this psychiatric symptom (that has been interpreted psychodynamically for many years) can have an organic basis, which should be studied.

  8. The role of levosimendan in acute heart failure complicating acute coronary syndrome

    DEFF Research Database (Denmark)

    Nieminen, Markku S; Buerke, M.; Cohen-Solal, A.

    2016-01-01

    Acute heart failure and/or cardiogenic shock are frequently triggered by ischemic coronary events. Yet, there is a paucity of randomized data on the management of patients with heart failure complicating acute coronary syndrome, as acute coronary syndrome and cardiogenic shock have frequently been...... defined as exclusion criteria in trials and registries. As a consequence, guideline recommendations are mostly driven by observational studies, even though these patients have a particularly poor prognosis compared to heart failure patients without signs of coronary artery disease. In acute heart failure...... adrenergic inotropes as a first line therapy for all ACS-AHF patients who are under beta-blockade and or when urinary output is insufficient after diuretics. Levosimendan can be used alone or in combination with other inotropic or vasopressor agents, but requires monitoring due to the risk of hypotension. (C...

  9. Bouveret's syndrome complicated by distal gallstone ileus after laser lithotropsy using Holmium: YAG laser

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    Rodgers John B

    2002-06-01

    Full Text Available Abstract Background Bouveret's syndrome is an unusual presentation of duodenal obstruction caused by the passage of a large gallstone through a cholecystoduodenal fistula. Endoscopic therapy has been used as first-line treatment, especially in patients with high surgical risk. Case presentation We report a 67-year-old woman who underwent an endoscopic attempt to fragment and retrieve a duodenal stone using a Holmium: Yttrium-Aluminum-Garnet Laser (Ho:YAG which resulted in small bowel obstruction. The patient successfully underwent enterolithotomy without cholecystectomy or closure of the fistula. Conclusion We conclude that, distal gallstone obstruction, due to migration of partially fragmented stones, can occur as a possible complication of laser lithotripsy treatment of Bouveret's syndrome and might require urgent enterolithotomy.

  10. Superior vena cava syndrome: A rare complication of percutaneous nephrolithotripsy laser lithotripsy

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    Qing Ji

    2010-01-01

    Full Text Available Purpose: To describe a case of acute superior vena cava syndrome during percutaneous nephrolithotomy (PCNL, and to review the associated clinical features, management and complications. Clinical features: A 34-year-old man, diagnosed as right renal calculi and nodal tachycardia, was admitted to receive percutaneous nephroscope laser lithotripsy. Shortly after stone disintegration, he suffered acute hypoxic and hypotension, and showed cyanoderma of face and chest skin, ocular proptosis, jugular filling and ventricular fibrillation. Dopamine and adrenaline was intravenously injected. The patient was turned over to supine position and external cardiac massage and electric defibrillation were carried out immediately. The patient finally cardioverted. His vital signs subsequently became stable and cyanoderma faded. The patient was eventually discharged from the intensive care unit three days following the event. Conclusion: Severe complications such as cardiac arrest could happen during PCNL. Close monitoring the vital signs is essential for early finding and quick response to rescue.

  11. Efficacy of metformin on pregnancy complications in women with polycystic ovary syndrome: a meta-analysis.

    Science.gov (United States)

    Feng, Li; Lin, Xiao-Fang; Wan, Zhi-Hua; Hu, Dan; Du, Yu-Kai

    2015-01-01

    To evaluate the efficacy of metformin administration throughout pregnancy on pregnancy-related complications in women with polycystic ovary syndrome (PCOS). MEDLINE and ScienceDirect were searched to retrieve relevant trials. The endpoint was the incidence of complications of pregnancy, gestational diabetes mellitus (GDM), pre-eclampsia (PE), miscarriage and premature birth included. Five studies with 502 PCOS patients with metformin administration throughout pregnancy and 427 controls who used metformin just to get conception were included in our meta-analysis. In study group, a significantly lower change of emerging miscarriage and premature birth was observed, the pooled relative risk (RR) was 0.32 (95% confidence interval (CI): 0.19-0.56) for miscarriage and 0.40 (95%CI: 0.18-0.91) for premature birth. No significant difference was demonstrated in emerging GDM and PE. Metformin therapy throughout pregnancy can reduce the RR of miscarriage and premature birth incidence in PCOS patients with no serious side effects.

  12. Bilateral gluteal compartment syndrome complicated by rhabdomyolysis and acute kidney injury in a patient with alcohol intoxication

    Directory of Open Access Journals (Sweden)

    Jae Young Cho

    2012-12-01

    Full Text Available Bilateral gluteal compartment syndrome is a rare clinical entity that can be complicated by rhabdomyolysis or acute kidney injury (AKI. We report the a case of a 30-year-old woman without any comorbid diseases who was diagnosed with bilateral gluteal compartment syndrome complicated by rhabdomyolysis and dialysis-requiring AKI, which was caused by prolonged immobilization under the influence of alcohol. Although the patient’s renal function recovered fully after 5 sessions of hemodialysis, sciatic neuropathy caused by gluteal compartment syndrome led to permanent foot drop.

  13. Complications in mechanically ventilated patients of Guillain–Barre syndrome and their prognostic value

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    Archana Becket Netto

    2017-01-01

    Full Text Available Introduction: The spectrum of various complications in critically ill Guillain–Barre syndrome (GBS and its effect on the prognosis is lacking in literature. This study aimed at enumerating the complications in such a cohort and their significance in the prognosis and mortality. Materials and Methods: Retrospective case record analysis of all consecutive mechanically ventilated patients of GBS in neurology Intensive Care Unit (ICU of a tertiary care institute for 10 years was done. Demographic, laboratory, and treatment details and outcome parameters were recorded. Results: Among the 173 patients were 118 men and 55 women (2.1:1, aged 1–84 years. The average number of ICU complications per patient was 6.8 ± 1.8 (median = 7, range = 1–12. The most common complication was tracheobronchitis (128. Other pulmonary complications were found in 36 patients. The next was metabolic hyponatremia (115 hypokalemia (67, hypocalcemia (13, stress hyperglycemia (10, hyperkalemia (8, hypernatremia (9. Sepsis (40, UTI (47, dysautonomia (27, hypoalbuminemia (76, anemia (75, seizures (8, paralytic ileus (5, bleeding (4, anoxic encephalopathy (3, organ failures (12, deep vein thrombosis (7, and drug rashes (1 were also noted. The complications, considered significant in causing death, Hughes scale ≤ 3 at discharge, prolonged mechanical ventilation (>21 days and hospitalization (>36 days were pneumonia, hyponatremia, hypokalemia, urinary infection, tracheobronchial infections, hypoalbuminemia, sepsis, anemia dysautonomia. Conclusion: Active monitoring and appropriate and early intervention by the clinician will improve the quality of life of these patients and reduce the cost of prolonged mechanical ventilation and ICU stay.

  14. Hepatitis and pneumonitis during adalimumab therapy in Crohn disease: mind the histoplasmosis!

    Science.gov (United States)

    Pinheiro, Bruno do Valle; Delgado, Aureo de Almeida; Chebli, Julio Maria Fonseca

    2014-01-01

    Tumor necrosis factor-alpha (TNF-α) inhibitor therapy plays a pivotal role in the management of moderate to severe inflammatory bowel disease. Because of the role of TNF-α in the host defenses, anti-TNF therapy has been associated with an increase the risks of granulomatous infections. To report the first case of adalimumab-associated invasive histoplasmosis presenting as an acute hepatitis-like syndrome and febrile pneumonitis in a patient with Crohn's disease. Case report of a patient with progressive histoplasmosis confirmed by percutaneous fine needle aspiration biopsy lung and urine Histoplasma antigen. We present the case of a young man with CD who developed pneumonia and acute hepatitis-like features caused by Histoplasma capsulatum infection during adalimumab therapy. To the best of our knowledge, this acute hepatitis-like manifestation has never been reported as a presentation of the histoplasmosis in patients with Crohn's disease. This case underscores the potential risk for serious infection that may arise in this setting and should alert clinicians to the need to consider the histoplasmosis diagnosis in patients presenting with acute hepatitis-like syndrome associated with prolonged febrile illness or pneumonitis during therapy with anti-TNF-α antibodies.

  15. HEPATITIS AND PNEUMONITIS DURIN ADALIMUMAB THERAPY IN CROHN? DISEASE: mind the histoplasmosis!

    Directory of Open Access Journals (Sweden)

    Bruno do Valle PINHEIRO

    2014-03-01

    Full Text Available Context Tumor necrosis factor-alpha (TNF-α inhibitor therapy plays a pivotal role in the management of moderate to severe inflammatory bowel disease. Because of the role of TNF-α in the host defenses, anti-TNF therapy has been associated with an increase the risks of granulomatous infections. Objective To report the first case of adalimumab-associated invasive histoplasmosis presenting as an acute hepatitis-like syndrome and febrile pneumonitis in a patient with Crohn’s disease. Method Case report of a patient with progressive histoplasmosis confirmed by percutaneous fine needle aspiration biopsy lung and urine Histoplasma antigen. Results We present the case of a young man with CD who developed pneumonia and acute hepatitis-like features caused by Histoplasma capsulatum infection during adalimumab therapy. To the best of our knowledge, this acute hepatitis-like manifestation has never been reported as a presentation of the histoplasmosis in patients with Crohn’s disease. Conclusions This case underscores the potential risk for serious infection that may arise in this setting and should alert clinicians to the need to consider the histoplasmosis diagnosis in patients presenting with acute hepatitis-like syndrome associated with prolonged febrile illness or pneumonitis during therapy with anti-TNF-α antibodies.

  16. The Effectiveness of GECB Pastille in Reducing Complications of Dry Socket Syndrome

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    Abbas Haghighat

    2012-01-01

    Full Text Available Background and Purpose. Dry socket syndrome is one of the most irritating complications after tooth extraction. This study aims to investigate the efficacy of pastille GECB compared to ZOE. Materials and Methods. 30 patients with dry socket syndrome were selected and divided into two groups. GECB pastille was produced with 3% Guaiacol, 3% Eugenol 1.6% Chlorobutanol, sized 3×7×10 mm. GECB was applied in one group, and ZOE was used for the other group. Duration of pain after treatment and painkiller intake values were recorded within 20 days. The data were analyzed with independent samples t-test, Mann-Whitney, and Chi-Square tests. Results. Pain persisted for 45.53±33.34 minutes in patients treated with ZOE and 19.87±21.80 minutes in those treated with GECB (=0.19. Patients in the ZOE group reported more painkiller intake within 20 days (=0.031. Conclusion. GECB showed more significant efficacy in reducing complications after tooth extraction.

  17. [PRES (Posterior Reversible Encephalopathy Syndrome): potential complication of hypertensive crisis. Case report and literature review].

    Science.gov (United States)

    Vergura, Michele; Prencipe, Michele; Del Giudice, Antonio Maria; Grifa, Rachele; Miscio, Filomena; Pennelli, Anna Maria; Popolizio, Teresa; Simeone, Anna; Ferrara, Mariangela; Leone, Maurizio; Aucella, Filippo

    2017-04-01

    Posterior reversible encephalopathy syndrome (PRES) is a clinical-radiological syndrome, usually reversible, characterized by vasogenic oedema in cerebral posterior regions in patients with autoimmune diseases, nephropathies, hypertensive crisis, eclampsia and exposure to cytotoxic drugs. The main symptoms are: headache, nausea, vomiting, seizures, visual disturbance and altered consciousness. Complications as cerebral hemorrhage and recurrences are rare. We describe a case of a 65 years old woman, affected by chronic kidney disease, recently exacerbated, diabetes and hypertension in treatment, who showed an heterogeneous clinical presentation with vomiting, headache, blurred vision and impaired consciousness during an episode of acute hypertension. After an adjustement of the antihypertensive treatment we observed a regression of symptoms in one week. FLAIR sequences on MRI showed cerebral bilateral vasogenic oedema in posterior regions, typical for PRES. This case was suggestive for PRES and a prompt adjustement of the antihypertensive treatment was critical for clinical recovery. Brain MRI was crucial for diagnosis. It is important for clinicians to recognize PRES as a possible complication of renal disease and hypertensive crisis. Copyright by Società Italiana di Nefrologia SIN, Rome, Italy.

  18. [Two cases of rapidly progressive nephritic syndrome complicated with alcoholic liver cirrhosis].

    Science.gov (United States)

    Kaneko, Tomohiro; Arima, Ryuji; Arakawa, Yusuke; Aoki, Michiko; Fukuda, Kumiko; Fukui, Megumi; Hirama, Akio; Fujita, Emiko; Mii, Akiko; Utsumi, Koichi; Shimizu, Akira; Iino, Yasuhiko

    2011-01-01

    It has been reported that glomerulosclerosis with IgA deposition is likely to be complicated with alcoholic liver cirrhosis. On the other hand, it is said that complications of nephrotic syndrome or rapidly progressive glomerulonephritis (RPGN) are relatively rare. We experienced two patients with alcoholic liver cirrhosis complicated with RPGN syndrome who had obtained favorable outcomes through the use of steroids and immune system suppressors. Case 1 was a 55-year-old male. He was being treated for alcoholic liver cirrhosis, but as bloody urine was noticed macroscopically, his renal function rapidly decreased. Specimens from a renal biopsy showed endocapillary proliferative lesions accompanying necrotic lesions. Granular deposition of IgA (IgA1) and C3 was seen along the capillary walls and in the mesangial areas. After the combined treatments of bilateral palatotonsillectomy, three courses of steroid semi-pulse therapy and post-therapy with steroids and mizoribin (MZR)were started, his hematuria and proteinuria disappeared and renal function improved markedly. Case 2 was a 37-year-old male with alcoholic liver cirrhosis complicated with hepatic encephalopathy. Although he was being treated at another hospital, nephritic syndrome occurred with rapidly worsening renal function and massive ascites. After continuous drainage of the ascites, we performed a renal biopsy. Mild proliferative lesions and notable wrinkling, thickening and doubling of the basal membrane were seen. Crescent formations were found in about half of the glomeruli. The fluorescent antibody technique showed positive pictures of IgA (IgA1) and C3. When three courses of steroid semi-pulse therapy and post therapy with steroids and MZR were combined, his proteinuria and serum Cre level decreased and stagnated ascites markedly decreased. The two cases were diagnosed as having secondary IgA nephropathy induced by the deposition of the IgA1 derived mainly from the intestinal tract, which had increased

  19. Deterimental complication of meconium aspiration syndrome and their impact on outcome

    International Nuclear Information System (INIS)

    Shaikh, M.; Waheed, K.A.I.; Javaid, S.; Gul, R.

    2016-01-01

    Meconium aspiration syndrome (MAS) is respiratory distress in an infant born through meconium stained amniotic fluid (MSAF) whose signs cannot be otherwise explained. MAS is associated with different complications. Many studies have been conducted in developed world to find the outcome associated with MAS but data from developing countries is lacking. Present study was conducted to determine the impact of chemical pneumonitis, pulmonary hypertension (PHN) and air leak on outcome of new-borns with MAS. Methods: This cross sectional descriptive study was conducted in a tertiary care Neonatology unit. The babies diagnosed with MAS were included in the study. All patients were monitored for development of complications. Outcome in terms of mortality was recorded. Outcome was compared by chi-square test and p-value <0.05 is considered significant. Results: Seventy two babies were included in the study. Mean gestation was 37 ± 0.56 weeks and birth weight was 2.87 ± 0.49 kg. Male to female ratio was 1.57:1 and mean age of admission was 9.59 ± 5.27 hours. Chemical pneumonitis, PHN and chemical pneumonitis with PHN were observed in 23.6%, 20.8% and 26.4% neonates respectively. Mortality rate was 19.44%. The mortality was highest in babies having chemical pneumonitis with PHN (p-value=0.013) followed by chemical pneumonitis group (p-value=0.02) and PHN group (p-value=0.032). Conclusion: About three fourth babies with MAS developed one or more complications. Mortality is significantly increases with the development of complications so as more the complications more is the mortality. (author)

  20. Detrimental Complications Of Meconium Aspiration Syndrome And Their Impact On Outcome.

    Science.gov (United States)

    Shaikh, Mehmood; Irfan Waheed, Khawaja Ahmad; Javaid, Sulman; Gul, Rafia; Hashmi, Muhammad Almas; Fatima, Syeda Tehseen

    2016-01-01

    Meconium aspiration syndrome (MAS) is respiratory distress in an infant born through meconium stained amniotic fluid (MSAF) whose signs cannot be otherwise explained. MAS is associated with different complications. Many studies have been conducted in developed world to find the outcome associated with MAS but data from developing countries is lacking. Present study was conducted to determine the impact of chemical pneumonitis, pulmonary hypertension (PHN) and air leak on outcome of new-borns with MAS. This cross sectional descriptive study was conducted in a tertiary care Neonatology unit. The babies diagnosed with MAS were included in the study. All patients were monitored for development of complications. Outcome in terms of mortality was recorded. Outcome was compared by chisquare test and p-value <0.05 is considered significant. Seventy two babies were included in the study. Mean gestation was 37±0.56 weeks and birth weight was 2.87±0.49 kg. Male to female ratio was 1.57:1 and mean age of admission was 9.59±5.27 hours. Chemical pneumonitis, PHN and chemical pneumonitis with PHN were observed in 23.6%, 20.8% and 26.4% neonates respectively. Mortality rate was 19.44%. The mortality was highest in babies having chemical pneumonitis with PHN (p-value=0.013) followed by chemical pneumonitis group (p-value=0.02) and PHN group (p-value=0.032). About three fourth babies with MAS developed one or more complications. Mortality is significantly increases with the development of complications so as more the complications more is the mortality.

  1. Acute abdominal compartment syndrome complicating a colonoscopic perforation: a case report

    Directory of Open Access Journals (Sweden)

    Souadka Amine

    2012-02-01

    Full Text Available Abstract Introduction A perforation occurring during colonoscopy is an extremely rare complication that may be difficult to diagnose. It can be responsible for acute abdominal compartment syndrome, a potentially lethal complex pathological state in which an acute increase in intra-abdominal pressure may provoke the failure of several organ systems. Case presentation We report a case of acute abdominal compartment syndrome after perforation of the bowel during a colonoscopy in a 60-year-old North African man with rectal cancer, resulting in respiratory distress, cyanosis and cardiac arrest. Our patient was treated by needle decompression after the failure of cardiopulmonary resuscitation. An emergency laparotomy with anterior resection, including the perforated sigmoid colon, was then performed followed by immediate anastomosis. Our patient remains alive and free of disease three years later. Conclusion Acute abdominal compartment syndrome is a rare disease that may occasionally occur after a colonoscopic perforation. It should be kept in mind during colonoscopy, especially considering its simple salvage treatment.

  2. Benign acute childhood myositis complicating influenza B infection in a boy with idiopathic nephrotic syndrome.

    Science.gov (United States)

    Skrzypczyk, Piotr; Przychodzień, Joanna; Pańczyk-Tomaszewska, Małgorzata

    2016-01-01

    Benign acute childhood myositis (BACM) is an acute complication of an infection characterized by calf pain, limitation of lower limb mobility, an increase in serum creatine kinase, and a self-limiting course. No reports of BACM in children with idiopathic nephrotic syndrome (INS) can be found in the literature. A 5-year-old boy with steroid-sensitive INS presented with fever, leg pain, and problems with walking. Physical examination showed pharyngeal erythema, preserved movements in all joints, and weakness of leg muscles. Laboratory tests showed white blood cell count 3900/µl, albumin 2.3 g/dl, urea 25 mg/dl, creatinine 0.3 mg/dl, increased transaminases (AspAT 440 U/l, AlAT 100 U/l) and creatine kinase (10 817 U/l), and proteinuria 3500 mg/dl. The boy was diagnosed with an INS bout and BACM. Testing for infective causes of myositis showed evidence of an influenza B virus infection. Treatment included prednisone and oseltamivir. A rapid improvement of motor function was observed, with normalization of serum creatine kinase and transaminases, and resolution of proteinuria. 1. As influenza virus infection in a child with INS is a risk factor for complications and a disease bout, these patients should be vaccinated against influenza. 2. Differential diagnosis of leg pain and mobility limitation in a child with INS should include lower limb deep venous thrombosis, arthritis, post-infectious neurological complications (including Guillain-Barré syndrome), and BACM. 3. Serum creatine kinase level should be measured in all cases of motor disturbances in a child with symptoms of respiratory tract infection.

  3. [Cold autoimmune hemolytic anemia complicated with relapsed myelodysplastic syndrome after allogeneic hematopoietic cell transplantation].

    Science.gov (United States)

    Okamura, Hiroshi; Nakane, Takahiko; Fujino, Keizo; Koh, Shiro; Yoshimura, Takuro; Nishimoto, Mitsutaka; Hayashi, Yoshiki; Koh, Hideo; Nakao, Yoshitaka; Nakamae, Hirohisa; Hino, Masayuki

    2015-04-01

    Myelodysplastic syndrome (MDS) is known to often be complicated by a range of autoimmune diseases. We herein present a case with MDS complicated by cold autoimmune hemolytic anemia (cold AIHA). The patient was a 51-year-old woman. She was diagnosed with MDS (refractory cytopenia with multilineage dysplasia) in May 2009. In January 2010, she underwent unrelated allogeneic bone marrow transplantation but was re-admitted in October 2010 for treatment of relapsed MDS. Despite daily transfusions of red blood cells, her anemia failed to improve. Her laboratory examinations showed a low haptoglobin level and elevation of indirect bilirubin and LDH. The direct Coombs test was positive at a low and at room temperature and cold agglutinin was negative. After confirming the diagnosis of cold AIHA, all transfusion fluids were warmed but her anemia still failed to improve. In addition to the warmed transfusion fluids, we administered corticosteroids, immunosuppressive agents and high-dose intravenous immunoglobulin infusions. This management strategy ameliorated the patient's hemolytic anemia. To our knowledge, MDS cases complicated by cold AIHA are rare. Our patient thus provides a valuable contribution to medical knowledge.

  4. Four Cases of Atopic Dermatitis Complicated by Sjogren's Syndrome: Link between Dry Skin and Autoimmune Anhidrosis

    Directory of Open Access Journals (Sweden)

    Shun Kitaba

    2011-01-01

    Full Text Available We report four adult cases of atopic dermatitis (AD complicated by Sjogren's syndrome (SS. The patients fulfilled diagnostic criteria for AD and SS. All cases showed persistent itchy dry skin and eczematous lesions complicated by sicca symptoms including dry eyes and dry mouth with moderate joint pain. One case manifested annular erythema and another manifested widespread discoid erythema. To investigate the underlying cause of dry skin in these cases, sweating function was evaluated using a quantitative sudomotor axon reflex test (QSART in which the axon reflex is stimulated by acetylcholine iontophoresis. The sweating latency time was significantly prolonged in eczematous skin of AD and AD/SS compared to normal controls. Axon reflex (AXR sweat volume was also significantly reduced in AD (normal and eczematous skin and AD/SS (normal and eczema compared to normal control. In contrast, the direct sweat volume of lesional or non-lesional AD skin induced by direct stimulation with acetylcholine was only slightly reduced compared to that in normal controls, but not in SS and lesional skin of AD/SS patients. These results suggest that the impaired sweat response in AD is attributable to an abnormal sudomotor axon reflex, which is accelerated and modulated when complicated by SS resulting in dry skin in the present cases.

  5. Tonic dystonia: an uncommon complication of reflex sympathetic dystrophy syndrome. A review of five cases.

    Science.gov (United States)

    Morelet, Aude; Gagneux-Lemoussu, Laurence; Brochot, Pascal; Ackah-Miezan, Stanley; Colmet-Daage, Jean-François; Gaillard, François; Boyer, François; Eschard, Jean-Paul; Etienne, Jean-Claude

    2005-05-01

    Tonic dystonia is an underrecognized complication of reflex sympathetic dystrophy syndrome (RSDS) characterized by an increase in muscle tone at the site of injury. Case-reports.- We describe five cases of tonic dystonia complicating RSDS of the lower extremity. There were four women and one man, with a mean age of 52 years. In addition to the typical features of RSDS, the patients had fixed equinovarus of the foot with hyperextension or hyperflexion of the great toe. In two patients, examination after spinal anesthesia showed that the deformity was reducible. Spontaneous resolution of the dystonia occurred in one patient. Another patient failed to experience meaningful improvement after a motor block followed by botulinic toxin injections. In two patients, the same treatment was followed by a slight improvement. Treatment options are still being evaluated in the last patient. Discussion.- Tonic dystonia is an underrecognized complication of RSDS that often develops after a minor injury yet causes prolonged pain and disability. Spread of the dystonia to other sites is not infrequent. The underlying mechanisms remain unclear but may involve dysfunction of the central or peripheral nervous system or psychogenic factors. Suggested treatments include motor block, intrathecal baclofen, sympathetic block, and sympathectomy. However, none of these treatments has been proved effective. Conclusion.- The five cases described here provide useful information on RSDS-associated tonic dystonia, a condition that runs a protracted course and remains difficult to manage.

  6. Anterior pituitary lobe atrophy as late complication of hemorrhagic fever with renal syndrome

    Directory of Open Access Journals (Sweden)

    Jovanović Dragan

    2009-01-01

    Full Text Available Introduction. Hemorrhagic fever with renal syndrome (HFRS is acute infective multisystemic disease followed by febrility, hemorrhages and acute renal insufficiency. Bleeding in the anterior pituitary lobe leading to tissue necrosis occurs in acute stage of severe clinical forms of HFRS, while atrophy of the anterior pituitary lobe with diminution of the gland function occurs after recovery stage. Case report. We presented a patient with the development of chronic renal insufficiency and hypopituitarism as complication that had been diagnosed six years after Hantavirus infection. Magnetic resonance of the pituitary gland revealed atrophy and empty sella turcica. Conclusion. Regarding frequency of this viral infection and its endemic character in some parts of our country partial and/or complete loss of pituitary function should be considered during the late stage of HFRS.

  7. Bronchiolitis obliterans complicating a pneumothorax after Stevens-Johnson syndrome induced by lamotrigine

    Directory of Open Access Journals (Sweden)

    Wu-ping Wang

    2015-03-01

    Full Text Available Bronchiolitis obliterans (BO was defined as a nonreversible obstructive lung disease in which the bronchioles are always compressed and narrowed by fibrosis or inflammation. In the severe event of lung collapse after BO, surgical intervention is often recommended, and conservative therapy is thought to be ineffective. Here, we report the case of a 9-year old girl clinically diagnosed as having bronchiolitis obliterans with abrupt occlusion of the right B4b bronchus. After a lamotrigine-induced Stevens-Johnson syndrome (SJS occurred, she presented with total collapse of the right lung on admission, which was subsequently complicated by a pneumothorax during conservative treatment, but with the re-expansion of the right upper lobe after intervention. The case indicates the possibility of reversing pulmonary atelectasis in BO. Thus, surgery may not be necessary.

  8. Bronchiolitis obliterans complicating a pneumothorax after Stevens-Johnson syndrome induced by lamotrigine.

    Science.gov (United States)

    Wang, Wu-ping; Ni, Yun-feng; Wei, Yao-ning; Li, Xiao-fei; Cheng, Qing-shu; Lu, Qiang

    2015-03-01

    Bronchiolitis obliterans (BO) was defined as a nonreversible obstructive lung disease in which the bronchioles are always compressed and narrowed by fibrosis or inflammation. In the severe event of lung collapse after BO, surgical intervention is often recommended, and conservative therapy is thought to be ineffective. Here, we report the case of a 9-year old girl clinically diagnosed as having bronchiolitis obliterans with abrupt occlusion of the right B4b bronchus. After a lamotrigine-induced Stevens-Johnson syndrome (SJS) occurred, she presented with total collapse of the right lung on admission, which was subsequently complicated by a pneumothorax during conservative treatment, but with the re-expansion of the right upper lobe after intervention. The case indicates the possibility of reversing pulmonary atelectasis in BO. Thus, surgery may not be necessary. Copyright © 2012. Published by Elsevier B.V.

  9. Visual Loss Induced by Adalimumab Used for Plaque Psoriasis

    Directory of Open Access Journals (Sweden)

    Norman Saffra

    2017-03-01

    Full Text Available A 61-year-old Caucasian male with severe plaque psoriasis without joint involvement was initiated on adalimumab therapy. Shortly thereafter he presented to the emergency room with acute loss of vision in the right eye. A comprehensive systemic workup was instituted which included magnetic resonance imaging (MRI with and without gadolinium of the brain and orbits. MRI revealed findings that were consistent with CNS demyelination and retrobulbar optic neuritis. Immediate cessation of adalimumab was instituted without any other systemic therapy. Complete return of vision occurred within 6 weeks. No additional psoriatic or neurologic treatment was instituted, and the patient has remained stable now for 14 months.

  10. Unambiguous molecular detections with multiple genetic approach for the complicated chromosome 22q11 deletion syndrome

    Directory of Open Access Journals (Sweden)

    Lin Lung-Huang

    2009-02-01

    Full Text Available Abstract Background Chromosome 22q11 deletion syndrome (22q11DS causes a developmental disorder during the embryonic stage, usually because of hemizygous deletions. The clinical pictures of patients with 22q11DS vary because of polymorphisms: on average, approximately 93% of affected individuals have a de novo deletion of 22q11, and the rest have inherited the same deletion from a parent. Methods using multiple genetic markers are thus important for the accurate detection of these microdeletions. Methods We studied 12 babies suspected to carry 22q11DS and 18 age-matched healthy controls from unrelated Taiwanese families. We determined genomic variance using microarray-based comparative genomic hybridization (array-CGH, quantitative real-time polymerase chain reaction (qPCR and multiplex ligation-dependent probe amplification (MLPA. Results Changes in genomic copy number were significantly associated with clinical manifestations for the classical criteria of 22q11DS using MPLA and qPCR (p Conclusion Both MLPA and qPCR could produce a clearly defined range of deleted genomic DNA, whereas there must be a deleted genome that is not distinguishable using MLPA. These data demonstrate that such multiple genetic approaches are necessary for the unambiguous molecular detection of these types of complicated genomic syndromes.

  11. Use of lung ultrasound in detection of complications of respiratory distress syndrome.

    Science.gov (United States)

    Sawires, Happy K; Abdel Ghany, Eman A; Hussein, Nouran F; Seif, Hadeel M

    2015-09-01

    Repeated chest radiography is required for the diagnosis and follow-up of neonates with respiratory distress syndrome (RDS) and carries the risk of radiation hazards. Lung ultrasound (LUS) is a non-invasive bedside diagnostic tool that has proven to be effective in the diagnosis of RDS. Our aim was to assess the role of LUS with respect to the standard chest X-ray (CXR) in the detection of complications of RDS in neonates. Ninety premature newborns of both genders with RDS (mean gestational age = 29.91 ± 1.33 wk) and 40 premature babies as a control group were involved in this study. All patients underwent initial clinical assessment as well as CXR and LUS. Those who presented with respiratory distress and/or exhibited deterioration of oxygenation parameters were followed by CXR and, within 4 h, by LUS. Alveolo-interstitial syndrome and pleural line abnormalities were detected in all cases (100%) in the initial assessment, patchy consolidation was detected in 34 cases and white lung was detected in 80 cases. Alveolo-interstitial syndrome was detected in 19 controls. In follow-up of the patients, LUS was superior to CXR in detection of consolidation and sub-pleural atelectasis, but not in detection of pneumothorax. We concluded that bedside LUS is a good non-hazardous alternative tool in the early detection and follow-up of RDS in the neonatal intensive care unit; it could be of value in reducing exposure to unnecessary radiation. Copyright © 2015 World Federation for Ultrasound in Medicine & Biology. Published by Elsevier Inc. All rights reserved.

  12. A Case of Acute Budd-Chiari Syndrome Complicating Primary Antiphospholipid Syndrome Presenting as Acute Abdomen and Responding to Tight Anticoagulant Therapy

    Directory of Open Access Journals (Sweden)

    Naofumi Chinen

    2016-01-01

    Full Text Available A 34-year-old woman with primary antiphospholipid syndrome was admitted to the Gastroenterology Department of our hospital with fever, acute abdomen, watery diarrhea, and extremely high levels of inflammatory parameters. She had a history of left lower limb deep vein thrombosis and pulmonary embolism and was taking warfarin potassium. Acute gastroenteritis was suspected and an antibiotic was administered, but symptoms progressed. Abdominal ultrasonography showed occlusion of the left hepatic vein and the middle hepatic vein and her D-dimer level was high. Accordingly, Budd-Chiari syndrome was diagnosed and high-dose intravenous infusion of heparin was initiated. Her abdominal symptoms improved and the levels of inflammatory parameters and D-dimer decreased rapidly. It is known that antiphospholipid syndrome can be complicated by Budd-Chiari syndrome that usually occurs as subacute or chronic onset, but acute onset is rare. It is difficult to diagnose acute Budd-Chiari syndrome complicating antiphospholipid syndrome and this complication generally has a poor outcome. However, the present case can get early diagnosis and successful treatment with tight anticoagulant therapy.

  13. [Clinical analysis of pregnancy and postpartum cardiovascular complications in Marfan syndrome].

    Science.gov (United States)

    Xie, Jin-sheng; Li, Bin; Sun, Yan-qing; Guan, Xin-liang; Xu, Bao-wei; Qiao, Zhi-yu

    2007-11-01

    To investigate the management of pregnancy and cardiovascular complications in women with Marfan syndrome (MFS). From October 1994 to September 2006, 30 patients with MFS undergoing cardiovascular surgery were studied retrospectively. In the labor of 46 offsprings given birth by 30 women, 5 cases (11%) were performed elective cesarean section because of the existence of aortic complication, and 12 (26%) were diagnosed as MFS. The gestation in two patients was terminated due to deterioration of aortic abnormalities during their third trimester, and they received surgical treatment with Bentall procedure. Two developed acute aortic dissection during labor and post delivery respectively. With the manipulation of anticoagulation peripartum, one who had the implantation with mechanical prosthesis went through pregnancy and delivery uneventfully. The average duration between delivery and cardiovascular surgery was (15 +/- 9) years. Vaginal delivery can be done safely in patients with the MFS who do not have or have mild cardiovascular system abnormalities, aortic dissection, or other important cardiac abnormalities, cesarean section should be the preferred method of delivery. Women with MFS are at increased risk for dissection and congestive heart failure during pregnancy and should be counseled before pregnancy about these risks, as well as the inheritance of the condition.

  14. PRES (posterior reversible encephalopathy syndrome), a rare complication of tacrolimus therapy.

    LENUS (Irish Health Repository)

    Hodnett, P

    2009-11-01

    With increasing numbers of solid organ and hematopoietic stem cell transplantations being performed, there have been significant increases in the use of immunosuppressive agents such as cyclosporine and tacrolimus. Posterior reversible encephalopathy syndrome (PRES) is a serious complication of immunosuppressive therapy use following solid organ or stem cell transplants. Clinical findings including headache, mental status changes, focal neurological deficits, and\\/or visual disturbances. Associated with these are characteristic imaging features of subcortical white matter lesions on computed tomography (CT) or magnetic resonance imaging (MRI). The changes in the subcortical white matter are secondary to potentially reversible vasogenic edema, although conversion to irreversible cytotoxic edema has been described. These imaging findings predominate in the territory of the posterior cerebral artery. Many studies have shown that the neurotoxicity associated with tacrolimus may occur at therapeutic levels. In most cases of PRES, the symptom complex is reversible by reducing the dosage or withholding the drug for a few days. While PRES is an uncommon complication, it is associated with significant morbidity and mortality if it is not expeditiously recognized. MRI represents the most sensitive imaging technique for recognizing PRES. This report highlights the value of MRI in prompt recognition of this entity, which offers the best chance of avoiding long-term sequelae.

  15. [Jugular vein thrombosis and Lemierre's syndrome--severe complications of oropharyngeal infections].

    Science.gov (United States)

    Maier, W; Lohnstein, P U; Schipper, J; Boedeker, C

    2010-09-01

    Acute oral or pharyngeal infections usually heal under adequate therapy within a few days. Therefore severe regionary or systemic complications are not regularly seen. We report on 3 patients in whom during or after apparent recovery from a pharyngeal or perioral infection a one-sided painful swelling of the neck associated with fever and leucocytosis developed. Color Doppler sonography (CDS) revealed unilateral thrombosis of the internal jugular vein (IJV) in all cases, whereupon we initiated high-dosed parenteral antibiotic therapy and therapeutic heparinisation. Furthermore, we drained detectable abscess formations. Nonetheless, in one patient fever attacks occurred postoperatively, accompanied by septic-embolic lung infiltrates, corresponding to Lemierre's syndrome. In all cases, we achieved clinical recovery and remission of infection. The course was significantly prolonged in the patient with pulmonary involvement and in this patient no reperfusion of the IJV was achieved. Even today serious complications may occur unexpectedly in presumed everyday oral or pharyngeal infections. CDS is a suitable procedure to disclose a jugular vein thrombosis (JVT) promptly and non-invasively. Parenteral antibiotic therapy for at least 10 days is usually the therapy of choice for JVT; additional full-heparinisation is controversially discussed in the professional literature. Septic pulmonary embolism following pharyngeal infection and JVT, as described by Lemierre, was associated with a high rate of mortality in the pre-antibiotic era, and even today may be fatal in spite of appropriate and maximal therapy. © Georg Thieme Verlag KG Stuttgart · New York.

  16. Long-term treatment of rheumatoid arthritis with adalimumab

    Directory of Open Access Journals (Sweden)

    Murdaca G

    2013-05-01

    Full Text Available Giuseppe Murdaca, Francesca Spanò, Francesco PuppoDepartment of Internal Medicine, Clinical Immunology Unit, University of Genoa, Genoa, ItalyAbstract: Rheumatoid arthritis (RA is a chronic inflammatory disease that is associated with joint damage and progressive disability, an increased risk of morbidity related to comorbid conditions and substantial socioeconomic costs. Tumor necrosis factor-alpha (TNF-α is a proinflammatory cytokine known to have a central role in the initial host response to infection and in the pathogenesis of various immune-mediated diseases, such as RA, ankylosing spondylitis, psoriasis and/or psoriatic arthritis, Crohn’s disease, and systemic lupus erythematosus. Five TNF-α inhibitors are available for the clinical use: infliximab; adalimumab; etanercept; golimumab; and certolizumab pegol. Infliximab is a chimeric human/murine IgG1 monoclonal antibody (mAb; adalimumab, and golimumab are human mAbs; certolizumab pegol is composed of the fragment antigen-binding anti-binding domain of a humanized anti-TNF-α mAb, combined with polyethylene glycol to increase its half-life in the body; etanercept is a fusion protein that acts as a “decoy receptor” for TNF-α. In this paper, we will briefly review the current data on efficacy and safety of adalimumab in patients with RA, its potential beneficial effects upon comorbid conditions, such as endothelial dysfunction and accelerated atherosclerosis in RA, and the immunogenicity.Keywords: adalimumab, efficacy, safety, rheumatoid arthritis, VEGF, immunogenicity, infections

  17. Two phase 3 trials of adalimumab for hidradenitis suppurativa

    DEFF Research Database (Denmark)

    Kimball, Alexa Boer; Okun, Martin; Williams, David A

    2016-01-01

    BACKGROUND Hidradenitis suppurativa is a painful, chronic inflammatory skin disease with few options for effective treatment. In a phase 2 trial, adalimumab, an antibody against tumor necrosis factor á, showed efficacy against hidradenitis suppurativa. METHODS PIONEER I and II were similarly desi...

  18. Adalimumab for the treatment of moderate to severe hidradenitis suppurativa

    DEFF Research Database (Denmark)

    Kimball, A.B.; Kerdel, F.; Adams, D.

    2012-01-01

    Trials.gov: NCT00918255) Setting: 26 academic and private practice medical centers in the United States and Europe. Patients: 154 adult patients with moderate to severe HS who were unresponsive or intolerant to oral antibiotics. Intervention: Patients were assigned in a 1:1:1 ratio to adalimumab, 40 mg...

  19. Adalimumab effect in a cohort of Libyan patients with rheumatic ...

    African Journals Online (AJOL)

    Four patients had psoriatic arthritis, mean age was 40 years, two were females and two were males. One patient had AOSD; she was a female aged 58 years. All RA patients were on prednisolone and/or one or two DMARD before starting adalimumab and failed to show a response. Fourteen AS patients were on one or two ...

  20. The use of adalimumab in severe fistulising Crohn's disease

    DEFF Research Database (Denmark)

    Bang, U.C.; Nielsen, Anette Mertz

    2008-01-01

    So far infliximab is the only approved anti-TNF-alpha antibody for patients with Crohn's disease. Development of antibodies to infliximab may result in allergic reactions or reduced effect. We report three patients who received adalimumab, which induced longstanding remission in all three patients...

  1. Use of sequential diagnostic pain blocks in a patient of posttraumatic complex regional pain syndrome-not otherwise specified complicated by myofascial trigger points and thoracolumbar pain syndrome

    Directory of Open Access Journals (Sweden)

    Kailash Kothari

    2017-01-01

    Full Text Available We are presenting a case of posttraumatic lower limb Complex regional pain syndrome – Not otherwise specified (CRPS – NOS. As it was not treated in acute phase, the pain became chronic and got complicated by myofascial and thoracolumbar pain syndrome. This case posed us a diagnostic challenge. We used sequential diagnostic pain blocks to identify the pain generators and successfully treat the patient. We used diagnostic blocks step by step to identify and treat pain generators – T12,L1 and L2 Facet joints, Lumbar sympathetic block for CRPS NOS and Trigger point injection with dry needling for myofascial pain syndrome. This case highlights the facet that additional pain generators unrelated to original pain may complicate the presentation. Identifying these pain generators requires out of box thinking and high index of suspicion.

  2. [Diabetes mellitus as a rare complication of hemolytic uremic syndrome--case report].

    Science.gov (United States)

    Rogowska-Kalisz, Anna; Tkaczyk, Marcin; Szałapska-Zawodniak, Małgorzata

    2010-01-01

    A 4-year-old girl was hospitalized in a local hospital with bloody diarrhoea, vomitus and abdominal pain. Because of acute abdominal symptoms she underwent appendectomy after which convulsions and acute respiratory distress were noticed. The child was transferred to the intensive care unit. During the examination she was unconscious, pale, oedematous with scattered ecchymoses, severe hypertension and urine output diminished to several ml per day. Routine blood tests showed microangiopathic anaemia, thrombocytopenia (52000/ul.) and uremia. Proteinuria and hematuria were revealed on urine examination. Among coagulation parameters kaolin-kefalin time (69 s) and D-dimers (2000-4000/ul.) were abnormal. On the strength of history, clinical and laboratory investigation the diagnosis of D-positive hemolytic uremic syndrome was established. Controlled artificial respiration (for 10 weeks), total parenteral alimentation (TPN), antihypertensive treatment and diuretics (furosemide, dopamine) were introduced. Daily temporary access hemodialyses were performed for 4 weeks. Subsequently peritoneal dialysis was started for 2 weeks. Despite the appropriate TPN glucose blood levels were unexpectedly high from first days from admission (200-330 mg%). Intensive intravenous insulin therapy was performed for 50 days. The child was discharged after 72 days with moderate renal function impairment (blood urea-53 mg%, creatinine-1,2 mg%), mild hypertension and proteinuria. Additional factor prone to thrombotic events was the 4G/4G genotype responsible for increased PAI-1 blood concentration, which may result in intensified fibrinolysis inhibition. Diabetes mellitus as a rare immunological complication of haemolytic uremic syndrome was suspected on the following evidence: positive anti-GAD antibodies (ELISA), elevated levels of glycosylated haemoglobin A1c, three-fold reduction of blood C-peptide concentration, negative family history for diabetes. After 12 y of follow up glucose and C

  3. Pericardial tamponade complicated by interventional management for Budd-Chiari syndrome: clinical analysis and treatment

    International Nuclear Information System (INIS)

    Zhang Luxi; Zu Maoheng; Wu Jinping; Xu Hao; Jiao Xudong; Chen Zhengkan

    2011-01-01

    Objective: To discuss the cases and treatment of pericardial tamponade (PT) occurred in the interventional management for Budd-Chiari syndrome (BCS). Methods: During the period from 1990 to 2006, interventional treatment was performed in 812 patients with BCS. Pericardial tamponade occurred in nine patients during the period of interventional treatment. The clinical data, including angiographic findings, clinical symptoms, management and outcomes, of the nine patients were retrospectively analyzed. The possible causes of pericardial tamponade were discussed. Results: Of the nine patients occurring pericardial tamponade, successful treatment was obtained in eight and death occurred in one. The lesions of BCS in the nine cases included inferior vena cava obstruction type (n=7), hepatic venous obstruction type (n=1) and mixed type (n=1). Pericardial tamponade was caused by mistakenly puncturing into pericardium (n=5), mistakenly puncturing together with laceration of pericardium by balloon (n=3), and breaking of pericardium by displaced stent (n=1). Conventional pericardicentesis was employed in one case, surgery was carried out in three cases, and infra-xiphoid catheterization and drainage using Seldinger technique was performed in two cases. Conservative treatment was adopted in one case and aspiration through the wrongly inserted catheter was tried in one case. In the remaining one case, aspiration through the wrongly inserted catheter together with infra-xiphoid catheterization and drainage by using Seldinger technique was carried out. Conclusion: The pericardial tamponade is an severe complication occurred in the interventional management for Budd-Chiari syndrome, although it is rarely seen. Preoperative prevention, prompt detection and rational treatment are the keys avoid serious consequences. (authors)

  4. Identification of a Monoclonal Antibody That Attenuates Antiphospholipid Syndrome-Related Pregnancy Complications and Thrombosis

    Science.gov (United States)

    Mineo, Chieko; Lanier, Lane; Jung, Eunjeong; Sengupta, Samarpita; Ulrich, Victoria; Sacharidou, Anastasia; Tarango, Cristina; Osunbunmi, Olutoye; Shen, Yu-Min; Salmon, Jane E.; Brekken, Rolf A.; Huang, Xianming; Shaul, Philip W.

    2016-01-01

    In the antiphospholipid syndrome (APS), patients produce antiphospholipid antibodies (aPL) that promote thrombosis and adverse pregnancy outcomes. Current therapy with anticoagulation is only partially effective and associated with multiple complications. We previously discovered that aPL recognition of cell surface β2-glycoprotein I (β2-GPI) initiates apolipoprotein E receptor 2 (apoER2)-dependent signaling in endothelial cells and in placental trophoblasts that ultimately promotes thrombosis and fetal loss, respectively. Here we sought to identify a monoclonal antibody (mAb) to β2-GPI that negates aPL-induced processes in cell culture and APS disease endpoints in mice. In a screen measuring endothelial NO synthase (eNOS) activity in cultured endothelial cells, we found that whereas aPL inhibit eNOS, the mAb 1N11 does not, and instead 1N11 prevents aPL action. Coimmunoprecipitation studies revealed that 1N11 decreases pathogenic antibody binding to β2-GPI, and it blocks aPL-induced complex formation between β2-GPI and apoER2. 1N11 also prevents aPL antagonism of endothelial cell migration, and in mice it reverses the impairment in reendothelialization caused by aPL, which underlies the non-thrombotic vascular occlusion provoked by disease-causing antibodies. In addition, aPL inhibition of trophoblast proliferation and migration is negated by 1N11, and the more than 6-fold increase in fetal resorption caused by aPL in pregnant mice is prevented by 1N11. Furthermore, the promotion of thrombosis by aPL is negated by 1N11. Thus, 1N11 has been identified as an mAb that attenuates APS-related pregnancy complications and thrombosis in mice. 1N11 may provide an efficacious, mechanism-based therapy to combat the often devastating conditions suffered by APS patients. PMID:27463336

  5. Bone marrow necrosis and fat embolism syndrome: a dreadful complication of hemoglobin sickle cell disease.

    Science.gov (United States)

    Targueta, Eduardo Pelegrineti; Hirano, André Carramenha de Góes; de Campos, Fernando Peixoto Ferraz; Martines, João Augusto Dos Santos; Lovisolo, Silvana Maria; Felipe-Silva, Aloisio

    2017-01-01

    Sickle cell disease encompasses a wide range of genotypic presentation with particular clinical features. The entity affects millions of people, particularly those whose ancestors came from sub-Saharan Africa and other countries in the Western Hemisphere, Saudi Arabia, and India. Currently, the high frequency of S and C genes reflects natural selection through the protection of heterozygotes against severe malaria, the high frequency of consanguineous marriages, improvement of some public health policies and the nutritional standards in the poorer countries where newborns are now living long enough to present for diagnosis and management. Although there is a high burden of the disease, in many countries, the new-born sickle cell screening test is being performed and is rendering an early diagnosis; however, it is still difficult for sickle cell patients to find proper treatment and adequate follow-up. Moreover, in many countries, patients are neither aware of their diagnosis nor the care they should receive to prevent complications; also, they do not receive adequate genetic counseling. Hemoglobin SC (HbSC) disease is the most frequent double sickle cell heterozygosis found in Brazil. The clinical course tends to be more benign with fewer hospitalizations compared with double homozygotic SS patients. However, HbSC patients may present severe complications with a fatal outcome. We report the case of a 36-year-old man who presented to the emergency care facility with symptoms consistent with the diagnosis of sickling crisis. The outcome was unfavorable and death occurred just hours after admission. The autopsy revealed a generalized vaso-occlusive crisis by sickled red cells, bone marrow necrosis, and fat embolism syndrome.

  6. Case series of thromboembolic complications in childhood nephrotic syndrome: Hacettepe experience.

    Science.gov (United States)

    Tavil, Betul; Kara, Fehime; Topaloglu, Rezan; Aytac, Selin; Unal, Sule; Kuskonmaz, Baris; Cetin, Mualla; Besbas, Nesrin; Gumruk, Fatma

    2015-06-01

    The aim of this study was to describe the incidence and contributory risk factors for thromboembolic complications in children with nephrotic syndrome (NS) and thrombosis. Among 188 children with the diagnosis of NS (80 girls; mean age: 12.6 ± 5.4 years) followed up in our hospital for the last 5 years, 17 (9.0 %) children (16 boys) identified as having thromboembolic complications. All 17 children with NS and thrombosis were screened for laboratory risk factors for thrombosis. The diagnosis was confirmed by cranial magnetic resonance imaging, doppler ultrasonography, and echocardiography. Among 17 children with thrombosis, 14 (82.3 %) were found to have focal segmental glomerulosclerosis (FSGS) as underlying pathology by renal biopsy. The mean age of the thrombotic children was 4.5 ± 3.2 years at the diagnosis of NS and that was 7.1 ± 4.9 years at the time of thrombosis. The mean time from NS diagnosis to the first thrombosis development was 2.6 ± 2.3 years. Thrombosis occurred during the first year of NS in 9/17 (52.9 %) children. Most of the children (88.2 %) had venous thrombosis. Among the screened risk factors, high factor VIII level (64.7 %) was the leading factor followed by decreased antithrombin III level (29.4 %). Furthermore, 4 children had central venous catheters and 2 had infection as clinical risk factors for thrombosis. In this case series, subtype of FSGS, active disease state of NS, central venous catheters, and some inherited and acquired thrombotic risk factors have been identified as contributory factors for the development of thrombosis in children with NS.

  7. Acute ventilatory failure complicating obesity hypoventilation: update on a 'critical care syndrome'.

    Science.gov (United States)

    BaHammam, Ahmed

    2010-11-01

    Obesity can result in serious complications, including obesity hypoventilation syndrome (OHS). OHS patients may present with acute-on-chronic ventilatory failure, necessitating acute care management. The purpose of this review is to discuss the recent literature on acute ventilatory failure in OHS patients. Obese persons can develop acute hypercapnic respiratory failure and sleep hypoventilation due to disorders in lung mechanics, ventilatory drive, and neurohormonal and neuromodulators of breathing. Although there are no clearly defined predictors for OHS patients who are likely to develop acute hypercapnic respiratory failure, most such patients are middle-aged (mid-50s), morbidly obese, and have daytime hypercapnia, hypoxemia, and low serum pH values. Immediate ventilatory support, without sleep study confirmation, is necessary in most such patients. Patients with respiratory acidemia (pH care unit monitoring. Noninvasive application of bilevel positive airway pressure therapy is the recommended initial ventilatory support under close monitoring. Prompt initiation of noninvasive positive pressure ventilation reduces the need for invasive mechanical ventilation and rapidly improves the levels of blood gases. Obese patients with sleep hypoventilation have an increased risk of acute hypercapnic respiratory failure. Early diagnosis and implementation of noninvasive positive pressure ventilation is recommended for these patients.

  8. Vestibular rehabilitation following surgical repair for Superior Canal Dehiscence Syndrome: A complicated case report.

    Science.gov (United States)

    Carender, Wendy J; Grzesiak, Melissa

    2018-02-01

    Superior Canal Dehiscence Syndrome (SCDS) causes auditory and vestibular symptoms. Following surgical repair of the dehiscence, patients often experience dizziness and imbalance. This case report describes a postoperative vestibular exercise program, focusing on the principles of central compensation and habituation, and how it was modified for a patient with delayed progress secondary to strabismus and visual vertigo. A 63-year-old male with history of strabismus eye surgery, right hearing loss, aural fullness, and sensitivity to loud sounds was referred for vestibular rehabilitation (VR). He was seen for one preoperative and six postoperative PT visits over eight months. Outcome measures two weeks postoperative were as follows: Dizziness Handicap Inventory (DHI) 38/100; Timed Up & Go (TUG) 9.92 seconds; Dynamic Gait Index (DGI) 16/24; and a 3-line difference in Dynamic Visual Acuity (DVA). Improved outcomes at discharge included: DHI 18/100; TUG 6.87 seconds; DGI 23/24; and 1-line difference in DVA. He was able to return to work and previously enjoyed recreational activities. Postoperative vestibular rehabilitation programs are functionally and symptomatically beneficial following surgical repair for SCDS. Deviations from expected recovery should be addressed to achieve optimal outcomes as demonstrated in this complicated case report.

  9. Improvement of overlapping hidradenitis suppurativa and ankylosing spondylitis after the introduction of adalimumab.

    Science.gov (United States)

    Bosnić, Dubravka; Žarković, Branimir; Barešić, Marko; Zarkovic, Maja; Anić, Branimir

    2016-01-01

    Hidradenitis suppurativa is a chronic inflammatory disorder characterized by occlusion of the follicular pilosebaceous units of the skin. The treatment options are sometimes very limited and unpleasant odor and abundant drainage complicate the disease. Ankylosing spondylitis is a form of seronegative spondyloarthritis with predominantly axial but also peripheral joint involvement. Both of the conditions lower the patient's quality of life and affect everyday activities. We describe a 39-year-old male patient with both diseases treated with different medications with only a modest result. After the initiation of a tumor necrosis factor α (TNF-α) inhibitor (adalimumab) the patient experienced first the musculoskeletal and later on the skin improvement. The introduction of TNF-α inhibitors should be considered early in the treatment of overlapping hidradenitis suppurativa and the spondyloarthritis spectrum of conditions. Available medical data confirm the positive results and beneficial effect on disease course, activity and, most importantly, quality of life.

  10. Increased serum levels of hyaluronic acid in pregnancies complicated by preeclampsia or hemolysis, elevated liver enzymes, and low platelets syndrome.

    Science.gov (United States)

    Osmers, R G; Schütz, E; Diedrich, F; Wehry, B; Krauss, T; Oellerich, M; Kuhn, W

    1998-02-01

    Fifteen percent of patients who later have hemolysis, elevated liver enzymes, and low platelets syndrome develop initially have nonspecific symptoms. Early diagnosis could ensure adequate obstetric management; however, prognostic biochemical tests are lacking. We hypothesized that elevated hyaluronic acid serum levels might be an early indicator of hemolysis, elevated liver enzymes, and low platelets syndrome because it is known to be a sensitive marker of liver cell function. Hyaluronic acid in serum was measured in patients with normal pregnancies (n = 109) and in those patients with pregnancies complicated by preeclampsia (n = 14) or hemolysis, elevated liver enzymes, and low platelets syndrome (n = 11). A significant increase in hyaluronic acid serum concentrations was observed in patients with hemolysis, elevated liver enzymes, and low platelets syndrome or with preeclampsia (p hyaluronic acid serum levels in hemolysis, elevated liver enzymes, and low platelets syndrome correlated with the clinical severity of the individual course of disease as measured by intensive care unit time (r = 0.72; p hyaluronic acid in preeclampsia and hemolysis, elevated liver enzymes, and low platelets syndrome are significantly elevated and might play an important diagnostic and prognostic role in patients with hemolysis, elevated liver enzymes, and low platelets syndrome.

  11. Clinical correlates of complicated grief among individuals with acute coronary syndromes

    Directory of Open Access Journals (Sweden)

    Pini S

    2015-10-01

    Full Text Available Stefano Pini,1 Camilla Gesi,1 Marianna Abelli,1 Alessandra Cardini,1 Lisa Lari,1 Francesca Felice,2 Rossella Di Stefano,2 Gianfranco Mazzotta,3 Francesco Bovenzi,4 Daniele Bertoli,5 Lucia Borelli,4 Paola Michi,1 Claudia Oligeri,3 Alberto Balbarini,2 Vijaya Manicavasagar6 1Department of Clinical and Experimental Medicine, Psychiatry Sector, University of Pisa, Pisa, 2Department of Surgical, Medical, Molecular and Critical Area Pathology, University of Pisa, Pisa, 3Unit of Cardiology, Ospedale Sant’Andrea, La Spezia, 4Cardio-Respiratory Department, Ospedale Campo di Marte, Lucca, 5Unit of Cardiology, Ospedale San Bartolomeo, Sarzana (SP, Italy; 6Black Dog Institute, University of New South Wales, Sydney, NSW, Australia Objective: The study aimed at exploring bereavement and complicated grief (CG symptoms among subjects without a history of coronary heart disease (CHD at the time of a first acute coronary syndrome (ACS and to evaluate the relationship of CG symptoms and ACS. Method: Overall, 149 subjects with ACS (namely, acute myocardial infarct with or without ST-segment elevation or unstable angina, with no previous history of CHD, admitted to three cardiac intensive care units were included and evaluated by the Structured Clinical Interview for Complicated Grief (SCI-CG, Hamilton Depression Rating Scale, Hamilton Anxiety Rating Scale, and the 36-item Short-Form Health Survey (MOS-SF-36. Results: Of the total sample of 149 subjects with ACS, 118 (79.2% met criteria for DSM-5 persistent complex bereavement disorder. Among these, subjects who lost a partner, child, or sibling were older (P=0.008, less likely to be working (P=0.032, and more likely to be suffering from hypertension (P=0.021, returned higher scores on the SCI-CG (P=0.001 and developed the index ACS more frequently between 12 and 48 months after the death than those who lost a parent or another relative (P≤0.0001. The occurrence of ACS 12–48 months (P=0.019 after the loss was

  12. Os peroneum friction syndrome complicated by sesamoid fatigue fracture: a new radiological diagnosis? Case report and literature review.

    Science.gov (United States)

    Bashir, Waseem A; Lewis, Steve; Cullen, Nicholas; Connell, David A

    2009-02-01

    Injuries to the peroneal tendons are relatively common worldwide but tendon rupture without significant trauma is uncommon. Ankle mechanics can be seriously affected by disruption of one or both of the peroneal tendons although complete rupture can also remain asymptomatic. Accessory ossicles are sesamoid bones and are common findings in routine radiology of the foot and ankle. Although in the vast majority these "os" are normal variants of anatomy, they can lead to painful syndromes and suffer fractures and even undergo degenerative changes in response to overuse and trauma. Although similar syndromes have been discussed in the surgical literature, there is a lack of literature describing the use of modern imaging in the accurate diagnosis and its subsequent assistance towards appropriate management of os peroneum friction syndrome complicated by sesamoid fatigue syndrome. This article presents the plain film, sonographic and magnetic resonance imaging findings in a case of os peroneum friction syndrome complicated by a sesamoid fatigue fracture as well as reviewing the pertinent literature.

  13. Os peroneum friction syndrome complicated by sesamoid fatigue fracture: a new radiological diagnosis? Case report and literature review

    International Nuclear Information System (INIS)

    Bashir, Waseem A.; Connell, David A.; Lewis, Steve; Cullen, Nicholas

    2009-01-01

    Injuries to the peroneal tendons are relatively common worldwide but tendon rupture without significant trauma is uncommon. Ankle mechanics can be seriously affected by disruption of one or both of the peroneal tendons although complete rupture can also remain asymptomatic. Accessory ossicles are sesamoid bones and are common findings in routine radiology of the foot and ankle. Although in the vast majority these ''os'' are normal variants of anatomy, they can lead to painful syndromes and suffer fractures and even undergo degenerative changes in response to overuse and trauma. Although similar syndromes have been discussed in the surgical literature, there is a lack of literature describing the use of modern imaging in the accurate diagnosis and its subsequent assistance towards appropriate management of os peroneum friction syndrome complicated by sesamoid fatigue syndrome. This article presents the plain film, sonographic and magnetic resonance imaging findings in a case of os peroneum friction syndrome complicated by a sesamoid fatigue fracture as well as reviewing the pertinent literature. (orig.)

  14. Os peroneum friction syndrome complicated by sesamoid fatigue fracture: a new radiological diagnosis? Case report and literature review

    Energy Technology Data Exchange (ETDEWEB)

    Bashir, Waseem A.; Connell, David A. [Royal National Orthopaedic Hospital NHS Trust, Department of Radiology, London, Middlesex (United Kingdom); Lewis, Steve [Craven Cottage, Fulham Football Club, London (United Kingdom); Cullen, Nicholas [The Royal National Orthopaedic Hospital NHS Trust, Department of Orthopaedics, London, Middlesex (United Kingdom)

    2009-02-15

    Injuries to the peroneal tendons are relatively common worldwide but tendon rupture without significant trauma is uncommon. Ankle mechanics can be seriously affected by disruption of one or both of the peroneal tendons although complete rupture can also remain asymptomatic. Accessory ossicles are sesamoid bones and are common findings in routine radiology of the foot and ankle. Although in the vast majority these ''os'' are normal variants of anatomy, they can lead to painful syndromes and suffer fractures and even undergo degenerative changes in response to overuse and trauma. Although similar syndromes have been discussed in the surgical literature, there is a lack of literature describing the use of modern imaging in the accurate diagnosis and its subsequent assistance towards appropriate management of os peroneum friction syndrome complicated by sesamoid fatigue syndrome. This article presents the plain film, sonographic and magnetic resonance imaging findings in a case of os peroneum friction syndrome complicated by a sesamoid fatigue fracture as well as reviewing the pertinent literature. (orig.)

  15. Complicated Onychomycosis in Patients with Metabolic Syndrome: Clinical Aspects of Deviation in the Laboratory Signs of Endothelial Dysfunction and Polyhypovitaminosis

    Directory of Open Access Journals (Sweden)

    O.N. Nadashkevich

    2015-08-01

    Full Text Available The study involved 93 patients aged 52 to 85 years with metabolic syndrome and concomitant complicated fungal nail infection. In all patients we have measured the levels of nitric oxide and endothelin-1. The content of B1, B2, PP, B6 and C vitamins in the blood, and urine excretion of derivatives before and during the treatment of patients with destructive onychomycosis were studied. It is noted that in the majority of patients (aged over 50 years with polyоnychomycosis and comorbid underlying metabolic syndrome has endothelial dysfunction and multivitamin deficiency that requires correction.

  16. Embryonic natural orifice transluminal endoscopic surgery in the treatment of severe acute pancreatitis complicated by abdominal compartment syndrome.

    Science.gov (United States)

    Zhu, Hui-Ming; Guo, Shao-Qing; Liao, Xiu-Min; Zhang, Li; Cai, Li

    2015-01-01

    The study aimed to estimate the value of embryonal natural orifice transluminal endoscopic surgery (ENOTES) in treating severe acute pancreatitis (SAP) complicated with abdominal compartment syndrome (ACS). The patients, who were randomized into an ENOTES group and an operative group, underwent ENOTES and laparotomy, respectively. The results and complications of the two groups were compared. Enterocinesia was observed earlier in the ENOTES group than in the operative group. Acute Physiology and Chronic Health Evaluation II (APACHE II) score of patients in the ENOTES group was lower than that of the operative group on the 1st, 3rd and 5th post-operative day (Pcomplications and mortality between the two groups (Pcomplications.

  17. Yellow Fever Vaccination of a Primary Vaccinee During Adalimumab Therapy.

    Science.gov (United States)

    Nash, Esther R; Brand, Myron; Chalkias, Spyridon

    2015-01-01

    In this case report, we describe a 63-year-old female with Crohn's disease since age 16 years, and on adalimumab therapy, who inadvertently received a yellow fever vaccine (YFV) 4 days before her next dose of adalimumab. She had never received YFV. Her next dose of tumor necrosis factor (TNF) antagonist was held. She did not report any adverse effects referable to the vaccine. Reverse transcriptase-polymerase chain reaction (RT-PCR) for yellow fever (YF) viral RNA on days 12 and 18 postvaccination was negative. Neutralizing antibody to YF virus vaccine was immunoprotective on day 18 following vaccination, which further increased by day 26. A neutralizing antibody obtained 2 years following vaccination also remained immunoprotective. © 2015 International Society of Travel Medicine.

  18. Patients with a high risk for obstructive sleep apnea syndrome: postoperative respiratory complications.

    Science.gov (United States)

    Pereira, H; Xará, D; Mendonça, J; Santos, A; Abelha, F J

    2013-01-01

    STOP-BANG score (snore; tired; observed apnea; arterial pressure; body mass index; age; neck circumference and gender) can predict the risk of a patient having Obstructive Syndrome Apnea (OSA). The aim of this study was to evaluate the incidence STOP-BANG score≥3, in surgical patients admitted to the Post-Anesthesia Care Unit (PACU). Observational, prospective study conducted in a post-anesthesia care unit (PACU) during three weeks (2011). The study population consisted of adult patients after noncardiac and non-neurological surgery. Patients were classified as high risk of OSA (HR-OSA) if STOP-BANG score≥3 and Low-risk of OSA (LR-OSA) if STOP-BANG score<3 (LR-OSA). Patient demographics, intraoperative and postoperative data were collected. Patient characteristics were compared using Mann-Whitney U-test, t-test for independent groups, and chi-square or Fisher's exact test. A total of 357 patients were admitted to PACU; 340 met the inclusion criteria. 179 (52%) were considered HR-OSA. These patients were older, more likely to be masculine, had higher BMI, higher ASA physical status, higher incidence of ischemic heart disease, heart failure, hypertension, dyslipidemia and underwent more frequently insulin treatment for diabetes. These patients had more frequently mild/moderated hypoxia in the PACU (9% vs. 3%, p=0.012) and had a higher incidence of residual neuromuscular blockade (NMB) (20% vs. 16%, p=0.035). Patients with HR-OSA had a longer hospital stay. Patients with HR-OSA had an important incidence among patients scheduled for surgery in our hospital. These patients had more co-morbidities and were more prone to post-operative complications. Copyright © 2012 Sociedade Portuguesa de Pneumologia. Published by Elsevier España. All rights reserved.

  19. Systemic inflammation: a key factor in the pathogenesis of cardiovascular complications in obstructive sleep apnoea syndrome?

    LENUS (Irish Health Repository)

    Ryan, S

    2012-02-01

    Obstructive sleep apnoea syndrome (OSAS) is a highly prevalent disease and is recognised as a major public health burden. Large-scale epidemiological studies have demonstrated an independent relationship between OSAS and various cardiovascular disorders. The pathogenesis of cardiovascular complications in OSAS is not completely understood but a multifactorial aetiology is likely. Inflammatory processes have emerged as critical in the pathogenesis of atherosclerosis at all stages of atheroma formation. Increased levels of various circulating markers of inflammation including tumour necrosis factor alpha (TNFalpha), interleukin 6 (IL6), IL-8 and C-reactive protein (CRP) have been reported as associated with future cardiovascular risk. There is increasing evidence of elevated inflammatory markers in OSAS with a significant fall after effective treatment with continuous positive airway pressure. This evidence is particularly strong for TNFalpha, whereas studies on IL6 and CRP have yielded conflicting results possibly due to the confounding effects of obesity. Cell culture and animal studies have significantly contributed to our understanding of the underlying mechanisms of the association between OSAS and inflammation. Intermittent hypoxia, the hallmark of OSAS, results in activation of pro-inflammatory transcription factors such as nuclear factor kappa B (NF-kappaB) and activator protein (AP)-1. These promote activation of various inflammatory cells, particularly lymphocytes and monocytes, with the downstream consequence of expression of pro-inflammatory mediators that may lead to endothelial dysfunction. This review provides a critical analysis of the current evidence for an association between OSAS, inflammation and cardiovascular disease, discusses basic mechanisms that may be responsible for this association and proposes future research possibilities.

  20. Delayed recognition of Wolfram syndrome frequently misdiagnosed as type 1 diabetes with early chronic complications.

    Science.gov (United States)

    Zmyslowska, A; Borowiec, M; Fichna, P; Iwaniszewska, B; Majkowska, L; Pietrzak, I; Szalecki, M; Szypowska, A; Mlynarski, W

    2014-01-01

    Improvements in diagnostic methods and greater genetic awareness have brought remarkable progress in the recognition of monogenic forms of diabetes, including Wolfram syndrome (WFS). WFS is diagnosed based on clinical criteria of coexistence of diabetes mellitus and optic atrophy, and confirmed by molecular analysis; however, the condition is still sometimes misdiagnosed. To begin to understand the reasons for misdiagnosis, we conducted a retrospective analysis of WFS patients who were originally misdiagnosed. The medical histories of 13 pediatric patients with clinical misdiagnosis of type 1 diabetes and early chronic complications made in the years 1995-2010 and who were subsequently correctly diagnosed with WFS based on genetic testing in 2008-2011 were analyzed. The average age of the patients at diabetes onset was 5 (4.4-6.3) years, and the mean HbA1c level at diagnosis was 9.1±2.3%. Initially, all of these patients were treated as having type 1 diabetes with progressive visual impairment despite good metabolic control (mean HbA1c 7.5±1.3%). Diagnosis of optic atrophy was made at an average age of 9 (5.9-11.5) years, which corresponds to 4 years after diabetes recognition (p=0.002). At the time of genetic analysis, the average age of the patients was 16 (12-18.7) years, which corresponds to 7 years after recognition of coexistence of diabetes mellitus and optic atrophy (p=0.007). Delays of at least 7 years occurred before recognition of WFS among a cohort of pediatric patients with diabetes. All patients with WFS were primarily misdiagnosed as having type 1 diabetes. © J. A. Barth Verlag in Georg Thieme Verlag KG Stuttgart · New York.

  1. Thoracic outlet syndrome after the Nuss procedure for pectus excavatum: Is it a rare complication?

    Science.gov (United States)

    Nagasao, Tomohisa; Morotomi, Tadaaki; Kuriyama, Motone; Kogure, Tetsukuni; Kudo, Hirro; Hamamoto, Yusuke; Tamai, Motoki

    2017-10-01

    The present study aims to elucidate the frequency of thoracic outlet syndrome after the Nuss procedure for pectus excavatum and the conditions in which thoracic outlet syndrome is likely to develop. A retrospective study including 85 pectus excavatum patients (58 males and 27 females) was conducted. Thoracic outlet syndrome was defined as a condition in which the patient has numbness, lassitude, or pain of the upper limbs at rest or during motion of the upper limbs. The frequency of the thus-defined thoracic outlet syndrome was evaluated in 85 patients. Age, sex, Haller indices, and the positions of the correction bars were compared between the patients who developed thoracic outlet syndrome and those who did not. Preadolescent patients (18 out of 85) did not develop postoperative thoracic outlet syndrome. In total, 15.2% of adult male patients (7 out of 46) and 33% of adult female patients (7 out of 21) developed postoperative thoracic outlet syndrome. For both male and female groups, Haller indices were significantly greater for patients who had postoperative thoracic outlet syndrome than for those who did not. Correction bars were generally placed at higher intercostal spaces in patients who developed postoperative thoracic outlet syndrome than in those who did not. A considerable percentage of adult patients develop thoracic outlet syndrome after the Nuss procedure for pectus excavatum. Maturity of the thoracic wall, femininity, severity of the deformity (represented by greater Haller indices), and placement of correction bars at superior intercostal spaces are risk factors for postoperative thoracic outlet syndrome. Copyright © 2017 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.

  2. A case of Mallory-Weiss syndrome complicating pregnancy in a patient with scleroderma.

    Science.gov (United States)

    Cho, Kyu-Hyun; Heo, Seong-Wook; Chung, Seung-Hie; Kim, Chae-Gi; Kim, Ho Gak; Choe, Jung-Yoon

    2003-12-01

    The majority of patients with scleroderma have gastrointestinal involvement, and a few experience gastrointestinal hemorrhage, however, gastrointestinal hemorrhage due to Mallory-Weiss syndrome is very rare. We report upon a 24-year-old pregnant woman with scleroderma who had gastrointestinal hemorrhage due to Mallory-Weiss syndrome.

  3. A Case of Mallory-Weiss Syndrome Complicating Pregnancy in a Patient with Scleroderma

    OpenAIRE

    Cho, Kyu-Hyun; Heo, Seong-Wook; Chung, Seung-Hie; Kim, Chae-Gi; Kim, Ho Gak; Choe, Jung-Yoon

    2003-01-01

    The majority of patients with scleroderma have gastrointestinal involvement 1) , and a few experience gastrointestinal hemorrhage, however, gastrointestinal hemorrhage due to Mallory-Weiss syndrome is very rare 2,3). We report upon a 24-year-old pregnant woman with scleroderma who had gastrointestinal hemorrhage due to Mallory-Weiss syndrome.

  4. Severe Renal Hemorrhage in a Pregnant Woman Complicated with Antiphospholipid Syndrome: A Case Report

    Directory of Open Access Journals (Sweden)

    Shohei Kawaguchi

    2011-01-01

    Full Text Available Antiphospholipid syndrome is a systemic autoimmune disease with thrombotic tendency. Consensus guidelines for pregnancy with antiphospholipid syndrome recommend low-dose aspirin combined with unfractionated or low-molecular-weight heparin because antiphospholipid syndrome causes habitual abortion. We report a 36-year-old pregnant woman diagnosed with antiphospholipid syndrome receiving anticoagulation treatment. The patient developed left abdominal pain and gross hematuria at week 20 of pregnancy. An initial diagnosis of left ureteral calculus was made. Subsequently abdominal-pelvic computed tomography was required for diagnosis because of the appearance of severe contralateral pain. Computed tomography revealed serious renal hemorrhage, and ureteral stent placement and pain control by patient-controlled analgesia were required. After treatment, continuance of pregnancy was possible and vaginal delivery was performed safely. This is the first case report of serious renal hemorrhage in a pregnant woman with antiphospholipid syndrome receiving anticoagulation treatment and is an instructive case for urological and obstetrical practitioners.

  5. Efficacy of Adalimumab as a long term maintenance therapy in ulcerative colitis.

    LENUS (Irish Health Repository)

    McDermott, Edel

    2013-03-01

    Adalimumab is a recombinant human IgG1 monoclonal antibody to TNF-alpha. There are limited data with regard to its efficacy in ulcerative colitis. We report experience of adalimumab in ulcerative colitis in a single centre with a focus on the ability of this agent to maintain response and avoid colectomy in the medium to long-term.

  6. A Rare Side Effect due to TNF-Alpha Blocking Agent: Acute Pleuropericarditis with Adalimumab

    Directory of Open Access Journals (Sweden)

    Hakan Ozkan

    2013-01-01

    Full Text Available Tumor necrosis factor-alpha antagonism is an important treatment strategy in patients with rheumatoid arthritis, psoriatic arthritis, vasculitis, and ankylosing spondylitis. Adalimumab is one of the well-known tumor necrosis factor-alpha blocking agents. There are several side effects reported in patients with adalimumab therapy. Cardiac side effects of adalimumab are rare. Only a few cardiac side effects were reported. A 61-year-old man treated with adalimumab for the last 6 months due to psoriatic arthritis presented with typically acute pleuropericarditis. Chest X-ray and echocardiography demonstrated marked pericardial effusion. Patient was successfully evaluated for the etiology of acute pleuro-pericarditis. Every etiology was excluded except the usage of adalimumab. Adalimumab was discontinued, and patient was treated with 1200 mg of ibuprofen daily. Control chest X-ray and echocardiography after three weeks demonstrated complete resolution of both pleural and pericardial effusions. This case clearly demonstrated the acute onset of pericarditis with adalimumab usage. Acute pericarditis and pericardial effusion should be kept in mind in patients with adalimumab treatment.

  7. Primary biliary cirrhosis complicated by transverse myelitis in a patient without Sjögren's syndrome

    Directory of Open Access Journals (Sweden)

    Micheli A

    2005-01-01

    Full Text Available Transverse myelitis is an acute inflammatory process, affecting one or more segments of the spinal cord. Its association with primary biliary cirrhosis has been documented in only four cases - all along with Sjögren's syndrome. Herein, we report for the first time, a patient who developed recurrent acute transverse myelitis in association with primary biliary cirrhosis without any clinical or histological indication of Sjögren's syndrome. A 42-year-old woman with primary biliary cirrhosis developed acute onset quadriparesis and urinary retention. Diagnostic evaluation excluded the presence of Sjögren's syndrome, other autoimmune syndromes, infections and multiple sclerosis. Magnetic resonance imaging of the spinal cord disclosed signal intensity abnormalities from C1 to T2 after gadolinium enhancement. As diagnosis of acute transverse myelitis was prominent, the patient was treated with intravenous methylprednisolone. The patient had a fair outcome despite an early recurrence of the symptoms after treatment withdrawal.

  8. A case of Alagille syndrome complicated by intraocular lens subluxation and rhegmatogenous retinal detachment

    Science.gov (United States)

    Fukumoto, Masanori; Ikeda, Tsunehiko; Sugiyama, Tetsuya; Ueki, Mari; Sato, Takaki; Ishizaki, Eisuke

    2013-01-01

    This case report describes a case of Alagille syndrome with developing intraocular lens subluxation and rhegmatogenous retinal detachment 4 years after cataract surgery. A 15-year-old female patient with Alagille syndrome-associated cataracts in both eyes underwent phacoemulsification aspiration and intraocular lens implantation. Four years postoperative, intraocular lens subluxation developed in her left eye. For treatment, extraction of the dislocated intraocular lens, anterior vitrectomy, and intraocular lens fixation was performed. Three weeks later, the patient developed rhegmatogenous retinal detachment, which was well-treated by pars plana vitrectomy. Cataract surgery needs to be performed carefully in patients with Alagille syndrome due to the weakness of the zonule of Zinn. Careful postoperative observation is necessary for patients with Alagille syndrome who have undergone intraocular surgery in order to facilitate early detection of a possible rhegmatogenous retinal detachment. PMID:23898221

  9. [Epidural pharmacotherapy in the treatment of the pain syndrome in osteochondrosis complicated by intervertebral discs protrusion of lumbar part of the vertebral column].

    Science.gov (United States)

    Khizhniak, M V; Priĭmak, E V

    2013-03-01

    Experience of application of intralaminar epidural pharmacotherapy for the treatment of the pain syndrome in the patients, suffering osteochondrosis of lumbar portion of vertebral column, complicated by intervertebral discs protrusion, was summarized. The method introduction permits to escape the operative intervention performance for complicated osteochondrosis.

  10. Anesthetic Implications of Emergent Cesarean Section in a Parturient with Marfan Syndrome Complicated by Ascending Aortic Aneurysm and Heart Failure

    Directory of Open Access Journals (Sweden)

    Young Sung Kim

    2014-07-01

    Full Text Available Cardiovascular comorbidities to the Marfan syndrome may induce hemodynamic instability especially in the parturients during labor or delivery. For anesthesiologists, it is challenging to maintain hemodynamic stability during Cesarean section in those patients with Marfan syndrome. Remifentanil is an ultra-short-acting opioid with rapid onset and offset of action which provides cardiovascular stability during surgery. Together with remifentanil, the use of a laryngeal mask airway can reduce the risk of hypertensive response followed by tracheal intubation. We describe the successful administration of remifentanil and application of laryngeal mask airway for emergent Cesarean section performed under general anesthesia in a patient with Marfan syndrome complicated by ascending aortic aneurysm and heart failure. The use of remifentanil (loading dose of 1 μg/kg for 1 min, 2 min before induction; thereafter continuous infusion dose of 0.1 μg/kg/min was useful to maintain hemodynamic stability of the parturient throughout the surgery without neonatal respiratory depression. Keywords: Ascending Aortic Aneurysm; Cesarean section; Laryngeal mask airway; Marfan syndrome; Remifentanil

  11. Membranoproliferative glomerulonephritis in the setting of multicentric angiofollicular lymph node hyperplasia (Castleman's disease) complicated by Evan's syndrome.

    Science.gov (United States)

    Gakiopoulou, Hariklia; Korkolopoulou, Penelope; Paraskevakou, Helen; Marinaki, Smaragdi; Voulgarelis, Michael; Stofas, Anastasios; Lelouda, Maria; Lazaris, Andreas C; Boletis, John; Patsouris, Efstratios

    2010-06-01

    Systemic Castleman's disease is a lymphoproliferative disorder with various clinical presentations and incompletely understood aetiology. The authors report on a rare case of the plasma cell variant of Castleman's disease associated with autoimmune haemolytic anaemia and autoimmune thrombocytopenia (Evan's syndrome) and complicated by mixed nephrotic-nephritic syndrome and acute renal failure due to an underlying glomerulopathy with microscopic and immunofluorescence findings suggestive of membranoproliferative glomerulonephritis (MPGN) type I. Immunocomplexed glomerulonephritis is rare in Castleman's disease, while, to the best of our knowledge, constellation of all these autoimmune phenomena is reported for the first time suggesting that apart from the putative role of VEGF and IL-6 in the pathogenesis of the disease, a more generalised immunological disturbance occurs, probably through autoantibodies induced by active polyclonal B cells raised from Castleman's disease tumour.

  12. A case of Alagille syndrome complicated by intraocular lens subluxation and rhegmatogenous retinal detachment

    OpenAIRE

    Fukumoto, Masanori; Ikeda,Tsunehiko; Sugiyama,Tetsuya; Ueki,Mari; Isizaki,Eisuke; Satou,Takaki

    2013-01-01

    Masanori Fukumoto, Tsunehiko Ikeda, Tetsuya Sugiyama, Mari Ueki, Takaki Sato, Eisuke Ishizaki Department of Ophthalmology, Osaka Medical College, Takatsuki City, Japan Abstract: This case report describes a case of Alagille syndrome with developing intraocular lens subluxation and rhegmatogenous retinal detachment 4 years after cataract surgery. A 15-year-old female patient with Alagille syndrome-associated cataracts in both eyes underwent phacoemulsification aspiration and intraocular lens ...

  13. Adalimumab for the treatment of moderate to severe hidradenitis suppurativa

    DEFF Research Database (Denmark)

    Kimball, A.B.; Kerdel, F.; Adams, D.

    2012-01-01

    with moderate to severe HS. Design: Phase 2, parallel, randomized, placebo-controlled trial consisting of a blinded 16-week period (period 1) and an open-label 36-week period (period 2). All study personnel, investigators, and patients remained blinded to treatment group throughout the study. (Clinical......Trials.gov: NCT00918255) Setting: 26 academic and private practice medical centers in the United States and Europe. Patients: 154 adult patients with moderate to severe HS who were unresponsive or intolerant to oral antibiotics. Intervention: Patients were assigned in a 1:1:1 ratio to adalimumab, 40 mg...

  14. Patients with a high risk for obstructive sleep apnea syndrome: Postoperative respiratory complications

    Directory of Open Access Journals (Sweden)

    H. Pereira

    2013-07-01

    Full Text Available Background: STOP-BANG score (snore; tired; observed apnea; arterial pressure; body mass index; age; neck circumference and gender can predict the risk of a patient having Obstructive Syndrome Apnea (OSA. The aim of this study was to evaluate the incidence STOP-BANG score ≥ 3, in surgical patients admitted to the Post-Anesthesia Care Unit (PACU. Methods: Observational, prospective study conducted in a post-anesthesia care unit (PACU during three weeks (2011. The study population consisted of adult patients after noncardiac and non-neurological surgery. Patients were classified as high risk of OSA (HR-OSA if STOP-BANG score ≥ 3 and Low-risk of OSA (LR-OSA if STOP-BANG score  3, em pacientes cirúrgicos internados na Unidade de Cuidados Pós-Anestésica (UCPA. Métodos: Estudo observacional e prospectivo conduzido numa UCPA, durante três semanas (2011. A população de estudo consistiu em doentes adultos após cirurgia não cardíaca e não neurológica. Os doentes foram considerados com alto risco de SAOS (AR-SAOS se tinham um score de STOP-BANG ≥3 e de baixo risco de SAOS (BR-SAOS se tinham score de STOP-BANG <3. Foram avaliados dados demográficos dos doentes e colhidas variáveis intraoperatórias e pós-operatórias. As características dos doentes foram comparadas através do teste de Mann-Whitney, teste t, qui-quadrado ou teste exato de Fisher. Resultados: Um total de 357 doentes foram admitidos de UCPA e 340 preencheram os critérios de inclusão. Cento e setenta e nove (52% tinham AR-SAOS. Estes doentes eram mais velhos, tinham maior probabilidade de serem do sexo masculino, tinham um Índice Massa Corporal superior, tiveram uma classificação maior no estado físico American Society Anesthesiologists, uma maior incidência de doença cardíaca isquémica, insuficiência cardíaca, hipertensão, dislipidemia e eram mais frequentemente doentes

  15. Multiple organ dysfunction syndrome, an unusual complication of heroin intoxication: a case report and review of literature.

    Science.gov (United States)

    Feng, Gang; Luo, Qiancheng; Guo, Enwei; Yao, Yulan; Yang, Feng; Zhang, Bingyu; Li, Longxuan

    2015-01-01

    Multiple organ dysfunction syndrome (MODS) has rarely been described in patients with heroin intoxication. Here, we report a rare case of MODS involving six organs, due to heroin intoxication. The patient was a 32-year-old Chinese man with severe heroin intoxication complicated by acute pulmonary edema and respiratory insufficiency, shock, myocardial damage and cardiac insufficiency, rhabdomyolysis and acute renal insufficiency, acute liver injury and hepatic insufficiency, toxic leukoencephalopathy, and hypoglycemia. He managed to survive and was discharged after 10 weeks of intensive care. The possible pathogenesis and therapeutic measures of MODS induced by heroin intoxication and some suggestions for preventing and treating severe complications of heroin intoxication, based on clinical evidence and the pertinent literature, are discussed in this report.

  16. Rituximab for childhood-onset, complicated, frequently relapsing nephrotic syndrome or steroid-dependent nephrotic syndrome: a multicentre, double-blind, randomised, placebo-controlled trial.

    Science.gov (United States)

    Iijima, Kazumoto; Sako, Mayumi; Nozu, Kandai; Mori, Rintaro; Tuchida, Nao; Kamei, Koichi; Miura, Kenichiro; Aya, Kunihiko; Nakanishi, Koichi; Ohtomo, Yoshiyuki; Takahashi, Shori; Tanaka, Ryojiro; Kaito, Hiroshi; Nakamura, Hidefumi; Ishikura, Kenji; Ito, Shuichi; Ohashi, Yasuo

    2014-10-04

    Rituximab could be an effective treatment for childhood-onset, complicated, frequently relapsing nephrotic syndrome (FRNS) and steroid-dependent nephrotic syndrome (SDNS). We investigated the efficacy and safety of rituximab in patients with high disease activity. We did a multicentre, double-blind, randomised, placebo-controlled trial at nine centres in Japan. We screened patients aged 2 years or older experiencing a relapse of FRNS or SDNS, which had originally been diagnosed as nephrotic syndrome when aged 1-18 years. Patients with complicated FRNS or SDNS who met all other criteria were eligible for inclusion after remission of the relapse at screening. We used a computer-generated sequence to randomly assign patients (1:1) to receive rituximab (375 mg/m(2)) or placebo once weekly for 4 weeks, with age, institution, treatment history, and the intervals between the previous three relapses as adjustment factors. Patients, guardians, caregivers, physicians, and individuals assessing outcomes were masked to assignments. All patients received standard steroid treatment for the relapse at screening and stopped taking immunosuppressive agents by 169 days after randomisation. Patients were followed up for 1 year. The primary endpoint was the relapse-free period. Safety endpoints were frequency and severity of adverse events. Patients who received their assigned intervention were included in analyses. This trial is registered with the University Hospital Medical Information Network clinical trials registry, number UMIN000001405. Patients were centrally registered between Nov 13, 2008, and May 19, 2010. Of 52 patients who underwent randomisation, 48 received the assigned intervention (24 were given rituximab and 24 placebo). The median relapse-free period was significantly longer in the rituximab group (267 days, 95% CI 223-374) than in the placebo group (101 days, 70-155; hazard ratio: 0·27, 0·14-0·53; pchildhood-onset, complicated FRNS and SDNS. Japanese Ministry of

  17. Cochlear implantation in Pendred syndrome and non-syndromic enlarged vestibular aqueduct – clinical challenges, surgical results, and complications

    DEFF Research Database (Denmark)

    Mey, Kristianna; Bille, Michael; Cayé-Thomasen, Per

    2016-01-01

    or on populations with mixed cochlear malformations. PS/NSEVA accounts for up to 10% of congenital SNHL, rendering this a large group of cochlear implant candidates. The abnormal inner ear anatomy of these patients may be associated with a lower surgical success rate and a higher rate of complications. STUDY DESIGN...

  18. Efficacy of Noninvasive Ventilation in a Patient with Hypercapnic Respiratory Failure Complicating Eisenmenger’s Syndrome

    Directory of Open Access Journals (Sweden)

    Ana Jaureguizar Oriol

    2017-08-01

    Full Text Available Eisenmenger’s syndrome is a severe type of congenital heart disease characterized by severe pulmonary arterial hypertension. In the cases that the pressure in pulmonary circulation exceeds the systemic pressure, there appears a right-to-left shunting of blood. Consequently, the syndrome exists hypoxemia and cyanosis. Hypercapnia is not common in these patients; however, it might coexist with hypoxemic failure if there are other restrictive pathologies associated. Meanwhile, it has been described high prevalence of sleep disorders in Down syndrome. There is no evidence about the role of noninvasive ventilation in the management of these patients. We present a 39-year-old man, suffering of Down and Eisenmenger’s syndrome with multiple cardiac decompensations and obstructive sleep apnea (OSA, who was admitted to hospital due to severe somnolence, edema, and dyspnea. We observed a hypercapnic respiratory acidosis that ameliorated with noninvasive ventilation (NIV. The patient returned home with nocturnal NIV as a new treatment, and no further admission to hospital was seen in the following two years. To our knowledge, this is the first report about the utility of NIV in Eisenmenger’s and Down syndrome patients.

  19. A case of Alagille syndrome complicated by intraocular lens subluxation and rhegmatogenous retinal detachment

    Directory of Open Access Journals (Sweden)

    Fukumoto M

    2013-07-01

    Full Text Available Masanori Fukumoto, Tsunehiko Ikeda, Tetsuya Sugiyama, Mari Ueki, Takaki Sato, Eisuke Ishizaki Department of Ophthalmology, Osaka Medical College, Takatsuki City, Japan Abstract: This case report describes a case of Alagille syndrome with developing intraocular lens subluxation and rhegmatogenous retinal detachment 4 years after cataract surgery. A 15-year-old female patient with Alagille syndrome-associated cataracts in both eyes underwent phacoemulsification aspiration and intraocular lens implantation. Four years postoperative, intraocular lens subluxation developed in her left eye. For treatment, extraction of the dislocated intraocular lens, anterior vitrectomy, and intraocular lens fixation was performed. Three weeks later, the patient developed rhegmatogenous retinal detachment, which was well-treated by pars plana vitrectomy. Cataract surgery needs to be performed carefully in patients with Alagille syndrome due to the weakness of the zonule of Zinn. Careful postoperative observation is necessary for patients with Alagille syndrome who have undergone intraocular surgery in order to facilitate early detection of a possible rhegmatogenous retinal detachment. Keywords: Alagille syndrome, cataract, retina, surgery

  20. Severe Plasmodium ovale malaria complicated by acute respiratory distress syndrome in a young Caucasian man.

    Science.gov (United States)

    D'Abramo, Alessandra; Gebremeskel Tekle, Saba; Iannetta, Marco; Scorzolini, Laura; Oliva, Alessandra; Paglia, Maria Grazia; Corpolongo, Angela; Nicastri, Emanuele

    2018-04-02

    Although Plasmodium ovale is considered the cause of only mild malaria, a case of severe malaria due to P. ovale with acute respiratory distress syndrome is reported. A 37-year old Caucasian man returning home from Angola was admitted for ovale malaria to the National Institute for Infectious Diseases Lazzaro Spallanzani in Rome, Italy. Two days after initiation of oral chloroquine treatment, an acute respiratory distress syndrome was diagnosed through chest X-ray and chest CT scan with intravenous contrast. Intravenous artesunate and oral doxycycline were started and he made a full recovery. Ovale malaria is usually considered a tropical infectious disease associated with low morbidity and mortality. However, severe disease and death have occasionally been reported. In this case clinical failure of oral chloroquine treatment with clinical progression towards acute respiratory distress syndrome is described.

  1. Gradenigo syndrome, a rare complication of the otitis media: a case report

    International Nuclear Information System (INIS)

    Aribal, S.; Incedayo, M.; Sivrioglu, A.

    2012-01-01

    Full text: 5-year-old child was admitted to our hospital's pediatrics service with the symptoms of acute otitis media. After 10 days antibiotherapy, he was readmitted with headache, diplopia and right sided retroauricular pain. In accordance with these symptomatologies, cranial MRI was performed to demonstrate the pathology and to make a further evaluation. According to clinical and MRI findings he was accepted as Gradenigo Syndrome and treated surgically. We just aimed to present the Gradenigo Syndrome's MRI findings in this case report

  2. Infliximab and adalimumab drug levels in Crohn's disease: contrasting associations with disease activity and influencing factors.

    Science.gov (United States)

    Ward, M G; Warner, B; Unsworth, N; Chuah, S-W; Brownclarke, C; Shieh, S; Parkes, M; Sanderson, J D; Arkir, Z; Reynolds, J; Gibson, P R; Irving, P M

    2017-07-01

    Discriminative drug level thresholds for disease activity endpoints in patients with Crohn's disease. have been consistently demonstrated with infliximab, but not adalimumab. To identify threshold concentrations for infliximab and adalimumab in Crohn's disease according to different disease endpoints, and factors that influence drug levels. We performed a cross-sectional service evaluation of patients receiving maintenance infliximab or adalimumab for Crohn's disease. Serum drug levels were at trough for infliximab and at any time point for adalimumab. Endpoints included Harvey-Bradshaw index, C-reactive protein and faecal calprotectin. 6-tioguanine nucleotide (TGN) concentrations were measured in patients treated with thiopurines. A total of 191 patients (96 infliximab, 95 adalimumab) were included. Differences in infliximab levels were observed for clinical (P=.081) and biochemical remission (P=.003) and faecal calprotectin normalisation (Pinfliximab dose, body mass index and colonic disease independently accounted for 31% of the variation in infliximab levels, and weekly dosing, albumin and weight accounted for 23% of variation in adalimumab levels. TGN levels did not correlate with drug levels. Infliximab drug levels are associated with the depth of response/remission in patients with Crohn's disease, but no such relationship was observed for adalimumab. More data are needed to explain the variation in drug levels. © 2017 John Wiley & Sons Ltd.

  3. Compartment syndrome as a rare complication of iloprost infusion for peripheral vascular disease.

    Science.gov (United States)

    Thomas, Gregory P; Kadam, Sameer; Mohammed, Mohammed; Andrews, Brian T E

    2011-05-01

    We report a case of acute compartment syndrome associated with the use of iloprost in the treatment of Buerger's disease. After a four-compartmental fasciotomy of the affected lower limb, the patient made a complete recovery. Copyright © 2011. Published by Elsevier Inc.

  4. Broken Heart Syndrome – An intra operative complication

    African Journals Online (AJOL)

    Zara Wani

    2017-03-09

    Mar 9, 2017 ... diomyopathy, Stress Cardiomyopathy1 is an acute reversible left ventricular dysfunction in the absence ... acute coronary syndrome with charac- teristics features of typical chest pain, pulmonary edema, ... Beta-blockers5 and intensive care is the prime mode of treatment, which provides good recovery and ...

  5. Early Disseminated Lyme Disease with Carditis Complicated by Posttreatment Lyme Disease Syndrome

    OpenAIRE

    Novak, Cheryl; Harrison, Andrew; Aucott, John

    2017-01-01

    Lyme disease is an infectious disease caused by the bacterium Borrelia burgdorferi. When untreated, infection may spread to the heart, nervous system, and joints. Cardiac involvement usually manifests as abnormalities of the conduction system and bradycardia. Treatment of Lyme disease is generally effective, with a subset of patients experiencing persistent, sometimes long-term symptoms called posttreatment Lyme disease syndrome.

  6. Obstetrical complications in dichorionic twin pregnancies in women with polycystic ovary syndrome

    DEFF Research Database (Denmark)

    Jonsdottir, Fjola; Nilas, Lisbeth; Andreasen, Kirsten R

    2017-01-01

    INTRODUCTION: Both women with polycystic ovary syndrome (PCOS) and women with twin pregnancies have increased risk of adverse pregnancy outcome. The aim of this study was to investigate the impact of PCOS and maternal androgen levels on the outcome of dichorionic twin pregnancy. MATERIAL...

  7. The prevalence of chronic diabetic complications and metabolic syndrome is not associated with maternal type 2 diabetes

    Directory of Open Access Journals (Sweden)

    R.S. Scheffel

    2008-12-01

    Full Text Available The maternal history of type 2 diabetes mellitus (DM has been reported more frequently in patients with type 2 DM than paternal history. The aim of the present study was to determine if there was an association between maternal history of DM and the presence of chronic complications or metabolic syndrome (MetS in patients with type 2 DM. A cross-sectional study was conducted with 1455 patients with type 2 DM. All outpatients with type 2 diabetes attending the endocrine clinics who fulfilled the eligibility criteria were included. Familial history of DM was determined with a questionnaire. Diabetic complications were assessed using standard procedures. The definition of MetS used was that of the World Health Organization and the National Cholesterol Education Program's Adult Treatment Panel III report criteria. Maternal history of DM was present in 469 (32.3%, absent in 713 (49.1% and unknown in 273 patients (18.7%. Paternal history of DM was positive in 255 (17.6%, negative in 927 (63.8% and unknown in 235 patients (16.1%. The frequency of microvascular chronic complications in patients with and without a positive maternal history of DM was similar: diabetic nephropathy (51.5 vs 52.5%, diabetic retinopathy (46.0 vs 41.7%, and diabetic sensory neuropathy (31.0 vs 37.1%. The prevalence of macrovascular chronic complications and MetS was also similar. Patients with type 2 DM were more likely to have a maternal than a paternal history of DM, although maternal history of DM was not associated with an increased prevalence of chronic complications or MetS.

  8. [Ogilvie syndrome with cecal perforation. A rare complication after isolated thoracic trauma. Case report and current literature review].

    Science.gov (United States)

    Meier, C; Di Lazzaro, M; Decurtins, M

    2000-01-01

    In reviewing 750 cases of acute colonic pseudo-obstruction from the literature, the most commonly associated disorders are listed and the therapeutical management is critically discussed. The authors describe an additional case of a 77-year-old male with ogilvie's syndrome following isolated thoracic trauma. Ogilvie's syndrome has been used synonymously with acute colonic pseudo-obstruction (ACPO) of the colon, first defined by Sir Heneage Ogilvie in 1948. If inappropriately managed, the massive colonic dilatation may lead to caecal ischaemia and perforation with a high mortality rate. Aetiology and pathogenesis of the syndrome are still unknown, but mostly ACPO is associated with a wide variety of medical conditions. Often it appears to be a complication after abdominal or pelvic surgery or trauma. In few cases the pseudo-obstruction is isolated and therefore truly idiopathic. Conservative treatment is indicated if the caecum is less than 12 cm in diameter. If there is a progressive increase in diameter or no improvement is seen, the colon should be decompressed without further delay. The indications for surgery are failure of conservative treatment and colonoscopy, signs for ceacal ischaemia or perforation. The choice of procedure, coecostomy or resection, is dictated by the state of the caecum. Due to a high mortality rate (up to 50%) if the caecum is perforated, an aggressive therapeutical management should be applied.

  9. Predictive value of uterine artery velocity waveforms in pregnancies complicated by systemic lupus erythematosus and the antiphospholipid syndrome.

    Science.gov (United States)

    Benifla, J L; Tchobroutsky, C; Uzan, M; Sultan, Y; Weill, B J; Laumond-Barny, S

    1992-01-01

    The objective of this study was to see if determination of uterine artery velocity waveforms between 20 and 30 weeks in lupus pregnancy and the antiphospholipid syndrome (APS) have a good predictive value for later fetal distress before labor, intrauterine growth retardation, and preeclampsia. Uterine and umbilical artery blood flow velocity waveforms were determined in 21 pregnancies complicated by systemic lupus erythematosus (SLE): 12 with antiphospholipid antibodies (aPL), 9 without aPL. We also studied 7 pregnancies with APS. This retrospective study was running from January 1st 1986 to July 31st 1991, at the Port-Royal Maternity, Paris, France. Abnormal uterine artery blood flow velocity waveforms were found in 10 out of 28 pregnancies at the first examination performed between 20 and 30 weeks gestational age. All the later adverse fetal and neonatal events were predicted by an abnormal uterine artery blood flow velocity waveform. From the 7 cases of fetal distress diagnosed during pregnancy, 6 were predicted by abnormal uterine waveforms and all of these pregnancies resulted in induced delivery before 32 weeks of gestational age. Twelve pregnancies with aPL and normal uterine artery waveforms were uncomplicated. Only 1 out of 7 pregnancies with abnormal uterine artery waveform and aPL ended without complication. Determination of uterine artery flow velocity waveform is a good adjunct to the management of pregnancies complicated by SLE or aPL. This determination has a better predictive value than the presence of aPL.

  10. Four cases of atopic dermatitis complicated by Sjögren's syndrome: link between dry skin and autoimmune anhidrosis.

    Science.gov (United States)

    Kitaba, Shun; Matsui, Saki; Iimuro, Eriko; Nishioka, Megumi; Kijima, Akiko; Umegaki, Noriko; Murota, Hiroyuki; Katayama, Ichiro

    2011-09-01

    We report four adult cases of atopic dermatitis (AD) complicated by Sjögren's syndrome (SS). The patients fulfilled diagnostic criteria for AD and SS. All cases showed persistent itchy dry skin and eczematous lesions complicated by sicca symptoms including dry eyes and dry mouth with moderate joint pain. One case manifested annular erythema and another manifested widespread discoid erythema. To investigate the underlying cause of dry skin in these cases, sweating function was evaluated using a quantitative sudomotor axon reflex test (QSART) in which the axon reflex is stimulated by acetylcholine iontophoresis. The sweating latency time was significantly prolonged in eczematous skin of AD and AD/SS compared to normal controls. Axon reflex (AXR) sweat volume was also significantly reduced in AD (normal and eczematous skin) and AD/SS (normal and eczema) compared to normal control. In contrast, the direct sweat volume of lesional or non-lesional AD skin induced by direct stimulation with acetylcholine was only slightly reduced compared to that in normal controls, but not in SS and lesional skin of AD/SS patients. These results suggest that the impaired sweat response in AD is attributable to an abnormal sudomotor axon reflex, which is accelerated and modulated when complicated by SS resulting in dry skin in the present cases.

  11. Beneficial Role of Bitter Melon Supplementation in Obesity and Related Complications in Metabolic Syndrome

    Science.gov (United States)

    Subhan, Nusrat; Rahman, Md Mahbubur; Jain, Preeti; Reza, Hasan Mahmud

    2015-01-01

    Diabetes, obesity, and metabolic syndrome are becoming epidemic both in developed and developing countries in recent years. Complementary and alternative medicines have been used since ancient era for the treatment of diabetes and cardiovascular diseases. Bitter melon is widely used as vegetables in daily food in Bangladesh and several other countries in Asia. The fruits extract of bitter melon showed strong antioxidant and hypoglycemic activities in experimental condition both in vivo and in vitro. Recent scientific evaluation of this plant extracts also showed potential therapeutic benefit in diabetes and obesity related metabolic dysfunction in experimental animals and clinical studies. These beneficial effects are mediated probably by inducing lipid and fat metabolizing gene expression and increasing the function of AMPK and PPARs, and so forth. This review will thus focus on the recent findings on beneficial effect of Momordica charantia extracts on metabolic syndrome and discuss its potential mechanism of actions. PMID:25650336

  12. Kawasaki disease shock syndrome: a rare and severe complication of Kawasaki disease.

    Science.gov (United States)

    Çakan, Mustafa; Gemici, Hakan; Aktay-Ayaz, Nuray; Keskindemirci, Gonca; Bornaun, Helen; İkizoğlu, Tarkan; Çeliker, Alpay

    2016-01-01

    Kawasaki disease is an acute systemic vasculitis that occurs most commonly in young children. It affects medium-sized muscular arteries and the coronary arteries are the predominant site of involvement. Morbidity and mortality is generally due to coronary artery aneurysms that develop during the chronic phase. Although it is well known that Kawasaki disease can cause myocarditis, tachycardia and heart failure during acute stage, Kawasaki disease shock syndrome has been recently described. It is characterized by hypotension, signs and symptoms of poor perfusion and a shock-like state. Herein we describe two cases of Kawasaki disease shock syndrome that were treated in the pediatric intensive care unit and followed a course without morbidity or mortality.

  13. Beneficial Role of Bitter Melon Supplementation in Obesity and Related Complications in Metabolic Syndrome

    Directory of Open Access Journals (Sweden)

    Md Ashraful Alam

    2015-01-01

    Full Text Available Diabetes, obesity, and metabolic syndrome are becoming epidemic both in developed and developing countries in recent years. Complementary and alternative medicines have been used since ancient era for the treatment of diabetes and cardiovascular diseases. Bitter melon is widely used as vegetables in daily food in Bangladesh and several other countries in Asia. The fruits extract of bitter melon showed strong antioxidant and hypoglycemic activities in experimental condition both in vivo and in vitro. Recent scientific evaluation of this plant extracts also showed potential therapeutic benefit in diabetes and obesity related metabolic dysfunction in experimental animals and clinical studies. These beneficial effects are mediated probably by inducing lipid and fat metabolizing gene expression and increasing the function of AMPK and PPARs, and so forth. This review will thus focus on the recent findings on beneficial effect of Momordica charantia extracts on metabolic syndrome and discuss its potential mechanism of actions.

  14. Sticky platelet syndrome: an important cause of life-threatening thrombotic complications.

    Science.gov (United States)

    Kubisz, Peter; Stanciakova, Lucia; Stasko, Jan; Dobrotova, Miroslava; Skerenova, Maria; Ivankova, Jela; Holly, Pavol

    2016-01-01

    Sticky platelet syndrome (SPS) is a prothrombotic thrombocytopathy with familial occurrence, characterized by hyperaggregability of platelets in response to adenosine diphosphate (ADP), epinephrine (EPI) or both. The syndrome has been identified in approximately 21% of unexplained arterial thrombotic episodes, regarded to be the most common thrombophilia in arterial thrombosis and 13.2% of unexplained venous thromboembolism (VTE). The relatively young age at the first manifestation, relation to fertility and pregnancy, seriousness of the symptoms, easy and effective management of the disorder indicate to the necessity to take it into account in the differential diagnosis of the underlying cause of the thrombotic event. As the various localizations of the thrombosis in SPS have been reported, its management often requires a multidisciplinary approach. This review deals with the clinical aspects of thrombophilia, its etiopathogenesis, diagnosis as well as novel advances in the treatment and outlines the challenges for the further research.

  15. Occam paradox? A variation of tapia syndrome and an unreported complication of guidewire-assisted pedicle screw insertion.

    Science.gov (United States)

    Emohare, Osa; Peterson, Erik; Slinkard, Nathaniel; Janus, Seth; Morgan, Robert

    2013-10-01

    Study Design Case report. Clinical Question The clinical aim is to report on a previously unknown association between guidewire-assisted pedicle screw insertion and neuropraxia of the recurrent laryngeal nerve (RLN), and how this may overlap with the signs of Tapia syndrome; we also report our approach to the clinical management of this patient. Methods A 17-year-old male patient with idiopathic scoliosis experienced Tapia syndrome after posterior instrumentation and arthrodesis at the level of T1-L1. After extubation, the patient had a hoarse voice and difficulty in swallowing. Imaging showed a breach in the cortex of the anterior body of T1 corresponding to the RLN on the right. Results Otolaryngological examination noted right vocal fold immobility, decreased sensation of the endolarynx, and pooling of secretions on flexible laryngoscopy that indicated right-sided cranial nerve X injury and left-sided tongue deviation. Aspiration during a modified barium swallow prompted insertion of a percutaneous endoscopic gastrostomy tube before the patient was sent home. On postoperative day 20, a barium swallow demonstrated reduced aspiration, and the patient reported complete resolution of symptoms. The feeding tube was removed, and the patient resumed a normal diet 1 month later. Tapia syndrome, or persistent unilateral laryngeal and hypoglossal paralysis, is an uncommon neuropraxia, which has previously not been observed in association with a breached vertebral body at T1 along the course of the RLN. Conclusion Tapia syndrome should be a differential diagnostic consideration whenever these symptoms persist postoperatively and spine surgeons should be aware of this as a potential complication of guidewires in spinal instrumentation.

  16. The administration of metformin during pregnancy reduces polycystic ovary syndrome related gestational complications.

    Science.gov (United States)

    De Leo, V; Musacchio, M C; Piomboni, P; Di Sabatino, A; Morgante, G

    2011-07-01

    To prospectively evaluate the safety of metformin administration during pregnancy in a group of PCOS patients by assessing its effect on the prevalence of gestational complications and neonatal outcome. Our prospective, single centre study included 98 pregnant women with PCOS treated with metformin throughout pregnancy and 110 normal pregnant controls. All PCOS patients were hyperinsulinemic and received metformin (1700-3000 mg/day) before conception and until 37 weeks' gestation. Metformin treatment in the pregnant PCOS patients resulted in significant decrease in miscarriage rate (9.1% vs 20%; pmetformin therapy throughout pregnancy resulted in significant reduction in pregnancy complications with concomitant improved neonatal outcome, with no serious deleterious side effects. Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.

  17. Acute Abdominal Compartment Syndrome as a Complication of Percutaneous Nephrolithotomy: Two Cases Reports and Literature Review

    Directory of Open Access Journals (Sweden)

    Jing Tao

    2016-09-01

    Full Text Available Percutaneous nephrolithotomy (PCNL is a technique commonly used to remove large or multiple kidney stones and stones in the inferior calyx, with the advantages of lower morbidity rates, decrease in post-operative pain with faster recovery. Intra-abdominal irrigation fluid extravasation which leads to abdominal hypertension is a rare complication of PCNL with little reports. Early detection of intra-abdominal extravagation is very important to prevent morbidity and mortality. We present two cases and review the literature.

  18. An Unexpected Innocent Complication Associated with Azacitidine Treatment of Myelodysplastic Syndrome: Erythema Annulare Centrifugum

    Directory of Open Access Journals (Sweden)

    Esra Turan Erkek

    2016-03-01

    Full Text Available Skin lesions accompanying hematological malignancies can be formed due to either direct tumor infiltration of the skin or indirect effects. Indirectly developing lesions may be a component of paraneoplastic syndrome. Erythema annulare centrifugum (EAC is considered to be a hypersensitivity reaction developed against various antigens associated with infections, drugs, and endocrine diseases. EAC, rarely seen in neoplastic diseases, has been reported in lymphoma, leukemia, histiocytosis, and prostate cancer. Here we report EAC in a patient using a hypomethylating agent, azacitidine. A 69-year-old female patient was admitted to our polyclinic with weakness and ecchymosis in her legs existing for 3 months. She was considered as having refractory anemia with excess blasts-2 according to myelodysplastic syndrome (MDS classification [1]. Because there was only hyperdiploidy in conventional cytogenetic examination, she was classified in group intermediate-2 of the International Prognostic Scoring System. She had a history of radical mastectomy and adjuvant chemoradiotherapy for breast cancer 3 years ago. She said that variously sized round and oval erythematous, itching, painless lesions had formed in the abdominal region on the 4th day of azacitidine usage (75 mg/m2/day, 7 days, s.c. (Figure 1 and 2. There were no concomitant complaints or physical examination findings except fatigue. After azacitidine was stopped, a skin biopsy was taken. In the biopsy, mild perivascular inflammatory infiltration accompanying vascular ectasia in the papillary dermis was detected. The possibility of paraneoplastic syndrome was excluded due to the disappearance of all lesions by 1 week after cessation of treatment. During the second course of azacitidine, the lesions reoccurred on the second day. Subsequently to the second course, the patient died of sepsis, which developed after pneumonia.

  19. D-Lactic Acidosis: An Underrecognized Complication of Short Bowel Syndrome

    OpenAIRE

    Kowlgi, N. Gurukripa; Chhabra, Lovely

    2015-01-01

    D-lactic acidosis or D-lactate encephalopathy is a rare condition that occurs primarily in individuals who have a history of short bowel syndrome. The unabsorbed carbohydrates act as a substrate for colonic bacteria to form D-lactic acid among other organic acids. The acidic pH generated as a result of D-lactate production further propagates production of D-lactic acid, hence giving rise to a vicious cycle. D-lactic acid accumulation in the blood can cause neurologic symptoms such as delirium...

  20. Thoracic air-leakage syndrome in allogeneic stem cell transplant recipients as a late complication of chronic graft-versus-host disease: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Park, Jae Wook; Kim, Song Soo; Jo, Daeg Yeon; Yun, Hwan Jung; Lee, Hyo Jin; Kim, Jin Hwan [Chungnam National University Hospital, Chungnam National University School of Medicine, Daejeon (Korea, Republic of)

    2016-08-15

    Air-leakage syndrome associated with graft-versus-host disease (GVHD) is a rare complication, but it is also reported as an independent predictor of a worse survival rate after stem cell transplantation. We report two cases of air-leakage syndrome associated with GVHD after allogeneic stem cell transplantation in acute leukemia patients who presented with spontaneous pneumomediastinum and subcutaneous emphysema, and finally death due to respiratory failure seven to eight months later.

  1. [Anesthetic Management of a Patient Complicated with Marfan Syndrome and Suffering from Stanford Type A Aortic Dissection during Pregnancy].

    Science.gov (United States)

    Uozaki, Nako; Mizuno, Kaori; Shiraishi, Yoshito; Doi, Matsuyuki; Sato, Shigehito

    2015-04-01

    We report a case of a 36-year-old woman at 34 weeks of gestation complicated with Marfan syndrome who underwent Bentall type aortic replacement surgery due to Stanford type A aortic dissection after undergoing caesarean section. Since this patient exhibited severe hypotension before coming to the operating room, it was very difficult to determine whether the cardiac surgery or caesarean section should be performed first. In this case, the caesarean section was performed first, followed by Bentall's surgery. Although intra-aortic balloon pumping and percutaneous cardiopulmonary support were required after weaning from the cardiopulmonary bypass, she was discharged on post-operative day (POD) 40 and the baby was discharged on POD 60, without signs of cerebral palsy. Unfortunately, this patient died on POD 57, due to heart failure. We discuss how to determine the priority of surgeries for patients who require emergency surgery for cardiovascular disease during pregnancy.

  2. Cervical sympathetic chain schwannoma masquerading as a carotid body tumour with a postoperative complication of first-bite syndrome.

    LENUS (Irish Health Repository)

    Casserly, Paula

    2012-01-31

    Carotid body tumours (CBT) are the most common tumours at the carotid bifurcation. Widening of the bifurcation is usually demonstrated on conventional angiography. This sign may also be produced by a schwannoma of the cervical sympathetic plexus. A 45-year-old patient presented with a neck mass. Investigations included contrast-enhanced CT, MRI and magnetic resonance arteriography with contrast enhancement. Radiologically, the mass was considered to be a CBT due to vascular enhancement and splaying of the internal and external carotid arteries. Intraoperatively, it was determined to be a cervical sympathetic chain schwannoma (CSCS). The patient had a postoperative complication of first-bite syndrome (FBS).Although rare, CSCS should be considered in the differential diagnosis for tumours at the carotid bifurcation. Damage to the sympathetic innervation to the parotid gland can result in severe postoperative pain characterised by FBS and should be considered in all patients undergoing surgery involving the parapharyngeal space.

  3. Extranodal marginal zone B cell lymphoma: An unexpected complication in children with Sjögren's syndrome.

    Science.gov (United States)

    Collado, Paz; Kelada, Aml; Cámara, Maria; Zeft, Andrew; Flagg, Aron

    2017-03-08

    Sjögren's syndrome (SS) is a systemic autoimmune disease characterized by the infiltration of lymphocytes into exocrine glands, resulting in the typical sicca symptoms. Unlike adults, primary SS is a very rare condition in childhood, and the risk of malignancy in juvenile SS (JSS) has not been defined. We report the detection of extranodal marginal zone B-cell lymphoma (EMZL) occurring in two children with SS. Fine needle aspiration of the salivary glands (SG) showed nonspecific findings that led to delayed diagnosis of SS. The diagnosis of B-cell lymphoma associated with JSS was based on morphologic and immunohistochemical staining done during the biopsy. To highlight awareness of EMZL as a timely and appropriate update of an unusual complication in children with SS. Copyright © 2017 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.

  4. Ipilimumab-induced Guillain-Barré Syndrome Presenting as Dysautonomia: An Unusual Presentation of a Rare Complication of Immunotherapy.

    Science.gov (United States)

    Kelly Wu, W; Broman, Kristy K; Brownie, Evan R; Kauffmann, Rondi M

    2017-06-01

    Immune-related adverse events are common and well-documented in patients treated with ipilimumab, a cytotoxic T-lymphocyte antigen-4 monoclonal antibody approved for the treatment of metastatic and stage III melanoma. Neurological complications are rare, but widely variable and potentially devastating. Here, we discuss a case of a patient who was treated with a single dose of ipilimumab for resected stage III melanoma. She subsequently developed pandysautonomia that manifested as a tonically dilated pupil, gastrointestinal dysmotility, urinary retention, and profound orthostatic hypotension. Guillain-Barré syndrome was diagnosed on electromyography. She was treated with intravenous immunoglobulin, droxidopa, and supportive care, with prolonged but eventual recovery. Given the broadening use of ipilimumab in the treatment of advanced and metastatic melanoma, awareness and recognition of its profound immune-mediated adverse effects are essential.

  5. Do clinical and laboratory parameters effect maternal and fetal outcomes in pregnancies complicated with hemolysis, elevated liver enzymes, and low platelet count syndrome?

    Science.gov (United States)

    Bezircioğlu, Incim; Baloğlu, Ali; Cetinkaya, Burcu; Pirim, Betül

    2012-01-01

    The aim of the study was to investigate whether the clinical features and laboratory parameters affect maternal and fetal outcomes in pregnancies complicated with HELLP syndrome. The medical records of pregnant patients complicated with HELLP syndrome were analyzed retrospectively between June 01, 2003 and June 01, 2010. The demographic data, medical history, admission symptoms, clinical and laboratory findings and recovery time were evaluated. The adverse maternal outcomes including eclampsia, placental abruption, disseminated intravascular coagulation, postpartum hemorrhage, pulmonary complications, cerebral edema and visual loss were recorded. Fetal growth restriction, necessity for neonatal intensive care unit admission and perinatal mortality were recorded as an adverse fetal outcome. The incidence of HELLP syndrome was 0.52%. The mean age of the patients was 28.93±7.90 (range 17-45). HELLP syndrome was diagnosed on average in the 33.68±4.41(th) (ranged 24-40) week of gestation. Eighteen cases (40.9%) were nullipara and twenty-six cases (59.1%) multipara. The most common complications were eclampsia (40.9%) and abruption placenta (15.9%). Pregnancy was terminated within 48 hours in all patients. The rate of cesarean section was 90.9%. Perinatal mortality rate in HELLP syndrome was 31.8%. There was no maternal mortality. Neither clinical characteristics nor laboratory parameters was found effective for prediction of adverse maternal and fetal outcomes.

  6. Wernicke-Korsakoff syndrome complicated by subacute beriberi neuropathy in an alcoholic patient.

    Science.gov (United States)

    Di Marco, Salvatore; Pilati, Laura; Brighina, Filippo; Fierro, Brigida; Cosentino, Giuseppe

    2018-01-01

    Thiamine (vitamin B1) deficiency is a common condition in alcohol abusers, which can lead to damage of both the peripheral and the central nervous systems. Here we describe the case of an alcoholic patient who presented with acute onset of ataxia, severe weakness of the four limbs, and hypoesthesia and dysesthesia of the distal portion of the upper and lower extremities. The clinical picture also included mental confusion and amnesia. A diagnosis of Wernicke-Korsakoff syndrome was made based on clinical symptoms and brain RMI findings. Electromyography and electroneurography revealed signs of subacute axonal sensory-motor polyneuropathy that were compatible with a rare acute presentation of beriberi. Patient immediately received parenteral thiamine administration, which resulted in rapid clinical amelioration of ataxia and confusion and also in a significant improvement of motor and sensory deficits. The association between Wernicke-Korsakoff syndrome and acute axonal polyneuropathy is a very rare condition that could make less recognizable the clinical picture of a thiamine deficiency. However, the diagnosis of thiamine deficiency should be suspected in every alcoholic patient presenting with acute onset symptoms of central and/or peripheral nervous system involvement. This because the immediate replacement treatment can be life-saving and reverse the clinical symptoms. Copyright © 2017 Elsevier B.V. All rights reserved.

  7. Increased risk of malignancy with adalimumab combination therapy, compared with monotherapy, for Crohn's disease.

    Science.gov (United States)

    Osterman, Mark T; Sandborn, William J; Colombel, Jean-Frederic; Robinson, Anne M; Lau, Winnie; Huang, Bidan; Pollack, Paul F; Thakkar, Roopal B; Lewis, James D

    2014-04-01

    Few studies have assessed the risk of malignancy from anti-tumor necrosis factor monotherapy or combination therapy for Crohn's disease (CD). We determined the relative risk of malignancy in patients with CD who received adalimumab monotherapy, compared with the general population. We also compared the risk of malignancy associated with combination adalimumab and immunomodulator therapy with that of adalimumab monotherapy. We performed a pooled analysis of data from 1594 patients with CD who participated in clinical trials of adalimumab (CLASSIC I and II, CHARM, GAIN, EXTEND, and ADHERE studies; 3050 patient-years of exposure). We calculated rates of malignancy among patients; the expected rates of malignancy, based on the general population, were derived from the Surveillance, Epidemiology, and End Results registry and National Cancer Institute survey. Compared with the general population, patients receiving adalimumab monotherapy did not have a greater than expected incidence of nonmelanoma skin cancer (NMSC) or other cancers, whereas those receiving combination therapy had a greater than expected incidence of malignancies other than NMSC (standardized incidence ratio, 3.04; 95% confidence interval [CI], 1.66-5.10) and of NMSC (standardized incidence ratio, 4.59; 95% CI, 2.51-7.70). Compared with patients receiving adalimumab monotherapy, those patients receiving combination therapy had an increased risk of malignancy other than NMSC (relative risk, 2.82; 95% CI, 1.07-7.44) and of NMSC (relative risk, 3.46; 95% CI, 1.08-11.06). In patients with CD, the incidence of malignancy with adalimumab monotherapy was not greater than that of the general population. Co-administration of immunomodulator therapy and adalimumab was associated with an increased risk of NMSC and other cancers. Copyright © 2014 AGA Institute. Published by Elsevier Inc. All rights reserved.

  8. Lethality of patients with rheumatoid arthritis depending on adalimumab administration: imitation modeling

    Directory of Open Access Journals (Sweden)

    D V Goryachev

    2009-01-01

    Full Text Available Lethality of pts with rheumatoid arthritis (RA exceeds mortality values in general population. Possibility of disease modifying anti-rheumatic drugs (DMARD influence on RA pts lethality has been widely discussed lately in scientific works. Objective. To determine possible lethality diminishment in Russian population of RA pts with one of biological drugs TNFα antagonist adalimumab. Material and methods. Model construction is based on the fact of lethality dependence on pt functional state assessed by HAQ. Model simulating progression of functional disability in pts with RA visiting medical institutions of Russia was made (RAISER study. 3 model variants for imitation of consecutive change of DMARDs including adalimumab were done. First consecution assessed DMARD change in the next chain: adalimumab-methotrexate-sulfasalazine-leflunomide-azathioprine-cyclosporine-palliative therapy. Second consecution: adalimumab administration after failure of first 3 DMARDs. Third consecution considered only change of synthetic DMARDs without adalimumab inclusion. Model imitated participation of 3000 pts in every consecution. Prognosis horizon was 12 years. Age of pts and initial HAQ distribution were get from results of epidemiological RAISER study. Calculation was done on the base of elevation of standardized lethality level (SLL in population of RA pts in average from 135% to 300%. SLL values from 80 to 320% were used depending on functional disability degree with converting to Russian values of age-specific lethality coefficient for 1999. Results. Lethality in treatment consecutions including adalimumab was significantly lower. To the end of 12th year in group not using adalimumab, using it at once and using it after 376 DMARDs respectively 65,1%, 71,6% and 71,1% of pts were still alive. Conclusion. Significant decrease of lethality with adalimumab inclusion in consecution of DMARD change during treatment of RA pts was demonstrated with imitation modeling

  9. Efficacy of treatment intensification with adalimumab, etanercept and infliximab in rheumatoid arthritis

    DEFF Research Database (Denmark)

    Eng, Grith; Stoltenberg, Michael B; Szkudlarek, Marcin

    2013-01-01

    To summarize the empirical evidence regarding the effect of treatment intensification on clinical outcomes in patients with rheumatoid arthritis treated with one of the TNF-α-inhibitors, adalimumab, etanercept or infliximab.......To summarize the empirical evidence regarding the effect of treatment intensification on clinical outcomes in patients with rheumatoid arthritis treated with one of the TNF-α-inhibitors, adalimumab, etanercept or infliximab....

  10. The effects of pregnancy on the pharmacokinetics of infliximab and adalimumab in inflammatory bowel disease.

    Science.gov (United States)

    Seow, C H; Leung, Y; Vande Casteele, N; Ehteshami Afshar, E; Tanyingoh, D; Bindra, G; Stewart, M J; Beck, P L; Kaplan, G G; Ghosh, S; Panaccione, R

    2017-05-01

    Transplacental transfer of infliximab and adalimumab results in detectable drug levels in the cord blood and infant. To determine if pregnancy influenced the pharmacokinetics of anti-TNF agents in women with inflammatory bowel disease. Twenty-five women from the University of Calgary inflammatory bowel disease(IBD) pregnancy clinic on maintenance infliximab or adalimumab were recruited prospectively with serum bio-banking performed each trimester. Infliximab trough and adalimumab steady-state levels were the outcomes of interest and were analysed using the ANSER infliximab and adalimumab assays. Multivariate linear mixed-effects models were constructed to assess infliximab and adalimumab drug levels during pregnancy adjusting for the clinical covariates of albumin, BMI and CRP. Fifteen women (eight Crohn's disease, seven ulcerative colitis) received infliximab and 10 women with 11 pregnancies were treated with adalimumab. Median age was 29.6 years (IQR: 27.6-31.2 years). Median disease duration was 9.2 years (IQR: 3.16-15.0 years). Median trough infliximab concentrations were 8.50 μg/mL (IQR: 7.23-10.07 μg/mL), 10.31 μg/mL (IQR: 7.66-15.63 μg/mL) and 21.02 μg/mL (IQR: 16.01-26.70 μg/mL) at trimesters 1, 2 and 3 respectively. Significant changes in albumin and BMI (P 0.05) were documented throughout pregnancy. After adjusting for albumin, BMI and CRP, infliximab trough levels increased during pregnancy, by 4.2 μg/mL per trimester (P = 0.02), while adalimumab drug levels remained stable (P > 0.05). Infliximab levels rise during pregnancy, whereas adalimumab levels remain stable after accounting for changes in albumin, BMI and CRP. Therapeutic drug monitoring in the second trimester may be useful in guiding dosing in the third trimester. © 2017 John Wiley & Sons Ltd.

  11. Late-onset cytomegalovirus infection complicated by Guillain-Barre syndrome in a kidney transplant recipient: case report and review of the literature.

    Science.gov (United States)

    Shaban, E; Gohh, R; Knoll, B M

    2016-04-01

    Cytomegalovirus (CMV) infection remains a common infection after solid-organ transplantation. In the general population CMV disease is associated with Guillain-Barre syndrome (GBS), an autoimmune disease leading to an acute peripheral neuropathy, in 1 of 1000 cases. Interestingly, GBS is a rarely observed complication in solid-organ transplant recipients, possibly related to maintenance immunosuppression. We describe a case of CMV infection complicated by GBS in a kidney transplant recipient and review the literature.

  12. A complicated case of antepartum eclamptic fit with HELLP syndrome, acute renal failure and multiple intracranial hemorrhages: A mortality report

    Directory of Open Access Journals (Sweden)

    Ahmed Samy El-agwany

    2016-04-01

    Full Text Available HELLP is an acronym for hemolysis, elevated liver enzymes and low platelets count, affecting 0.2–12% of all pregnancies or 4–12% of those with preeclampsia. The maternal mortality reported from the literature is up 4% due to disseminated intravascular coagulation, placental abruption, acute renal failure, eclampsia, and cerebral hemorrhage. A 20 year old, G2P1, at 36 weeks of gestation, was referred to our hospital because of postictal coma state with bilateral mydriasis and epistaxis due to repeated antepartum eclamptic fits. Elevated blood pressure level 170/110 mmHg was accompanied with massive proteinuria. Cesarean section was performed and female newborn were delivered. Laboratory findings were characteristic of preeclampsia, HELLP syndrome and renal failure. The patient developed an intraventricular hematoma and an intracerebral hemorrhage with subarachnoid one, which were not suitable to neurosurgical treatment. The patient died from refractory hemolytic anemia, spontaneous bleeding of multiple organs, renal failure and intracranial hemorrhage. Preeclampsia, HELLP syndrome, and acute fatty liver of pregnancy might overlap and be associated with potentially fatal complications, including intracranial hemorrhage, as in the present case. Early detection and diagnosis are crucial to ensure appropriate management and treatment success.

  13. Cytotoxicity and genotoxicity of intravitreal adalimumab administration in rabbit retinal cells

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    Álcio Coutinho de Paula

    2015-04-01

    Full Text Available Purpose: To assess the cytotoxicity and genotoxicity of intravitreal adalimumab treatment in an animal experimental model using cytological and molecular techniques. Methods: Eighteen rabbits were randomly assigned to three groups: control, adalimumab treatment, and placebo. Cytotoxicity on retinal cells was evaluated using flow cytometry assays to determine the level of apoptosis and necrosis. Genotoxicity was evaluated by comet assays to assess DNA damage, and quantitative real-time polymerase chain reaction (qPCR was used to evaluate expression of apoptosis-inducing caspases (8 and 3. Results: No cytotoxicity or genotoxicity was observed in any of the two treatment groups (adalimumab and placebo following intravitreal administration compared with the control group. Flow cytometry analysis revealed that more than 90% of the cells were viable, and only a low proportion of retinal cells presented apoptotic (~10% or necrotic (<1% activity across all groups. Molecular damage was also low with a maximum of 6.4% DNA degradation observed in the comet assays. In addition, no increase in gene expression of apoptosis-inducing caspases was observed on retinal cells by qPCR in both the adalimumab and placebo groups compared with the control group. Conclusion: The use of adalimumab resulted in no detectable cytotoxicity or genotoxicity on retinal cells for up to 60 days upon administration. These results therefore indicate that adalimumab may be a safe option for intravitreal application to treat ocular inflammatory diseases in which TNF-α is involved.

  14. Value of OCT Angiography in the Diagnosis of Choroidal Neovascularization Complicating Multiple Evanescence White Dot Syndrome.

    Science.gov (United States)

    Nozaki, Miho; Hamada, Satoshi; Kimura, Masayo; Yoshida, Munenori; Ogura, Yuichiro

    2016-06-01

    This report describes a 19-year-old patient with the rare association of multiple evanescent white dot syndrome (MEWDS) and choroidal neovascularization (CNV). Despite the initial diagnosis of MEWDS, her vision significantly decreased. Fluorescein angiography showed dye leakage and pooling at the macula, whereas optical coherence tomography (OCT) showed cystoid macular edema and serous neuroepithelial elevation. The dye leakage and pooling made it impossible to diagnose CNV. However, OCT angiography (OCTA) revealed a vascular structure in the outer retina, thereby leading to the CNV diagnosis. Visual acuity improved after an anti-vascular endothelial growth factor injection. OCTA proved to be useful in the diagnosis of CNV. [Ophthalmic Surg Lasers Imaging Retina. 2016;47:580-584.]. Copyright 2016, SLACK Incorporated.

  15. Successful Management of Pregnancy Complicated by Klippel-Trenaunay Syndrome Using MR Angiography-Based Evaluation

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    Reiko Tanaka

    2011-01-01

    Full Text Available Klippel-Trenaunay syndrome (KTS is a rare congenital disease, and extensive cutaneous hemangiomas and abnormal venous vessels are characteristic. In our case, to manage her pregnancy with KTS, whole-body MRA was performed before delivery. A 29-year-old woman was referred at 28 weeks because of prominent vulvovaginal varicosities due to KTS. At 35 weeks, hypertrophy and multiple venous varicosities of her leg as well as massive vulvovaginal varicosities became prominent with a normal coagulation profile. Systematic MRAs revealed hemangiomas and varicosities in the right leg, the lower abdomen, and the pubic region, while no obvious AVM was detected around the bronchial tube and spine. We decided to deliver her baby by cesarean section at 37 weeks under general anesthesia, and a healthy baby was delivered. No blood transfusion was required. Prophylaxis against thrombosis was performed after the operation. She was discharged with her baby. Her vulvovaginal varicosities shrunk considerably one month later.

  16. Cholecystitis and nephrotic syndrome complicating Epstein-Barr virus primary infection.

    Science.gov (United States)

    Rodà, Diana; Huici, Malka; Ricart, Sílvia; Vila, Jordi; Fortuny, Clàudia; Alsina, Laia

    2017-02-01

    Epstein-Barr virus (EBV) infection results in a spectrum of clinical manifestations. The host immune response to EBV plays a key role in the extent and degree of clinical features, which in children under 4 years of age are usually mild, non-specific and self-limiting. A 2-year-old boy in whom no known immune disorder could be found presented with acute acalculous cholecystitis, renal dysfunction with massive proteinuria, ascites, pleural effusion, minimal peripheral oedema and a severe systemic inflammatory response. Improvement occurred after initiation of corticosteroids and antiviral treatment with gancyclovir. In severely symptomatic or complicated EBV infection, a primary immunodeficiency must be suspected. If a primary immunodeficiency has been ruled out, the correct management of severe EBV infection in the immunocompetent host remains controversial.

  17. Osteomalacia complicating renal tubular acidosis in association with Sjogren′s syndrome

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    Zohra El Ati

    2014-01-01

    Full Text Available Renal involvement in Sjogren′s syndrome (SS is not uncommon and may precede other complaints. Tubulointerstitial nephritis is the most common renal disease in SS and may lead to renal tubular acidosis (RTA, which in turn may cause osteomalacia. Nevertheless, osteomalacia rarely occurs as the first manifestation of a renal tubule disorder due to SS. We herewith describe a 43-year-old woman who was admitted to our hospital for weakness, lumbago and inability to walk. X-ray of the long bones showed extensive demineralization of the bones. Laboratory investigations revealed chronic kidney disease with serum creatinine of 2.3 mg/dL and creatinine clearance of 40 mL/min, hypokalemia (3.2 mmol/L, hypophosphatemia (0.4 mmol/L, hypocalcemia (2.14 mmol/L and hyperchloremic metabolic acidosis (chlorine: 114 mmol/L; alkaline reserve: 14 mmol/L. The serum alkaline phosphatase levels were elevated. The serum levels of 25-hydroxyvitamin D and 1,25-dihydroxy vitamin D were low and borderline low, respectively, and the parathyroid hormone level was 70 pg/L. Urinalysis showed inappropriate alkaline urine (urinary PH: 7, glycosuria with normal blood glucose, phosphaturia and uricosuria. These values indicated the presence of both distal and proximal RTA. Our patient reported dryness of the mouth and eyes and Schirmer′s test showed xerophthalmia. An accessory salivary gland biopsy showed changes corresponding to stage IV of Chisholm and Masson score. Kidney biopsy showed diffuse and severe tubulo-interstitial nephritis with dense lymphoplasmocyte infiltrates. Sicca syndrome and renal interstitial infiltrates indicated SS as the underlying cause of the RTA and osteomalacia. The patient received alkalinization, vitamin D (Sterogyl ®, calcium supplements and steroids in an initial dose of 1 mg/kg/day, tapered to 10 mg daily. The prognosis was favorable and the serum creatinine level was 1.7 mg/dL, calcium was 2.2 mmol/L and serum phosphate was 0.9 mmol/L.

  18. Classification of and risk factors for hematologic complications in a French national cohort of 102 patients with Shwachman-Diamond syndrome

    Science.gov (United States)

    Donadieu, Jean; Fenneteau, Odile; Beaupain, Blandine; Beaufils, Sandrine; Bellanger, Florence; Mahlaoui, Nizar; Lambilliotte, Anne; Aladjidi, Nathalie; Bertrand, Yves; Mialou, Valérie; Perot, Christine; Michel, Gérard; Fouyssac, Fanny; Paillard, Catherine; Gandemer, Virginie; Boutard, Patrick; Schmitz, Jacques; Morali, Alain; Leblanc, Thierry; Bellanné-Chantelot, Christine

    2012-01-01

    Background Patients with the Shwachman-Diamond syndrome often develop hematologic complications. No risk factors for these complications have so far been identified. The aim of this study was to classify the hematologic complications occurring in patients with Shwachman-Diamond syndrome and to investigate the risk factors for these complications. Design and Methods One hundred and two patients with Shwachman-Diamond syndrome, with a median follow-up of 11.6 years, were studied. Major hematologic complications were considered in the case of definitive severe cytopenia (i.e. anemia <7 g/dL or thrombocytopenia <20×109/L), classified as malignant (myelodysplasia/leukemia) according to the 2008 World Health Organization classification or as non-malignant. Results Severe cytopenia was observed in 21 patients and classified as malignant severe cytopenia (n=9), non-malignant severe cytopenia (n=9) and malignant severe cytopenia preceded by non-malignant severe cytopenia (n=3). The 20-year cumulative risk of severe cytopenia was 24.3% (95% confidence interval: 15.3%–38.5%). Young age at first symptoms (<3 months) and low hematologic parameters both at diagnosis of the disease and during the follow-up were associated with severe hematologic complications (P<0.001). Fifteen novel SBDS mutations were identified. Genotype analysis showed no discernible prognostic value. Conclusions Patients with Shwachman-Diamond syndrome with very early symptoms or cytopenia at diagnosis (even mild anemia or thrombocytopenia) should be considered at a high risk of severe hematologic complications, malignant or non-malignant. Transient severe cytopenia or an indolent cytogenetic clone had no deleterious value. PMID:22491737

  19. D-Lactic Acidosis: An Underrecognized Complication of Short Bowel Syndrome

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    N. Gurukripa Kowlgi

    2015-01-01

    Full Text Available D-lactic acidosis or D-lactate encephalopathy is a rare condition that occurs primarily in individuals who have a history of short bowel syndrome. The unabsorbed carbohydrates act as a substrate for colonic bacteria to form D-lactic acid among other organic acids. The acidic pH generated as a result of D-lactate production further propagates production of D-lactic acid, hence giving rise to a vicious cycle. D-lactic acid accumulation in the blood can cause neurologic symptoms such as delirium, ataxia, and slurred speech. Diagnosis is made by a combination of clinical and laboratory data including special assays for D-lactate. Treatment includes correcting the acidosis and decreasing substrate for D-lactate such as carbohydrates in meals. In addition, antibiotics can be used to clear colonic flora. Although newer techniques for diagnosis and treatment are being developed, clinical diagnosis still holds paramount importance, as there can be many confounders in the diagnosis as will be discussed subsequently.

  20. Sinus Venosus Atrial Septal Defect Complicated by Eisenmenger Syndrome and the Role of Vasodilator Therapy

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    Amornpol Anuwatworn

    2016-01-01

    Full Text Available Sinus venosus atrial septal defect is a rare congenital, interatrial communication defect at the junction of the right atrium and the vena cava. It accounts for 5–10% of cases of all atrial septal defects. Due to the rare prevalence and anatomical complexity, diagnosing sinus venous atrial septal defects poses clinical challenges which may delay diagnosis and treatment. Advanced cardiac imaging studies are useful tools to diagnose this clinical entity and to delineate the anatomy and any associated communications. Surgical correction of the anomaly is the primary treatment. We discuss a 43-year-old Hispanic female patient who presented with dyspnea and hypoxia following a laparoscopic myomectomy. She had been diagnosed with peripartum cardiomyopathy nine years ago at another hospital. Transesophageal echocardiography and computed tomographic angiography of the chest confirmed a diagnosis of sinus venosus atrial septal defect. She was also found to have pulmonary arterial hypertension and Eisenmenger syndrome. During a hemodynamic study, she responded to vasodilator and she was treated with Ambrisentan and Tadalafil. After six months, her symptoms improved and her pulmonary arterial hypertension decreased. We also observed progressive reversal of the right-to-left shunt. This case illustrates the potential benefit of vasodilator therapy in reversing Eisenmenger physiology, which may lead to surgical repair of the atrial septal defect as the primary treatment.

  1. Infectious Mimicry Complicates Diagnosis in Hemophagocytic Syndrome Caused by Anaplastic Large-Cell Lymphoma

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    Michael J. Peluso

    2012-01-01

    Full Text Available Hemophagocytic syndrome (HPS arises secondary to genetic, rheumatologic, neoplastic, and infectious causes. We discuss a patient whose presentation was consistent with systemic infection but was discovered to have HPS of unknown etiology. The presenting symptoms, as well as unremarkable malignancy and rheumatologic workups, led to the pursuit of an infectious cause, but the patient was ultimately discovered to have an occult anaplastic large-cell lymphoma (ALCL. This case demonstrates the diagnostic challenges that result from infectious mimicry in the context of HPS—first, in distinguishing noninfectious HPS from the systemic inflammation that can result from a widespread infectious process, second, in the identification of the precipitating cause of HPS. While evidence of these challenges has been suggested by the limited literature on HPS and ALCL, our case illustrates the diagnostic dilemma that arises when tissue biopsy does not quickly reveal an etiology. It is important that all physicians be aware that HPS can mimic infection and be prepared to redirect the workup when an infectious etiology for HPS cannot be identified.

  2. Life-Threatening Postpneumonectomy Syndrome Complicated with Right Aortic Arch after Left Pneumonectomy.

    Science.gov (United States)

    Karasaki, Takahiro; Tanaka, Makoto

    2015-01-01

    A 54-year-old man with right aortic arch underwent left lower lobectomy and lingular segmentectomy, followed by complete pneumonectomy, for refractory nontuberculous mycobacterial infection. Three months after the pneumonectomy, he developed acute respiratory distress. Computed tomography showed an excessive mediastinal shift with an extremely narrowed bronchus intermedius and right lower bronchus compressed between the right pulmonary artery and the right descending aorta. Soon after the nearly obstructed bronchus intermedius was observed by bronchoscopy, he began to exhibit frequent hypoxic attacks, perhaps due to mucosal edema. Emergent surgical repositioning of the mediastinum and decompression of the bronchus was indicated. After complete adhesiolysis of the left thoracic cavity was performed, to maintain the proper mediastinal position, considering the emergent setting, an open wound thoracostomy was created and piles of gauze were inserted, mildly compressing the heart and the mediastinum to the right side. Thoracoplasty was performed three months later, and he was eventually discharged without any dressings needed. Mediastinal repositioning under thoracostomy should be avoided in elective cases because of its extremely high invasiveness. However, in the case of life-threatening postpneumonectomy syndrome in an emergent setting, mediastinal repositioning under thoracostomy may be an option to save life, which every thoracic surgeon could attempt.

  3. Nephrotic Syndrome Complicated by Life-Threatening Pulmonary Embolism in an Adult Patient

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    Szu-Chia Chen

    2010-02-01

    Full Text Available We report a 23-year-old male presenting with edema. He was originally admitted for an elective renal biopsy for diagnosis of renal pathology. Unfortunately, because of acute abdominal pain an exploratory laparotomy was done. Progressive azotemia and oliguria then developed, and he required temporary hemodialysis. However, he suffered from sudden-onset severe respiratory distress, and blood gas analysis showed profound hypoxemia with a marked arterial–alveolar oxygen difference. Assessment of a pulmonary embolism by radioisotope imaging was not possible because of his dependence on mechanical ventilation. Subcutaneous low molecular weight heparin and intravenous methylprednisolone were given to treat the presumed pulmonary embolism and the underlying nephrotic syndrome. His partial oxygen level gradually increased after continuous heparin and steroid administration. Complete obliteration of one major pulmonary artery and partial obliteration of other smaller arteries were revealed by magnetic resonance angiography. He was discharged and followed-up as an outpatient, and was given oral warfarin and prednisolone. Follow-up magnetic resonance angiography 5 months later showed a normal pulmonary tree with no residual lesions.

  4. High-Risk Coronary Plaques Complicated with Acute Coronary Syndrome in Young Patients

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    Opincariu Diana

    2017-06-01

    Full Text Available Coronary computed tomography angiography (CCTA has evolved notably over the last decade, gaining an increased amount of temporo-spatial resolution in combination with decreased radiation exposure. The importance of CCTA is emerging especially in vulnerable and young patients who might not have developed a viable collateral vascular network to sustain the circulation to an infarction area during a major adverse coronary event. There are a few well-known markers by which a vulnerable plaque can be assessed and that can predict the subsequent events of sudden myocardial ischemia, such as an increased positive remodeling index (cut-off >1.4, low-attenuation plaque (cut-off 0.7, and napkin-ring sign (NRS. This manuscript presents a series of 3 clinical cases of young patients experiencing symptoms and signs of myocardial ischemia who underwent CCTA in order to assess the composition and functional characteristics of atherosclerotic plaques and their repercussion in developing an acute coronary syndrome.

  5. Clinical features and pregnancy outcome in antiphospholipid syndrome patients with history of severe pregnancy complications.

    Science.gov (United States)

    Matsuki, Yuko; Atsumi, Tatsuya; Yamaguchi, Koushi; Hisano, Michi; Arata, Naoko; Oku, Kenji; Watanabe, Noriyoshi; Sago, Haruhiko; Takasaki, Yoshinari; Murashima, Atsuko

    2015-03-01

    Abstract Objective. To clarify the clinical significance of antiphospholipid antibody (aPL) profile in patients with obstetric antiphospholipid syndrome (APS). Methods. Clinical records of 13 pregnant patients (15 pregnancies) with obstetrical APS were reviewed over 10 years. Patients who met the Sapporo Criteria fully were studied, whereas those with only early pregnancy loss were excluded. In addition to classical aPL: lupus anticoagulant (LA), anticardiolipin antibody (aCL), and anti-β2-glycoprotein I (aβ2GPI); phosphatidylserine-dependent anti-prothrombin antibody (aPS/PT) and kininogen-dependent anti-phosphatidylethanolamine antibody (aPE) were also examined in each case. Results. Cases were divided into two groups according to patient response to standard treatment: good and poor outcome groups. All cases with poor outcome presented LA, with IgG aβ2GPI and IgG aPS/PT were also frequently observed. IgG aPE did not correlate with pregnancy outcome. Conclusion. aPL profile may predict pregnancy outcome in patients with this subset of obstetric APS.

  6. Pre- and perinatal complications in relation to Tourette syndrome and co-occurring obsessive-compulsive disorder and attention-deficit/hyperactivity disorder

    NARCIS (Netherlands)

    Abdulkadir, Mohamed; Tischfield, Jay A.; King, Robert A.; Fernandez, Thomas V.; Brown, Lawrence W.; Cheon, Keun-Ah; Coffey, Barbara J.; de Bruijn, Sebastian F. T. M.; Elzerman, Lonneke; Garcia-Delgar, Blanca; Gilbert, Donald L.; Grice, Dorothy E.; Hagstrom, Julie; Hedderly, Tammy; Heyman, Isobel; Hong, Hyun Ju; Huyser, Chaim; Ibanez-Gomez, Laura; Kim, Young Key; Kim, Young-Shin; Koh, Yun-Joo; Kook, Sodahm; Kuperman, Samuel; Lamerz, Andreas; Leventhal, Bennett; Ludolph, Andrea G.; Madruga-Garrido, Marcos; Maras, Athanasios; Messchendorp, Marieke D.; Mir, Pablo; Morer, Astrid; Munchau, Alexander; Murphy, Tara L.; Openneer, Thaira J. C.; Plessen, Kerstin J.; Rath, Judith J. G.; Roessner, Veit; Frundt, Odette; Shin, Eun-Young; Sival, Deborah A.; Song, Dong-Ho; Song, Jungeun; Stolte, Anne-Marie; Tubing, Jennifer; van den Ban, Els; Visscher, Frank; Wanderer, Sina; Woods, Martin; Zinner, Samuel H.; State, Matthew W.; Heiman, Gary A.; Hoekstra, Pieter J.; Dietrich, Andrea

    2016-01-01

    Pre- and perinatal complications have been implicated in the onset and clinical expression of Tourette syndrome albeit with considerable inconsistencies across studies. Also, little is known about their role in co-occurring obsessive-compulsive disorder (OCD) and attention-deficit/hyperactivity

  7. Pre- and perinatal complications in relation to Tourette syndrome and co-occurring obsessive-compulsive disorder and attention-deficit/hyperactivity disorder

    NARCIS (Netherlands)

    Abdulkadir, M. (Mohamed); J.A. Tischfield (Jay); King, R.A. (Robert A.); T.V. Fernandez (Thomas); Brown, L.W. (Lawrence W.); Cheon, K.-A. (Keun-Ah); Coffey, B.J. (Barbara J.); S.F. de Bruijn (S.); Elzerman, L. (Lonneke); Garcia-Delgar, B. (Blanca); D.L. Gilbert (D.); S.F.J. Le Grice; Hagstrøm, J. (Julie); T. Hedderly (Tammy); Heyman, I. (Isobel); Hong, H.J. (Hyun Ju); J. Huyser (Jochanan); Ibanez-Gomez, L. (Laura); Kim, Y.K. (Young Key); Kim, Y.-S. (Young-Shin); Koh, Y.-J. (Yun-Joo); Kook, S. (Sodahm); Kuperman, S. (Samuel); Lamerz, A. (Andreas); Leventhal, B. (Bennett); Ludolph, A.G. (Andrea G.); Madruga-Garrido, M. (Marcos); A. Maras (Athanasios); Messchendorp, M.D. (Marieke D.); Mir, P. (Pablo); Morer, A. (Astrid); A. Münchau (Alexander); Murphy, T.L. (Tara L.); Openneer, T.J.C. (Thaïra J.C.); Plessen, K.J. (Kerstin J.); S. Rath (Santosh); V. Rœssner (Veit); Fründt, O. (Odette); Shin, E.-Y. (Eun-Young); D.A. Sival (Deborah); Song, D.-H. (Dong-Ho); Song, J. (Jungeun); Stolte, A.-M. (Anne-Marie); Tübing, J. (Jennifer); van den Ban, E. (Els); F. Visscher (Frank); Wanderer, S. (Sina); Woods, M. (Martin); Zinner, S.H. (Samuel H.); M.W. State (Matthew); M.L. Heiman (Mark); P.J. Hoekstra (Pieter); A. Dietrich (Andrea)

    2016-01-01

    textabstractPre- and perinatal complications have been implicated in the onset and clinical expression of Tourette syndrome albeit with considerable inconsistencies across studies. Also, little is known about their role in co-occurring obsessive-compulsive disorder (OCD) and

  8. Ictal central apnea and bradycardia in temporal lobe epilepsy complicated by obstructive sleep apnea syndrome

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    Yoko Nishimura

    2015-01-01

    Full Text Available We describe the case of a 12-year-old boy who developed temporal lobe epilepsy (TLE with daily complex partial seizures (CPS and monthly generalized seizures. Moreover, he frequently snored while asleep since early childhood. Polysomnography (PSG revealed severe obstructive sleep apnea with apnea–hypopnea index (AHI of 37.8/h. Video-PSG with simultaneous electroencephalography (EEG recording captured two ictal apneic episodes during sleep, without any motor manifestations. The onset of rhythmic theta activity in the midtemporal area on EEG was preceded by the onset of apnea by several seconds and disappeared soon after cessation of central apnea. One episode was accompanied by ictal bradycardia of <48 beats/min which persisted for 50 s beyond the end of epileptic activity. After treatment with carbamazepine and tonsillectomy/adenoidectomy, the seizures were well controlled and AHI decreased to 2.5/h. Paroxysmal discharges also disappeared during this time. Uncontrolled TLE complicated by sleep apnea should be evaluated for the presence of ictal central apnea/bradycardia.

  9. Medical complications of bariatric surgery: focus on malabsorption and dumping syndrome.

    Science.gov (United States)

    Hammer, Heinz F

    2012-01-01

    Gastroenterologists increasingly see patients with symptoms after bariatric surgery. A number of gastrointestinal or extra-gastrointestinal symptoms should raise the suspicion of malabsorption or dumping syndrome. Little is known about long-term consequences of disordered intestinal anatomy and physiology resulting from bariatric surgical procedures. The latency phase of clinical problems is unknown, but may potentially be long, and postoperative courses over many decades have to be considered regarding the consequences of surgical alterations of gastrointestinal structure and function. Long-term nutritional requirements in patients with bariatric procedures are incompletely understood. This review focuses on the pathophysiology of long-limb Roux-en-Y gastric bypass (RYGB) because it has become the most common bariatric procedure in many parts of the world. Although several potential mechanisms for nutritional deficiencies after RYGB like deficiency of dietary intake, lack of gastric secretions, exclusion of proximal duodenum and jejunum, or asynergia between food bolus and biliopancreatic secretions have been postulated, it was only very recently that in-depth studies have been carried out to measure the extent to which the long-limb RYGB causes malabsorption. In order to improve care for these patients, specialists who are trained in understanding pathophysiological changes in digestion and absorption after bariatric surgery and who recognize and treat clinical symptoms and nutritional deficits after bariatric surgery are needed. In addition, clinical researchers should take advantage of the experimental setups provided by standardized surgical procedures, and scientific societies should design courses and scientific meetings which combine the expertise in gastroenterology, surgery and nutrition. Copyright © 2012 S. Karger AG, Basel.

  10. Sjögren's syndrome complicated by interstitial cystitis: A case series and literature review.

    Science.gov (United States)

    Darrieutort-Laffite, Christelle; André, Vincent; Hayem, Gilles; Saraux, Alain; Le Guern, Véronique; Le Jeunne, Claire; Puéchal, Xavier

    2015-07-01

    To characterize the interstitial cystitis (IC) associated with Sjögren's syndrome (SS). Report of three new cases. Only cases fulfilling the American-European consensus criteria for SS and the European Society for the Study of Interstitial Cystitis criteria with positive histological findings for IC were included. Thirteen cases of SS and IC have been reported in women, including the three reported here, with a mean age of 54 years. SS appeared first in 77% (n=10) of cases, a mean of 6.6 years before IC. The symptoms of IC included pollakiuria (n=11), lower abdominal pain (n=8), urinary urgency (n=5), painful micturition (n=6), hematuria (n=3) and dysuria (n=3). Urinary dilatation occurred in three cases, leading to acute renal failure in two patients. The diagnosis of IC was confirmed by anatomical evidence of cystitis inflammation on bladder biopsy in all (n=13) patients. Treatment was reported for nine patients, seven of whom (78%) received corticosteroid treatment, which was partially or completely effective in six cases. Immunosuppressive treatment was added in three cases (cyclosporine, n=2; azathioprine, n=1; cyclophosphamide, n=1). Local bladder treatments were performed, with hydraulic distension in five cases and DMSO instillation in one patient. A urinary catheter was inserted in the two cases of acute obstructive renal failure. Urinary symptoms without infection should lead the physician to consider a diagnosis of IC in SS patients. Urinary dilatation may occur, leading to acute obstructive renal failure. Corticosteroid treatment may be effective and local treatments have been tried. Copyright © 2015 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved.

  11. Hunter Syndrome

    Science.gov (United States)

    ... in girls. There's no cure for Hunter syndrome. Treatment of Hunter syndrome involves management of symptoms and complications. Symptoms Hunter syndrome is one type of a group of inherited metabolic disorders called mucopolysaccharidoses (MPSs), and Hunter syndrome is ...

  12. Adalimumab for the treatment of Crohn’s disease

    Directory of Open Access Journals (Sweden)

    Andrea Cassinotti

    2008-09-01

    Full Text Available Andrea Cassinotti, Sandro Ardizzone, Gabriele Bianchi PorroDepartment of Clinical Sciences, Chair of Gastroenterology, “Luigi Sacco” University Hospital, Milan, ItalyIntroduction: Crohn’s disease (CD is a chronic inflammatory bowel disease characterized by a relapsing-remitting course with trans-mural inflammation of potentially any section of the digestive tract. Adalimumab (ADA is a subcutaneously administered, recombinant, fully human, IgG1 monoclonal antibody that binds with high affinity and specificity to human TNF-alpha, thus modulating its biologic functions and its proinflammatory effects.Aims: To review the available data on ADA in CD for biological properties, efficacy, and safety.Methods: Electronic searches were conducted using the Pubmed and SCOPUS databases from the earliest records to April 2008. The search terms used were “adalimumab”, “anti-TNF”, “TNF-alpha”, “biologicals”, “inflammatory bowel disease”, and “Crohn’s disease”. Reference lists of all relevant articles were searched for further studies.Results: Available studies suggest that ADA has the potential to induce and maintain clinical response and remission in moderate-severe CD, both in anti-TNF-naïve patients and in subjects who lost their response and/or became intolerant to infliximab (IFX. ADA seems also effective in maintaining corticosteroid-free remission and obtaining complete fistula closure (although no specific randomized trials are available. No concomitant immunosuppressors seem to be necessary. Side effects appear similar to IFX, while site-injection reactions are frequent and specific. Data on immunogenicity and its clinical impact are uncertain.Conclusions: ADA appears to be effective in inducing and maintain clinical remission in CD, including patients not manageable with IFX. Successive clinical practice and further on going trials will confirm a positive role for ADA as a new anti-TNF treatment in CD. The impact on

  13. Long-term use of adalimumab in the treatment of rheumatic diseases

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    Charalampos Papagoras

    2009-05-01

    Full Text Available Charalampos Papagoras, Paraskevi V Voulgari, Alexandros A DrososRheumatology Clinic, Department of Internal Medicine, Medical School, University of Ioannina, Ioannina, GreeceAbstract: Adalimumab, a fully humanized monoclonal antibody against tumor necrosis factor-alpha (TNFα, has been evaluated in various randomized placebo-controlled trials in rheumatoid arthritis, ankylosing spondylitis, psoriatic arthritis and juvenile idiopathic arthritis. In the short time frame of these trials adalimumab has been shown to be effective in reducing disease activity, slowing radiographic disease progression and improving patients’ quality of life, while at the same time demonstrating an acceptable safety profile. Furthermore, release of adalimumab on the market, prospective observational studies, as well as open-label extensions of the original double-blind trials have provided experience and data about the long-term efficacy and safety of the drug. Initial effectiveness, in terms of reducing disease activity, is sustained, while in most cases patients treated with adalimumab experienced a slower radiographic progression and consequently less disability and improved health-related quality-of-life outcomes. Moreover, long-standing treatment of thousands of patients with adalimumab outside the controlled context of clinical trials was not related to new safety signals, with the most common adverse events being respiratory infections. The most common serious adverse events seem to be tuberculosis reactivation, while a putative association with malignant lymphoma development is not yet proven. Besides, both of these adverse reactions pertain to the whole TNFα blocker group. In conclusion, adalimumab is a safe and effective option for the treatment of patients with rheumatoid arthritis, ankylosing spondylitis, psoriatic arthritis and juvenile idiopathic arthritis. Keywords: adalimumab, tumor necrosis factor-alpha, rheumatoid arthritis, ankylosing spondylitis

  14. Efficacy of adalimumab in sarcoidosis patients who developed intolerance to infliximab.

    Science.gov (United States)

    Crommelin, Heleen A; van der Burg, Leone M; Vorselaars, Adriane D M; Drent, Marjolein; van Moorsel, Coline H M; Rijkers, Ger T; Deneer, Vera H M; Grutters, Jan C

    2016-06-01

    Tumor necrosis factor-alpha (TNF-α) inhibitors are regarded as the third-line therapy in sarcoidosis, the first choice generally being infliximab. To date, data regarding response to adalimumab in sarcoidosis patients intolerant to infliximab are lacking. The objective of this retrospective observational study was to establish if adalimumab could achieve stabilization or improvement of the disease in refractory sarcoidosis patients who developed intolerance to infliximab. Sarcoidosis patients referred to St Antonius Interstitial Lung Diseases Center of Excellence, Nieuwegein, The Netherlands, between January 2008 and April 2015 who switched from infliximab to adalimumab were included. Changes in organ function, inflammatory biomarker levels, and adverse events were retrieved from medical records. Out of 142 infliximab treated patients, 18 (13%) had to discontinue treatment due to antibody formation or severe adverse events and switched to adalimumab therapy. Organ function improved in 7 patients (39%), was stable in 6 patients (33%), and worsened in 5 patients (28%) after 12 months of treatment or after 6 months if evaluation after 12 months was not available (n = 4). In none of the patients biomarker levels of soluble interleukin-2 receptor (sIL-2R) deteriorated. Median decrease in sIL-2R was 3614 pg/mL. Most reported adverse event was infection (n = 10). Adalimumab is an effective alternative for patients intolerant to infliximab. The switch to adalimumab achieved clinical improvement in 39% and stabilization in 33% of patients intolerant to infliximab. Further research is needed to develop guidelines on how to use adalimumab for sarcoidosis in terms of dosing regimen. Copyright © 2016 Elsevier Ltd. All rights reserved.

  15. Temporal trends in the epidemiology, management, and outcome of patients with cardiogenic shock complicating acute coronary syndromes.

    Science.gov (United States)

    De Luca, Leonardo; Olivari, Zoran; Farina, Andrea; Gonzini, Lucio; Lucci, Donata; Di Chiara, Antonio; Casella, Gianni; Chiarella, Francesco; Boccanelli, Alessandro; Di Pasquale, Giuseppe; De Servi, Stefano; Bovenzi, Francesco Maria; Gulizia, Michele Massimo; Savonitto, Stefano

    2015-11-01

    Despite advances in the management of patients with acute coronary syndrome (ACS), cardiogenic shock (CS) remains the leading cause of death in these patients. We describe the evolution of clinical characteristics, in-hospital management, and outcome of patients with CS complicating ACS. We analysed data from five Italian nationwide prospective registries, conducted between 2001 and 2014, including consecutive patients with ACS. Out of 28 217 ACS patients enrolled, 1209 (4.3%) had CS: 526 (44%) at the time of admission and 683 (56%) later on during hospitalization. Over the years, a reduction in the incidence of CS was observed, even though this was not statistically significant (P for trend = 0.17). The proportions of CS patients with a history of heart failure declined, whereas the proportion of those with hypertension, renal dysfunction, previous PCI, and AF significantly increased. The use of PCI considerably increased from 2001 to 2014 [19% to 60%; percentage change 41, 95% confidence interval (CI) 29-51]. In-hospital mortality of CS patients decreased from 68% (95% CI 59-76) in 2001 to 38% (95% CI 29-47) in 2014 (percentage change -30, 95% CI -41 to -18). Compared with 2001, the risk of death was significantly lower in all of the registries, with reductions in adjusted mortality between 45% and 66%. Over the last 14 years, substantial changes occurred in the clinical characteristics and management of patients with CS complicating ACS, with a greater use of PCI and a significant reduction in adjusted mortality rate. © 2015 The Authors European Journal of Heart Failure © 2015 European Society of Cardiology.

  16. Apical ballooning syndrome complicated by acute severe mitral regurgitation with left ventricular outflow obstruction – Case report

    Directory of Open Access Journals (Sweden)

    Celermajer David S

    2007-02-01

    Full Text Available Abstract Background Apical ballooning syndrome (or Takotsubo cardiomyopathy is a syndrome of transient left ventricular apical ballooning. Although first described in Japanese patients, it is now well reported in the Caucasian population. The syndrome mimicks an acute myocardial infarction but is characterised by the absence of obstructive coronary disease. We describe a serious and poorly understood complication of Takotsubo cardiomyopathy. Case Presentation We present the case of a 65 year-old lady referred to us from a rural hospital where she was treated with thrombolytic therapy for a presumed acute anterior myocardial infarction. Four hours after thrombolysis she developed acute pulmonary oedema and a new systolic murmur. It was presumed she had acute mitral regurgitation secondary to a ruptured papillary muscle, ischaemic dysfunction or an acute ventricular septal defect. Echocardiogram revealed severe mitral regurgitation, left ventricular apical ballooning, and systolic anterior motion of the mitral valve with significant left ventricular outflow tract gradient (60–70 mmHg. Coronary angiography revealed no obstructive coronary lesions. She had an intra-aortic balloon pump inserted with no improvement in her parlous haemodynamic state. We elected to replace her mitral valve to correct the outflow tract gradient and mitral regurgitation. Intra-operatively the mitral valve was mildly myxomatous but there were no structural abnormalities. She had a mechanical mitral valve replacement with a 29 mm St Jude valve. Post-operatively, her left ventricular outflow obstruction resolved and ventricular function returned to normal over the subsequent 10 days. She recovered well. Conclusion This case represents a serious and poorly understood association of Takotsubo cardiomyopathy with acute pulmonary oedema, severe mitral regurgitaton and systolic anterior motion of the mitral valve with significant left ventricular outflow tract obstruction. The

  17. Clinical Features of Systemic Lupus Erythematosus Patients Complicated With Evans Syndrome: A Case-Control, Single Center Study.

    Science.gov (United States)

    Zhang, Lili; Wu, Xiuhua; Wang, Laifang; Li, Jing; Chen, Hua; Zhao, Yan; Zheng, Wenjie

    2016-04-01

    The aim of the study was to investigate the clinical features of systemic lupus erythematous (SLE) complicated with Evans syndrome (ES). We conducted a retrospective case-control study to compare the clinical and laboratory features of age- and gender-matched lupus patients with and without ES in 1:3 ratios. In 5724 hospitalized SLE patients, we identified 27 (0.47%, 22 women and 5 men, average age 34.2 years) SLE patients complicated with ES. Fifteen patients (55.6%) presented with hematologic abnormalities initially, including 6 (22.2%) cases of isolated ITP, 4 (14.8%) cases of isolated AIHA, and 5 (18.5%) cases of classical ES. The median intervals between hematological presentations the diagnosis of SLE was 36 months (range 0-252). ES developed after the SLE diagnosis in 4 patients (14.8%), and concomitantly with SLE diagnosis in 8 patients (29.6%). Systemic involvements are frequently observed in SLE patients with ES, including fever (55.6%), serositis (51.9%), hair loss (40.7%), lupus nephritis (37%), Raynaud phenomenon (33.3%), neuropsychiatric (33.3%) and pulmonary involvement (25.9%), and photosensitivity (25.9%). The incidence of photosensitivity, hypocomplementemia, elevated serum IgG level, and lupus nephritis in patients with ES or without ES was 25.9% vs 6.2% (P = 0.007), 88.9% vs 67.1% (P = 0.029), 48.1% vs 24.4% (P = 0.021), and 37% vs 64.2% (P = 0.013), respectively. Twenty-five (92.6%) patients achieved improvement following treatment of glucocorticoids and immunosuppressants as well as splenectomy, whereas 6 patients experienced the relapse and 1 patient died from renal failure during the follow-up. ES is a relatively rare complication of SLE. Photosensitivity, hypocomplementemia, and elevated serum IgG level were frequently observed in ES patients, but lupus nephritis was less observed. More than half of patients presented with hematological manifestation at onset, and progress to typical lupus over months to years. Therefore

  18. ADALIMUMAB FOR ULCERATIVE COLITIS: RESULTS OF A BRAZILIAN MULTICENTER OBSERVATIONAL STUDY

    Directory of Open Access Journals (Sweden)

    Patrícia ZACHARIAS

    2017-10-01

    Full Text Available ABSTRACT BACKGROUND: Adalimumab is a monoclonal antibody, tumor necrosis factor-alpha (TNFα inhibitor that has efficacy for inducing and maintaining remission in moderate-to-severe ulcerative colitis. Real world studies with adalimumab in Latin American ulcerative colitis patients are scarce. OBJECTIVE: To assess the clinical remission rates in induction and maintenance with adalimumab therapy in ulcerative colitis. METHODS: Observational, multicenter and retrospective study on a case series of patients with moderate-to-severe ulcerative colitis under adalimumab therapy. The variables analyzed were: demographic data, previous infliximab status, concomitant drugs, the Montreal Classification, disease activity (Mayo score at weeks 0, 8, 26 and 52, or until the last follow-up. Clinical remission was defined as a partial Mayo score ≤2 and Last observation carried forward (LOCF and Non responder imputation (NRI analysis were used. RESULTS: Thirty-six patients were included in the study. With LOCF analysis, remission rates at weeks 8, 26 e 52 were of 41.7%, 47.2% and 47.2%, respectively. With NRI analysis, remission rates at weeks 8, 26 and 52 were of 41.7%, 41.7% and 27.8%, respectively. CONCLUSION: Adalimumab was effective in the treatment of moderate-to-severe ulcerative colitis. Clinical remission was observed in approximately 40% of the patients at weeks 8 and 26, and in almost a quarter of the patients after 1 year of follow up.

  19. The impact of prior biologic therapy on adalimumab response in patients with rheumatoid arthritis.

    Science.gov (United States)

    Feuchtenberger, Martin; Kleinert, Stefan; Scharbatke, Eva-Christina; Gnann, Holger; Behrens, Frank; Wittig, Bianca M; Greger, Gerd; Tony, Hans-Peter

    2015-01-01

    The aim of this study is to use data from a non-interventional study of adalimumab in patients with rheumatoid arthritis (RA) during routine clinical practice to evaluate the impact of prior treatment with biologics on the effectiveness of current therapy. Efficacy parameters were evaluated for all patients with values at baseline and month 12. Subgroup analyses were performed on patients with 0, 1, or ≥2 prior biologic agents. Key outcome measures included Disease Activity Score- 28 joints (DAS28) and Funktionsfragebogen Hannover (FFbH) functional ability score. A total of 4700 RA adalimumab-treated patients were included in this analysis. Baseline disease activity increased with an increasing number of prior biologic agents and therapeutic response diminished. After 12 months of adalimumab therapy, DAS28 and FFbH scores showed improvements in all groups, but the group with 0 prior biologic agents had the best outcomes, while the group with ≥2 prior biologic agents had the worst. Clinical response (EULAR and DAS28-dcrit) and remission rates showed a similar pattern. Nevertheless, 44% to 67% of patients treated with ≥2 prior biologic agents achieved a clinical response. Multiple regression analyses identified prior biologic therapy as a significant negative predictor for response to therapy. Treatment with adalimumab leads to decreases in disease activity and improvements in function. Improvements are most pronounced in patients with 0 or 1 prior biologic agent, but a substantial proportion of patients treated with ≥2 prior biologic agents experience significant benefit from adalimumab therapy.

  20. Frequency of anesthesia-related complications in children with Down syndrome under general anesthesia for noncardiac procedures.

    Science.gov (United States)

    Borland, Lawrence M; Colligan, Jacqueline; Brandom, Barbara W

    2004-09-01

    Craniofacial and cardiac anomalies of Down syndrome (DS; trisomy 21) would seem to place these patients at higher risk of anesthesia-related complications (ARCs), but to date no comprehensive large-scale study has quantified this risk. A retrospective chart review was conducted on all patients with DS undergoing anesthesia between April 1, 1988, and May 31, 1995, at Children's Hospital of Pittsburgh. In addition, the Anesthesiology Department Quality Assurance (QA) System database of concurrently collected anesthesia information on all patients undergoing anesthesia at the hospital since 1985 was analyzed. Of the total 74,021 anesthetic encounters during the study period, 930 anesthetic encounters in 488 patients with DS undergoing noncardiac procedures were analyzed. The most frequent ARCs were bradycardia (severe) (3.66%), natural airway obstruction (1.83%), difficult intubation (0.54%), postintubation croup (1.83%), and bronchospasm (0.43%). Comprehensive reporting is needed to capture all significant adverse events. The incidences of bradycardia on induction, natural airway obstruction, and postintubation (or instrumentation) croup were significantly higher in the DS noncardiac group compared with the remaining population. Current anesthesia techniques and agents must be compared using quantitative QA data to ensure use of the safest options for each patient.

  1. A functional variant in the OAS1 gene is associated with Sjögren's syndrome complicated with HBV infection.

    Science.gov (United States)

    Liu, Xianjun; Xing, Hongcun; Gao, Wenjing; Yu, Di; Zhao, Yuming; Shi, Xiaoju; Zhang, Kun; Li, Pingya; Yu, Jiaao; Xu, Wei; Shan, Hongli; Zhang, Kaiyu; Bao, Wanguo; Fu, Xueqi; Yang, Sirui; Wang, Shafeng

    2017-12-14

    Hepatitis B virus (HBV) has been suspected to contribute to several autoimmune diseases, including Sjögren's syndrome (SS), although the exact mechanism is unknown. The 2'-5' oligoadenylate synthetase (OAS1) is one of the most important components of the immune system and has significant antiviral functions. We studied a polymorphism rs10774671 of OAS1 gene in Han Chinese descent. The minor allele G was significantly associated with a decreased risk for SS, anti-SSA-positive SS, and anti-SSA-positive SS complicated with HBV infection, which have not been seen in anti-SSA-negative SS and HBcAb-negative SS patients. Gene expression analysis showed that the risk-conferring A allele was correlated with lower expression of p46 and increased expression of p42, p48, and p44. A functional study of enzymatic activities revealed that the p42, p44, and p48 isoforms display a reduced capacity to inhibit HBV replication in HepG2 cells compared to the normal p46 isoform. Our data demonstrated that the functional variant, rs10774671, is associated with HBV infection and anti-SSA antibody-positive SS. The SAS variant switches the primary p46 isoform to three alternatives with decreased capacities to inhibit HBV replication. These data indicated that individuals harboring the risk allele might be susceptible to hepatitis B infection and SS development.

  2. Plasma Lipid Profiling of Patients with Chronic Ocular Complications Caused by Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis.

    Science.gov (United States)

    Saito, Kosuke; Ueta, Mayumi; Maekawa, Keiko; Sotozono, Chie; Kinoshita, Shigeru; Saito, Yoshiro

    2016-01-01

    Stevens-Johnson syndrome (SJS) and its severe variant, toxic epidermal necrolysis (TEN), are drug-induced acute inflammatory vesiculobullous reactions of the skin and mucous membranes, including the ocular surface. Even after recovery from skin symptoms, some SJS/TEN patients continue to suffer with severe ocular complications (SOCs). Therefore, this study aims to understand the pathophysiology of chronic SOCs. Because plasma lipid profiling has emerged as a useful tool to understand pathophysiological alterations in the body, we performed plasma lipid profiling of 17 patients who suffered from SJS/TEN-associated chronic SOCs. A lipidomics approach yielded 386 lipid molecules and demonstrated that plasma levels of inflammatory oxylipins increased in patients with SJS/TEN-associated chronic SOCs. In addition, oxidized phosphatidylcholines and ether-type diacylglycerols increased in the patients with chronic SOCs, while phosphoglycerolipids decreased. When we compared these lipidomic profiles with those of patients with atopic dermatitis, we found that patients with chronic SOCs, specifically, had decreased levels of ether-type phosphatidylcholines (ePCs) containing arachidonic acid (AA), such as PC(18:0e/20:4) and PC(20:0e/20:4). To confirm our finding, we recruited additional patients, who suffered from SOC associated with SJS/TEN (up to 51 patients), and validated the decreased plasma levels of AA-containing ePCs. Our study provides insight into the alterations of plasma lipidomic profiles in chronic SOCs and into the pathophysiology of SJS/TEN-associated chronic SOCs.

  3. Clinical and radiographic outcome of a treat-to-target strategy using methotrexate and intra-articular glucocorticoids with or without adalimumab induction

    DEFF Research Database (Denmark)

    Hørslev-Petersen, K; Hetland, M L; Ørnbjerg, L M

    2015-01-01

    . RESULTS: One year after adalimumab withdrawal, treatment profiles and clinical responses did not differ between groups. In the DMARD/DMARD+adalimumab groups, the median 2-year methotrexate dose was 20/20 mg/week (p=0.45), triple DMARD therapy had been initiated in 33/27 patients (p=0.49), adalimumab...

  4. [Life Threatening Complications in Patients With ST-Elevation Acute Coronary Syndrome-Dependence on Reperfusion Interventions (Data of Federal Register of Patients With Acute Coronary Syndrome)].

    Science.gov (United States)

    Oshchepkova, E V; Dmitriev, V V

    2016-04-01

    to study rate and type of life-threatening complications (LC) in patients with ST-Elevation (STE) Acute Coronary Syndrome (ACS) and their relation to implementation of reperfusion measures. Database of the Federal Register of patients with ACS (FRACS) which functioned from 01/01/2009 to 01/01/2014 contains information on 212304 patients with verified diagnosis of ACS. From this pool using random number generator we selected a cohort comprising 10348 patients with STEACS (60% men, mean age 63.5+/-0.1 clinical systolic and diastolic arterial pressure [AP] 135.2+/-0.3 and 81.9+/-0.2 mm Hg, respectively). Killip class was used for determination of degree of acute heart failure. Most frequent forms of LC were heart rhythm disturbances (6.2%) and cardiogenic shock (5.4%). Patients subjected to percutaneous coronary intervention (PCI) including those in whom pharmacoinvasive approach was used had less LC and lower hospital mortality than patients who received only thrombolytic therapy (TLT). Reperfusion measures were administered mostly to patients with class I-II acute heart failure. Hospital mortality was highest (9.47%) among patients not subjected to reperfusion measures and lowest (1.09%) among patients treated with PCI+TLT. FRACS assesses LC in patients with STEACS in the context of implemented reperfusion measures. Data of FRACS can be used by the health service authorities for improvement of medical aid to patients with STEACS.

  5. Developing a Therapeutic Range of Adalimumab Serum Concentrations in Management of Psoriasis: A Step Toward Personalized Treatment

    NARCIS (Netherlands)

    Menting, Stef P.; Coussens, Emma; Pouw, Mieke F.; van den Reek, Juul M. P. A.; Temmerman, Linda; Boonen, Hugo; de Jong, Elke M. G. J.; Spuls, Phyllis I.; Lambert, Jo

    2015-01-01

    Adalimumab has proven to be effective in suppressing psoriasis disease activity and is administered in a standard dose. To establish a therapeutic range for adalimumab trough levels in the treatment of plaque-type psoriasis, leading to a more personalized treatment. A multicenter, prospective,

  6. Improvement of Work Ability, Quality of Life, and Fatigue in Patients With Rheumatoid Arthritis Treated With Adalimumab

    NARCIS (Netherlands)

    Herenius, Marieke M. J.; Hoving, Jan L.; Sluiter, Judith K.; Raterman, Hennie G.; Lems, Willem F.; Dijkmans, Ben A. C.; Tak, Paul Peter; Nurmohamed, Mike T.; Voskuyl, Alexandre E.; Frings-Dresen, Monique H. W.

    2010-01-01

    Objective: To assess the effect of 12-month treatment with adalimumab on work ability, quality of life, and fatigue in patients with active rheumatoid arthritis ( RA). Methods: One hundred twenty-six patients with active RA started treatment with adalimumab. Primary outcome measurements were work

  7. Prospective comparison of preference and efficacy of adalimumab and infliximab for treating ulcerative colitis naive to antitumor necrosis factor therapy.

    Science.gov (United States)

    Mizoshita, Tsutomu; Katano, Takahito; Tanida, Satoshi; Hirano, Atsuyuki; Miyaki, Tomokatsu; Ozeki, Keiji; Suzuki, Yuka; Sugimura, Naomi; Kataoka, Hiromi; Joh, Takashi

    2017-08-01

    There have been few reports on 2 tumor necrosis factor alpha inhibitors, infliximab and adalimumab, with respect to patient preference and efficacy in ulcerative colitis (UC).We used questionnaires to evaluate the preference and reasons for drug choice between infliximab and adalimumab in UC patients naive to antitumor necrosis factor alpha therapy. We also analyzed the efficacy of infliximab and adalimumab prospectively and endoscopically before treatment and at 14 and 54 weeks.Of the 25 UC patients, infliximab and adalimumab were chosen by 10 (40%) and 15 (60%), respectively. Patients who favored infliximab considered "fear of syringes" (7/10, 70%) as the most important influencing factor, whereas patients who favored adalimumab considered "ease of administration" (10/15, 66.7%) and "time required for therapy" (10/15, 66.7%) as the most important factors. There were no statistical differences in remission induction and maintenance between the infliximab and adalimumab groups with regard to response, remission, mucosal healing, steroid-free, and steroid-free remission rates at weeks 14 and 54.The efficacy of adalimumab in remission induction and maintenance was equivalent to that of infliximab in UC patients naive to antitumor necrosis factor alpha therapy in this prospective study, but more patients preferred adalimumab.

  8. A Giant Hepatic Hemangioma Complicated by Kasabach-Merritt Syndrome: Findings of Tc-99m RBC Scintigraphy and SPECT Including a Total Body Blood Pool Imaging Study

    Energy Technology Data Exchange (ETDEWEB)

    Sohn, Myung Hee; Jeong, Hwan Jeong; Lim, Seok Tae; Kim, Dong Wook; Yim, Chang Yeol [Chonbuk National University Medical School, Jeonju (Korea, Republic of)

    2009-02-15

    Kasabach-Merritt syndrome (KMS) consists of thrombocytopenia, microangiopathic hemolytic anemia, and localized consumption coagulopathy that develops within vascular hemangioma. This syndrome may also be associated with occult hemangiomas located at various sites. Tc-99m RBC scintigraphy and SPECT have proven to be reliable for confirming or excluding hemangioma. Total body blood pool imaging study during the scintigraphy also provides a means of screening for occult lesions. The authors report the case of a 29-year-old man who presented with a giant hepatic hemangioma complicated by KMS, and underwent Tc-99m RBC scintigraphy and SPECT including a total body blood pool imaging study.

  9. Vascular Ehlers-Danlos Syndrome With a Novel Missense COL3A1 Mutation Present With Pulmonary Complications and Iliac Arterial Dissection.

    Science.gov (United States)

    Gu, Guangchao; Yang, Hang; Cui, Lijia; Fu, Yuanyuan; Li, Fangda; Zhou, Zhou; Zheng, Yuehong

    2018-02-01

    Vascular Ehlers-Danlos syndrome (vEDS) is a life-threatening connective tissue disorder due to its high tendency of arterial and organ rupture. Pulmonary complications in vEDS are rare. We present a young male patient with vEDS who developed severe pulmonary complications and severe rupture of the iliac artery at different stages of his life. Vascular Ehlers-Danlos syndrome was diagnosed based on clinical manifestations and confirmed by the identification of COL3A1 gene mutation. Due to high bleeding tendency and weak cardiopulmonary capacity, conservative treatment was taken for him. To our knowledge, this is the first report of vEDS case in which the patient developed both pulmonary complications and dissection of large arteries. Our report emphasizes the importance of considering vEDS when an adolescent develops unexplained pulmonary cysts with fragility of lung tissues. Genetic counseling and close monitoring should be performed for earlier diagnosis and prevention of severe complications of large arteries. The typical presentations of vEDS were also discussed by means of a review of case reports on vEDS with pulmonary complications.

  10. Asymptomatic choroidal tubercle in a patient with Crohn's disease on adalimumab treatment.

    Science.gov (United States)

    Asensio-Sánchez, V M; Díaz-Cabanas, L; Martín-Prieto, A; Haro-Álvarez, B

    2018-03-01

    Adalimumab, an anti-tumour necrosis factor alpha therapy for active Crohn's disease (CD), is associated with increased risks of tuberculosis infection. The case is presented of a 48 year-old male with active CD on treatment with adalimumab. After three months, he developed a miliary pulmonary tuberculosis infection, with a solitary non-reactive choroidal tubercle temporal-superior to the optic disc being found in an ophthalmological study. Fluorescein angiography showed late hyperfluorescence in a staining pattern. Optic coherence tomography showed a flat mass without serous retinal detachment. The choroidal tubercle slowly regressed with antituberculosis therapy. Choroidal tubercles with no vitreo-retinal symptomatology can be present in patients with CD and on treatment with adalimumab. Copyright © 2017 Sociedad Española de Oftalmología. Publicado por Elsevier España, S.L.U. All rights reserved.

  11. Acute and delayed hypersensitivity reactions to infliximab and adalimumab in a patient with Crohn's disease

    DEFF Research Database (Denmark)

    Steenholdt, Casper; Svenson, Morten; Bendtzen, Klaus

    2012-01-01

    A 61 year old woman with active luminal Crohn's disease was successfully treated with infliximab induction therapy followed by 5 infusions every 8 weeks. However, symptoms returned in the weeks preceding the 7th and 8th infusions. The 9th infusion was therefore given only 4 weeks after the 8th...... infusion, but an acute severe anaphylactoid reaction occurred immediately after start of the infusion. Anti-infliximab IgG antibody concentration was high (100 U/ml) prior to the 8th infusion and up to 1 year after infliximab discontinuation (81 U/ml). Anti-infliximab IgE antibodies were not found......, and the anti-infliximab antibodies did not cross react with adalimumab. One week after the anaphylactoid reaction to infliximab, adalimumab therapy was initiated. Twelve days after the first adalimumab administration (80 mg), a delayed hypersensitivity reaction occurred. This was likely caused by rapidly...

  12. Effectiveness of Adalimumab in Non-radiographic Axial Spondyloarthritis

    Science.gov (United States)

    Cantarini, Luca; Fabbroni, Marta; Talarico, Rosaria; Costa, Luisa; Caso, Francesco; Cuneo, Gian Luca; Frediani, Bruno; Faralli, Gabriele; Vitale, Antonio; Brizi, Maria Giuseppina; Sabadini, Luciano; Galeazzi, Mauro

    2015-01-01

    Abstract The primary aim of the study was to evaluate the long-term effectiveness of adalimumab (ADA) in a cohort of non-radiographic axial spondyloarthritis (nr-axSpA), and the secondary aims were to identify predictive factors of response and evaluate radiological progression. We evaluated 37 patients (male/female: 12/25; mean age 49 ± 14; mean disease duration: 6.3 ± 5.8) with active nr-axSpA (Assessment of SpondyloArthritis International Society criteria), despite the treatment with ≥1 nonsteroidal anti-inflammatory drug for at least 3 months, initiating the treatment with ADA 40 mg every other week. Patients were treated for 24 months, and evaluated at baseline, 6, 12, and 24 months. Outcome measures included Ankylosing Spondylitis Disease Activity Score, Bath Ankylosing Spondylitis Disease Activity Index (BASDAI), and Bath Ankylosing Spondylitis Functional Index. Radiograph of the spine and sacroiliac joints and magnetic resonance of the sacroiliac joints were performed at baseline and according to the standard of assessment for the disease. The proportion of patients that achieved a BASDAI50 response at 6, 12 and 24 months was 51.3%, 70.3%, and 76.8%, respectively. Treatment was well tolerated with no unexpected adverse events and/or serious adverse events. All patients remained on treatment for 2 years, with a good compliance. We did not identify any predictive factor of response to therapy. Moreover, modified Stoke Ankylosing Spondylitis Spine Score and Spondyloarthritis Research Consortium of Canada scores showed a trend of improvement during the study period. ADA was effective on clinical and radiological outcomes at 2-year follow-up; thus, early treatment with ADA may prevent radiographic damage and be associated with low disease activity or remission. Moreover, data from this cohort study have confirmed safety and tolerability profile of ADA in nr-axSpA in the long term. PMID:26222847

  13. Possible macrophage activation syndrome following initiation of ...

    African Journals Online (AJOL)

    Macrophage activation syndrome (MAS) has been rarely reported in the course of adult-onset Still's disease (AOSD) and in the majority of cases, it was triggered by an infection. Here, we report, to our knowledge, the first case of MAS occurring after adalimumab treatment initiation and not triggered by an infection.

  14. Concentrations of Adalimumab and Infliximab in Mothers and Newborns, and Effects on Infection

    DEFF Research Database (Denmark)

    Julsgaard, Mette; Christensen, Lisbet Ambrosius; Gibson, Peter R.

    2016-01-01

    and in umbilical cords, and in infants for every 3 months until the drug was no longer detected.  Results  The time from last exposure to anti-TNF agent during pregnancy correlated inversely with the concentration of the drugs in the umbilical cord (adalimumab: r = -0.64, P =.0003; infliximab: r = -0.77, P ... the drugs until 12 months of age. There was an inverse correlation between the time from last exposure during pregnancy and drug concentration in the umbilical cord. Infliximab was cleared more slowly than adalimumab from the infants. The combination of an anti-TNF agent and thiopurine therapy during...

  15. Pregnancy Complications: HELLP Syndrome

    Science.gov (United States)

    ... premature birth The newborn intensive care unit (NICU) Birth defects & other health conditions Loss & grief Tools & Resources Frequently asked health questions Calculating your due date Ovulation calendar Order bereavement materials News Moms Need Blog Stories & Media ...

  16. [Frequency and maternal complications of the criteria of hemolysis in preeclamptic patients with HELLP syndrome treated in an intensive care unit].

    Science.gov (United States)

    Vázquez-Rodríguez, J G; Ríos-Gutiérrez, C D; Paredes-Lozano, E P; García-Flores, A

    2016-01-01

    HELLP syndrome is an aggressive form of preeclampsia related with hemolysis and its complications. To determine the frequency of the appearance of criteria of hemolysis and maternal complications in preeclamptic patients with HELLP syndrome treated in an intensive care unit. We carried out a cross-sectional study in 50 preeclamptic women with HELLP syndrome admitted to intensive care unit to determine the presence of the following criteria of hemolysis: peripheral blood schistocytes, anemia (hemoglobin ≤ 10 g/dL), lactate dehydrogenase ≥ 600 U/L, indirect bilirubin ≥ 0.6 mg/dL and hemoglobinuria. We also studied maternal complications in patients with lactate dehydrogenase ≥ 600 U/L and positive for schistocytes. Descriptive (mean, median, range, standard deviation) and inferential (Student t test) statistics were used. Lactate dehydrogenase ≥ 600 U/L was found in 36%, indirect bilirubin 0.6 mg/dL in 20%, positive schistocytes in 16%, hemoglobinuria in 4% and anemia in 0%. Patients with lactate dehydrogenase ≥ 600 U/L had more clinical and laboratory deterioration as well as prolonged intensive care unit stay (p = 0.0025). Patients positive for schistocytes did not demonstrate adverse effects. Biochemical criteria of hemolysis were more frequent than schistocytes, hemoglobinuria and anemia. Patients with lactate dehydrogenase ≥ 600 U/L was alterations more serious than patients with positive schistocytes so its usefulness as a biomarker may be higher.

  17. Adalimumab administration after infliximab therapy is a successful treatment strategy for generalized pustular psoriasis.

    Science.gov (United States)

    Matsumoto, Ai; Komine, Mayumi; Karakawa, Masaru; Kishimoto, Megumi; Ohtsuki, Mamitaro

    2017-02-01

    We report a case of a 70-year-old woman with generalized pustular psoriasis (GPP) who responded well to infliximab therapy and adalimumab therapy after secondary failure of infliximab therapy, but did not respond to ustekinumab therapy. We speculate that the pathogenic factor in this case favored anti-tumor necrosis factor (TNF)-α therapy to anti-interleukin-12/23 therapy. Herein, we also briefly present three additional cases of treatment with adalimumab after secondary failure of infliximab. GPP is often difficult to treat, and no placebo-controlled trials have been conducted to guide the use of biologics against it because of a paucity of cases. Infliximab and adalimumab are anti-TNF-α antibodies that specifically block the interaction of TNF-α with its receptors. Infliximab has been reported to be effective, with a rapid clearance of symptoms, even in cases of severe GPP. Adalimumab could be a good biologic candidate that can be administrated after secondary failure of infliximab therapy. © 2016 Japanese Dermatological Association.

  18. Treatment persistence during therapeutic sequences with adalimumab and infliximab in the treatment of Crohn's disease

    Directory of Open Access Journals (Sweden)

    Carlos Taxonera

    Full Text Available Background and aim: Tumor necrosis factor (TNF inhibitors have demonstrated efficacy and safety in the treatment Crohn's disease (CD. However, the loss of response over time means that they are usually used sequentially. The aim of this study was to compare treatment persistence with different sequences of TNF inhibitors in patients with active luminal CD. Methods: A Markov model (3-month cycles was developed to simulate the therapeutic sequences of beginning biological treatment with infliximab or adalimumab, with a time horizon of three years. Each state of the model represented treatment (induction, standard dose or escalated dose with each TNF inhibitor or the state without biological treatment. The transition probabilities between states were determined by the clinical response to TNF inhibitors obtained from the literature. The likelihood of discontinuation due to adverse effects was also considered. Results: After three years, the percentage of CD patients receiving infliximab and adalimumab as a first TNF inhibitor that remained in treatment was 52.8% and 59.3% (p = 0.1 respectively. Median time to discontinuation of the standard dose was 26.26 months in patients who started with adalimumab and 24.39 months in patients who started with infliximab. Conclusion: In the model, there were no significant differences in persistence after three years with the initial drug among patients with active luminal CD starting treatment with infliximab or adalimumab.

  19. Adalimumab Dose Tapering in Psoriasis: Predictive Factors for Maintenance of Complete Clearance.

    Science.gov (United States)

    Hansel, Katharina; Bianchi, Leonardo; Lanza, Francesco; Bini, Vittorio; Stingeni, Luca

    2017-03-10

    Psoriasis can be managed successfully with long-term biologics. Real-life clinical practice may require dose tapering as a therapeutic option to reduce the risk of drug-exposure and to increase cost-effectiveness. The responsiveness to extended intervals between adalimumab doses and the possible predictive factors of maintenance of complete clearance were studied in a retrospective 7-year single-centre analysis. Thirty patients who achieved complete clearance with adalimumab underwent dose tapering, progressively extending between-dose intervals (to 21-28 days). Sixty percent of subjects (group A) maintained complete clearance, whereas 40.0% (group B) relapsed and were switched back to the standard dosage to re-achieve complete clearance. Body mass index (BMI) and time to achieve Psoriasis Area Severity Index (PASI-100) with adalimumab standard treatment before dose tapering were significantly lower in group A than in group B (multi-variate Cox regression: p < 0.05, Kaplan-Meier analysis: p < 0.001, respectively). This study suggests that patients with lower BMI and shorter time to achieve PASI-100 with adalimumab standard dose were significantly more likely to be candidates for dose tapering.

  20. False diagnosis of type 1 diabetes mellitus and its complications in Wolfram syndrome--is it the reason for the low number of reported cases of this abnormality?

    Science.gov (United States)

    Homa, Katarzyna; Stefański, Adam; Zmysłowska, Agnieszka; Molęda, Piotr; Bryśkiewicz, Marta Ewa; Majkowska, Liliana

    2014-01-01

    Wolfram syndrome (WS), also known as DIDMOAD (Diabetes Insipidus, Diabetes Mellitus, Optic Atrophy and Deafness), is a rare autosomal recessive syndrome (1/770,000 in the United Kingdom), characterised by juvenile onset of diabetes mellitus, optic nerve atrophy, diabetes insipidus, sensorineural deafness, renal tract and neurological abnormalities, and primary gonadal atrophy. WS is caused mainly by biallelic mutations in the WFS1 gene, which encodes wolframin. Wide tissue distribution of wolframin and many mutations in the wolframin gene resulting in Wolfram syndrome may contribute to different phenotypes and the unusual combinations of clinical features. We describe a female patient with Wolfram syndrome diagnosed at the age of 25, with a previous false diagnosis of type 1 diabetes mellitus and misdiagnosed diabetic complications. The patient was found to be a compound heterozygote for two novel mutations in exon 8 of WFS1 gene: a 2-bp deletion AT at nt 1539 leading to a frameshift (Y513fs) and a single-base substitution 1174C > T resulting in a stop codon (Q392X). A detailed analysis of the patient's medical history and a review of the literature suggest that many cases of Wolfram syndrome may remain undiagnosed due to misdiagnosis as type 1 diabetes mellitus and incorrect interpretation of clinical symptoms of neurodegenerative abnormalities, especially in their early stages.

  1. Observational study on the efficacy of adalimumab for the treatment of ulcerative colitis and predictors of outcome.

    Science.gov (United States)

    García-Bosch, Orlando; Gisbert, Javier P; Cañas-Ventura, Alex; Merino, Olga; Cabriada, José L; García-Sánchez, Valle; Gutiérrez, Ana; Nos, Pilar; Peñalva, Mireia; Hinojosa, Joaquin; García-Planella, Esther; Muñoz, Fernando; Calvet, Xavier; Panés, Julián

    2013-10-01

    Information on efficacy and predictors of response to adalimumab in ulcerative colitis (UC) clinical practice is limited. Assessment of response to adalimumab and its predictors in an observational cohort study. Retrospective cohort study based on data obtained from ENEIDA registry. All patients diagnosed with UC treated with adalimumab were included. Response to adalimumab was evaluated at weeks 12, 28, and 54 according to the partial Mayo score, and requirement of colectomy until end of follow-up. 48 patients with UC treated with adalimumab were included; 39 (81.3%) had previously received infliximab. Response rates at weeks 12, 28 and 54 were 70.8%, 43.2% and 35% respectively. Response to prior treatment with infliximab was the only predictive factor of response to adalimumab at week 12, which was obtained in 90% of infliximab remitters, 53.8% of responders and 33.3% of primary non-responders (p=0.01). Colectomy was required in 11 patients (22.9%), after a mean time of 205 days. The only clinical independent predictor of colectomy was non-response to adalimumab at week 12: colectomy rates were 5/34 (14.7%) in responders and 6/14 (42.9%) in non-responders (p=0.035), time free of colectomy was significantly reduced in non-responders (p=0.01). Adalimumab withdrawal due to adverse events occurred in 4.2% of patients. This study shows that adalimumab is an effective treatment in patients with UC. If used as a second anti-TNF, previous achievement of remission with the first anti-TNF predicts response, and failure to achieve response at week 12 predicts colectomy. Copyright © 2012 European Crohn's and Colitis Organisation. Published by Elsevier B.V. All rights reserved.

  2. Efficiency of adalimumab, etanercept and infliximab in ankylosing spondylitis in clinical practice.

    Science.gov (United States)

    Escudero-Vilaplana, Vicente; Ramírez-Herráiz, Esther; Alañón-Plaza, Estefanía; Trovato-López, Nicolás; García-Vicuña, Rosario; Carreño-Pérez, Luis; Morell-Baladrón, Alberto; Sanjurjo-Sáez, María

    2015-10-01

    Information on the use of ankylosing spondylitis (AS) therapies in clinical practice is a key factor in decision making, as more efficient treatments may involve substantial savings while maintaining the clinical benefits for the patient. To assess the mean annual doses and associated costs of the three main anti-tumour necrosis factor agents used in Spanish daily clinical practice in ankylosing spondylitis patients and to correlate these costs with disease activity. This retrospective, observational study included adult ankylosing spondylitis patients over a 4-year period that had been treated for at least 6 months with adalimumab, etanercept or infliximab at two University Hospitals in Spain. Disease activity was estimated with Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) scores at the start of anti-tumour necrosis factor (anti-TNF) therapy and in the last visit or whenever the drug was switched. Mean costs were estimated for a 52-week horizon from the delivered doses registered by pharmacy records. Outcomes were the doses and costs of anti TNFs administered to each patient, and the BASDAI score. A total of 119 patients (137 cases) were included (28 cases treated with adalimumab, 48 cases with etanercept and 61 with infliximab). Mean doses of adalimumab and etanercept were 92.8 and 88.8% of the initially prescribed doses, respectively, while the mean dose of infliximab administered was 102%. There were no statistical differences among treatments in terms of clinical effectiveness. Associated mean patient-year costs were significantly higher in the infliximab group (€14,235), compared to the other treatments [adalimumab €11,934; etanercept €10,516; (P ankylosing spondylitis patients, doses and associated costs of biological therapies can be reduced while controlling disease activity. Mean doses used in our clinical practice vary from the recommended doses and are significantly lower for adalimumab and etanercept than for infliximab. These

  3. Comparison of patients with avian influenza A (H7N9) and influenza A (H1N1) complicated by acute respiratory distress syndrome.

    Science.gov (United States)

    Li, Hongyan; Weng, Heng; Lan, Changqing; Zhang, Hongying; Wang, Xinhang; Pan, Jianguang; Chen, Lulu; Huang, Jinbao

    2018-03-01

    The aim of this study was to compare the clinical features of patients with avian influenza A (H7N9) and influenza A (H1N1) complicated by acute respiratory distress syndrome (ARDS).The clinical data of 18 cases of H7N9 and 26 cases of H1N1 with ARDS were collected and compared in the respiratory intensive care unit (RICU) of Fuzhou Pulmonary Hospital of Fujian from March 2014 to December 2016.Patients with H7N9 had a higher acute physiology and chronic health evaluation-II score (P H1N1 group (P H1N1 group. Patients with H7N9 had higher rates of invasive mechanical ventilation; serious complications, including alimentary tract hemorrhage, pneumothorax or septum emphysema, hospital-acquired pneumonia (HAP) and multiple organ dysfunction syndrome (MODS); and hospital mortality (P H1N1 group (P H1N1 influenza viral infection (odds ratio 8.29, 95% confidence interval [CI] 1.53-44.94; P H1N1, patients with H7N9 complicated by ARDS had much more severe disease. During long-term follow-up, more changes in pulmonary fibrosis were observed in patients with H7N9 than in patients with H1N1 during the convalescent stage.

  4. E valuation and clinical significance of serum C-reactive protein and homocysteine level in obstructive sleep apnea/hypopnea syndrome complicated with coronary heart disease patients

    International Nuclear Information System (INIS)

    Su Yingrui; Zha Jinshun; Xu Chaoxiang; Chen Xiaoyang; Wang Yaoguo; Du Xinqing

    2011-01-01

    Objective: To explore the relationship between homocysteine (HCY) and C-reactive protein (CRP) in obstructive sleep apnea/hypopnea syndrome (OSAHS) patients and OSAHS patients complicated with coronary heart disease by detecting the scrum level of HCY and CRP on the mechanism of OSAHS complicated with coronary heart disease. Methods: Ninety-one patients were divided into three groups, 30 patients as control group, 36 patients as OSAHS group, and 25 patients as OSAHS complicated with CHD group. Serum HCY level was detected through chemiluminescence. Serum CRP level was detected through radioimmunity. The serum level of HCY and CRP was compared among these groups. OSAHS patients were divided into mild OSAHS subgroup, moderate OSAHS subgroup and severe OSAHS subgroup. The morbidity rate of CHD and the serum level of HCY and CRP were compared among these subgroups. Meanwhile the parameters of polysomnogram such as activity apnea-hypopnea index (AHI) and blood oxygen saturation (SaO 2 ) were compared between OSAHS group and OSAHS complicated with coronary heart disease group. Results: (1) There was significant difference among the serum level of HCY and CRP of control group, OSAHS group and OSAHS complicated with CHD group (F HCY =15.80, F CRP =19.21, P all HCY =4.74, t CRP =5.14, P all HCY =7.31, t CRP =8.17, P all 2 =6.96, χ 2 =4.18, P HCY =16.38, F CRP =12.97, P all 2 of OSAHS group and OSAHS complicated with CHD group (t AHI =5.46, percentage of SaO 2 2 : t=4.68, average lowest SaO 2 : t=3.65, longest duration of disordered breathing events: t=4.73, P all<0.01 ). Conclusion: The serum level of HCY and CRP rose because of hypoxia in OSAHS patients,and might play an important role in the mechanism of OSAHS complicated with CHD. (authors)

  5. Complications of Sinusitis

    Science.gov (United States)

    ... Anatomy Virtual Anatomy Disclosure Statement CONDITIONS Adult Sinusitis Pediatric Sinusitis Fungal Sinusitis Sinusitis & Asthma Empty Nose Syndrome Cystic Fibrosis Sinusitis Q&A Complications of Sinusitis Epistaxis (Nosebleeds) Allergic Rhinitis (Hay Fever) Headaches and Sinus Disease Disorders of ...

  6. Complications of Sinus Surgery

    Science.gov (United States)

    ... Anatomy Virtual Anatomy Disclosure Statement CONDITIONS Adult Sinusitis Pediatric Sinusitis Fungal Sinusitis Sinusitis & Asthma Empty Nose Syndrome Cystic Fibrosis Sinusitis Q&A Complications of Sinusitis Epistaxis (Nosebleeds) Allergic Rhinitis (Hay Fever) Headaches and Sinus Disease Disorders of ...

  7. Formation of antibodies against infliximab and adalimumab strongly correlates with functional drug levels and clinical responses in rheumatoid arthritis

    DEFF Research Database (Denmark)

    Radstake, T R D J; Svenson, M; Eijsbouts, A M

    2008-01-01

    BACKGROUND: Tumour necrosis factor alpha (TNFalpha) neutralising antibody constructs are increasingly being used to treat rheumatoid arthritis (RA). OBJECTIVE: To determine potential differences in clinical responses, soluble drug levels and antibody formation between patients with RA receiving...... infliximab and adalimumab. METHODS: 69 patients with RA fulfilling the 1987 American College of Rheumatology criteria and about to start treatment with infliximab or adalimumab, were enrolled consecutively. All patients had active disease (28-joint count Disease Activity Score >3.2). Infliximab was given...... intravenously at 3 mg/kg at baseline and after 2, 6 and 14 weeks. Adalimumab was administered as 40 mg biweekly subcutaneously. Concomitant drug treatment was monitored and continued at constant dosage during the study. All serum samples were tested for infliximab/adalimumab levels and anti...

  8. Osler-Weber-Rendu syndrome complicated with pulmonary arteriovenous malformation: A case report and review of literatures

    Directory of Open Access Journals (Sweden)

    Kuan-Yu Wang

    2015-01-01

    Full Text Available Osler-Weber-Rendu syndrome is a hereditary disease which is diagnosed by criterions of clinical symptoms and examinations. Here, we report a definite case of Osler-Weber-Rendu syndrome who had epistaxis, skin telangiectasia, and pulmonary arteriovenous malformation (PAVM. His initial clinical presentations were growing pulmonary nodule with cough and occasional chest pain. PAVM with rupture of aneurysm was diagnosed histologically after wedge resection of the nodule in his right lower lung.

  9. Influence of Adalimumab on the Expression Profile of Genes Associated with the Histaminergic System in the Skin Fibroblasts In Vitro

    Science.gov (United States)

    Grabarek, Beniamin; Zmarzły, Nikola; Skubis, Aleksandra; Kruszniewska-Rajs, Celina; Gola, Joanna; Kucharz, Eugeniusz

    2018-01-01

    Objective The aim of this study was to evaluate the influence of adalimumab on expression profile of genes associated with the histaminergic system in Normal Human Dermal Fibroblast (NHDF) cells stimulated with 8.00 μg/ml of adalimumab and the identification of miRNAs regulating these genes' expression. Methods NHDFs were cultured with or without the presence of adalimumab for 2, 8, and 24 hours. The expression profile of genes and miRNA were determined with the use of microarray technology. Results Among 22283 ID mRNA, 65 are associated with the histaminergic system. It can be observed that 15 mRNAs differentiate NHDFs cultures with adalimumab form control. The analysis of miRNAs showed that, among 1105 ID miRNA, 20 miRNAs are differentiating in cells treated with adalimumab for 2 hours, 9 miRNA after 8 hours, and only 3 miRNAs after 24 hours. Conclusion It was also determined that miRNAs play certain role in the regulation of the expression of genes associated with the histaminergic system. The results of this study confirmed the possibility of using both genes associated with this system as well as miRNAs regulating their expression, as complementary molecular markers of sensitivity to the adalimumab treatment. PMID:29487864

  10. Immunogenicity, adalimumab levels and clinical response in ankylosing spondylitis patients during 24 weeks of follow-up.

    Science.gov (United States)

    Kneepkens, Eva L; Wei, James Cheng-Chung; Nurmohamed, Michael T; Yeo, Kai-Jieh; Chen, C Y; van der Horst-Bruinsma, Irene E; van der Kleij, Desiree; Rispens, Theo; Wolbink, Gertjan; Krieckaert, Charlotte L M

    2015-02-01

    Immunogenicity influences adalimumab levels and therefore clinical response in patients with rheumatic diseases. To study the relationship between clinical response, adalimumab levels and antidrug antibodies (ADAb) in ankylosing spondylitis (AS). Observational cohort study of 115 consecutive AS patients treated with adalimumab in the Netherlands (n=85) and Taiwan (n=30), monitored during 24 weeks. Adalimumab levels and ADAb titres were determined using an ELISA and an antigen binding test (ABT), respectively, designed by Sanquin Research, Amsterdam. Response to adalimumab treatment was defined as a Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) response, and disease activity was measured using the Ankylosing Spondylitis Disease Activity Score using C-reactive protein (CRP) (ASDAS). At baseline, median BASDAI (IQR) was 6.4 (4.5-7.6) and mean ASDAS (SD) was 3.5 (1.0). After 24 weeks, 49 (42.6%) patients were BASDAI50 responders and mean ASDAS (SD) for responders was 1.5 (1.0) vs 2.6 (1.0) for non-responders (p100 AU/mL). In these patients, CRP and erythrocyte sedimentation rate remained elevated during treatment. Adalimumab levels are related to clinical response in AS patients measured with ASDAS and are influenced by ADAb detectable with an ABT. 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.

  11. Effect of Adalimumab on Refractory Arthritis in Juvenile Idiopathic Inflammatory Myopathy with Anti-MDA5 Autoantibody

    Directory of Open Access Journals (Sweden)

    Takako Miyamae

    2018-01-01

    Full Text Available A 10-year-old girl manifested persistent fever, skin rash, leg pain, fatigue, and joint pain. Based on muscle weakness, elevated muscle-derived enzymes, magnetic resonance imaging, and skin biopsy results, the diagnosis was juvenile idiopathic inflammatory myopathies (JIIM. Chest CT was normal; the anti-melanoma differentiation-associated protein-5 (anti-MDA5 autoantibody was positive. Initial manifestations subsided after prednisolone (PSL and methotrexate treatment. After the PSL dosage was decreased, the patient presented with metacarpophalangeal (MCP joint pain and swelling in both index fingers, synovial fluid, and signals on power Doppler ultrasound. The arthritis was refractory to cyclosporine and tacrolimus. Radiography showed progressive MCP joint space narrowing and joint erosion. Adalimumab was initiated 14 months after disease onset. There was a mildly increased matrix metalloproteinase-3 (MMP3 level, an erythrocyte sedimentation ratio (ESR, and a normal CRP level. Adalimumab resulted in decreased MCP joint pain and swelling. PSL was discontinued 10 months after adalimumab initiation; after 9 more months of adalimumab, there were no significant ultrasonography findings. MMP3 and ESR levels normalized during treatment. Radiography after 2 years of adalimumab showed further progressive MCP joint space narrowing restricting dorsiflexion. This report clarified that anti-MDA5-positive JIIM joint manifestations were due to active synovitis and that adalimumab is required for severe cases. Further experience is needed to determine the pathology, severity, and prognosis of this type of arthritis.

  12. Long-Term Risk for Aortic Complications After Aortic Valve Replacement in Patients With Bicuspid Aortic Valve Versus Marfan Syndrome.

    Science.gov (United States)

    Itagaki, Shinobu; Chikwe, Joanna P; Chiang, Yuting P; Egorova, Natalia N; Adams, David H

    2015-06-09

    Bicuspid aortic valves are associated with valve dysfunction, ascending aortic aneurysm and dissection. Management of the ascending aorta at the time of aortic valve replacement (AVR) in these patients is controversial and has been extrapolated from experience with Marfan syndrome, despite the absence of comparative long-term outcome data. This study sought to assess whether the natural history of thoracic aortopathy after AVR in patients with bicuspid aortic valve disease is substantially different from that seen in patients with Marfan syndrome. In this retrospective comparison, outcomes of 13,205 adults (2,079 with bicuspid aortic valves, 73 with Marfan syndrome, and 11,053 control patients with acquired aortic valve disease) who underwent primary AVR without replacement of the ascending aorta in New York State between 1995 and 2010 were compared. The median follow-up time was 6.6 years. The long-term incidence of thoracic aortic dissection was significantly higher in patients with Marfan syndrome (5.5 ± 2.7%) compared with those with bicuspid valves (0.55 ± 0.21%) and control group patients (0.41 ± 0.08%, p Marfan syndrome (10.8 ± 4.4%) compared with those with bicuspid valves (4.8 ± 0.8%) and control group patients (1.4 ± 0.2%) (p Marfan syndrome were significantly more likely to undergo thoracic aortic surgery in late follow-up (10.4 ± 4.3%) compared with those with bicuspid valves (2.5 ± 0.6%) and control group patients (0.50 ± 0.09%) (p Marfan syndrome compared with those with bicuspid aortic valves confirm that operative management of patients with bicuspid aortic valves should not be extrapolated from Marfan syndrome and support discrete treatment algorithms for these different clinical entities. Copyright © 2015 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

  13. Cerebral tuberculoma in a patient receiving anti-TNF alpha (adalimumab) treatment.

    LENUS (Irish Health Repository)

    Lynch, Karen

    2010-10-01

    We report a case of a cerebral tuberculoma in a 60-year-old woman with rheumatoid arthritis while receiving the anti-tumor necrosis factor alpha monoclonal antibody, adalimumab (Humira), for active disease. MR brain imaging for dyspraxia revealed a left parietal ring-enhancing lesion, which on resection was shown to be a necrotizing granuloma. There were no associated pulmonary lesions, and the patient was systemically well. Sputum and urine cultures were negative for tuberculosis. The patient was treated with anti-tuberculous medications and made an excellent recovery. We consider this to be the first documented case of tuberculosis involving the central nervous system occurring in the setting of adalimumab treatment.

  14. Economic Impact of Above-Label Dosing with Etanercept, Adalimumab, or Ustekinumab in Patients with Psoriasis.

    Science.gov (United States)

    Feldman, Steven R; Zhao, Yang; Zhou, Huanxue; Herrera, Vivian; Tian, Haijun; Li, Yunfeng

    2017-05-01

    Patients with moderate-to-severe psoriasis may be treated with above-label doses of biologics in an attempt to optimize outcomes. Dose escalation will have an effect on the cost of treatment. To examine costs related to above-label use of etanercept, adalimumab, and ustekinumab among patients with moderate-to-severe psoriasis. A retrospective study was performed using a large U.S. claims database. Patients were included in the study if they were aged ≥ 18 years with a diagnosis of psoriasis (excluding psoriatic arthritis) and had at least 1 medication fill for etanercept, adalimumab, or ustekinumab between January 1, 2011, and June 30, 2012. In addition, patients were required to have continuous enrollment for 12 months before, and 18 months after, the first biologic use (index biologic) during the maintenance period (defined as the period following the induction period in which each agent was titrated to its recommended maintenance dose per label) and at least 1 prescription filled for the index biologic during the 18 months after the maintenance period. Extensive above-label use was defined as taking an above-label dose (at least 10% higher than indicated in the label) for ≥ 180 days over a 12-month period following the maintenance period. Percentages of patients with extensive above-label use, mean number of days of above-label use, and additional costs associated with extensive above-label use (abovelabel cost minus on-label cost) were examined. The study included 3,310 patients who started treatment with etanercept (n = 1,443), adalimumab (n = 1,447), or ustekinumab (n = 420). Extensive above-label use occurred in 20.0% of etanercept patients, 2.6% of adalimumab patients, and 14.8% of ustekinumab patients. The mean duration of extensive above-label use was roughly similar for the 3 biologics (mean days [±SD]: 282 [±55] for etanercept, 279 [±57] for adalimumab, and 305 [±43] for ustekinumab). Additional annual costs per patient because of extensive

  15. Antibodies to Infliximab and Adalimumab in Patients with Rheumatoid Arthritis in Clinical Remission

    DEFF Research Database (Denmark)

    Eng, Grith P; Bendtzen, Klaus; Bliddal, Henning

    2015-01-01

    Objective. To investigate if antibodies towards biological TNF-α inhibitors (anti-TNFi Abs) are present in patients with rheumatoid arthritis (RA) in clinical remission and to relate any anti-TNFi Abs to circulating level of TNF-α inhibitor (TNFi). Methods. Patients with RA, treated with infliximab....../44 patients (18%) treated with infliximab and 1/49 patients (2%) treated with adalimumab (p = 0.012). In the former group, anti-TNFi Abs corresponded with low levels of TNFi (p = 0.048). Anti-TNFi Ab-positive patients had shorter disease duration at initiation of TNFi therapy (p = 0.023) but were similar...... for the rest of the compared parameters. Conclusions. In RA patients in clinical remission, anti-TNFi Abs occur frequently in patients treated with infliximab, while they occur rarely in patients treated with adalimumab. Presence of anti-infliximab Abs is accompanied by low or undetectable levels of infliximab...

  16. [Adalimumab induction and maintenance therapy for Crohn's disease. An open-label study].

    Science.gov (United States)

    López Palacios, N; Mendoza, J L; Taxonera, C; Lana, R; Fuentes Ferrer, M; Díaz-Rubio, M

    2008-11-01

    adalimumab has been shown in placebo-controlled clinical trials and uncontrolled studies to be effective in luminal and perianal fistulizing CD. to evaluate the efficacy and safety of adalimumab for induction and maintenance therapy in CD. twenty-two patients with CD treated with adalimumab (16 for luminal disease and 6 for active perianal fistulizing disease) were included. Twenty-one patients had previously received IFX. All patients received induction therapy with 160 mg s.c. at week 0, and 80 mg s.c. at week 2. Responders received maintenance therapy with 40 mg s.c. every 14 days. Response was assessed at 4 weeks after the initial dose, and classified as remission, partial response, or non-response. after induction, 25% of patients with luminal disease had a complete remission, and 56.3% had a partial response. Clinical response was maintained in 71.6% of patients at 1 year, in 53.7% at 18 months, and in 35.8% at 48 months. No differences in response were observed between patients with hypersensitivity reactions or loss of response to IFX.All patients with perianal fistulizing disease (n = 6) had been previously treated with IFX. After induction 16.7% entered remission, and 66.7% had a partial response. All patients maintained remission or response over time, with a median follow-up of 15 months. adalimumab is an effective and safe treatment for the induction and maintenance of response in luminal and perianal fistulizing CD. These results confirm that the findings obtained in controlled clinical trials are reproducible in clinical practice.

  17. Comparative Effectiveness of Etanercept and Adalimumab in Patient Reported Outcomes and Injection-Related Tolerability.

    Directory of Open Access Journals (Sweden)

    Iris Navarro-Millán

    Full Text Available To describe patient preferences in selecting specific biologics and compare clinical response using patient reported outcomes (PROs among patients with rheumatoid arthritis (RA started on different anti-tumor necrosis factor (TNF therapies.Participants were enrollees in Kaiser Permanente Northern California. Patients with RA who had at least two provider visits and started a new anti-TNF therapy from 10/2010-8/2011, were eligible for participation in this longitudinal study. Using a telephone survey, patient preferences in biologic selection and RAPID3, MDHAQ, and SF-12 scores were collected at baseline and at 6 months. Patient scores rating injection/infusion-site burning and stinging (ISBS were collected at 6 months.In all, 267 patients with RA responded to the baseline survey, of whom 57% preferred an injectable biologic, 22% preferred an infused biologic, and 21% had no preference. Motivation for injectable biologics was convenience (92% and for infusion therapy was dislike or lack of self-efficacy for self-injection (16%. After 6 months of treatment with anti-TNF, 70% of the 177 patients who answered the ISBS question reported ISBS with the last dose; on a scale of 1 (none to 10 (worst, 41% of these reported a score of 2-5; and 29% reported a score of 6-10. Adalimumab users experienced 3.2 times (95% confidence interval 1.2-8.6 the level of ISBS that etanercept users experienced. There were no significant differences in RAPID3, MDHAQ, or SF-12 scores between etanercept or adalimumab initiators.Convenience and fear of self-injection were important considerations to patients selecting a biologic drug. Although more convenient, adalimumab associated with more ISBS than did etanercept, and this rate was higher than reported in clinical trials. At 6 months, PROs did not differ between etanercept and adalimumab users.

  18. Pre- and perinatal complications in relation to Tourette syndrome and co-occurring obsessive-compulsive disorder and attention-deficit/hyperactivity disorder.

    Science.gov (United States)

    Abdulkadir, Mohamed; Tischfield, Jay A; King, Robert A; Fernandez, Thomas V; Brown, Lawrence W; Cheon, Keun-Ah; Coffey, Barbara J; de Bruijn, Sebastian F T M; Elzerman, Lonneke; Garcia-Delgar, Blanca; Gilbert, Donald L; Grice, Dorothy E; Hagstrøm, Julie; Hedderly, Tammy; Heyman, Isobel; Hong, Hyun Ju; Huyser, Chaim; Ibanez-Gomez, Laura; Kim, Young Key; Kim, Young-Shin; Koh, Yun-Joo; Kook, Sodahm; Kuperman, Samuel; Lamerz, Andreas; Leventhal, Bennett; Ludolph, Andrea G; Madruga-Garrido, Marcos; Maras, Athanasios; Messchendorp, Marieke D; Mir, Pablo; Morer, Astrid; Münchau, Alexander; Murphy, Tara L; Openneer, Thaïra J C; Plessen, Kerstin J; Rath, Judith J G; Roessner, Veit; Fründt, Odette; Shin, Eun-Young; Sival, Deborah A; Song, Dong-Ho; Song, Jungeun; Stolte, Anne-Marie; Tübing, Jennifer; van den Ban, Els; Visscher, Frank; Wanderer, Sina; Woods, Martin; Zinner, Samuel H; State, Matthew W; Heiman, Gary A; Hoekstra, Pieter J; Dietrich, Andrea

    2016-11-01

    Pre- and perinatal complications have been implicated in the onset and clinical expression of Tourette syndrome albeit with considerable inconsistencies across studies. Also, little is known about their role in co-occurring obsessive-compulsive disorder (OCD) and attention-deficit/hyperactivity disorder (ADHD) in individuals with a tic disorder. Therefore, we aimed to investigate the role of pre- and perinatal complications in relation to the presence and symptom severity of chronic tic disorder and co-occurring OCD and ADHD using data of 1113 participants from the Tourette International Collaborative Genetics study. This study included 586 participants with a chronic tic disorder and 527 unaffected family controls. We controlled for age and sex differences by creating propensity score matched subsamples for both case-control and within-case analyses. We found that premature birth (OR = 1.72) and morning sickness requiring medical attention (OR = 2.57) were associated with the presence of a chronic tic disorder. Also, the total number of pre- and perinatal complications was higher in those with a tic disorder (OR = 1.07). Furthermore, neonatal complications were related to the presence (OR = 1.46) and severity (b = 2.27) of co-occurring OCD and also to ADHD severity (b = 1.09). Delivery complications were only related to co-occurring OCD (OR = 1.49). We conclude that early exposure to adverse situations during pregnancy is related to the presence of chronic tic disorders. Exposure at a later stage, at birth or during the first weeks of life, appears to be associated with co-occurring OCD and ADHD. Copyright © 2016 Elsevier Ltd. All rights reserved.

  19. Pre- and perinatal complications in relation to Tourette syndrome and co-occurring obsessive-compulsive disorder and attention-deficit/hyperactivity disorder

    Science.gov (United States)

    Abdulkadir, Mohamed; Tischfield, Jay A.; King, Robert A.; Fernandez, Thomas V.; Brown, Lawrence W.; Cheon, Keun-Ah; Coffey, Barbara J.; de Bruijn, Sebastian F. T. M.; Elzerman, Lonneke; Garcia-Delgar, Blanca; Gilbert, Donald L.; Grice, Dorothy E.; Hagstrøm, Julie; Hedderly, Tammy; Heyman, Isobel; Hong, Hyun Ju; Huyser, Chaim; Ibanez-Gomez, Laura; Kim, Young Key; Kim, Young-Shin; Koh, Yun-Joo; Kook, Sodahm; Kuperman, Samuel; Lamerz, Andreas; Leventhal, Bennett; Ludolph, Andrea G.; Madruga-Garrido, Marcos; Maras, Athanasios; Messchendorp, Marieke D.; Mir, Pablo; Morer, Astrid; Münchau, Alexander; Murphy, Tara L.; Openneer, Thaïra J. C.; Plessen, Kerstin J.; Rath, Judith J. G.; Roessner, Veit; Fründt, Odette; Shin, Eun-Young; Sival, Deborah A.; Song, Dong-Ho; Song, Jungeun; Stolte, Anne-Marie; Tübing, Jennifer; van den Ban, Els; Visscher, Frank; Wanderer, Sina; Woods, Martin; Zinner, Samuel H.; State, Matthew W.; Heiman, Gary A.; Hoekstra, Pieter J.; Dietrich, Andrea

    2016-01-01

    Pre- and perinatal complications have been implicated in the onset and clinical expression of Tourette syndrome albeit with considerable inconsistencies across studies. Also, little is known about their role in co-occurring obsessive-compulsive disorder (OCD) and attention–deficit/hyperactivity disorder (ADHD) in individuals with a tic disorder. Therefore, we aimed to investigate the role of pre- and perinatal complications in relation to the presence and symptom severity of chronic tic disorder and co-occurring OCD and ADHD using data of 1,113 participants from the Tourette International Collaborative Genetics study. This study included 586 participants with a chronic tic disorder and 527 unaffected family controls. We controlled for age and sex differences by creating propensity score matched subsamples for both case-control and within-case analyses. We found that premature birth (OR=1.72) and morning sickness requiring medical attention (OR=2.57) were associated with the presence of a chronic tic disorder. Also, the total number of pre- and perinatal complications was higher in those with a tic disorder (OR=1.07). Furthermore, neonatal complications were related to the presence (OR=1.46) and severity (b=2.27) of co-occurring OCD and also to ADHD severity (b=1.09). Delivery complications were only related to co-occurring OCD (OR=1.49). We conclude that early exposure to adverse situations during pregnancy is related to the presence of chronic tic disorders. Exposure at a later stage, at birth or during the first weeks of life, appears to be associated with co-occurring OCD and ADHD. PMID:27494079

  20. Comparative efficacy of golimumab, infliximab, and adalimumab for moderately to severely active ulcerative colitis: a network meta-analysis accounting for differences in trial designs.

    Science.gov (United States)

    Thorlund, Kristian; Druyts, Eric; Toor, Kabirraaj; Mills, Edward J

    2015-05-01

    To conduct a network meta-analysis (NMA) to establish the comparative efficacy of infliximab, adalimumab and golimumab for the treatment of moderately to severely active ulcerative colitis (UC). A systematic literature search identified five randomized controlled trials for inclusion in the NMA. One trial assessed golimumab, two assessed infliximab and two assessed adalimumab. Outcomes included clinical response, clinical remission, mucosal healing, sustained clinical response and sustained clinical remission. Innovative methods were used to allow inclusion of the golimumab trial data given the alternative design of this trial (i.e., two-stage re-randomization). After induction, no statistically significant differences were found between golimumab and adalimumab or between golimumab and infliximab. Infliximab was statistically superior to adalimumab after induction for all outcomes and treatment ranking suggested infliximab as the superior treatment for induction. Golimumab and infliximab were associated with similar efficacy for achieving maintained clinical remission and sustained clinical remission, whereas adalimumab was not significantly better than placebo for sustained clinical remission. Golimumab and infliximab were also associated with similar efficacy for achieving maintained clinical response, sustained clinical response and mucosal healing. Finally, golimumab 50 and 100 mg was statistically superior to adalimumab for clinical response and sustained clinical response, and golimumab 100 mg was also statistically superior to adalimumab for mucosal healing. The results of our NMA suggest that infliximab was statistically superior to adalimumab after induction, and that golimumab was statistically superior to adalimumab for sustained outcomes. Golimumab and infliximab appeared comparable in efficacy.

  1. The Value of Vedolizumab as Rescue Therapy in Moderate-Severe Crohn's Disease Patients with Adalimumab Non-response in the USA.

    Science.gov (United States)

    Erim, Daniel O; Mahendraratnam, Nirosha; Okafor, Phillip N; Wheeler, Stephanie B

    2015-08-01

    In May 2014, vedolizumab was approved by the Food and Drug Administration for the treatment of moderate-to-severe Crohn's disease. In clinical practice it is typically used in patients who are primary or secondary non-responders to adalimumab [Humira]. We aim to estimate the incremental benefits and costs of using vedolizumab as rescue therapy for adalimumab non-responders. A Markov model was used to simulate the clinical course of Crohn's disease in a hypothetical cohort of 10,000 patients over a 12-month period. The treatment strategies evaluated were adalimumab only [with and without dose intensification] and adalimumab and vedolizumab [with and without adalimumab dose intensification]. The base case strategy was adalimumab only with 25% of non-responders undergoing dose intensification. Our primary outcomes were changes in costs and quality of life measures over the analytical horizon. In a 1-year period, initiating vedolizumab as rescue therapy in adalimumab non-responders reduces the average total cost per patient by 10%, and increases the average amount of time spent in remission or mild disease by up to 2 months. Treating on-label adalimumab non-responders with vedolizumab can, in the short term, significantly improves the quality of life of Crohn's disease patients that do not respond to adalimumab. Copyright © 2015 European Crohn’s and Colitis Organisation (ECCO). Published by Oxford University Press. All rights reserved. For permissions, please email: journals.permissions@oup.com.

  2. Developments in the treatment of moderate to severe ulcerative colitis: focus on adalimumab

    Directory of Open Access Journals (Sweden)

    Freeman HJ

    2013-11-01

    Full Text Available Hugh J FreemanDepartment of Medicine (Gastroenterology, University of British Columbia, Vancouver, BC, Canada Abstract: In selected patients with moderate to severe active ulcerative colitis who have failed to respond or are poorly responsive to standard pharmacologic forms of treatment with corticosteroids and immunosuppressive agents, therapy with a biological agent may be considered. While infliximab is an established tumor necrosis factor blocker and has a longer history of clinical use, adalimumab is an alternative in the same class and may be employed as an initial biological agent, if indicated for treatment of the disease. Adalimumab may have special appeal to stable users able to self-inject in a home setting rather than a centralized infusion center. Short-term adverse effects have been limited, but long-term adverse events can be serious and remain less well defined. Recently, another agent, subcutaneous golimumab, has also been reported to induce and maintain clinical response and remission in clinical trials, but a large experience has not been accumulated to date in clinical practice. In the future, other biological agents with novel and distinct mechanisms of therapeutic action may become available. Keywords: ulcerative colitis, anti-tumor necrosis factor inhibitors, biological therapy, infliximab, adalimumab

  3. Severe alcohol withdrawal syndrome: Evolution of care and impact of adjunctive therapy on course and complications of 171 intensive care unit patients.

    Science.gov (United States)

    Puscas, Mircea; Hasoon, Mohammed; Eechevarria, Carlos; Cooper, Tracy; Tamura, Leslie; Chebbo, Ahmad; W Carlson, Richard

    2016-01-01

    This single site retrospective observational study assessed the evolution of sedation therapy for severe alcohol withdrawal syndrome in the intensive care unit. Patient records for 2 intervals were reviewed: Interval 1, which included 87 intensive care unit patients admitted January 2005 through September 2007, for whom benzodiazedpine monotherapy was utilized; and Interval 2, January 2010 through December 2010, for whom 54 of 84 (64.3%) intensive care unit patients, including all those intubated, received adjunctive agents, including dexmedetomidine or propofol. Clinical management was similar for both intervals, as well as prevalence of alcohol withdrawal syndrome versus total adult hospital admissions and comorbid conditions. Overall, respiratory failure (53 versus 39%), seizures (36 versus 18%), and pneumonia (51 versus 38%) were less frequent during Interval 2 (all p intensive care unit admission are excluded, the prevalence of these complications was similar (p = ns) for Interval 1 and Interval 2. Intensive care unit and hospital length of stay were not altered by adjunctive therapy, which was typically employed for more severely affected patients. High intensity sedation with adjunctive drugs led to few cardiovascular adverse events and may have facilitated management, but did not alter intensive care unit course of severe alcohol withdrawal syndrome.

  4. Increased Need for Gastrointestinal Surgery and Increased Risk of Surgery-Related Complications in Patients with Ehlers-Danlos Syndrome: A Systematic Review.

    Science.gov (United States)

    Kulas Søborg, Marie-Louise; Leganger, Julie; Rosenberg, Jacob; Burcharth, Jakob

    2017-01-01

    Ehlers-Danlos syndromes (EDSs) constitute a rare group of inherited connective tissue diseases, characterized by multisystemic manifestations and general tissue fragility. Most severe complications include vascular and gastrointestinal (GI) emergencies requiring acute surgery. The purpose of this systematic review was to assess the causes of GI-related surgery and related mortality and morbidity in patients with EDSs. A systematic search was conducted in PubMed, Embase, and Scopus to identify relevant studies. Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines for systematic reviews were followed. According to eligibility criteria, data were extracted and systematically screened by 2 authors. Screening process identified 11 studies with a total of 1,567 patients. Findings indicated that patients with EDSs had a higher occurrence of surgery demanding GI manifestations, including perforation, hemorrhage, rupture of intra-abdominal organs, and rectal prolapse. Most affected was the vascular subtype, of which up to 33% underwent GI surgery and suffered from a lowered average life expectancy of 48 years (range 6-78). Secondary complications of surgery were common in all patients with EDSs. Studies suggested that patients with EDSs present an increased need for GI surgery, but also an increased risk of surgery-related complications, most predominantly seen in the vascular subtype. © 2016 S. Karger AG, Basel.

  5. Complicated Crohn's-like colitis, associated with Hermansky-Pudlak syndrome, treated with Infliximab: a case report and brief review of the literature

    Directory of Open Access Journals (Sweden)

    Kouklakis George

    2007-12-01

    Full Text Available Abstract Introduction Hermansky-Pudlak syndrome (HPS is a rare autosomal recessive inherited disorder consisting of a triad of albinism, increased bleeding tendency secondary to platelet dysfunction, and systemic complications associated with ceroid depositions within the reticuloendothelial system. HPS has been associated with gastrointestinal (GI complications related to chronic granulomatous colitis with pathologic features suggestive of Crohn's disease. This colitis can be severe and has been reported to be poorly responsive to medical therapies including antibiotics, corticosteroids, sulfasalazine, mesalamine and azathioprine. Case presentation We report a patient with HPS which was complicated by inflammatory bowel disease with clinical and pathologic features of Crohn's disease, refractory to antibiotics, corticosteroids and azathioprine. A trial of infliximab was attempted and repeated infusions produced a complete response. Conclusion The occurrence of ileitis and perianal lesions and also the histopathological findings in our case suggest that HPS and Crohn's disease may truly be associated. Given this similarity and the failure of the standard medical therapy of corticosteroids and azathioprine, our patient received infliximab with marked clinical improvement.

  6. [Clinical effect of high-frequency oscillatory ventilation combined with pulmonary surfactant in treatment of neonatal severe meconium aspiration syndrome complicated by pulmonary hemorrhage].

    Science.gov (United States)

    Huang, Jing; Lin, Xin-Zhu; Zheng, Zhi

    2016-11-01

    To study the clinical effect and safety of high-frequency oscillatory ventilation (HFOV) combined with pulmonary surfactant (PS) in the treatment of neonatal severe meconium aspiration syndrome (MAS) complicated by neonatal pulmonary hemorrhage (NPH). A total of 48 children with severe MAS complicated by NPH were enrolled, and a retrospective analysis was performed for the clinical effects of HFOV+PS (trial group, 25 children) and HFOV alone (control group, 23 children). The blood gas parameters, oxygenation index (OI), PaO 2 /FiO 2 (P/F) value, duration of pulmonary hemorrhage, ventilation time, length of hospital stay, incidence of complications, and outcome were compared between the two groups. At 6, 12, 24, and 48 hours after treatment, the trial group had significantly better PaO 2 , OI, and P/F value than the control group (Phemorrhage (P0.05). HFOV combined with PS can better improve oxygenation function and shorten the duration of NPH and ventilation time. Meanwhile, it does not increase the incidence of adverse events. Therefore, it is a safe and effective therapy.

  7. Risk-benefit analysis of adalimumab versus traditional non-biologic therapies for patients with Crohn's disease.

    Science.gov (United States)

    Loftus, Edward V; Johnson, Scott J; Wang, Si-Tien; Wu, Eric; Mulani, Parvez M; Chao, Jingdong

    2011-01-01

    Adalimumab is indicated for the treatment of moderately to severely active Crohn's disease (CD). A systematic analysis of risks and benefits of adalimumab versus traditional non-biologic therapies for patients refractory to non-biologic therapy is lacking. A base-case analysis compared expected benefits of adalimumab therapy with a 12-week stopping rule for non-responders versus non-biologic therapies using data from clinical trials (CHARM, CLASSIC I). Adverse events (AEs) recorded in clinical trials (CHARM, CLASSIC I, CLASSIC II, GAIN, open-label extensions) were compiled. Sensitivity analyses incorporated all observed benefits of adalimumab and placebo (CHARM, CLASSIC I, GAIN) and observed AEs from a systematic literature review of non-biologic therapies (MEDLINE search of randomized trials 1990-2007). Distributional information from maintenance clinical trial observations and benefit model predictions were used in a probabilistic simulation. Incremental net benefits were estimated based on utility estimates from the literature. Average time in remission (i.e., CDAI traditional non-biologic therapies. Adalimumab was associated with fewer expected hospitalizations, better fistula closure rates, and lower AE rates. These findings were robust in sensitivity analyses. In the probabilistic simulation, with serious AEs as a composite of risks, adalimumab provided greater benefits with fewer AEs versus non-biologic therapies (P therapies. Adalimumab demonstrated greater benefits and lower rates of AEs versus traditional non-biologic therapies for patients with moderately to severely active CD who were refractory to non-biologic therapies. Copyright © 2010 Crohn's & Colitis Foundation of America, Inc.

  8. Treatment changes in patients with psoriasis on etanercept or adalimumab

    DEFF Research Database (Denmark)

    Lee, Erica B; Amin, Mina; Egeberg, Alexander

    2018-01-01

    , 2004 and September 8, 2017 was conducted. The first treatment change and reason for that change was documented. RESULTS: Of 140 patients, 103 patients had treatment changes, most commonly due to inefficacy, however complicating events and changes due to patient preference were also seen. CONCLUSION......: Although biologics are considered the gold standard for psoriasis, clinicians should still take individual variations in response and preferences into account....

  9. [Surgical treatment of duodenal ulcer associated with Mallory-Weiss syndrome and sliding hiatal hernia and complicated by hemorrhage].

    Science.gov (United States)

    Zaĭtsev, V T; Boĭko, V V; Donets, N P; Taraban, I A; Groma, V G

    1997-01-01

    Of 1061 patients with an acute gastro-intestinal haemorrhage the combination of duodenal ulcer disease, Mallory-Weiss syndrome and sliding hiatal hernia was noted in 22 (2.07%). Surgical treatment consisted of the bleeding ulcer excision, cardia defects suturing, lowering of the stomach acid production performing one kind of vagotomy, external and internal decompression of stomach accomplishment (nasogastric tube and pyloroplasty), correction of anatomic-topographic interrelations of cardio-esophageal zone (cruroraphy, fundoplication).

  10. Obesity Hypoventilation Syndrome

    Science.gov (United States)

    ... Home / Obesity Hypoventilation Syndrome Obesity Hypoventilation Syndrome Also known as Pickwickian Syndrome What ... your neck is larger than normal. Complications of Obesity Hypoventilation Syndrome When left untreated, OHS can cause ...

  11. Developing a Therapeutic Range of Adalimumab Serum Concentrations in Management of Psoriasis: A Step Toward Personalized Treatment.

    Science.gov (United States)

    Menting, Stef P; Coussens, Emma; Pouw, Mieke F; van den Reek, Juul M P A; Temmerman, Linda; Boonen, Hugo; de Jong, Elke M G J; Spuls, Phyllis I; Lambert, Jo

    2015-06-01

    Adalimumab has proven to be effective in suppressing psoriasis disease activity and is administered in a standard dose. To establish a therapeutic range for adalimumab trough levels in the treatment of plaque-type psoriasis, leading to a more personalized treatment. A multicenter, prospective, observational, daily practice cohort study conducted at an academic hospital with affiliated secondary care hospitals in Belgium (cohort 1) and 2 academic hospitals in the Netherlands (cohort 2). Both cohorts included adult patients treated with adalimumab for plaque-type psoriasis. Cohort 1 comprised 73 patients who were being treated with adalimumab for more than 24 weeks until 401 weeks. In cohort 2 (n = 62), serum samples were obtained between weeks 24 and 52 of treatment. Before the start of adalimumab therapy and at time of serum sampling, Psoriasis Area and Severity Index (PASI) scores were determined. Adalimumab trough level and PASI score at the time of serum sampling to determine the receiver-operator characteristics analyses and concentration effect curve. By means of receiver-operator characteristics analyses with an area under the curve of 0.756 (SD, 0.046; 95% CI, 0.666-0.847) and a sensitivity of 78% and a specificity of 70%, 3.51 mg/L was established as the lower margin for the therapeutic range. By means of a concentration effect curve, 7 mg/L was established as the upper margin. One-third of patients had an adalimumab trough concentration exceeding 7 mg/L. A therapeutic range of adalimumab trough levels of 3.51 mg/L to 7.00 mg/L, which corresponds to an optimal clinical effect, was identified. In one-third of patients, it was observed that trough concentrations exceeded the therapeutic window. Based on the established range, a therapeutic algorithm for adalimumab treatment for patients with psoriasis can be developed and validated in a prospective patient cohort. By identifying this range, a step has been taken toward a more rational use of biological

  12. Le syndrome d'ogilvie post c?sarienne: une complication myst?rieuse: ? propos d'un cas

    OpenAIRE

    Amourak, Sarah; Tayae, Mariam; Jayi, Sofia; Alaoui, Fatimazahra Fdili; Bouguern, Hakima; Chaara, Hikmat; Melhouf, Moulay Abdelilah

    2014-01-01

    Le syndrome d'ogilvie ou appeler encore Pseudo-occlusion colique aigu? (acute colonic pseudo-obstruction), d?crit par Sir William Ogilvie en 1948 et correspond ? une dilatation aigu? du colon ant?rieurement sain, survenant en l'absence d'obstruction m?canique avec un diam?tre c?cal > 9 cm, La symptomatologie correspond ? celle d'une occlusion intestinale basse, d'installation rapide et l'imagerie fait d'ASP et de TDM abdominale permet de mettre en ?vidence la distension c?cale dont la mesure ...

  13. Real-Life Treatment Paradigms Show Adalimumab Is Cost-Effective for the Management of Ulcerative Colitis.

    Science.gov (United States)

    Beilman, Candace L; Thanh, Nguyen Xuan; Ung, Victoria; Ma, Christopher; Wong, Karen; Kroeker, Karen I; Lee, Thomas; Wang, Haili; Ohinmaa, Arto; Jacobs, Phil; Halloran, Brendan P; Fedorak, Richard N

    2016-01-01

    Background . Adalimumab is effective for the maintenance of remission in patients with moderate-to-severe ulcerative colitis (UC). Currently, biologic therapies are used in cases where patients fail conventional medical therapies. If biologic therapies are not available, patients often choose to remain in an unwell state rather than undergo colectomy. Objective . The aim of the study was to evaluate the cost-effectiveness of adalimumab in patients with UC where adalimumab was readily available compared to not available. Methods . A previously validated Markov model was used to simulate disease progression of patients with UC who are corticosteroid-dependent and/or did not respond to thiopurine therapy. Utility scores and transition probabilities between health states were determined by using data from randomized controlled trials and real-life observational studies. Costs were obtained from the Ontario Case Costing Initiative and the Alberta Health Schedule of Medical Benefits. Results . The incremental cost-effectiveness ratios for readily available adalimumab treatment of UC were $40,000 and $59,000 per quality-adjusted life year, compared with ongoing medical therapy in an unwell state, at 5-year and 10-year treatment time horizons, respectively. Conclusion . Considering real-life patient preferences to avoid colectomy, adalimumab is cost-effective according to a willingness-to-pay threshold of $80,000 for treatment of UC.

  14. Real-Life Treatment Paradigms Show Adalimumab Is Cost-Effective for the Management of Ulcerative Colitis

    Directory of Open Access Journals (Sweden)

    Candace L. Beilman

    2016-01-01

    Full Text Available Background. Adalimumab is effective for the maintenance of remission in patients with moderate-to-severe ulcerative colitis (UC. Currently, biologic therapies are used in cases where patients fail conventional medical therapies. If biologic therapies are not available, patients often choose to remain in an unwell state rather than undergo colectomy. Objective. The aim of the study was to evaluate the cost-effectiveness of adalimumab in patients with UC where adalimumab was readily available compared to not available. Methods. A previously validated Markov model was used to simulate disease progression of patients with UC who are corticosteroid-dependent and/or did not respond to thiopurine therapy. Utility scores and transition probabilities between health states were determined by using data from randomized controlled trials and real-life observational studies. Costs were obtained from the Ontario Case Costing Initiative and the Alberta Health Schedule of Medical Benefits. Results. The incremental cost-effectiveness ratios for readily available adalimumab treatment of UC were $40,000 and $59,000 per quality-adjusted life year, compared with ongoing medical therapy in an unwell state, at 5-year and 10-year treatment time horizons, respectively. Conclusion. Considering real-life patient preferences to avoid colectomy, adalimumab is cost-effective according to a willingness-to-pay threshold of $80,000 for treatment of UC.

  15. Adalimumab (TNFα Inhibitor Therapy Exacerbates IgA Glomerulonephritis Acute Renal Injury and Induces Lupus Autoantibodies in a Psoriasis Patient

    Directory of Open Access Journals (Sweden)

    S. S. Wei

    2013-01-01

    Full Text Available Adalimumab (Humira is a tumour necrosis factor α (TNFα inhibitor that is approved for the treatment of rheumatoid arthritis, psoriasis, psoriatic arthritis, Crohn's disease, ankylosing spondylitis, and juvenile idiopathic arthritis (Sullivan and Preda (2009, Klinkhoff (2004, and Medicare Australia. Use of TNFα inhibitors is associated with the induction of autoimmunity (systemic lupus erythematosus, vasculitis, and sarcoidosis or sarcoid-like granulomas (Ramos-Casals et al. (2010. We report a patient with extensive psoriasis presenting with renal failure and seropositive lupus markers without classical lupus nephritis after 18 months treatment with adalimumab. He has renal biopsy proven IgA nephritis instead. Renal biopsy is the key diagnostic tool in patients presenting with adalimumab induced nephritis and renal failure. He made a remarkable recovery after adalimumab cessation and steroid treatment. To our knowledge, this is a unique case of a psoriasis patient presenting with seropositive lupus markers without classical lupus nephritis renal failure and had renal biopsy proven IgA glomerulonephritis after receiving adalimumab.

  16. Psoriasis in autoimmune polyendocrine syndrome type I: A possible complication or a non-endocrine minor component?

    Directory of Open Access Journals (Sweden)

    Shital Amin Poojary

    2015-01-01

    Full Text Available Introduction: Autoimmune polyendocrine syndrome type I (APS I is an autosomal recessive systemic autoimmune disorder, affecting primarily endocrine glands, in which chronic mucocutaneous candidiasis is an early and prominent manifestation. We describe the rare occurrence of unstable psoriasis (with onset of pustular lesions in a case of APS I without mucocutaneous candidiasis. A patient presenting with unstable psoriasis (with onset of pustular lesions was detected to have persistent hypocalcemia which led to the diagnosis of hypoparathyroidism. Subsequently he was found to have hypergonadotrophic hypogonadism, primary adrenal insufficiency (compensated, and coeliac disease, thus confirming the diagnosis of APS I. Psoriasis is very rarely reported in APS I, possibly due to the protective effect of antibodies to Th17 cytokines, which are responsible for the occurrence of candidiasis in this syndrome. However, psoriasis could occur in APS I patients without mucocutaneous candidiasis, who lack these antibodies. In our patient, possible factors aggravating psoriasis include hypocalcemia due to hypoparathyroidism as well as coeliac disease via anti-tissue transglutaminase antibodies. However, defining psoriasis as a possible minor component of APS I would require further studies of the autoimmune regulator (AIRE gene functions.

  17. Long-term outcome of childhood-onset complicated nephrotic syndrome after a multicenter, double-blind, randomized, placebo-controlled trial of rituximab.

    Science.gov (United States)

    Kamei, Koichi; Ishikura, Kenji; Sako, Mayumi; Aya, Kunihiko; Tanaka, Ryojiro; Nozu, Kandai; Kaito, Hiroshi; Nakanishi, Koichi; Ohtomo, Yoshiyuki; Miura, Kenichiro; Takahashi, Shori; Morimoto, Tetsuji; Kubota, Wataru; Ito, Shuichi; Nakamura, Hidefumi; Iijima, Kazumoto

    2017-11-01

    Although rituximab effectively prevents relapses of complicated frequently relapsing nephrotic syndrome (FRNS) and steroid-dependent nephrotic syndrome (SDNS), data of long-term outcomes and safety are limited. Fifty-one patients (age, 3-38 years) with childhood-onset complicated FRNS or SDNS, who received rituximab in investigator-initiated multicenter prospective trials were enrolled. Rituximab was administered at 375 mg/m 2 once weekly for 4 weeks, and immunosuppressive agents were discontinued according to the study protocol. We investigated relapses, re-administration of immunosuppressive agents, additional rituximab treatment, body height, renal function, and late adverse events during the observation period. Forty-eight patients (94%) developed relapses during the observation period (median, 59 months) and the 50% relapse-free survival was 261 days. Thirty patients (59%) developed SDNS, 44 (86%) required re-administration of immunosuppressive agents, and 22 (43%) received additional rituximab treatment. All patients who were receiving immunosuppressive agents at rituximab treatment required either immunosuppressive agents or additional rituximab treatment. On the contrary, 5 of the 13 patients without immunosuppressive agents at rituximab treatment required neither immunosuppressive agents nor additional rituximab treatment and 3 of them did not develop relapse during observation period. Growth failure due to steroid toxicity did not progress and none of the patients developed chronic renal insufficiency. None of the patients suffered from rituximab-related late adverse events. As most patients suffer from relapses after B-cell recovery, long-term immunosuppressive agents or additional rituximab treatment is necessary. However, some patients who can discontinue immunosuppressive agents before rituximab treatment may achieve long-term remission after rituximab treatment without immunosuppressive agents.

  18. Successful adalimumab treatment of a psoriasis vulgaris patient with hemodialysis for renal failure: A case report and a review of the previous reports on biologic treatments for psoriasis patients with hemodialysis for renal failure.

    Science.gov (United States)

    Kusakari, Yoshiyuki; Yamasaki, Kenshi; Takahashi, Toshiya; Tsuchiyama, Kenichiro; Shimada-Omori, Ryoko; Nasu-Tamabuchi, Mei; Aiba, Setsuya

    2015-07-01

    The efficacy and safety of biologic treatments have been established in patients with moderate to severe psoriasis, but there are few reports on biologic therapy for patients with psoriasis complicated by end-stage renal failure on hemodialysis (HD). In this report, we demonstrated the efficacy and safety of adalimumab for patients with severe psoriasis on HD. A 46-year-old Japanese man with a 14-year history of psoriasis was referred to our clinic in September 2009. He had developed hypertension and renal failure during a 7-year history of cyclosporin treatment. With the infliximab treatment, he achieved 75% improvement of the Psoriasis Area and Severity Index (PASI) score within 3 months from the PASI of 42.3 before the treatment. However, his renal failure gradually deteriorated, and HD was initiated at 1 year after the introduction of infliximab. Because of hydration during the i.v. injection of infliximab, he developed pulmonary edema with every infliximab treatment after starting HD. We switched to ustekinumab treatment, but his psoriasis was not improved. Then, we switched to adalimumab and achieved a PASI-100 response within 2 months. The patient received adalimumab treatment for more than a year without any adverse effects. In addition to our case, five articles reported cases of psoriasis patients with renal failure on HD who were treated with biologics. The psoriatic lesions were improved by biologics in these cases, and no severe adverse effects on the renal function were reported. Thus, biologics are a reasonable treatment option for patients with severe psoriasis with renal failure on HD. © 2015 Japanese Dermatological Association.

  19. Trends in the management and outcomes of patients admitted with acute coronary syndrome complicated by cardiogenic shock over the past decade: Real world data from the acute coronary syndrome Israeli survey (ACSIS).

    Science.gov (United States)

    Kalmanovich, Eran; Blatt, Alex; Brener, Svetlana; Shlezinger, Meital; Shlomo, Nir; Vered, Zvi; Hod, Hanoch; Goldenberg, Ilan; Elbaz-Greener, Gabby

    2017-06-27

    Registries and other cohorts have demonstrated that early revascularization improve the survival of patients presenting with Cardiogenic Shock (CS) completing Aute coronary syndrome (ACS). Our aim was to describe the change in the clinical characteristics of these patients and their management and their outcome. The study population comprised 224 patients who were admitted with ACS complicated by cardiogenic shock who were enrolled in the prospective biannual Acute Coronary Syndrome Israeli Surveys (ACSIS) between 2000 and 2013 (1.7% of all patients admitted with ACS during the study period). Survey periods were categorized as early (years 2000-2004) and late (year 2006-2013).The rate of cardiogenic shock complicated ACS declined from 1.8% between the years 2000-2004 to 1.5% during the years 2006-2013. The clinical presentation in both the early and late groups was similar. During the index hospitalization primary percutaneous coronary intervention (PPCI) was more frequently employed during the late surveys [31% vs. 58% (ppatients enrolled in the early surveys, those enrolled in the late survey group experienced significantly lower mortality rates at 7-days (44% vs. 30%, respectively; p=0.03). However, this difference was no longer statistically significant at 30-days (52.8% vs. 46.4%, respectively, p=0.34) and 1-year (63% vs. 53.2%, respectively, p=0.14). Similarly, the rate of major adverse cardiac events (MACE) at 30-days was similar between the two groups (57.4% vs. 47.4%, respectively, p=0.13).Our findings indicate that patients admitted with ACS complicated by cardiogenic shock still experience very high rates of MACE and mortality during follow-up, despite a significant increase in the use of PPCI in this population over the past decade.

  20. Clinical effects of pulse high-volume hemofiltration on severe acute pancreatitis complicated with multiple organ dysfunction syndrome.

    Science.gov (United States)

    Chu, La-Ping; Zhou, Jun-Jing; Yu, Ya-Fen; Huang, Yang; Dong, Wen-Xia

    2013-02-01

    To evaluate the effects of pulse high-volume hemofiltration (PHVHF) on severe acute pancreatitis (SAP) with multiple organ dysfunction syndrome (MODS). Thirty patients were divided into two groups: PHVHF group and continuous venovenous hemofiltration (CVVH) group. They were evaluated in terms of clinical symptoms, acute physiology and chronic health evaluation (APACHE) II score, sequential organ failure assessment (SOFA) score, simplified acute physiology (SAPS) II score and biochemical changes. The levels of IL-6, IL-10 and TNF-α in plasma were assessed by ELISA before and after treatment. The doses of dopamine used in shock patients were also analyzed. In the two groups, symptoms were markedly improved after treatment. Body temperature (BT), breath rate (BR), heart rate (HR), APACHE II score, SOFA score, SAPS II score, serum amylase, white blood cell count and C-reactive protein were decreased after hemofiltration (P treatment of SAP with MODS. © 2012 The Authors. Therapeutic Apheresis and Dialysis © 2012 International Society for Apheresis.

  1. Thymoma-associated multi-organ autoimmunity: A case of graft-versus-host disease-like erythroderma complicated by Good syndrome successfully treated by thymectomy.

    Science.gov (United States)

    Fukushima, Ayano; Ichimura, Yoshiko; Obata, Shoko; Kinoshita-Ise, Misaki; Fujio, Yumi; Takeno, Mitsuhiro; Konohana, Izumi

    2017-07-01

    Thymoma-associated multi-organ autoimmunity disease (TAMA) is a rare paraneoplastic disorder, clinicopathologically similar to graft-versus-host disease (GVHD). Many reported cases follow a difficult course; half of them die from serious infectious diseases subsequent to immunosuppression induced by chemotherapy for unresectable thymoma, or intensive therapies including systemic steroids for complicating autoimmune diseases and GVHD-like symptoms. We report a patient whose skin symptoms were improved subsequently to total thymectomy. The patient also presented with hypogammaglobulinemia, which led to the diagnosis of complicated Good syndrome. Taking account of her immunodeficient condition, antibiotics and i.v. immunoglobulin were administrated promptly on onset of bacterial pneumonia, which was successfully treated. According to a review of the published work, treatments with systemic steroids for skin symptoms have limited effects and may contribute to serious infection. Our case indicates that successful treatment of thymoma itself may lead to the amelioration of the disease. The management priority should be given to the treatment of thymoma and the control of subsequent immune abnormality other than GVHD-like erythroderma. © 2017 Japanese Dermatological Association.

  2. A Summation Analysis of Compliance and Complications of Compression Hosiery for Patients with Chronic Venous Disease or Post-thrombotic Syndrome.

    Science.gov (United States)

    Kankam, Hadyn K N; Lim, Chung S; Fiorentino, Francesca; Davies, Alun H; Gohel, Manj S

    2018-03-01

    Compression stockings are commonly prescribed for patients with a range of venous disorders, but are difficult to don and uncomfortable to wear. This study aimed to investigate compliance and complications of compression stockings in patients with chronic venous disease (CVD) and post-thrombotic syndrome (PTS). A literature search of the following databases was carried out: MEDLINE (via PubMed), EMBASE (via OvidSP, 1974 to present), and CINAHL (via EBSCOhost). Studies evaluating the use of compression stockings in patients with CVD (CEAP C2-C5) or for the prevention or treatment of PTS were included. After scrutinising full text articles, compliance with compression and associated complications were assessed. Compliance rates were compared based on study type and degree of compression. Good compliance was defined as patients wearing compression stockings for >50% of the time. From an initial search result of 4303 articles, 58 clinical studies (37 randomised trials and 21 prospective studies) were selected. A total of 10,245 limbs were included, with compression ranging from 15 to 40 mmHg (not stated in 12 studies) and a median follow-up of 12 months (range 1-60 months). In 19 cohorts, compliance was not assessed and in a further nine, compliance was poorly specified. Overall, good compliance with compression was reported for 5371 out of 8104 (66.2%) patients. The mean compliance, weighted by study size, appeared to be greater for compression ≤25 mmHg (77%) versus > 25 mmHg (65%) and greater in the randomised studies (74%) than in prospective observational studies (64%). Complications of stockings were not mentioned in 43 out of 62 cohorts reviewed. Where complications were considered, skin irritation was a common event. In published trials, good compliance with compression is reported in around two thirds of patients, with inferior compliance in those given higher degrees of compression. Further studies are required to identify predictors of non

  3. Efficacy of adalimumab for the treatment of extraintestinal manifestations of Crohn's disease Eficacia del adalimumab en el tratamiento de las manifestaciones extraintestinales de la enfermedad de Crohn

    Directory of Open Access Journals (Sweden)

    Manuel Barreiro-de-Acosta

    2012-09-01

    Full Text Available Background and aims: Crohn's disease (CD is commonly associated with inflammatory processes located in organs and systems others than the gut, which are known as extraintestinal manifestations (EIM of the disease. The aim of this study was to assess the effectiveness of adalimumab (ADA for the treatment of EIM in patients with CD. Methods: forty two consecutive CD patients with at least one EIM were prospectively included in a open-label study. Patients received ADA (160 mg at week zero, 80 mg at week two and 40 mg every other week over six months and the effectiveness and safety of ADA for EIMs were assessed. The influence of gender, age, smoking habits, family history of inflammatory bowel disease, phenotype and previous anti-TNF treatment on EIM resolution was also investigated. Results: at month six, 76.2% of the patients showed remission or response in CD (33.3% remission and 42.9% any response. EIM showed a parallel course with CD in most cases, and showed remission or response in 66.7% of patients (38.1% remission and 28.5% any response. Patients with any response of their EIM condition were younger than those with no response (p = 0.04. No relationship was found between sex, tobacco, family history of IBD, phenotype and previous treatment with anti-TNF, and EIM resolution. Conclusions: adalimumab is effective in reducing EIM of CD. Age but not tobacco, CD phenotype and anti-TNF-naïve status appears to influence the response.

  4. Clinical status, quality of life, and work productivity in Crohn's disease patients after one year of treatment with adalimumab.

    Science.gov (United States)

    Saro, Cristina; Ceballos, Daniel; Muñoz, Fernando; de la Coba, Cristóbal; Aguilar, María Dolores; Lázaro, Pablo; García-Sánchez, Valle; Hernández, Mariola; Barrio, Jesús; de Francisco, Ruth; Fernández, Luis I; Barreiro-de Acosta, Manuel

    2017-02-01

    Clinical trials have shown the efficacy of adalimumab in Crohn's disease, but the outcome in regular practice remains unknown. The aim of the study was to examine clinical status, quality of life, and work productivity of Crohn's disease patients receiving adalimumab for one year in the context of usual clinical practice. This was a prospective, observational study with a one-year follow-up. After baseline, Crohn's disease patients were evaluated at 1, 3, 6, 9, and 12 months after starting treatment with adalimumab. Outcome variables included: clinical status (measured with CDAI), quality of life (measured with EuroQoL-5D and IBDQ), and work productivity (measured with WPAI questionnaire). These outcome variables were compared using the Student's t test or Wilcoxon test for paired comparison data according to the data distribution. Statistical significance was set at two-sided p work productivity impact decreased from 53% to 24% (p quality of life and work productivity.

  5. Comparison of Infliximab and Adalimumab in Biologic-Naive Patients With Ulcerative Colitis: A Nationwide Danish Cohort Study

    DEFF Research Database (Denmark)

    Singh, Siddharth; Nyboe Andersen, Nynne; Andersson, Mikael

    2017-01-01

    BACKGROUND & AIMS: We compared the effectiveness and safety of infliximab and adalimumab in biologic-naïve patients with ulcerative colitis (UC), in a nationwide register-based propensity score-matched study of patients in Denmark. METHODS: We collected data from 1719 adults with UC, 15-75 years...... old, in Denmark who were treated with either infliximab or adalimumab as their first biologic agent. We compared rates of all-cause hospitalization, UC-related hospitalization, major abdominal surgery, and serious infections after a variable 2:1 propensity score matching, accounting for baseline...... clinical characteristics, disease severity, healthcare use, and use of UC-related medications. RESULTS: Compared to patients treated with infliximab, patients treated with adalimumab had a higher risk for all-cause hospitalization (hazard ratio [HR], 1.84; 95% CI, 1.18-2.85), with a trend towards higher...

  6. Regulatory T cells in ankylosing spondylitis and the response after adalimumab treatment.

    Science.gov (United States)

    Liao, Hsien-Tzung; Lin, Yuh-Feng; Tsai, Chang-Youh; Chou, Chung-Tei

    2015-12-01

    The aim of this study was to investigate the role of regulatory T cells (Tregs) in ankylosing spondylitis (AS). We included 69 AS patients (15 of them received anti-tumor necrosis factor-apha agent-adalimumab) in the study and used a questionnaire to record the demographic data, disease activity index, functional index, human leukocyte antigen-B27, erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). Thirty healthy subjects were used as controls. The peripheral blood mononuclear cells (PMBCs) were stained with anti-CD4, anti-CD25 and anti-Forkhead/winged helix transcription factor P3 (anti-FoxP3) antibodies and flow-cytometry was used to determine cell populations. The percentages of Tregs in PMBCs were significantly higher in AS patients than in healthy controls. In AS patients who had poor disease functional index with higher levels of ESR and CRP were positively and significantly correlated with Tregs percentages in PMBCs. After adalimumab treatment in 15 patients, the percentages of Tregs, the ESR/CRP levels and the Bath Ankylosing Spondylitis Disease Activity Index/Bath Ankylosing Spondylitis Functional Index were significantly and gradually decreased over time. The high expression of FoxP3 and CD25 on CD4(+) T cells in PBMCs in AS patients was noted, and could be reversed by adalimumab therapy. These findings suggest that Tregs may play a role in modulating the inflammatory process in AS. Whether Tregs can be taken as a predictor for disease activity or treatment outcome is unclear and requires further study. Copyright © 2015 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved.

  7. Clinical and Pharmacokinetic Factors Associated With Adalimumab-Induced Mucosal Healing in Patients With Crohn's Disease.

    Science.gov (United States)

    Watanabe, Kenji; Matsumoto, Takayuki; Hisamatsu, Tadakazu; Nakase, Hiroshi; Motoya, Satoshi; Yoshimura, Naoki; Ishida, Tetsuya; Kato, Shingo; Nakagawa, Tomoo; Esaki, Motohiro; Nagahori, Masakazu; Matsui, Toshiyuki; Naito, Yuji; Kanai, Takanori; Suzuki, Yasuo; Nojima, Masanori; Watanabe, Mamoru; Hibi, Toshifumi

    2017-11-11

    We previously reported results from a prospective randomized controlled trial comparing the efficacy of adalimumab monotherapy versus combination with azathioprine for patients with Crohn's disease (CD) who were naive to biologics and thiopurines. We performed a subanalysis of data from this study to evaluate factors associated with endoscopic response and mucosal healing in study participants. We compared simple endoscopic scores for CD in between patients with moderate to severe active CD randomly assigned groups that received adalimumab monotherapy (n = 85) or adalimumab in combination with azathioprine (n = 91), from June 2011 to June 2014 in Japan. We evaluated associations of simple endoscopic scores for CD with clinical factors and trough levels of adalimumab. Ultimately, 135 patients at Week 26 and 139 patients at Week 52 from 44 referral sites were analyzed for the present investigation. The odds for endoscopic response were significantly higher in the combination group than in the monotherapy group at Week 26 (odds ratio [OR], 2.12; 95% confidence interval [CI], 1.04-4.32) but not at Week 52 (OR, 1.50; 95% CI, 0.77-2.94). The odds of mucosal healing did not differ significantly between groups at Weeks 26 or 52. Simple endoscopic scores for CD at Week 0 was significantly associated with mucosal healing at Week 26 (OR, 0.80; 95% CI, 0.72-0.90) and at Week 52 (OR, 0.91; 95% CI, 0.84-0.99). Higher adalimumab trough level at Week 26 associated with mucosal healing at Week 52 (OR, 1.34; 95% CI, 1.14-1.58; P for trend = .001) and was significantly higher in patients with endoscopic response than in patients without endoscopic response at Weeks 26 and 52 (P < .001). In a post hoc analysis of data from a randomized controlled trial of patients with moderate to severe CD, we found that adalimumab in combination with azathioprine increased trough levels of adalimumab. Higher trough levels of adalimumab associated with endoscopic response and mucosal healing at

  8. Influence of obstructive sleep apnea syndrome on blood glucose control and occurrence of chronic diabetic complications in type 2 diabetes patients

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    Ran LIU

    2012-12-01

    Full Text Available Objective  To explore the influence of obstructive sleep apnea syndrome (OSAS on blood glucose control and chronic diabetic complications in type 2 diabetes patients. Methods  The type 2 diabetes patients hospitalized in 307 Hospital of PLA from September 2011 to July 2012 were screened for OSAS with a portable sleep respiration monitor ApneaLinkTM device. According to screening results mentioned above, patients were divided into two groups as OSAS group and control group, whose fasting blood glucose level, 2-h postprandial blood glucose level, HbA1c level, HOMA-IR index and prevalence of diabetic retinopathy (DR, and incidences of diabetic nephropathy (DN, diabetic peripheral neuropathy (DPN, hypertension, and hyperlipidemia were recorded and analyzed for evaluating the relationship between OSAS severity and aforesaid factors. Results  One hundred and forty-five patients completed the protocal with 114 cases (87 males and 27 females stratified in OSAS group and 31 cases (22 males and 9 females in control group. Compared with control group, the average fasting blood glucose level, 2-h postprandial blood glucose level, HbA1c level and HOMA-IR index were significantly higher in OSAS patients (P<0.05. The apnea-hypopnea index (AHI and body mass index (BMI were both independently and positively correlated with the fasting blood glucose (adjust R2=37.1%, AHI 31.1%, 2-h postprandial blood glucose (adjust R2=31.2%, AHI 26.2% and HbA1c levels (adjust R2=38.7%, AHI 33.1% with AHI as the predominant risk factor of patient's poor blood glucose control. HbA1c level (OR5.86, 95%CI 4.64-7.08, suffering from OSAS (OR2.38, 95%CI 1.44-3.32, complicated hypertension (OR1.04, 95%CI 1.02-1.06 and patient's diabetes duration (OR2.10, 95%CI 1.80-2.40 were also independently and positively correlated with DR, and the independent and positive correlation factor of DPN included HbA1c level (OR 8.56, 95%CI 5.86-10.64, OSAS (OR 3.21, 95%CI 1.79-4.62 and hypertension

  9. Prognostic value of echocardiographic and Doppler parameters in horses admitted for colic complicated by systemic inflammatory response syndrome.

    Science.gov (United States)

    Borde, Laura; Amory, Hélène; Grulke, Sigrid; Leroux, Aurélia A; Houben, Rosa M; Detilleux, Johanne; Sandersen, Charlotte C

    2014-01-01

    To assess the prognostic value of echocardiographic parameters of left ventricular (LV) function in horses with systemic inflammatory response syndrome (SIRS). Prospective observational study. Veterinary teaching hospital. Forty-one horses admitted for colic with clinical evidence of SIRS. All horses underwent Doppler echocardiographic examination on admission. LV echocardiographic parameters, including pulsed-wave tissue Doppler imaging parameters, were compared between nonsurvivors (n = 29) and horses that survived to discharge (n = 12). With comparable heart rate and LV preload estimate, LV stroke volume index, the velocity time integral, deceleration time, ejection time of Doppler aortic flow, and peak early diastolic myocardial velocity were lower in the nonsurviving than in the surviving horses, while pre-ejection period to ejection time ratio (PEP/ET) of Doppler aortic flow and the peak early diastolic filling velocity to peak early diastolic myocardial velocity ratio (E/Em) were higher (P < 0.05). A cut-off value of 0.26 for PEP/ET predicted mortality with 100% sensitivity and 42% specificity (area under the receiver operating characteristic curve: 0.71), whereas a cut-off value of 2.67 for E/Em predicted mortality with 100% sensitivity and 83% specificity (area under the receiver operating characteristic curve: 0.89). Echocardiography may provide prognostic information in colic horses with clinical evidence of SIRS. Especially, PEP/ET and E/Em could be useful markers of systolic and diastolic dysfunction, respectively, to detect horses with a high risk of death requiring more intensive cardiovascular monitoring as it has been reported in human patients with septic shock. © Veterinary Emergency and Critical Care Society 2014.

  10. Síndrome hemolítico-urêmica relacionada à infecção invasiva pelo Streptococcus pneumoniae Hemolytic-uremic syndrome complicating invasive pneumococcal disease

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    Anna Leticia de O. Cestari

    2008-03-01

    Full Text Available OBJETIVO: A doença pneumocócica é importante problema de saúde pública e raramente há associação desta infecção com a síndrome hemolítico-urêmica (SHU grave. O objetivo deste artigo é relatar o caso de um paciente com esta associação. DESCRIÇÃO DO CASO: Criança do sexo masculino, com 17 meses de idade, admitida no hospital com insuficiência respiratória aguda e necessitando de suporte ventilatório. O exame radiológico mostrava extensa opacidade homogênea em hemitórax direito. A hemocultura foi positiva para Streptococcus pneumoniae. Nos exames de admissão, notaram-se: hemoglobina de 6,5g/dL, 38.000 plaquetas/mm³, uréia de 79mg/dL e creatinina de 1,64mg/dL. No primeiro dia, apresentou oligoanúria e hipervolemia, necessitando de hemodiafiltração. Evoluiu com disfunção de múltiplos órgãos e óbito no sétimo dia. A necrópsia mostrou áreas extensas de necrose cortical e tubular renal, com depósito de fibrina nas arteríolas. COMENTÁRIOS: A SHU associada ao pneumococo apresenta morbidade e mortalidade elevadas. Em crianças com doença pneumocócica invasiva e acometimento hematológico ou renal grave, deve-se estar atento a esta rara complicação. Merecem investigação os seguintes aspectos relacionados à doença: a função da detecção precoce de antígenos T ativados no diagnóstico e terapêutica, o papel do fator H na patogênese, o método ideal de substituição renal e a definição do prognóstico em longo prazo.OBJECTIVE: Pneumococcal diseases are a major public health problem. Severe hemolytic-uremic syndrome is an uncommon complication. The aim of this study is to report a child with this complication. CASE DESCRIPTION: A male child with 17 months old was admitted to the hospital, due to acute respiratory failure, needing ventilatory support. Roentgenogram demonstrated massive condensation of right lung and Streptococcus pneumonia was isolated from blood cultures. Laboratory tests showed

  11. Lung-Protective Ventilation With Low Tidal Volumes and the Occurrence of Pulmonary Complications in Patients Without Acute Respiratory Distress Syndrome: A Systematic Review and Individual Patient Data Analysis.

    Science.gov (United States)

    Neto, Ary Serpa; Simonis, Fabienne D; Barbas, Carmen S V; Biehl, Michelle; Determann, Rogier M; Elmer, Jonathan; Friedman, Gilberto; Gajic, Ognjen; Goldstein, Joshua N; Linko, Rita; Pinheiro de Oliveira, Roselaine; Sundar, Sugantha; Talmor, Daniel; Wolthuis, Esther K; Gama de Abreu, Marcelo; Pelosi, Paolo; Schultz, Marcus J

    2015-10-01

    Protective mechanical ventilation with low tidal volumes is standard of care for patients with acute respiratory distress syndrome. The aim of this individual patient data analysis was to determine the association between tidal volume and the occurrence of pulmonary complications in ICU patients without acute respiratory distress syndrome and the association between occurrence of pulmonary complications and outcome in these patients. Individual patient data analysis. ICU patients not fulfilling the consensus criteria for acute respiratory distress syndrome at the onset of ventilation. Mechanical ventilation with low tidal volume. The primary endpoint was development of a composite of acute respiratory distress syndrome and pneumonia during hospital stay. Based on the tertiles of tidal volume size in the first 2 days of ventilation, patients were assigned to a "low tidal volume group" (tidal volumes ≤ 7 mL/kg predicted body weight), an "intermediate tidal volume group" (> 7 and tidal volume group" (≥ 10 mL/kg predicted body weight). Seven investigations (2,184 patients) were included. Acute respiratory distress syndrome or pneumonia occurred in 23% of patients in the low tidal volume group, in 28% of patients in the intermediate tidal volume group, and in 31% of the patients in the high tidal volume group (adjusted odds ratio [low vs high tidal volume group], 0.72; 95% CI, 0.52-0.98; p = 0.042). Occurrence of pulmonary complications was associated with a lower number of ICU-free and hospital-free days and alive at day 28 (10.0 ± 10.9 vs 13.8 ± 11.6 d; p tidal volumes is associated with a lower risk of development of pulmonary complications in patients without acute respiratory distress syndrome.

  12. Acrodermatitis continua of Hallopeau (ACH): two cases successfully treated with adalimumab.

    Science.gov (United States)

    Di Costanzo, Luisa; Napolitano, Maddalena; Patruno, Cataldo; Cantelli, Mariateresa; Balato, Nicola

    2014-12-01

    Acrodermatitis continua of Hallopeau (ACH), also known as dermatitis repens or acrodermatitis perstans, is a rare acropustular eruption, characterized by sterile pustules, paronychia and atrophic skin changes, onychodystrophy and osteolysis of the distal phalanges of the fingers and toes. While some consider ACH a distinct entity, many believe it to be a variant of pustular psoriasis, especially as cases of ACH progressing to generalized pustular psoriasis. The treatment options used are various; however, its typical cyclic recurrences, which induce important physical and psychological morbidity, may render this pathology difficult to treat. Hence, it was considered important to review the evolution of treatment options available thus far including use of biologics. Hereby, we report two patients with ACH who were successfully treated with adalimumab. By analogy to the efficacy of TNF-α antagonists in the treatment of generalized pustular psoriasis, the two patients we report illustrate the long-term efficacy and safety of adalimumab in the treatment of  Hallopeau's acrodermatitis refractory to therapies.

  13. Impact of adalimumab treatment on cardiovascular risk biomarkers in psoriasis: Results of a pilot study.

    Science.gov (United States)

    Gkalpakiotis, Spyridon; Arenbergerova, Monika; Gkalpakioti, Petra; Potockova, Jana; Arenberger, Petr; Kraml, Pavel

    2017-04-01

    Psoriasis is a chronic systemic immune-mediated inflammatory dermatosis associated with several comorbidities. Psoriasis patients are at increased risk of developing cardiovascular diseases (CVD), namely, coronary heart disease, stroke or peripheral vascular disease, and psoriasis seems to be an independent cardiovascular risk factor. Antipsoriatic systemic therapy, especially anti-tumor necrosis factor (TNF)-α, seems to exert a beneficial effect on these comorbidities. The purpose of this study was: (i) to measure the level of cardiovascular serum markers in psoriasis patients in comparison with healthy volunteers; and (ii) to compare the serum level of the same markers in patients before and 3 months after adalimumab therapy. We investigated six biomarkers connected to CVD: C-reactive protein (measured high sensitively, hsCRP), oxidized low-density lipoproteins (oxLDL), oxLDL/β-glycoprotein I complex (oxLDL/β2GPI), vascular endothelial adhesion molecule 1 (VCAM-1), E-selectin and interleukin (IL)-22. These biomarkers were measured in 21 patients with moderate/severe psoriasis before and after treatment with adalimumab and in healthy volunteers. hsCRP (P psoriasis patients but the difference did not reach statistical significance. A decrease of E-selectin (P psoriasis but also decreases serum cardiovascular biomarkers. E-selectin and IL-22 could serve for monitoring of the efficacy of antipsoriatic systemic therapy on cardiovascular risk. © 2016 Japanese Dermatological Association.

  14. Dose Escalation and Healthcare Resource Use among Ulcerative Colitis Patients Treated with Adalimumab in English Hospitals: An Analysis of Real-World Data.

    Directory of Open Access Journals (Sweden)

    Christopher M Black

    Full Text Available To describe the real-world use of adalimumab for maintenance treatment of ulcerative colitis (UC and associated healthcare costs in English hospitals.Retrospective cohort study.Analysis of NHS Hospital Episode Statistics linked with pharmacy dispensing data in English hospitals.Adult UC patients receiving ≥240mg during adalimumab treatment induction, subsequently maintained on adalimumab.Frequency and pattern of adalimumab use and dose escalation during maintenance treatment and associated healthcare costs (prescriptions and hospital visits.191 UC patients completed adalimumab treatment induction. 83 (43.46% dose escalated during maintenance treatment by ≥100% (equivalent to weekly dosing (median time to dose escalation: 139 days. 56 patients (67.47% subsequently de-escalated by ≥50% (median time to dose de-escalation: 21 days. Mean all-cause healthcare costs for all patients ≤12 months of index were £13,892. Dose escalators incurred greater mean healthcare costs than non-escalators ≤12 months of index (£14,596 vs. £13,351. Prescriptions accounted for 96.49% of UC-related healthcare costs (£11,090 of £11,494 in all patients.Within the cohort, 43.46% of UC patients escalated their adalimumab dose by ≥100% and incurred greater costs than non-escalators. The apparent underestimation of adalimumab dose escalation in previous studies may have resulted in underestimated costs in healthcare systems.

  15. Ex vivo inhibited cytokine profiling may explain inferior treatment response to golimumab after adalimumab failure in rheumatoid arthritis

    NARCIS (Netherlands)

    Tweehuysen, L; Schraa, K.; Netea, M.G.; Hoogen, F.H.J. van den; Joosten, L.A.B.; Broeder, A.A. den

    2018-01-01

    OBJECTIVES: Clinical data suggest that the response of rheumatoid arthritis patients to treatment with golimumab is much lower among those who switched from adalimumab than among those who switched from etanercept. To elucidate the mechanism behind this difference in response to sequential biologic

  16. Personalised treatment using serum drug levels of adalimumab in patients with rheumatoid arthritis : An evaluation of costs and effects

    NARCIS (Netherlands)

    Krieckaert, C. L M; Nair, S. C.; Nurmohamed, M. T.; Van Dongen, C. J J; Lems, W. F.; Lafeber, F. P J G; Bijlsma, J. W J; Koffijberg, H.; Wolbink, G.; Welsing, P. M J

    2015-01-01

    Objective: To evaluate the cost-effectiveness of personalised treatment for rheumatoid arthritis (RA) using clinical response and serum adalimumab levels. Methods: A personalised treatment algorithm defined, based on clinical (European League Against Rheumatism) response and drug levels at 6 months,

  17. Adalimumab for induction of clinical remission in moderately to severely active ulcerative colitis: results of a randomised controlled trial

    NARCIS (Netherlands)

    Reinisch, W.; Sandborn, W.J.; Hommes, D.W.; D'Haens, G.; Hanauer, S.; Schreiber, S.; Panaccione, R.; Fedorak, R.N.; Tighe, M.B.; Huang, B.; Kampman, W.; Lazar, A.; Thakkar, R.

    2011-01-01

    Objective The aim of this study was to assess the efficacy and safety of adalimumab (ADA), a recombinant human monoclonal antibody against tumour necrosis factor alpha (TNF), for the induction of clinical remission in anti-TNF naive patients with moderately to severely active ulcerative colitis.

  18. Long-term use of adalimumab in the treatment of moderate to severe plaque psoriasis: a review of the literature

    Directory of Open Access Journals (Sweden)

    Angela Y Moore

    2010-04-01

    Full Text Available Angela Y Moore, Blakely S RichardsonArlington Center for Dermatology, Arlington, Texas, USAAbstract: Psoriasis is a chronic T-cell-mediated inflammatory disease that primarily affects the skin and joints. Patients with moderate to severe psoriasis constitute about 30% of the psoriasis population. Treatment of this group is challenging due to the long-term side effects, toxicities and inconvenience of conventional treatments such as phototherapy, methotrexate and cyclosporine. However, recent advances in our understanding of the pathogenesis of psoriasis have led to the popular use of biologics, which offer a safer, more convenient and effective targeted therapy. Adalimumab was originally approved for treating rheumatoid arthritis. Currently, adalimumab is also approved for treatment of adult patients with moderate to severe chronic plaque psoriasis who are candidates for systemic therapy or phototherapy or when other systemic therapies are medically less appropriate. Since the onset of the use of biologics, there have been concerns over safety and efficacy when used as long-term therapy. This paper reviews all publications, posters and abstracts reporting original data on the efficacy and/or safety of adalimumab in patients treated for chronic plaque psoriasis for more than 1 year.Keywords: psoriasis, adalimumab, biologics

  19. Complication with intraosseous access

    DEFF Research Database (Denmark)

    Hallas, Peter; Brabrand, Mikkel; Folkestad, Lars

    2013-01-01

    .5%), and extravasation (3.7%). Compartment syndrome and osteomyelitis occurred in 0.6% and 0.4% of cases respectively. CONCLUSION: In users' recollection of real-life IO use, perceived complications were more frequent than usually reported from model studies. The perceived difficulties with using IO could affect...

  20. Prader-Willi Syndrome

    Science.gov (United States)

    ... syndrome can be helpful in genetic counseling. Complications Obesity-related complications In addition to having constant hunger, ... result from Prader-Willi syndrome include: Effects of binge eating. Eating large amounts of food quickly, called binge ...

  1. Possible familial case of Birt-Hogg-Dubé syndrome complicated with lung cancer: A possible link between these two disease entities.

    Science.gov (United States)

    Nishida, Chinatsu; Yatera, Kazuhiro; Yamasaki, Kei; Torii, Ryo; Kawanami, Yukiko; Kawanami, Toshinori; Ishimoto, Hiroshi; Shibuya, Ryo; Takenaka, Masaru; Yamada, Sohusuke; Kasai, Takahiko; Tanaka, Fumihiro; Mukae, Hiroshi

    2015-07-01

    A 65-year-old Japanese woman was introduced to our hospital for an examination of multiple pulmonary cystic lesions and a pulmonary nodule in the left lower lobe. She had a smoking history of 25 pack-years, and her two younger brothers had suffered from pneumothorax; one of them additionally had lung cancer with pulmonary multiple cystic lesions. A surgical biopsy specimen obtained from her left lower lobe revealed adenocarcinoma surrounded by a single epithelial layer that was covered with collagen fibers. The pathological features were compatible with the findings of the cystic lesions in the patients with Birt-Hogg-Dubé syndrome (BHDS). A diagnosis of BHDS was eventually made according to the detection of a folliculin gene mutation. This is the first report of a possible familial case of BHDS complicated with primary lung cancer. We herein reviewed the previously reported cases of BHDS with lung cancer and other tumors and discussed a potential mechanism of tumorigenesis and carcinogenesis in the lung in the patients with BHDS. Copyright © 2015 Elsevier Ltd. All rights reserved.

  2. Otalgia and eschar in the external auditory canal in scrub typhus complicated by acute respiratory distress syndrome and multiple organ failure

    Directory of Open Access Journals (Sweden)

    Hu Sung-Yuan

    2011-03-01

    Full Text Available Abstract Background Scrub typhus, a mite-transmitted zoonosis caused by Orientia tsutsugamushi, is an endemic disease in Taiwan and may be potentially fatal if diagnosis is delayed. Case presentations We encountered a 23-year-old previously healthy Taiwanese male soldier presenting with the right ear pain after training in the jungle and an eleven-day history of intermittent high fever up to 39°C. Amoxicillin/clavulanate was prescribed for otitis media at a local clinic. Skin rash over whole body and abdominal cramping pain with watery diarrhea appeared on the sixth day of fever. He was referred due to progressive dyspnea and cough for 4 days prior to admission in our institution. On physical examination, there were cardiopulmonary distress, icteric sclera, an eschar in the right external auditory canal and bilateral basal rales. Laboratory evaluation revealed thrombocytopenia, elevation of liver function and acute renal failure. Chest x-ray revealed bilateral diffuse infiltration. Doxycycline was prescribed for scrub typhus with acute respiratory distress syndrome and multiple organ failure. Fever subsided dramatically the next day and he was discharged on day 7 with oral tetracycline for 7 days. Conclusion Scrub typhus should be considered in acutely febrile patients with multiple organ involvement, particularly if there is an eschar or a history of environmental exposure in endemic areas. Rapid and accurate diagnosis, timely administration of antibiotics and intensive supportive care are necessary to decrease mortality of serious complications of scrub typhus.

  3. Spontaneous complete regression of hypothalamic pilocytic astrocytoma after partial resection in a child, complicated with Stevens-Johnson syndrome: a case report and literature review.

    Science.gov (United States)

    Samadian, Mohammad; Bakhtevari, Mehrdad Hosseinzadeh; Haddadian, Karim; Alavi, Hossein Afshin; Rezaei, Omidvar

    2016-04-01

    Pilocytic astrocytoma (PA) is the most common pediatric central nervous system glial neoplasm and the most common pediatric cerebellar tumor. The spontaneous regression that occurs after partial/subtotal resection is multifactorial, depending on multiple factors, as for the case of humoral and cell-mediated immune responses of the host to the implanted tumor. A 7-year-old boy was referred to a neurosurgery clinic with headache. Further imaging workup revealed hypothalamic PA. Partial resection of the lesions was performed with right-side pterional approach. The patient developed a severe panmucositis [Stevens-Johnson syndrome (SJS)] and respiratory failure plus conjunctivitis, due to phenytoin allergy. During the patient's 6-month follow-up, postoperative magnetic resonance imaging (MRI) revealed a residual tumor, and about 9 months later (at 15 months postoperatively), the MRI showed total regression of the tumor. Clinically, symptomatic PA may undergo spontaneous regression after partial resection. We report a well-documented case of spontaneous regression hypothalamic PA after partial resection that complicated with SJS. Immune system reaction in SJS may have a role in tumor behavior and spontaneous regression. Multiple studies confirmed spontaneous regression in PA after partial/subtotal resection. This phenomenon occurs due to humoral and cell-mediated host immune responses to the implanted tumor. The immune system reaction in SJS may have a role in tumor behavior and spontaneous regression.

  4. Complications encountered in patients bearers with crest syndrome in rheumatological service of the Hospital San Juan de Dios until September 2009

    International Nuclear Information System (INIS)

    Mendez Rodriguez, Alexis

    2010-01-01

    Systematic sclerosis is a disease that has caused much morbidity and dependence in patients. Despite not being the disease most prevalent in rheumatological practice, it has been perhaps one that has generated more interest in the complex pathophysiology; but, also a great sense of frustration with the great therapeutic limitations especially when is diagnosed in advanced stages. The research was conducted in order to motivate early clinical search of the major complications found in patients CREST syndrome, in rheumatological service of the Hospital San Juan de Dios until September 2009 the total of cases with a diagnosis of CREST were reviewed in outpatient records, the study population have been of 41 patients. The different clinical manifestations of the patient were taken into account, among other aspects: immunological studies, established treatments and diagnosis methods as conventional radiology, endoscopic studies, echocardiogram, capillaroscopy. This job has determined among other things, that the majority of patients with CREST come from Desamparados and the Southern Zone, representing 31.7 and 29.2%, respectively, 98% are women and 76% of patients engaged in domestic chores and no mortality case was found in relation to CREST [es

  5. Adalimumab impact on fatigue measured by FACIT-F: a study of rheumatoid arthritis patients in Saudi Arabia

    Directory of Open Access Journals (Sweden)

    Janoudi N

    2017-10-01

    Full Text Available Nahed Janoudi,1 Narges Omran,2 Waleed Hussain,3 Hanan Al-osaimi,4 Matouqa Baamer,5 Muhammad Irfanullah Siddiqui,6 Omar Fathaldin,7 Hani Almoallim1,4,8 1Department of Medicine, Dr. Soliman Fakeeh Hospital, Jeddah, 2Department of Medicine, Alnoor General Hospital, 3Department of Medicine, Heraa General Hospital, 4Alzaidi Chair of Research in Rheumatic Diseases, Umm Al-Qura University, Makkah, 5Department of Medicine, King Abdulaziz Hospital & Oncology Center, Jeddah, 6Department of Community Medicine and Public Health, Faculty of Medicine, Umm Al-Qura University, Makkah, 7Department of Medicine, Faculty of Medicine, King Abdulaziz University, Jeddah, 8Department of Medicine, Faculty of Medicine, Umm Al-Qura University, Makkah, Saudi Arabia Objective: The objective of the study was to assess the effectiveness of adalimumab as a treatment for fatigue in patients with rheumatoid arthritis (RA. Methods: Fatigue was monitored in patients with RA who were already on an adalimumab treatment regimen. Fatigue, general well-being, comorbidities, and disease activity were measured at baseline and ~8, 16, and 24 weeks, thereafter. Results: Significant reductions in fatigue scores and disease activity were observed from baseline to 6 months after. A predictive regression model of fatigue severity was proposed and was found to be significant, with RA disease activity as the most significant predictor of fatigue severity. Conclusion: This quasi-experimental study is a good starting point for research on the efficacy of adalimumab in treating fatigue in RA patients. The results here suggest that a randomized controlled trial assessing adalimumab as a treatment option for RA patients suffering from fatigue is warranted. Keywords: rheumatoid arthritis, fatigue, adalimumab, disease activity, Saudi Arabia, FACIT-F 

  6. Abdominal Complications after Severe Burns

    Science.gov (United States)

    2009-05-01

    abdominal compartment syndrome, schemic bowel, biliary disease , peptic ulcer disease and astritis requiring laparotomy, small bowel obstruction, rimary fungal...complications in- luded trauma exploratory laparotomy, abdominal com- artment syndrome, ischemic bowel, biliary disease , peptic lcer disease and gastritis, large...abdominal complications was 25%, with Curl- ng’s ulcer the most common malady (54% of the total), ollowed by esophageal lesions (17%), hemorrhagic

  7. Economic impact of adalimumab treatment in Japanese patients with rheumatoid arthritis from the adalimumab non-interventional trial for up-verified effects and utility (ANOUVEAU) study.

    Science.gov (United States)

    Tanaka, Yoshiya; Yamazaki, Kiyotaka; Nakajima, Ryo; Komatsu, Shuichi; Igarashi, Ataru; Tango, Toshiro; Takeuchi, Tsutomu

    2018-01-01

    The objective of this study is to evaluate the economic impact of adalimumab (ADA) on Japanese rheumatoid arthritis (RA) patients. ANOUVEAU was a 48-week multicenter, prospective, observational, single-cohort study. Work-related outcomes including absenteeism, presenteeism, overall work impairment (OWI), and activity impairment (AI) were evaluated using the RA-related work productivity and activity impairment (WPAI/RA). The amount of productivity loss was estimated via multiplication of absenteeism, presenteeism and OWI by the national average occupational wage for paid worker (PW) and part time worker (PTW), and via multiplication of AI by the estimated wage for domestic work for home maker (HM). In this analysis, 1196 patients were included. At week 48, measures of productivity loss due to absenteeism, presenteeism, OWI, and AI were significantly improved by administrating ADA to RA patients in all employment types (PW, PTW, and HM), compared to baseline (p < .01). Productivity loss of Japanese society by RA disease was estimated to be $9.80 billion. The annual decrease in productivity loss through ADA administration to Japanese RA patients was estimated to be $3.76 billion. The socioeconomic burden of RA is high, but ADA treatment may reduce productivity loss related to RA.

  8. Antiphosphatidylserine/prothrombin antibodies (aPS/PT) as potential diagnostic markers and risk predictors of venous thrombosis and obstetric complications in antiphospholipid syndrome.

    Science.gov (United States)

    Shi, Hui; Zheng, Hui; Yin, Yu-Feng; Hu, Qiong-Yi; Teng, Jia-Lin; Sun, Yue; Liu, Hong-Lei; Cheng, Xiao-Bing; Ye, Jun-Na; Su, Yu-Tong; Wu, Xin-Yao; Zhou, Jin-Feng; Norman, Gary L; Gong, Hui-Yun; Shi, Xin-Ming; Peng, Yi-Bing; Wang, Xue-Feng; Yang, Cheng-De

    2018-03-28

    The aim of the study was to determine the prevalence and clinical associations of antiphosphatidylserine/prothrombin antibodies (aPS/PT) with thrombosis and pregnancy loss in Chinese patients with antiphospholipid syndrome (APS) and seronegative APS (SNAPS). One hundred and eighty six Chinese patients with APS (67 primary, 119 secondary), 48 with SNAPS, 176 disease controls (79 systemic lupus erythematosus [SLE], 29 Sjogren's syndrome [SS], 30 ankylosing spondylitis [AS], 38 rheumatoid arthritis [RA]) and 90 healthy donors were examined. IgG and IgM aPS/PT, IgG/IgM/IgA anticardiolipin (aCL) and IgG/IgM/IgA anti-β2-glycoprotein I (anti-β2GPI) antibodies were tested by ELISA. One hundred and sixty (86.0%) of APS patients were positive for at least one aPS/PT isotype. One hundred and thirty five (72.6%) were positive for IgG aPS/PT, 124/186 (66.7%) positive for IgM aPS/PT and 99 (53.2%) positive for both. Approximately half of the SNAPS patients were positive for IgG and/or IgM aPS/PT. Highly significant associations between IgG aPS/PT and venous thrombotic events (odds ratio [OR]=6.72) and IgG/IgM aPS/PT and pregnancy loss (OR=9.44) were found. Levels of IgM aPS/PT were significantly different in APS patients with thrombotic manifestations and those with fetal loss (p=0.014). The association between IgG/IgM aPS/PT and lupus anticoagulant (LAC) was highly significant (pAPS was 101.6. Notably, 91.95% (80/87) of LAC-positive specimens were positive for IgG and/or IgM aPS/PT, suggesting aPS/PT is an effective option when LAC testing is not available. Anti-PS/PT antibody assays demonstrated high diagnostic performance for Chinese patients with APS, detected some APS patients negative for criteria markers and may serve as potential risk predictors for venous thrombosis and obstetric complications.

  9. Digital Vasculitis in a Patient with Rheumatoid Arthritis Responded Well to Adalimumab

    Directory of Open Access Journals (Sweden)

    Senol Kobak

    2014-01-01

    Full Text Available 42-year-old old female patient, followed up with diagnosis of rheumatoid arthritis for 15 years, was admitted with necrotising ulcer of left hand 1st and 2nd fingertips and pain, swelling, limitation of movement, and morning stiffness at bilateral wrist, and metacarpophalangeal and proximal interphalangeal joints. Laboratory tests revealed elevated acute phase reactants. Radial and ulnar arteries were clear in upper extremity Doppler ultrasound. The patient was diagnosed as RA activation and digital ulcer and administered iloprost infusion for five days and 1 mg/kg corticosteroid and 20 mg/week methotrexate (MTX. After one month, a partial regression of clinical and laboratory findings was observed. However, 6 months later, due to relapsed and increased complaints and findings, adalimumab 40 mg was administered. Two months later, clinical and laboratory findings apparently decreased.

  10. The number needed to treat for adalimumab, etanercept, and infliximab based on ACR50 response in three randomized controlled trials on established rheumatoid arthritis

    DEFF Research Database (Denmark)

    Kristensen, L E; Christensen, R; Bliddal, H

    2011-01-01

    To compare the efficacy of adalimumab, etanercept, and infliximab in patients with established rheumatoid arthritis (RA) taking concomitant methotrexate (MTX) by calculating the number needed to treat (NNT) using three different methods....

  11. Methotrexate Dose in Patients With Early Rheumatoid Arthritis Impacts Methotrexate Polyglutamate Pharmacokinetics, Adalimumab Pharmacokinetics, and Efficacy: Pharmacokinetic and Exposure-response Analysis of the CONCERTO Trial.

    Science.gov (United States)

    Goss, Sandra L; Klein, Cheri E; Jin, Ziyi; Locke, Charles S; Rodila, Ramona C; Kupper, Hartmut; Burmester, Gerd-Rudiger; Awni, Walid M

    2018-02-01

    Methotrexate (MTX) and adalimumab are well-recognized treatments of rheumatoid arthritis (RA), the efficacy of which may be driven by intracellular polyglutamates (PGs). The aim of this analysis was to characterize MTX PG concentrations and adalimumab pharmacokinetics in the CONCERTO trial. In addition, the relationships between MTX dose/pharmacokinetics, adalimumab pharmacokinetics, and efficacy were evaluated. CONCERTO was a double-blind, parallel-arm study in patients with early RA randomized to adalimumab 40 mg SC every other week plus blinded MTX 2.5, 5, 10, or 20 mg PO once weekly, for 26 weeks. Blood samples were obtained through week 26 for the determination of concentrations of MTX PG, adalimumab, and anti-adalimumab antibody (AAA). Clinical outcomes were also assessed. A total of 395 patients were included in the analysis (MTX, 329; adalimumab, 395). The mean time to steady-state MTX PG concentration was increased with MTX dose, from 8 to >26 weeks, depending on PG chain length. Dose proportionality changed with PG chain length. As MTX dose was increased, the percentage of short-chain PGs increased less than dose proportionally, while the percentage of long-chain PGs increased more than dose proportionally. For very-long-chain PGs, dose proportionality could not be assessed due to the nonmeasurable concentrations in the 2.5- and 5-mg MTX dose groups. As MTX dose increased, mean adalimumab concentrations also increased (P < 0.001). The percentage of patients with AAA decreased with increasing MTX dose, and at week 26, AAA + status was significantly correlated with MTX dose level (P = 0.005). In general, rates of response, defined using the 28-joint count disease activity score based on C-reactive protein (DAS28[CRP]; response, <3.2), were greater in the subgroup without AAA. The likelihood of a patient achieving a DAS28(CRP) response was related to the baseline measurement (P < 0.001) and to the concentration of adalimumab (P = 0.001), but not to the MTX

  12. Crohn's disease outpatients treated with adalimumab have an earlier secondary loss of response and requirement for dose escalation compared to infliximab: a real life cohort study.

    Science.gov (United States)

    Ma, Christopher; Huang, Vivian; Fedorak, Darryl K; Kroeker, Karen I; Dieleman, Levinus A; Halloran, Brendan P; Fedorak, Richard N

    2014-11-01

    The efficacy of anti-tumor necrosis factor alpha agents in maintaining remission in Crohn's disease may wane over time, leading to secondary loss of response that can often be overcome with dose escalation. Comparison of secondary loss of response of adalimumab and infliximab during long-term treatment of CD in a real-life IBD clinic has not been previously evaluated. A retrospective cohort study was conducted evaluating outpatients with CD on a maintenance regimen with adalimumab or infliximab from 200 to 2013 and who experienced a secondary loss of response. All infliximab-treated patients were anti-TNF naïve. Adalimumab-treated patients were stratified by prior anti-TNF exposure. Kaplan-Meier analysis was conducted to compare time to loss of response. 218 CD patients met inclusion criteria (117 infliximab, 101 adalimumab). Median follow-up duration was 170.0weeks for infliximab and 122.0weeks for adalimumab (p=0.61). The proportion of patients with secondary loss of response was similar for infliximab-treated - 51.3% (60/117) compared to adalimumab patients naïve to anti-TNF therapy - 60.5% (23/38) (p=0.32), and adalimumab patients with prior anti-TNF exposure - 65.1% (41/63) (p=0.08). Median time to secondary loss of response was longer for infliximab patients (99.3wk, IQR 55.7-168.5) compared to both adalimumab patients naïve to anti-TNF therapy (58.9wk, IQR 29.0-85.7) (p=0.03), and adalimumab patients with prior anti-TNF exposure (52.7wk, IQR 20.1-85.0) (p<0.001). Over 50% of CD patients treated with infliximab and adalimumab develop secondary loss of response. Time to loss of response was shorter in patients treated with adalimumab compared to those treated with infliximab. Prior anti-TNF exposure further accelerated time to loss of response. Copyright © 2014 European Crohn's and Colitis Organisation. Published by Elsevier B.V. All rights reserved.

  13. The number needed to treat for adalimumab, etanercept, and infliximab based on ACR50 response in three randomized controlled trials on established rheumatoid arthritis

    DEFF Research Database (Denmark)

    Kristensen, L E; Christensen, R; Bliddal, H

    2011-01-01

    To compare the efficacy of adalimumab, etanercept, and infliximab in patients with established rheumatoid arthritis (RA) taking concomitant methotrexate (MTX) by calculating the number needed to treat (NNT) using three different methods.......To compare the efficacy of adalimumab, etanercept, and infliximab in patients with established rheumatoid arthritis (RA) taking concomitant methotrexate (MTX) by calculating the number needed to treat (NNT) using three different methods....

  14. The number needed to treat for adalimumab, etanercept, and infliximab based on ACR50 response in three randomized controlled trials on established rheumatoid arthritis

    DEFF Research Database (Denmark)

    Kristensen, L E; Christensen, R; Bliddal, H

    2007-01-01

    To compare the efficacy of adalimumab, etanercept, and infliximab in patients with established rheumatoid arthritis (RA) taking concomitant methotrexate (MTX) by calculating the number needed to treat (NNT) using three different methods.......To compare the efficacy of adalimumab, etanercept, and infliximab in patients with established rheumatoid arthritis (RA) taking concomitant methotrexate (MTX) by calculating the number needed to treat (NNT) using three different methods....

  15. SAFETY PROFILE OF ANTI-TNF THERAPY IN CROHN'S DISEASE MANAGEMENT: A BRAZILIAN SINGLE-CENTER DIRECT RETROSPECTIVE COMPARISON BETWEEN INFLIXIMAB AND ADALIMUMAB.

    Science.gov (United States)

    Bau, Mariella; Zacharias, Patricia; Ribeiro, Diogo Araújo; Boaron, Larissa; Steckert Filho, Alvaro; Kotze, Paulo Gustavo

    2017-12-01

    Infliximab and adalimumab are considered effective drugs in the management of Crohn's disease. However, due to significant immunossupression, they can cause important adverse events, mostly infections. The aim of this study was to quantify and describe adverse events derived from adalimumab and infliximab use in Crohn's disease patients, and to compare the safety profile between these two agents. This was an observational, single-center, longitudinal, retrospective study with Crohn's disease patients under infliximab or adalimumab therapy. Variables analyzed: demographic characteristics (including the Montreal classification), type of agent used, concomitant immunomodulators, presence and types of adverse events observed. Patients were allocated in two groups (infliximab and adalimumab) and had their adverse events accessed and subsequently compared. A total of 130 patients were included (68 in infliximab and 62 in adalimumab groups, respectively). The groups were fully homogeneous in all baseline characteristics, with a median follow-up of 47.21±36.52 months in the infliximab group and 47.79±35.09 in the adalimumab group (P=0.512). Adverse events were found in 43/68 (63.2%) and 40/62 (64.5%) in each group, respectively (P=0.879). There were no differences between the groups regarding infections (P=0.094) or treatment interruption (P=0.091). There were higher rates of infusion reactions in the infliximab group (P=0.016). Cephalea and injection site reactions were more prevalent in adalimumab patients. Adverse events were found in approximately two thirds of Crohn's disease patients under anti-TNF therapy, and there were no significant differences between infliximab or adalimumab.

  16. Downregulation of interferon-γ-induced protein 10 in the tears of patients with Stevens-Johnson syndrome with severe ocular complications in the chronic stage.

    Science.gov (United States)

    Ueta, Mayumi; Nishigaki, Hiromi; Sotozono, Chie; Kinoshita, Shigeru

    2017-01-01

    Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are acute inflammatory vesiculobullous reactions of the skin and mucosa such as the ocular surface, oral cavity and genitals. Severe ocular complications (SOC) arise in some patients with SJS/TEN diagnosed by dermatologists. To investigate the pathophysiology of ocular surface inflammation in SJS/TEN with SOC in the chronic stage, we examined cytokines in the tears of patients with ocular surface diseases and healthy controls. SJS/TEN eyes in the chronic stage (n>30), healthy eyes (n>20, controls) and eyes (n>20) from patients with atopic keratoconjunctivitis representing different ocular surface inflammatory disorders. Tear samples were collected on Schirmer's measurement strips. To measure the level of various cytokines in the tears we used BD CBA Flex sets. An observational study (case-control study). We recorded the level of interleukin (IL)-6, IL-8, eotaxin, macrophage inflammatory protein (MIP)-1β, RANTES (regulated on activation, normal T cell expressed and secreted), interferon gamma (IFN)-γ, monocyte chemoattractant protein-1, IFN-γ-induced protein 10 (IP-10) and total IgE. We found that compared with the controls, in SJS/TEN with SOC, IL-6, IL-8, eotaxin and MIP-1β were significantly upregulated while IP-10 was significantly downregulated. Compared with atopic keratoconjunctivitis, IP-10 was significantly downregulated in SJS/TEN with SOC; on the other hand, total IgE was significantly upregulated in atopic keratoconjunctivitis compared with SJS/TEN with SOC. IP-10 in tears may be a biomarker to distinguish between chronic SJS/TEN with SOC and other ocular inflammatory disorders such as atopic keratoconjunctivitis.

  17. Abdominal Compartment Syndrome complicating massive ...

    African Journals Online (AJOL)

    shobha

    cycle. Unless the Intra-abdominal pressure is reduced quickly by urgent surgical or medical interventions, death is inevitable. We report a case of ACS resulting from an unrecognized slow but massive intra-abdominal bleeding caused by a ruptured ectopic pregnancy (REP) in an Arab woman. Due to the unusual nature of ...

  18. Abdominal Compartment Syndrome complicating massive ...

    African Journals Online (AJOL)

    L'operation chirurgicale a permis d' tablir que la tentative infructueuse de r animation par une perfusion agressive n'est pas en fait due une hypovol mie en soi, mais l'hypertension intra-abdominale provoquant le SCA. Les le ons tir es de ce cas mettent un accent sur la n cessit d'une prise de conscience ...

  19. [Complications of cocaine addiction].

    Science.gov (United States)

    Karila, Laurent; Lowenstein, William; Coscas, Sarah; Benyamina, Amine; Reynaud, Michel

    2009-06-20

    Addiction is a chronic relapsing disorder characterized by repetitive and compulsive drug-seeking behavior and drug abuse despite negative health or social consequences. Cocaine addiction is a significant worldwide public health problem, which has somatic, psychological, psychiatric, socio-economic and judicial complications. Some of the most frequent complications are cardiovascular effects (acute coronary syndrome, cardiac arrhythmias, increased blood pressure); respiratory effects (fibrosis, interstitial pneumonitis, pulmonary hypertension, alveolar haemorrhage, asthma exacerbation; emphysema), neurological effects (strokes, aneurysms, seizures, headaches); risk for contracting HIV/AIDS, hepatitis B and C, sexual transmitted disease and otolaryngologic effects. Other complications are not discussed here. The vast majority of studies indicate that there are cognitive deficits induced by cocaine addiction. Attention, visual and working memories, executive functioning are affected in cocaine users. Psychiatric complications found in clinical practice are major depressive disorders, cocaine-induced paranoia, cocaine-induced compulsive foraging and panic attacks.

  20. Tratamiento de inducción y mantenimiento con adalimumab en la enfermedad de Crohn: un estudio abierto Adalimumab induction and maintenance therapy from Crohn's diseas: An open-label study

    Directory of Open Access Journals (Sweden)

    N. López Palacios

    2008-11-01

    Full Text Available Introducción: el adalimumab ha demostrado, en ensayos clínicos controlados con placebo y en estudios no controlados, ser efectivo en la EC luminal y fistulosa perianal. Objetivo: evaluar la eficacia y seguridad del adalimumab como tratamiento de inducción y mantenimiento en la EC. Metodología: se incluyeron 22 pacientes con EC tratados con adalimumab (16 por enfermedad luminal y 6 por enfermedad fistulosa perianal activa. Veintiún pacientes habían recibido previamente IFX. Se realizó tratamiento de inducción con 160 mg s.c. en la semana 0 y 80 mg s.c. a las 2 semanas. Los respondedores recibieron 40 mg s.c. cada 14 días como tratamiento de mantenimiento. Se valoró la respuesta a las 4 semanas de la dosis inicial, y se clasificó la respuesta como remisión, respuesta parcial o ausencia de respuesta. Resultados: tras la inducción, el 25% de los pacientes con enfermedad luminal tuvieron remisión completa y el 56,3% respuesta parcial. La respuesta clínica se mantuvo al año en el 71,6% de los pacientes, a los 18 meses en el 53,7% y a los 48 meses en el 35,8%. No se objetivaron diferencias en la respuesta entre pacientes que presentaron reacciones de hipersensibilidad o pérdida de respuesta a IFX. Todos los pacientes con enfermedad fistulosa perianal (n = 6 habían recibido previamente tratamiento con IFX. Tras la inducción un 16,7% entran en remisión y un 66,7% presentan respuesta parcial. Todos los pacientes mantienen remisión o respuesta en el tiempo con una mediana de seguimiento de 15 meses. Conclusiones: el adalimumab es un tratamiento eficaz y seguro en la inducción y mantenimiento de la respuesta en la EC luminal y fistulosa perianal. Estos resultados confirman que los hallazgos obtenidos en los ensayos clínicos controlados son reproducibles en la práctica clínica diaria.Background: adalimumab has been shown in placebo-controlled clinical trials and uncontrolled studies to be effective in luminal and perianal fistulizing

  1. Clinical experience of the use of adalimumab in the management of hidradenitis suppurativa. Comparison of response rates with Crohn disease.

    Science.gov (United States)

    Moyano, B; Clemente, A; Marín-Jiménez, I; Martorell, A

    2016-09-01

    The recent approval of adalimumab as the first treatment to be approved for the management of hidradenitis suppurativa has represented a before and after in the control of this chronic inflammatory disease. Given the inflammatory burden of this cutaneous disease, in the last few years hidradenitis suppurativa has been compared with inflammatory bowel disease, particularly with Crohn disease, to the point of considering hidradenitis suppurativa as "Crohn disease of the skin". These two chronic inflammatory diseases show sufficient similarities to consider whether treatment response based on the inflammatory load could also be similar. The present article aims to analyse the efficacy of adalimumab in hidradenitis suppurativa in comparison with a truly comparable disease, Crohn disease, with a view to evaluating therapeutic response rates and to drawing conclusions on the therapeutic success obtained in this disabling cutaneous disease. Copyright © 2016 Elsevier España, S.L.U. y AEDV. All rights reserved.

  2. Crohn's Disease Activity and Concomitant Immunosuppressants Affect the Risk of Serious and Opportunistic Infections in Patients Treated With Adalimumab.

    Science.gov (United States)

    Osterman, Mark T; Sandborn, William J; Colombel, Jean-Frederic; Peyrin-Biroulet, Laurent; Robinson, Anne M; Zhou, Qian; Lewis, James D

    2016-12-01

    Anti-tumor necrosis factor (TNF) drugs are commonly used to treat moderate-to-severe Crohn's disease (CD). Both the activity of CD and the concomitant immunosuppressants (corticosteroids and immunomodulators) used with anti-TNF drugs could increase the risk of infection. We determined the relative risk of serious and opportunistic infections associated with increasing disease activity and concomitant immunomodulators and corticosteroids in patients with CD treated with adalimumab. This pooled analysis identified incident treatment-emergent serious and opportunistic infections among patients with CD in clinical trials of adalimumab. Disease activity was assessed with the Crohn's Disease Activity Index (CDAI). The analysis included 2,266 patients treated with adalimumab with median age 35 years. Higher disease activity was associated with significantly increased risks of both serious and opportunistic infections at 1 year, with each 100-point increase in CDAI associated with a >30% increased risk of each type of infection. Concomitant use of immunomodulators was associated with a significant >3-fold decreased risk of serious infection (hazard ratio (HR) 0.29 (0.08-0.98), P=0.045) by 1 year. Concomitant use of corticosteroids was associated with a significantly increased risk of serious infection by day 120 (HR 2.40 (1.33-4.35), P=0.004). Concomitant use of either category of immmunosuppressant was associated with numerically higher rates of opportunistic infection, 40% of which were due to herpes zoster, compared with adalimumab monotherapy. Higher disease activity in CD is associated with significantly increased risks of both serious and opportunistic infections. In addition to corticosteroid-sparing strategies, consideration should be given to expanding herpes zoster vaccination guidelines to include younger patients.

  3. [Complications of liver cirrhosis].

    Science.gov (United States)

    Bratović, Ismet; Jazić, Altaira

    2003-01-01

    Increase in portal venous pressure after anatomical or functional obstruction of portal venous system represents the most important complication of liver cirrhosis. Important sequels of portal hypertension are not dependable of etiology of liver disease. They are: increased collateral circulation in portal system and low pressure venous system (esophageal and gastric varices, portal hypertensive gastropathy and colopathy, hemorrhoids, collateral circulation through anterior abdominal wall, increased lymphatic flow, ascites, splenomegaly with occasional hypersplenismus, hepatic encephalopathy, hepatorenal syndrome.

  4. HLA-A*0206 with TLR3 polymorphisms exerts more than additive effects in Stevens-Johnson syndrome with severe ocular surface complications.

    Directory of Open Access Journals (Sweden)

    Mayumi Ueta

    Full Text Available BACKGROUND: Stevens-Johnson syndrome (SJS is an acute inflammatory vesiculobullous reaction of the skin and mucosa, often including the ocular surface, and toxic epidermal necrolysis (TEN occurs with its progression. Although SJS/TEN is thought to be initiated by certain types of medication coupled with possible infection. In the present study we examined the multiplicative interaction(s between HLA-A*0206 and 7 Toll-like receptor 3 (TLR3 Single-nucleotide polymorphisms (SNPs in patients with SJS/TEN. PRINCIPAL FINDINGS: We analyzed the genotypes for HLA-A and 7 TLR3 SNPs in 110 Japanese SJS/TEN patients with severe ocular complications and 206 healthy volunteers to examine the interactions between the two loci. We found that HLA-A*0206 exhibited a high odds ratio for SJS/TEN (carrier frequency: OR = 5.1; gene frequency: OR = 4.0 and that there was a strong association with TLR3 rs.5743312T/T SNP (OR = 7.4, TLR3 rs.3775296T/T SNP (OR = 5.8, TLR3 rs.6822014G/G SNP (OR = 4.8, TLR3 rs.3775290A/A SNP (OR = 2.9, TLR3 rs.7668666A/A SNP (OR = 2.7, TLR3 rs.4861699G/G SNP (OR = 2.3, and TLR3 rs.11732384G/G SNP (OR = 1.9. There was strong linkage disequilibrium (LD between rs.3775296 and rs.5743312 and between rs.7668666 and rs.3775290. The results of interaction analysis showed that the pair, HLA-A*0206 and TLR3 SNP rs3775296T/T, which exhibited strong LD with TLR3 rs.5743312, exerted more than additive effects (OR = 47.7. The other pairs, HLA-A*0206 and TLR3 rs.3775290A/A SNP (OR = 11.4 which was in strong LD with TLR3 rs7668666A/A SNP, and TLR3 rs4861699G/G SNP (OR = 7.6 revealed additive effects. Moreover, the combination HLA-A*0206 and TLR3 rs3775296T/T was stronger than the TLR3 rs6822014G/G and TLR3 rs3775290A/A pair, which reflected the interactions within the TLR3 gene alone. SIGNIFICANCE: By interaction analysis, HLA-A*0206 and TLR3 SNP rs3775296T/T, which were in strong LD with TLR3 SNP rs5743312T/T, manifested more than additive effects that

  5. Maintenance of improvement in spinal mobility, physical function and quality of life in patients with ankylosing spondylitis after 5 years in a clinical trial of adalimumab.

    Science.gov (United States)

    van der Heijde, Désirée; Breban, Maxime; Halter, Dale; DiVittorio, Gino; Bratt, Johan; Cantini, Fabrizio; Kary, Sonja; Pangan, Aileen L; Kupper, Hartmut; Rathmann, Suchitrita S; Sieper, Joachim; Mease, Phillip J

    2015-07-01

    Chronic pain and progressive loss of physical function with AS may adversely affect health-related quality of life (HRQoL). The objective of this study was to assess the 5-year data regarding spinal mobility, physical function and HRQoL in patients with AS who participated in the Adalimumab Trial Evaluating Long-term Efficacy and Safety for AS (ATLAS) study. Patients received blinded adalimumab 40 mg or placebo every other week for 24 weeks, then open-label adalimumab for up to 5 years. Spinal mobility was evaluated using linear BASMI (BASMIlin). BASDAI, total back pain, CRP, BASFI, Short Form-36 and AS quality of life (ASQoL) were also assessed. Correlations between BASMIlin and clinical, functional and ASQoL outcomes after 12 weeks and after 5years of adalimumab exposure were evaluated using Spearman's rank correlation. Associations were further analysed using multivariate regression. Three hundred and eleven patients received ≥1 dose of adalimumab; 125 of the 208 patients originally randomized to adalimumab received treatment for 5 years. Improvements in BASMIlin were sustained through 5 years, with a mean change of -0.6 from baseline in the population who completed 5 years of treatment with adalimumab. Improvements in disease activity, physical function and ASQoL were also sustained through 5 years. BASMIlin was significantly correlated with all evaluated clinical outcomes (P mobility, disease activity, physical function and HRQoL in patients with active AS. Spinal mobility was significantly associated with short- and long-term physical function in these patients. Clinicaltrials.gov; https://clinicaltrials.gov/NCT00085644. © The Author 2014. Published by Oxford University Press on behalf of the British Society for Rheumatology.

  6. Head-to-head comparison of subcutaneous abatacept versus adalimumab for rheumatoid arthritis: two-year efficacy and safety findings from AMPLE trial.

    Science.gov (United States)

    Schiff, Michael; Weinblatt, Michael E; Valente, Robert; van der Heijde, Désirée; Citera, Gustavo; Elegbe, Ayanbola; Maldonado, Michael; Fleischmann, Roy

    2014-01-01

    To compare over 2 years the safety, efficacy and radiographic outcomes of subcutaneous abatacept versus adalimumab, in combination with methotrexate (MTX), in patients with rheumatoid arthritis (RA). AMPLE is a phase IIIb, 2-year, randomised, investigator-blinded study with a 1-year primary endpoint. Biologic-naive patients with active RA and an inadequate response to MTX were randomised to 125 mg abatacept weekly or 40 mg adalimumab bi-weekly, both with a stable dose of MTX. Of 646 patients randomised, 79.2% abatacept and 74.7% adalimumab patients completed year 2. At year 2, efficacy outcomes, including radiographic, remained comparable between groups and with year 1 results. The American College Rheumatology 20, 50 and 70 responses at year 2 were 59.7%, 44.7% and 31.1% for abatacept and 60.1%, 46.6% and 29.3% for adalimumab. There were similar rates of adverse events (AEs) and serious adverse events (SAEs). More serious infections occurred with adalimumab (3.8% vs 5.8%) including two cases of tuberculosis with adalimumab. There were fewer discontinuations due to AEs (3.8% vs 9.5%), SAEs (1.6% vs 4.9%) and serious infections (0/12 vs 9/19 patients) in the abatacept group. Injection site reactions (ISRs) occurred less frequently with abatacept (4.1% vs 10.4%). Through 2 years of blinded treatment in this first head-to-head study between biologic disease-modifying antirheumatic drugs in RA patients with an inadequate response to MTX, subcutaneous abatacept and adalimumab were similarly efficacious based on clinical, functional and radiographic outcomes. Overall, AE frequency was similar in both groups but there were less discontinuations due to AEs, SAEs, serious infections and fewer local ISRs with abatacept.

  7. Long-term safety and efficacy of adalimumab for intestinal Behçet's disease in the open label study following a phase 3 clinical trial

    Directory of Open Access Journals (Sweden)

    Nagamu Inoue

    2017-07-01

    Full Text Available Background/Aims: Intestinal Behçet's disease (BD is an immune-mediated inflammatory disorder. We followed up the patients and evaluated safety profile and effectiveness of adalimumab for the treatment of intestinal BD through 100 weeks rolled over from the 52 week clinical trial (NCT01243671.Methods: Patients initiated adalimumab therapy at 160 mg at week 0, followed by 80 mg at week 2, followed by 40 mg every other week until the end of the study. Long-term safety and all adverse events (AEs were examined. The efficacy was assessed on the basis of marked improvement (MI and complete remission (CR using a composite efficacy index, which combined global gastrointestinal symptoms and endoscopic assessments.Results: Twenty patients were enrolled in this study; 15 patients received adalimumab treatment until study completion. The incidence of AEs through week 100 was 544.4 events/100 person-years, which was comparable to the incidence through week 52 (560.4 events/100 person-years. No unexpected trend was observed and adalimumab was well tolerated. At weeks 52 and 100, 60.0% and 40.0% of patients showed MI, respectively, and 20.0% and 15.0% of patients showed CR, respectively.Conclusions: This report demonstrates 2 years safety and effectiveness of adalimumab in intestinal BD patients. Patients with intestinal BD refractory to conventional treatment receiving up to 2 years of adalimumab treatment demonstrated safety outcomes consistent with the known profile of adalimumab, and the treatment led to sustained reduction of clinical and endoscopic disease activity.

  8. Outpatient Ulcerative Colitis Primary Anti-TNF Responders Receiving Adalimumab or Infliximab Maintenance Therapy Have Similar Rates of Secondary Loss of Response.

    Science.gov (United States)

    Ma, Christopher; Huang, Vivian; Fedorak, Darryl K; Kroeker, Karen I; Dieleman, Levinus A; Halloran, Brendan P; Fedorak, Richard N

    2015-09-01

    To compare the proportion of secondary loss of response to adalimumab and infliximab during maintenance treatment of ulcerative colitis (UC) after primary response to induction therapy. The efficacy of anti-tumor necrosis factor-α (TNF-α) therapy used to maintain response in patients with UC after primary response to induction therapy wanes with time, resulting in secondary loss of response. A retrospective cohort study evaluating anti-TNF-naive UC outpatients who were primary responders to adalimumab and infliximab induction therapy and who advanced onto a maintenance regimen with the respective anti-TNF agent from 2003 to 2013 was conducted. The primary outcome was the proportion of patients in each treatment group that had secondary loss of response. The secondary outcome was time to secondary loss of response, analyzed by the Kaplan-Meier method analysis. A total of 102 UC primary anti-TNF responders met inclusion criteria. Thirty-six patients (35.3%) were treated with adalimumab and 66 patients (64.7%) with infliximab. Mean follow-up was 139.0 weeks for adalimumab and 158.8 weeks for infliximab. A total of 21/36 (58.3%) adalimumab-treated patients and 39/66 (59.1%) infliximab-treated patients experienced a secondary loss of response during maintenance therapy. Mean time to secondary loss of response was similar for adalimumab (55.8 wk) and infliximab (59.4 wk) (P=0.82). Sex, extent of colitis, previous or concomitant azathioprine, and concurrent corticosteroids with anti-TNF induction were not associated with increased risk of secondary loss of response. In this real-life cohort of anti-TNF-naive primary responders with UC, the proportion of secondary loss of response and the time to secondary loss of response are similar for adalimumab and infliximab.

  9. The Biochemical, Histopathological and Clinical Comparison of the Neuroprotective Effects of Subcutaneous Adalimumab and Intravenous Methylprednisolone in an Experimental Compressive Spinal Cord Trauma Model.

    Science.gov (United States)

    Celik, Haydar; Karatay, Mete; Erdem, Yavuz; Yildirim, Ali Erdem; Sertbas, Idris; Karatay, Eylem; Kul, Halil; Guvenc, Yahya; Koksal, Ismet; Menekse, Guner; Alagoz, Fatih; Kertmen, Huseyin Hayri; Caydere, Muzaffer

    To evaluate the neuroprotective effects of adalimumab in an experimental spinal cord injury model and compare them with those of the widely-used methylprednisolone. Forty male Wistar rats were divided into 5 as the sham, trauma, adalimumab, methylprednisolone, and adalimumab+methylprednisolone groups. Only laminectomy was performed in the sham group. Laminectomy and trauma was performed to the trauma group but no treatment was given. A single dose of 40 mg/kg subcutaneous adalimumab was administered after the laminectomy and trauma to group 3. A single dose of intravenous 30 mg/kg methylprednisolone was administered right after laminectomy and trauma to group 4. Single doses of 40 mg/kg adalimumab and 30 mg/kg methylprednisolone were administered together after laminectomy and trauma to group 5. Serum malondialdehyde (MDA), TNF-α, IL-1β and IL-6 levels were measured and sections were obtained for histopathological study at the end of the 7 th day. MDA, TNF-α, IL-1β and IL-6 levels in serum were significantly decreased in the adalimumab group with clinical and histopathological improvement not less than the methylprednisolone group. The serum MDA levels were similar when the two drugs were given together or separately but there was a statistically quite significant decrease in TNF-α, IL-1β and IL-6 levels with concurrent use. Statistically significantly better results were obtained on histopathological evaluation with the use of both drugs together. This study revealed that adalimumab is as effective as methylprednisolone in compressive spinal cord injury in rats.

  10. Postpartum complications

    International Nuclear Information System (INIS)

    Kronthal, A.J.; Kuhlman, J.E.; Fishman, E.K.

    1990-01-01

    This paper reports the CT findings of major postpartum complications and determine what role CT plays in their evaluation. The CT scans of nine patients with major postpartum complications were retrospectively reviewed. Patients had been referred to CT for evaluation of postpartum fever, abdominal pain, and elevated results of liver function tests. Complications identified at CT included hepatic infarctions (n = 2), endometritis (n = 2), postoperative wound abscess (n = 1), massive abdominal hemorrhage (n = 1), septic thrombophlebitis (n = 1), and renal vein thrombosis (n = 1). CT findings of hepatic infarction included wedge-shaped areas of decreased enhancement conforming to a vascular distribution

  11. Post dengue neurological complication

    Directory of Open Access Journals (Sweden)

    Hizlinda Tohid

    2015-12-01

    Full Text Available Dengue infection is highly endemic in many tropical countries including Malaysia. However, neurological complications arising from dengue infection is not common; Gullain–Barre syndrome (GBS is one of these infrequent complications. In this paper, we have reported a case in which a 39-year-old woman presented with a neurological complication of dengue infection without typical symptoms and signs of dengue fever. She had a history of acute gastroenteritis (AGE followed by an upper respiratory tract infection (URTI weeks prior to her presentation rendering GBS secondary to the post viral URTI and AGE as the most likely diagnosis. Presence of thrombocytopenia was the only clue for dengue in this case.

  12. [Sample representativeness and incidence of liver biopsy complications caused by needles of bigger diameter (1.6 mm) and smaller diameter (1.2 mm) in children with cholestatic syndrome].

    Science.gov (United States)

    Bosković, Aleksandra; Kitić, Ivana; Prokić, Dragan; Stanković, Ivica

    2013-01-01

    Percutaneous liver biopsy and histomorphological analysis of liver tissue is an important diagnostic procedure in the investigation of neonates and infants with cholestatic syndrome. This study has been aimed at determining whether there is a difference in the incidence of complications after liver biopsy performed by Menghini technique using a needle of 1.6 mm as compared to 1.2 mm diameter and if there is a difference in the sample representativeness of liver tissue after liver biopsy with those two different needle diameters. We retrospectively reviewed medical records of 156 neonates and infants with chronic cholestatic syndrome, hospitalized at Mother and Child Health Care Institute, Serbia. One hundred and fifty six children underwent liver biopsy. There was no difference in frequency of liver biopsy complications performed by Menghini technique using a larger diameter needle (1.6 mm) as compared to 1.2 mm diameter. The mortality after liver biopsy was 0% while the frequency of complications with a needle of 1.6 mm in diameter was 3.8% the percentage of serious complications being 0.6%. Among the samples of liver biopsy taken by a larger diameter needle (1.6 mm), 108/109 were representative samples (> 5 portal areas), and among those taken by a smaller diameter needle (1.2 mm), 34/47 were representative samples. Of 109 liver biopsy specimens obtained by Menghini technique using a needle of larger diameter (1.6 mm), 109/109 were representative samples (> 3 portal areas), and when a smaller diameter needle (1.2 mm) was used, 42/47 were representative samples. Our results indicate that the sample representativeness was significantly higher when a larger diameter needle was used for liver biopsy by Menghini technique; however, no difference in the incidence of complications was observed.

  13. Complications of cirrhosis. A 50 years flashback

    DEFF Research Database (Denmark)

    Møller, Søren; Bendtsen, Flemming

    2015-01-01

    In patients with cirrhosis and portal hypertension, it is largely the frequency and severity of complications relating to the diseased liver, degree of portal hypertension and hemodynamic derangement that determine the prognosis. It can be considered as a multiple organ failure that apart from th...... complications such as renal failure precipitating the hepatorenal syndrome. Patients with end-stage liver disease and related complications as for example the hepatopulmonary syndrome can only radically be treated by liver transplantation....

  14. [Bone metabolism and cardiovascular function update. α-klotho/FGF23 system; a new insight into the field of mineral homeostasis and the pathogeneses of aging-associated syndromes and the complications of chronic kidney disease].

    Science.gov (United States)

    Nabeshima, Yo Ichi

    2014-07-01

    α-klotho (α-kl) was first identified as an aging gene and was later shown to be a regulator of mineral homeostasis. α-kl (- / -) mice display multiple aging related phenotypes including atherosclerosis, cardiovascular/soft tissue calcifications, pulmonary emphysema, osteopenia, and senile atrophy of skin ; such age-related organ pathologies are associated with biochemical changes in blood, including severe hyperphosphatemia, elevated serum FGF23 and1,25 (OH) 2 Vitamin D levels. Of significance, advanced stage patients suffering chronic kidney disease (CKD) develop multiple complications quite resembling phenotypes observed in α-kl (- / -) mice, and high serum phosphate, the major cause of abnormalities of α-kl (- / -) mice, has been reported to be closely associated with high levels of cardiovascular disease morbidity and mortality in patients with CKD, particularly in patients with end-stage renal disease. In addition, the expressions of α-kl mRNA and α-Kl protein were severely reduced in these patients. These results suggest the involvement of α-Kl and FGF23 in the pathogeneses of not only aging-associated syndromes but also the complications of CKD. Here, the unveiling of the molecular functions of α-Klotho and FGF23 has recently given new insight into the field of mineral homeostasis and the pathogeneses of aging-associated syndromes and the complications of CKD.

  15. Diphtheria Complications

    Science.gov (United States)

    ... Search Form Controls Cancel Submit Search The CDC Diphtheria Note: Javascript is disabled or is not supported ... message, please visit this page: About CDC.gov . Diphtheria Home About Diphtheria Causes and Transmission Symptoms Complications ...

  16. Complicated Pancreatitis

    NARCIS (Netherlands)

    Bakker, O.J.

    2015-01-01

    Research questions addressed in this thesis: What is the accuracy of serum blood urea nitrogen as early predictor of complicated pancreatitis? ; What is difference in clinical outcome between patients with pancreatic parenchymal necrosis and patients with extrapancreatic necrosis without necrosis

  17. Nephrotic syndrome due to minimal change disease secondary to spider bite: clinico-pathological case of a non-described complication of latrodectism.

    Science.gov (United States)

    Méndez, Gonzalo P; Enos, Daniel; Moreira, José Luis; Alvaredo, Fátima; Oddó, David

    2017-04-01

    The patient was an 18-year-old man who developed nephrotic syndrome after a 'wheat spider' bite ( Latrodectus mactans ). Due to this atypical manifestation of latrodectism, a renal biopsy was performed showing minimal change disease. The nephrotic syndrome subsided after 1 week without specific treatment. This self-limited evolution suggests that the mechanism of podocyte damage was temporary and potentially mediated by a secondary mechanism of hypersensitivity or direct effect of the α-latrotoxin. The patient did not show signs of relapse in subsequent checkup. This is the first reported case of nephrotic syndrome due to a minimal change lesion secondary to latrodectism.

  18. No overall damage progression despite persistent inflammation in adalimumab-treated psoriatic arthritis patients

    DEFF Research Database (Denmark)

    Poggenborg, René Panduro; Wiell, Charlotte; Bøyesen, Pernille

    2014-01-01

    OBJECTIVE: In a comparative conventional MRI, dynamic contrast-enhanced (DCE)-MRI, CT and radiography study, the authors aimed to monitor whether inflammation is reduced or even eliminated and damage halted in PsA patients receiving anti-TNF therapy. METHODS: A 48-week prospective open-label inve......OBJECTIVE: In a comparative conventional MRI, dynamic contrast-enhanced (DCE)-MRI, CT and radiography study, the authors aimed to monitor whether inflammation is reduced or even eliminated and damage halted in PsA patients receiving anti-TNF therapy. METHODS: A 48-week prospective open......-label investigator-initiated trial of 41 biologic-naive patients treated with 40 mg adalimumab every other week. Hand CT, MRI (according to the PsA MRI scoring system method) and radiography (Sharp-van der Heijde method) were obtained at weeks 0, 6 (only MRI), 24 and 48. Clinical response was assessed by the Ps...... as the standard reference for detecting bone erosions/proliferations, sensitivity, specificity and accuracy were 100%/40%, 83%/93% and 84%/86%, respectively, for MRI, whereas corresponding values for radiography were 17%/26%, 98%/96%, and 93%/87%, respectively. Erosive progression as assessed by CT was found in 6...

  19. Comparing the effects of inorganic nitrate and allopurinol in renovascular complications of metabolic syndrome in rats: role of nitric oxide and uric acid.

    Science.gov (United States)

    Essawy, Soha S; Abdel-Sater, Khaled A; Elbaz, Amani A

    2014-06-29

    The epidemic of metabolic syndrome is increasing worldwide and correlates with elevation in serum uric acid and marked increase in total fructose intake. Fructose raises uric acid and the latter inhibits nitric oxide bioavailability. We hypothesized that fructose-induced hyperuricemia may have a pathogenic role in metabolic syndrome and treatment of hyperuricemia or increased nitric oxide may improve it. Two experiments were performed. Male Sprague-Dawley rats were fed a control diet or a high-fructose diet to induce metabolic syndrome. The latter received either sodium nitrate or allopurinol for 10 weeks starting with the 1(st) day of fructose to evaluate the preventive role of the drugs or after 4 weeks to evaluate their therapeutic role. A high-fructose diet was associated with significant (p metabolic syndrome and obesity due to its ability to raise uric acid. Either sodium nitrate or allopurinol can prevent this pathological condition by different mechanisms of action.

  20. Prediction of successful dose reduction or discontinuation of adalimumab, etanercept, or infliximab in rheumatoid arthritis patients using serum drug levels and antidrug antibody measurement.

    Science.gov (United States)

    Bouman, Cam; van Herwaarden, N; van den Hoogen, Fhj; van der Maas, A; van den Bemt, Bjf; den Broeder, A A

    2017-06-01

    To evaluate if TNF inhibitor serum drug levels (DL) or anti-drug antibodies (ADAb) can predict successful dose reduction (in patients with high DL) or discontinuation (in patients with no/low DL or ADAb) in rheumatoid arthritis (RA) patients. RA patients that were using adalimumab (n = 42), etanercept (n = 76) or infliximab (n = 51) and were doing well, were tapered until discontinuation or flare (1-1.5 year follow up). Random timed DL for adalimumab and etanercept and trough DL for infliximab were measured before dose reduction: Receiver-Operator-Curves (ROC) analyses with optimal cut-off DL were determined. No predictive value of adalimumab and infliximab DL for all outcomes were found, except for an inverse association of lower etanercept DL and higher chance for successful dose reduction (Area Under the Curve (AUC) 0.36, 95% CI 0.23-0.49; cut-off 7.8). ADAb were infrequent and not predictive of successful discontinuation. No predictive value of baseline adalimumab, etanercept and infliximab DL or ADAb for successful dose reduction or discontinuation in RA was found in this context, with the possible exception of high adalimumab trough levels for successful dose reduction.

  1. Human Leukocyte Antigen Class I Genes Associated With Stevens-Johnson Syndrome and Severe Ocular Complications Following Use of Cold Medicine in a Brazilian Population.

    Science.gov (United States)

    Wakamatsu, Tais H; Ueta, Mayumi; Tokunaga, Katsushi; Okada, Yukinori; Loureiro, Renata R; Costa, Karita A; Sallum, Juliana Maria F; Milhomens, José Arthur; Inoue, Chikara; Sotozono, Chie; Gomes, José Álvaro P; Kinoshita, Shigeru

    2017-04-01

    Describing the association with human leukocyte antigen (HLA) alleles could facilitate the understanding of increased risk factors for development of Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) in patients with severe ocular complications (SOCs). To investigate the association between HLA class I genes and cold medicine (CM)-associated SJS/TEN with SOCs. This case-control study was conducted between February 8, 2013, and August 29, 2014. Thirty-nine Brazilian patients with CM-SJS/TEN of 74 patients with SJS/TEN with SOCs and 133 healthy Brazilian volunteers were enrolled. Human leukocyte antigen class I genes (HLA-A, HLA-B, and HLA-C) were examined to determine whether there was a genetic predisposition for CM-SJS/TEN with SOC. Patients were interviewed to identify possible etiologic factors. Data analysis was performed from April 14, 2013, to August 29, 2014. Genetic predisposition for CM-SJS/TEN with SOCs by analysis of HLA class I genes. Of 74 patients included in the analysis, 32 (43%) were male; mean (SD) age was 36.01 [15.42] years. HLA-A*66:01 (odds ratio [OR], 24.0; 95% CI, 2.79-206.0; P < .001), HLA-B*44:03 (OR, 2.71; 95% CI, 1.11-6.65; P = .04), and HLA-C*12:03 (OR, 5.6; 95% CI, 1.67-18.80; P = .006) were associated with Brazilian CM-SJS/TEN with SOCs, and HLA-A*11:01 (OR, 0.074; 95% CI, 0.004-1.26; P = .008), HLA-B*08:01 (OR, 0.15; 95% CI, 0.02-1.15; P = .048), and HLA-B*51:01 (OR, 0.23; 95% CI, 0.05-1.03; P = .045) were inversely associated with Brazilian CM-SJS/TEN with SOCs (39 cases: 19 Pardo and 16 European ancestry; 14 males and 25 females; age, 35.2 [14.4] years; and 133 controls: 66 Pardo and 61 European ancestry; 55 males and 78 females; age, 41.2 [12.9] years). When multiple test correction within the HLA locus, HLA-A*66:01 and HLA-C*12:03 demonstrated associations. When participants were segregated into Pardo and locus is considered, HLA-A*66:01 was associated with CM-SJS/TEN with SOC among

  2. Relation of serum magnesium level to microvascular complications and the components of metabolic syndrome in patients with type 2 diabetes mellitus

    Directory of Open Access Journals (Sweden)

    Heba M Yossef

    2017-01-01

    Hypomagnesemia is prevalent in diabetic patients. It is associated with diabetic complications and poor glycemic control. High plasma triglycerides, total cholesterol, creatinine level, albuminuria, HbA1c, FBS, and 2-h PPBS are independent correlates of hypomagnesemia.

  3. Unusual Complications of Quinalphos Poisoning

    Directory of Open Access Journals (Sweden)

    Stalin Viswanathan

    2013-01-01

    Full Text Available This 40-year-old man was treated for suicidal quinalphos 25%EC consumption. He developed intermediate syndrome with normal response to repetitive nerve stimulation, pancreatitis with high enzyme elevations, and normal computed tomography and excreted black, brown, and orange urine sequentially over the first nine days of hospitalization. The last complication has not been previously reported with any organophosphate compound. He finally succumbed to complication of ventilator associated pneumonia related septic shock and ventricular tachycardia.

  4. Vascular complications (splenic and hepatic artery aneurysms) in the occipital horn syndrome: report of a patient and review of the literature

    Energy Technology Data Exchange (ETDEWEB)

    Mentzel, H.-J. [Institute of Diagnostic and Interventional Radiology, University of Jena (Germany)]|[Institute of Diagnostic and Interventional Radiology, Bachstrasse 18, D-07 740 Jena (Germany); Seidel, J.; Vogt, L. [Department of Paediatrics, University of Jena, Friedrich-Schiller-Universitaet Jena, Jena/Thueringen (Germany); Vogt, S.; Kaiser, W.A. [Institute of Diagnostic and Interventional Radiology, University of Jena (Germany)

    1999-01-01

    We report an 18-year-old boy with occipital horn syndrome who developed aneurysms of the splenic and hepatic arteries. Occipital horn syndrome, also called X-linked cutis laxa or Ehlers-Danlos syndrome (EDS) type IX, is characterised by a skeletal dysplasia which includes occipital horns, broad clavicles, deformed radii, ulnae and humeri, narrow rib cage, undercalcified long bones and coxa valga. Distinctive features common to all patients are unusual facial appearance, hypermobility of finger joints, limitation of extension of elbows, chronic diarrhoea and genitourinary abnormalities. In this case report we describe the difficulties encountered in the diagnostic management of patients with EDS-related vascular lesions. (orig.) With 5 figs., 2 tabs., 12 refs.

  5. Incidence of Antidrug Antibodies in Rheumatoid Arthritis Patients From Argentina Treated With Adalimumab, Etanercept, or Infliximab in a Real-World Setting.

    Science.gov (United States)

    Maid, Pablo J; Xavier, Ricardo; Real, Rosa M; Pedersen, Ron; Shen, Qi; Marshall, Lisa; Solano, Gaston; Borlenghi, Cecilia Elena; Hidalgo, Rodolfo Pardo

    2017-12-12

    Biologic agents may induce immune responses that could impact drug action. The aims of this study were to assess antidrug antibodies (ADAs) in patients with rheumatoid arthritis (RA) from Argentina treated with etanercept, adalimumab, or infliximab at a single visit and correlate it with efficacy outcomes. In this subset analysis of a noninterventional, multinational, cross-sectional study (NCT01981473), adult patients with RA treated continuously for 6 to 24 months with etanercept, adalimumab, or infliximab were evaluated for ADAs and trough drug concentrations of 2 days or less prior to the next scheduled dose. Efficacy measurements included Disease Activity Score based on a 28-joint count-erythrocyte sedimentation rate, low disease activity, and Health Assessment Questionnaire-Disability Index. Targeted medical history of injection site/infusion reactions, serum sickness, and thromboembolic events were reported. Baseline demographics, disease characteristics, and duration of treatment of the 119 patients (etanercept: n = 54, adalimumab: n = 52, infliximab: n = 13) were similar across all groups. No etanercept-treated patient tested positive for ADAs compared with 19 (36.5%) of 52 patients and 4 (30.8%) of 13 patients treated with adalimumab and infliximab, respectively. In adalimumab- and infliximab-treated patients, ADA presence correlated negatively with trough drug levels. A greater proportion of ADA-negative patients achieved Health Assessment Questionnaire-Disability Index of 0.5 or less and had better composite efficacy measures compared with ADA-positive patients. The rate of targeted medical events reported was low. In this subset analysis, RA patients from Argentina treated with adalimumab or infliximab, but not etanercept, tested positive for ADAs. Antidrug antibody-negative patients showed a tendency toward better clinical outcomes compared with ADA-positive patients.

  6. Clinical efficacy of adalimumab versus infliximab and the factors associated with recurrence or aggravation during treatment of anal fistulas in Crohn's disease

    Directory of Open Access Journals (Sweden)

    Cheng-Chun Ji

    2017-04-01

    Full Text Available Background/Aims: Infliximab has proven to be effective in the treatment of perianal fistulas in Crohn's disease (CD but the efficacy of adalimumab is still unclear. The aim of this study is to assess the clinical efficacy of adalimumab and compare the results with those for infliximab.Methods: Forty-seven CD patients treated for perianal fistulas with infliximab from September 2005 to December 2010 (n=31, or with adalimumab from November 2010 to May 2012 (n=16, were enrolled in this retrospective study. The following patient characteristics were analyzed; intestinal lesion site, fistula classification, seton placement, index of inflammatory bowel disease, C-reactive protein level, follow-up period, and the cumulative rate of nonrecurrence or aggravation of fistula.Results: There were no significant differences in the intestinal lesion site, fistula classification, inflammatory bowel disease index, C-reactive protein level, and the frequency of injection between the infliximab group and the adalimumab group. The cumulative rate of nonrecurrence or aggravation of fistula was 62.5% in the adalimumab group and 83.9% in the infliximab group at 24 months after treatment (P=0.09. The risk factors for recurrence or aggravation may be related to seton placement (P=0.02, gender (P=0.06, and fistula classification (P=0.07.Conclusions: There was no significant difference in the clinical efficacy of adalimumab and infliximab in the treatment of perianal fistulas in CD. However, fistula classification may be an important risk factor for recurrence or aggravation. The preliminary findings in this study show that further research is warranted.

  7. Similar Improvements in Patient-Reported Outcomes Among Rheumatoid Arthritis Patients Treated with Two Different Doses of Methotrexate in Combination with Adalimumab: Results From the MUSICA Trial.

    Science.gov (United States)

    Kaeley, Gurjit S; MacCarter, Daryl K; Goyal, Janak R; Liu, Shufang; Chen, Kun; Griffith, Jennifer; Kupper, Hartmut; Garg, Vishvas; Kalabic, Jasmina

    2018-03-24

    In patients with rheumatoid arthritis (RA), combination treatment with methotrexate (MTX) and adalimumab is more effective than MTX monotherapy. From the patients' perspective, the impact of reduced MTX doses upon initiating adalimumab is not known. The objective was to evaluate the effects of low and high MTX doses in combination with adalimumab initiation on patient-reported outcomes (PROs), in MTX-inadequate responders (MTX-IR) with moderate-to-severe RA. MUSICA was a randomized, double-blind, controlled trial evaluating the efficacy of 7.5 or 20 mg/week MTX, in combination with adalimumab for 24 weeks in MTX-IR RA patients receiving prior MTX ≥ 15 mg/week for ≥ 12 weeks. PROs were recorded at each visit, including physical function, health-related quality-of-life, work productivity, quality-of-sleep, satisfaction with treatment medication, sexual impairment due to RA, patient global assessment of disease activity (PGA), and patient pain. Last observation carried forward was used to account for missing values. At baseline, patients in both MTX dosage groups had similar demographics, disease characteristics, and PRO scores. Overall, initiation of adalimumab led to significant improvements from baseline in the PROs assessed for both MTX dosage groups. Improvements in presenteeism from baseline were strongly correlated with corresponding improvements in SF-36 (vitality), pain, and physical function. Physical and mental well-being had a good correlation with improvement in sleep. Overall, improvements in disease activity from baseline were correlated with improvements in several PROs. The addition of adalimumab to MTX in MTX-IR patients with moderate-to-severe RA led to improvements in physical function, quality-of-life, work productivity, quality of sleep, satisfaction with treatment medication, and sexual impairment due to RA, regardless of the concomitant MTX dosage. AbbVie. Clinicaltrials.gov identifier, NCT01185288.

  8. Chylous Ascites in a Patient with HIV/AIDS: A Late Complication of Mycobacterium avium Complex-Immune Reconstitution Inflammatory Syndrome

    Directory of Open Access Journals (Sweden)

    Imam H. Shaik

    2014-01-01

    Full Text Available Chylous ascites is very rare in HIV/AIDS and its association with Mycobacterium avium complex-immune reconstitution inflammatory syndrome (MAC-IRIS has been rarely reported. Here, we report a case of a young African-American male who developed chylous ascites as a late sequela to immune reconstitution inflammatory syndrome while on treatment for MAC. Antiretroviral drug-naive patients who start HAART in close proximity to the diagnosis of an opportunistic infection and have a rapid decline in HIV RNA level should be monitored for development of IRIS. Although the long term prognosis is poor, early diagnosis and treatment help to improve quality of life.

  9. The role of adalimumab in rheumatic and autoimmune disorders: comparison with other biologic agents

    Directory of Open Access Journals (Sweden)

    Reimold AM

    2012-05-01

    Full Text Available Andreas M ReimoldDallas Veterans Affairs Medical Center and Rheumatic Diseases Division, University of Texas Southwestern Medical Center, Dallas, TX, USAAbstract: Adalimumab (ADA is a biologic medication that dampens inflammatory pathways by binding to the cytokine tumor necrosis factor alpha. The US Food and Drug Administration has approved ADA as a medication for use in rheumatoid arthritis, psoriatic arthritis, ankylosing spondylitis, Crohn's disease, psoriasis, and juvenile idiopathic arthritis. This year marks 10 years of clinical experience with ADA. Long-term extension studies of some of the initial clinical trials, as well as data from large patient registries, are demonstrating ongoing benefit for responders. Potential side effects such as increased risk of infection, lymphoma, congestive heart failure, and demyelination continue to be examined, as the available data are not unanimous in showing an increase in incidence. In balancing both the advantages and the disadvantages of using ADA, the drug's overall effectiveness and its availability for use in patients with hepatic or renal comorbidities are weighed against the high cost. ADA is expected to have a leading role in the treatment of rheumatoid arthritis and other inflammatory conditions for years to come. Future studies will need to address the optimal sequence of disease-modifying antirheumatic drugs and biologics to use, combinations of disease-modifying antirheumatic drugs and biologics, and head-to-head comparisons of biologics in clinical trials. For those who go into clinical remission on an anti-tumor necrosis factor medication, unanswered questions remain about identifying the patients who can maintain the remission off all drugs, or at least off injected medication. Given the cost of biologic drugs, even studies that increase the interval between drug doses in well-controlled patients could provide financial benefits.Keywords: rheumatoid arthritis, Humira®, tumor

  10. Methotrexate Dosage Reduction Upon Adalimumab Initiation: Clinical and Ultrasonographic Outcomes from the Randomized Noninferiority MUSICA Trial.

    Science.gov (United States)

    Kaeley, Gurjit S; Evangelisto, Amy M; Nishio, Midori J; Goss, Sandra L; Liu, Shufang; Kalabic, Jasmina; Kupper, Hartmut

    2016-08-01

    To examine the clinical and ultrasonographic (US) outcomes of reducing methotrexate (MTX) dosage upon initiating adalimumab (ADA) in MTX-inadequate responders with moderately to severely active rheumatoid arthritis (RA). MUSICA (NCT01185288) was a double-blind, randomized, parallel-arm study of 309 patients with RA receiving MTX ≥ 15 mg/week for ≥ 12 weeks before screening. Patients were randomized to high dosage (20 mg/week) or low dosage (7.5 mg/week) MTX; all patients received 40 mg open-label ADA every other week for 24 weeks. The primary endpoint was Week 24 mean 28-joint Disease Activity Score based on C-reactive protein (DAS28-CRP) to test for noninferiority of low-dosage MTX using a 15% margin. US images were scored using a 10-joint semiquantitative system incorporating OMERACT definitions for pathology, assessing synovial hypertrophy, vascularity, and bony erosions. Rapid improvement in clinical indices was observed in both groups after addition of ADA. The difference in mean DAS28-CRP (0.37, 95% CI 0.07-0.66) comparing low-dosage (4.12, 95% CI 3.88-4.34) versus high-dosage MTX (3.75, 95% CI 3.52-3.97) was statistically significant and non-inferiority was not met. Statistically significant differences were not detected for most clinical, functional, and US outcomes. Pharmacokinetic and safety profiles were similar. In MUSICA, Week 24 mean DAS28-CRP, the primary endpoint, did not meet non-inferiority for the low-dosage MTX group. Although the differences between the 2 MTX dosage groups were small, our study findings did not support routine MTX reduction in MTX inadequate responders initiating ADA.

  11. Head-to-head comparison of subcutaneous abatacept versus adalimumab for rheumatoid arthritis: two-year efficacy and safety findings from AMPLE trial

    OpenAIRE

    Schiff, Michael; Weinblatt, Michael E; Valente, Robert; van der Heijde, Désirée; Citera, Gustavo; Elegbe, Ayanbola; Maldonado, Michael; Fleischmann, Roy

    2013-01-01

    Objectives To compare over 2 years the safety, efficacy and radiographic outcomes of subcutaneous abatacept versus adalimumab, in combination with methotrexate (MTX), in patients with rheumatoid arthritis (RA). Methods AMPLE is a phase IIIb, 2-year, randomised, investigator-blinded study with a 1-year primary endpoint. Biologic-naive patients with active RA and an inadequate response to MTX were randomised to 125 mg abatacept weekly or 40 mg adalimumab bi-weekly, both with a stable dose of MT...

  12. [Congenital disorders of the cardiovascular system and their complications in a 21-year-old woman with Turner syndrome--a case report].

    Science.gov (United States)

    Załuska, Roman; Kazłowski, Jacek; Gumkowska, Anna; Pikto-Pietkiewicz, Witold

    2008-01-01

    A 21-year-old woman with Turner syndrome was admitted to hospital because of progressive heart failure. Echocardiography revealed a bicuspid aortic valve with one cusp ruptured due to infective endocarditis and anomalous pulmonary venous drainage. The patient underwent successful aortic valve replacement.

  13. The obstetric antiphospholipid syndrome

    NARCIS (Netherlands)

    Derksen, R. H. W. M.; de Grootb, Ph. G.

    The association of persistent presence of circulating antiphospholipid antibodies and thromboembolic events, (recurrent) pregnancy loss or both is termed antiphospholipid syndrome. Pregnancies in women with the syndrome should be regarded as at high-risk for complications. Optimal management

  14. Pre- and perinatal complications in relation to Tourette syndrome and co-occurring obsessive-compulsive disorder and attention-deficit/hyperactivity disorder

    DEFF Research Database (Denmark)

    Abdulkadir, Mohamed; Tischfield, Jay A; King, Robert A

    2016-01-01

    -deficit/hyperactivity disorder (ADHD) in individuals with a tic disorder. Therefore, we aimed to investigate the role of pre- and perinatal complications in relation to the presence and symptom severity of chronic tic disorder and co-occurring OCD and ADHD using data of 1113 participants from the Tourette International...... Collaborative Genetics study. This study included 586 participants with a chronic tic disorder and 527 unaffected family controls. We controlled for age and sex differences by creating propensity score matched subsamples for both case-control and within-case analyses. We found that premature birth (OR = 1.......72) and morning sickness requiring medical attention (OR = 2.57) were associated with the presence of a chronic tic disorder. Also, the total number of pre- and perinatal complications was higher in those with a tic disorder (OR = 1.07). Furthermore, neonatal complications were related to the presence (OR = 1...

  15. [Pulse indicator continuous cardiac output measurement-guided treatment aids two pediatric patients with severe acute pancreatitis complicated with acute respiratory distress syndrome].

    Science.gov (United States)

    Yan, Weiyuan; Wang, Lijie

    2014-09-01

    To evaluate the clinical value of the pulse indicator continuous cardiac output (PiCCO) system in patients with severe acute pancreatitis (SAP) complicated with acute respiratory distress syndrome (ARDS). Two cases of SAP with ARDS were monitored using PiCCO during comprehensive management in the Pediatric Intensive Care Unit (PICU) of Shengjing Hospital, China Medical University. To guide fluid management, the cardiac index (CI) was measured to assess cardiac function, the global end-diastolic volume index (GEDVI) was used to evaluate cardiac preload, and the extravascular lung water index (EVLWI) was used to evaluate the pulmonary edema. Case 1 was diagnosed with type L2 acute lymphoblastic leukemia (intermediate risk) and received the sixth maintenance phases of chemotherapy this time. After a 1-week dosage of chemotherapeutic drugs (pegaspargase and mitoxantrone), he suffered SAP combined with ARDS. Except comprehensive treatment (life supporting, antibiotic, etc.) and applying continuous veno-venous hemodiafiltration (CVVHDF) to remove inflammatory mediators. PiCCO monitor was utilized to guide fluid management. During the early stage of PiCCO monitoring, the patient showed no significant manifestations of pulmonary edema in the bedside chest X-ray (bedside ultrasound showed left pleural effusion), and had an oxygenation index 223 mmHg (1 mmHg = 0.133 kPa), GEDVI 450 ml/m², and ELVWI 7 ml/kg. We increased cardiac output to increase tissue perfusion and dehydration speed of CVVHDF was set at 70 ml/h. Two hours later, GEDVI significantly increased to 600 ml/m² and ELVWI significantly increased to 10 ml/kg, the oxygenation index declined to 155 mmHg, the bedside chest X-ray showed a significant decrease of permeability (right lung) and PEEP was adjusted to 5 cmH₂O (1 cmH₂O = 0.098 kPa), indicating circulating overload. ARDS subsequently occurred, upon which the fluid infusion was halted, the dehydration rate of CVVHDF raised (adjusted to 100-200 ml/h). On

  16. Pulmonary complications in neurosurgical patients

    Directory of Open Access Journals (Sweden)

    Randeep Guleria

    2012-01-01

    Full Text Available Pulmonary complications are a major cause of morbidity and mortality in neurosurgical patients. The common pulmonary complications in neurosurgical patients include pneumonia, postoperative atelectasis, respiratory failure, pulmonary embolism, and neurogenic pulmonary edema. Postoperative lung expansion strategies have been shown to be useful in prevention of the postoperative complications in surgical patients. Low tidal volume ventilation should be used in patients who develop acute respiratory distress syndrome. An antibiotic use policy should be put in practice depending on the local patterns of antimicrobial resistance in the hospital. Thromboprophylactic strategies should be used in nonambulatory patients. Meticulous attention should be paid to infection control with a special emphasis on hand-washing practices. Prevention and timely management of these complications can help to decrease the morbidity and mortality associated with pulmonary complications.

  17. Further delineation of FKBP14-related Ehlers-Danlos syndrome: A patient with early vascular complications and non-progressive kyphoscoliosis, and literature review.

    Science.gov (United States)

    Dordoni, Chiara; Ciaccio, Claudia; Venturini, Marina; Calzavara-Pinton, Piergiacomo; Ritelli, Marco; Colombi, Marina

    2016-08-01

    FKBP14-related Ehlers-Danlos syndrome (EDS) is an extremely rare recessive connective tissue disorder described for the first time in 2012 by Baumann and coworkers. The causal gene, FKBP14, encodes a member of the F506-binding family of peptidyl-prolyl cis-trans isomerases. The paucity of patients described so far makes this disorder poorly defined at clinical level. Here, we report an additional pediatric patient, who is compound heterozygous for a recurrent and a novel FKBP14 mutation, and compare his phenotype with those available in literature. This evaluation confirms that kyphoscoliosis (either progressive or non-progressive), myopathy, joint hypermobility, and congenital hearing loss (sensorineural, conductive, or mixed) are the typical features of the syndrome. Since the patient showed a severe cardiovascular event in childhood and atlantoaxial instability, this report expands the phenotype of the disorder and the allelic repertoire of FKBP14. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

  18. Plasmodium vivax induced hemolytic uremic syndrome: An uncommon manifestation that leads to a grave complication and treated successfully with renal transplantation.

    Science.gov (United States)

    Jhorawat, Rajesh; Beniwal, Pankaj; Malhotra, Vinay

    2015-01-01

    We are reporting a case of hemolytic uremic syndrome, a rare manifestation of Plasmodium vivax malaria. A young driver was admitted with acute febrile illness, decreased urine output, anemia, thrombocytopenia, jaundice, and increased serum lactate dehydrogenase. He showed a partial response to antimalarial drugs. However, he was readmitted with worsening renal parameters. His kidney biopsy revealed chronic thrombotic microangiopathy. He remained dialysis dependent and later underwent renal transplantation successfully, with excellent graft function at 1-year.

  19. Onychomycosis in patients of nail psoriasis on biologic therapy: a randomized, prospective open label study comparing Etanercept, Infliximab and Adalimumab.

    Science.gov (United States)

    Al-Mutairi, Nawaf; Nour, Tarek; Al-Rqobah, Duha

    2013-05-01

    The association between patients of psoriasis on anti TNF therapy and onychomycosis has not been explored. The aim of this study was to determine the rate of onychomycosis in patients of psoriasis with nail involvement on anti TNF therapy. All patients of psoriasis with nail involvement seen between February 2007 - July 2012 were examined. All the patients with negative nail scrapings for fungus were enrolled. Patients found fit for biologics after investigations were randomly divided into 3 groups (Group A: Infliximab, Group B: Etanercept and Group C: Adalimumab). The patients were followed up every 4 weeks for 24 weeks. Repeat nail scrapings were done at week 24. The results were compared with controls. In total, 315 (178 males and 137 females) patients were enrolled. The mean age was 37.5 ± 11.4 years. The results for scraping for fungus at the end of 24 weeks were as follows: 33% (33/100) in patients on Infliximab followed by 15.45% (17/110), 13.33% (14/105) in patients on treatment with Etanercept and Adalimumab respectively as compared to 13.89% (25/180) among controls. Onychomycosis in association with nail psoriasis was more common in males. This study revealed statistically significant association between fungal infections of the nail in patients of psoriasis on treatment with Infliximab.

  20. Alcohol extract of North American ginseng (Panax quinquefolius) reduces fatty liver, dyslipidemia, and other complications of metabolic syndrome in a mouse model.

    Science.gov (United States)

    Singh, Ratnesh K; Lui, Edmund; Wright, David; Taylor, Adrian; Bakovic, Marica

    2017-09-01

    We investigated whether North American ginseng (Panax quinquefolius) could reduce development of the metabolic syndrome phenotype in a mouse model (ETKO) of the disease. Young ETKO mice have no disease but similar to humans start to develop the fatty liver, hypertriglyceridemia, obesity, and insulin resistance at 25-30 weeks of age, and the disease continues to progress with ageing. ETKO mice were orally given an ethanol extract of ginseng roots at 4 and 32 weeks of age. Treatments with ginseng eliminated the ETKO fatty liver, reduced hepatic and intestinal lipoprotein secretion, and reduced the level of circulating lipids. Improvements by ginseng treatments were manifested as a reduction in the expression of genes involved in the regulation of fatty acid and triglyceride (fat) synthesis and secretion by the lipoproteins on one hand, and the stimulation of fatty acid oxidation and triglyceride degradation by lipolysis on the other hand. These processes altogether improved glucose, fatty acid, and triglyceride metabolism, reduced liver fat load, and reversed the progression of metabolic syndrome. These data confirm that treatments with North American ginseng could alleviate metabolic syndrome through the maintenance of a better balance between glucose and fatty acid metabolism, lipoprotein secretion, and energy homeostasis in disease-prone states.

  1. Contemporary management of complicated monochorionic twins.

    Science.gov (United States)

    Moise, Karen Y; Kugler, Lisa; Jones, Tyra

    2012-01-01

    Monochorionic twins are at increased risk for unique complications including twin-twin transfusion syndrome (TTTS), selective intrauterine growth restriction (sIUGR), and twin-reversed arterial perfusion (TRAP) sequence. Twin-twin transfusion syndrome is treated with laser photocoagulation whereas selective reduction is an option in previable sIUGR or TRAP sequence. The nurse is integral in the management, education, care and support of women with complicated pregnancies. © 2012 AWHONN, the Association of Women's Health, Obstetric and Neonatal Nurses.

  2. Immunological effects of adalimumab in rheumatoid arthritis: Results of a Russian national study

    Directory of Open Access Journals (Sweden)

    Dmitri Evgenyevich Karateev

    2013-01-01

    Full Text Available A reduction in immunological parameters during therapy for rheumatoid arthritis (RA can yield new data on the mechanisms of anti-inflammatory action and be of great practical importance since this allows judgment of the depth of impact on the immunological process and therefore may be regarded as one of the components of improvement and remission. Objective: to study the effect of the tumor necrosis factor-a (TNF-а inhibitor adalimumab (ADA on a number of key immunological parameters in RA. Subjects and methods. The study included 100 patients (11 men and 89 women diagnosed with RA from 5 research centers. The patients were observed to have high RA activity: at baseline, DAS28 6.22+0.84 scores; C-reactive protein (CRP 37.1+34.7 mg/l. The mean number of disease-modifying antirheumatic drugs (DMARDs used by a patient in the history was 2.1. During 24-week treatment, the patients took ADA in a subcutaneous dose of 40 mg every 2 weeks both alone and in combination with DMARDs. The time course of changes in the serum levels of CRP, IGM rheumatoid factor (RF and in the concentration of anti-cyclic citrullinated peptide 2 (anti-CCP2 antibodies was determined by enzyme immunoassay (EIA using the Axis-Shield Diagnostics commercial kits (United Kingdom. The levels of TNF-a, interleukin (IL 6, 12, matrix metalloproteinase 3 (MMP3 were measured in pg/ml by EIA using the Bender MedSystems commercial kits (USA according to the manufacturer's directions. Results. During ADA therapy, there was a rapid reduction in the level of CRP from 34.3 to 11.317.2 mg/l following 2 weeks (p<0.001, which thereafter remained low (11.9 mg/l, with some fluctuations, until week 24 of the study. There was a significant reduction in blood RF levels from 169.24 to 111.97 at 24 weeks (p<0.001. After 12 weeks of ADA treatment, there was virtually a two-fold decrease in IL-6 content from 8.87 to 4.87 pg/ml and later on to 4.03 pg/ml at week 24 (p>0,05. The mean levels of anti-CCP2

  3. 'Happy' drug survival of adalimumab, etanercept and ustekinumab in psoriasis in daily practice care: results from the BioCAPTURE network

    NARCIS (Netherlands)

    Reek, J.M.P.A. van den; Zweegers, J.; Kievit, W.; Otero, M.E.; Lumig, P.P.M. van; Driessen, R.J.B.; Ossenkoppele, P.M.; Njoo, M.D.; Mommers, J.M.; Koetsier, M.I.A.; Arnold, W.P.; Sybrandy-Fleuren, B.A.M.; Kuijpers, A.L.A.; Andriessen, M.P.M.; Kerkhof, P.C.M. van de; Seyger, M.M.B.; Jong, E.M.G.J. de

    2014-01-01

    BACKGROUND: Drug survival is a marker for treatment success. To date, no analyses relating dermatological quality-of-life measures to drug survival have been published. OBJECTIVES: (i) To describe 1-year drug survival for adalimumab, etanercept and ustekinumab in a daily practice psoriasis cohort,

  4. A Prospective, Randomized, Placebo-Controlled Study to Identify Biomarkers Associated with Active Treatment in Psoriatic Arthritis: Effects of Adalimumab Treatment on Lesional and Nonlesional Skin

    NARCIS (Netherlands)

    de Groot, Marjan; Picavet, Daisy I.; van Kuijk, Arno W. R.; Tak, Paul P.; Bos, Jan D.; de Rie, Menno A.; Teunissen, Marcel B. M.

    2012-01-01

    Background: There is a need for biomarkers to screen the effectiveness of (novel) therapeutic agents for psoriasis at an early stage. Objective: We aimed to determine which of the changes in psoriatic skin correlates best with clinical improvement 4 weeks after effective adalimumab therapy. Methods:

  5. Adalimumab for nail psoriasis: Efficacy and safety from the first 26 weeks of a phase 3, randomized, placebo-controlled trial.

    Science.gov (United States)

    Elewski, Boni E; Okun, Martin M; Papp, Kim; Baker, Christopher S; Crowley, Jeffrey J; Guillet, Gérard; Sundaram, Murali; Poulin, Yves; Gu, Yihua; Geng, Ziqian; Williams, David A; Rich, Phoebe A

    2018-01-01

    Previous clinical trials have not evaluated improvement in nail psoriasis as a primary end point. This phase 3 trial evaluated the safety and efficacy of adalimumab in patients with moderate-to-severe fingernail psoriasis and moderate-to-severe plaque psoriasis. Patients were randomized 1:1 to 40 mg adalimumab every other week or placebo. The primary efficacy end point was at least 75% improvement in total-fingernail modified Nail Psoriasis Severity Index (NAPSI75) response rate at week 26. Ranked secondary end point scores evaluated at week 26 were total-fingernail NAPSI and modified NAPSI, nail pain, Nail Psoriasis Physical Functioning Severity, Brigham Scalp Nail Inverse Palmo-Plantar Psoriasis Index, and Physician's Global Assessment (fingernail psoriasis). Of the 217 randomized patients (108 received placebo and 109 received adalimumab), 188 (86.6%) completed 26 weeks of treatment (period A) or escaped early to the open-label period. The study met the primary end point (response rate of 3.4% with placebo vs 46.6% with adalimumab [P psoriasis versus with placebo and no new safety risks were identified. Copyright © 2017 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.

  6. Neurologic complications of metronidazole.

    Science.gov (United States)

    Sarna, Justyna R; Furtado, Sarah; Brownell, A Keith W

    2013-11-01

    Metronidazole (Flagyl®) is an antimicrobial agent commonly used in clinical practice. Although it is generally well tolerated with minimal side effects, there are a host of still under-recognized neurologic complications of metronidazole treatment. The following review is aimed at summarizing current literature pertaining to metronidazole-induced neurotoxicity including clinical syndromes, neuroradiological findings, prognosis and proposed pathophysiology. Recognition of the neurotoxic effects of metronidazole is critical as prompt discontinuation is generally associated with full clinical recovery and radiological resolution. Complications neurologiques du métronidazole. Le métronidazole (Flagyl®) est un agent antimicrobien utilisé couramment en pratique clinique. Bien qu'il soit généralement bien toléré et que ses effets secondaires soient minimes, il existe une myriade de complications neurologiques du traitement par le métronidazole qui ne sont pas toujours reconnues. Le but de cette revue constitue un sommaire de la littérature actuelle concernant la neurotoxicité induite par le métronidazole dont les syndromes cliniques, les constatations neuroradiologiques, le pronostic et l'hypothèse physiopathologique expliquant cette neurotoxicité. Il est important d'identifier ces effets neurotoxiques du métronidazole étant donné que l'arrět immédiat du traitement est généralement associé à une guérison clinique complète et à la disparition des signes radiologiques.

  7. Dizygotic monochorionic twin pregnancy conceived following intracytoplasmic sperm injection treatment and complicated by twin-twin transfusion syndrome and blood chimerism

    DEFF Research Database (Denmark)

    Ekelund, Charlotte Kvist; Skibsted, L.; Søgaard, Kirsten

    2008-01-01

    and zygocity determination were performed on amniotic fluid and showed the twins to be dizygotic with normal female and male karyotypes. There were clinical and sonographic signs of twin-twin transfusion syndrome (TTTS), and Cesarean delivery was performed at 32 weeks' gestation. At birth the twins were...... phenotypically a normal male and a normal female. Histology of the placenta showed it to be monochorionic diamniotic. Blood chimerism was found postnatally as both infants had the karyotypes 46,XX[13]/46,XY[17]. Chimerism was not found in cells from a buccal swab at 6 months of age. This is one of only a few...

  8. Cavitating mesenteric lymph node syndrome: a rare complication of celiac disease Síndrome del ganglio linfático mesentérico cavitado: una rara complicación de la enfermedad

    Directory of Open Access Journals (Sweden)

    Joaquín Rodríguez-Sánchez

    2011-12-01

    Full Text Available Among the many complications of celiac disease, mesenteric lymph node syndrome cavitated is considered one of the rarest, there is few case series published in the literature. The etiology and pathophysiology are unknown but because of its high mortality rate, estimated to be around 50%, it should recognize at an early stage in order to institute appropriate therapy as soon as possible.Dentro de las numerosas complicaciones de la enfermedad celiaca, el síndrome del ganglio linfático mesentérico cavitado se considera una de las más infrecuentes, siendo escasas las series de casos publicadas en la literatura. Su etiología y su mecanismo fisiopatológico se desconocen pero debido a su alta tasa de mortalidad, situada en torno al 50%, es conveniente reconocerla de forma precoz para así instaurar un tratamiento adecuado lo antes posible.

  9. Genome-wide association study using the ethnicity-specific Japonica array: identification of new susceptibility loci for cold medicine-related Stevens-Johnson syndrome with severe ocular complications.

    Science.gov (United States)

    Ueta, Mayumi; Sawai, Hiromi; Shingaki, Ryosei; Kawai, Yusuke; Sotozono, Chie; Kojima, Kaname; Yoon, Kyung-Chul; Kim, Mee Kum; Seo, Kyoung Yul; Joo, Choun-Ki; Nagasaki, Masao; Kinoshita, Shigeru; Tokunaga, Katsushi

    2017-04-01

    A genome-wide association study (GWAS) for cold medicine-related Stevens-Johnson syndrome (CM-SJS) with severe ocular complications (SOC) was performed in a Japanese population. A recently developed ethnicity-specific array with genome-wide imputation that was based on the whole-genome sequences of 1070 unrelated Japanese individuals was used. Validation analysis with additional samples from Japanese individuals and replication analysis using samples from Korean individuals identified two new susceptibility loci on chromosomes 15 and 16. This study might suggest the usefulness of GWAS using the ethnicity-specific array and genome-wide imputation based on large-scale whole-genome sequences. Our findings contribute to the understanding of genetic predisposition to CM-SJS with SOC.

  10. Thrombophilia and Pregnancy Complications

    Directory of Open Access Journals (Sweden)

    Louise E. Simcox

    2015-11-01

    Full Text Available There is a paucity of strong evidence associated with adverse pregnancy outcomes and thrombophilia in pregnancy. These problems include both early (recurrent miscarriage and late placental vascular-mediated problems (fetal loss, pre-eclampsia, placental abruption and intra-uterine growth restriction. Due to poor quality case-control and cohort study designs, there is often an increase in the relative risk of these complications associated with thrombophilia, particularly recurrent early pregnancy loss, late fetal loss and pre-eclampsia, but the absolute risk remains very small. It appears that low-molecular weight heparin has other benefits on the placental vascular system besides its anticoagulant properties. Its use is in the context of antiphospholipid syndrome and recurrent pregnancy loss and also in women with implantation failure to improve live birth rates. There is currently no role for low-molecular weight heparin to prevent late placental-mediated complications in patients with inherited thrombophilia and this may be due to small patient numbers in the studies involved in summarising the evidence. There is potential for low-molecular weight heparin to improve pregnancy outcomes in women with prior severe vascular complications of pregnancy such as early-onset intra-uterine growth restriction and pre-eclampsia but further high quality randomised controlled trials are required to answer this question.

  11. 'Happy' drug survival of adalimumab, etanercept and ustekinumab in psoriasis in daily practice care: results from the BioCAPTURE network.

    Science.gov (United States)

    van den Reek, J M P A; Zweegers, J; Kievit, W; Otero, M E; van Lümig, P P M; Driessen, R J B; Ossenkoppele, P M; Njoo, M D; Mommers, J M; Koetsier, M I A; Arnold, W P; Sybrandy-Fleuren, B A M; Kuijpers, A L A; Andriessen, M P M; van de Kerkhof, P C M; Seyger, M M B; de Jong, E M G J

    2014-11-01

    Drug survival is a marker for treatment success. To date, no analyses relating dermatological quality-of-life measures to drug survival have been published. (i) To describe 1-year drug survival for adalimumab, etanercept and ustekinumab in a daily practice psoriasis cohort, and (ii) to introduce the concept of 'happy' drug survival, defined as Dermatology Life Quality Index (DLQI) ≤ 5 combined with being 'on drug' at a specific time point. Data were extracted from a prospective registry. Drug survival was analysed using Kaplan-Meier estimates. 'Happy' drug survival was calculated, with data split into 'happy' (DLQI ≤ 5) vs. 'unhappy' (DLQI > 5) at baseline and months 3, 6, 9 and 12. 249 treatment episodes were included (101 adalimumab, 82 etanercept, 66 ustekinumab). The 1-year drug survival rates for ustekinumab, adalimumab and etanercept were 85%, 74% and 68%, respectively. Ustekinumab showed a better confounder-corrected drug survival vs. etanercept [hazard ratio (HR) 3·8, P = 0·02] and a trend towards better survival vs. adalimumab (HR 2·3, P = 0·1). At baseline, the majority (n = 115, 73%) was considered 'unhappy' and a minority 'happy' (n = 42, 27%) (ratio 'happy':'unhappy' was 1 : 2.7). The percentage of treatment episodes with 'happy' on-drug patients increased to 79% after 1 year. Ustekinumab showed a better overall drug survival than etanercept, and a trend towards a better overall drug survival than adalimumab. After 1 year, patients reported to be 'happy' in 79% of episodes and 'unhappy' in 21%. We introduced the new concept of 'happy' drug survival because the proportion of on-drug patients with good quality of life is an important indicator for treatment success. © 2014 British Association of Dermatologists.

  12. Adalimumab in acute sciatica reduces the long-term need for surgery: a 3-year follow-up of a randomised double-blind placebo-controlled trial.

    Science.gov (United States)

    Genevay, S; Finckh, A; Zufferey, P; Viatte, S; Balagué, F; Gabay, C

    2012-04-01

    Two subcutaneous injections of adalimumab in severe acute sciatica significantly reduced the number of back operations in a short-term randomised controlled clinical trial. To determine in a 3-year follow-up study whether the short-term benefit of adalimumab in sciatica is sustained over a longer period of time. The primary outcome of this analysis was incident discectomy. Three years after randomisation, information on surgery could be retrieved in 56/61 patients (92%).A multivariate Cox proportional hazard models, adjusted for potential confounders, was used to determine factors predisposing to surgery. Twenty-three (41%) patients had back surgery within 3 years, 8/29 (28%) in the adalimumab group and 15/27 (56%) in the placebo group, p=0.04. Adalimumab injections reduced the need for back surgery by 61% (HR)=0.39 (95% CI 0.17 to 0.92). In a multivariate model, treatment with a tumour necrosis factor-α antagonist remained the strongest protective factor (HR=0.17, p=0.002). Other significant predictors of surgery were a good correlation between symptoms and MRI findings (HR=11.6, p=0.04), baseline intensity of leg pain (HR=1.3, p=0.06), intensity of back pain (HR=1.4, p=0.03) and duration of sickness leave (HR=1.01 per day, p=0.03). A short course of adalimumab in patients with severe acute sciatica significantly reduces the need for back surgery.

  13. Pregnancy after oocyte donation in 45, X Turner syndrome women, complicated by gestational diabetes and polyhydramnios. Case report and mini-review of literature.

    Science.gov (United States)

    Czyzyk, Adam; Podfigurna-Stopa, Agnieszka; Katulski, Krzysztof; Breborowicz, Grzegorz H; Genazzani, Andrea R; Meczekalski, Blazej

    2016-08-01

    Patients suffering from Turner syndrome (TS) demonstrate characteristic clinical features, with a short stature and gonadal dysgenesis causing infertility in most patients. Spontaneous pregnancies in women with TS are quite rare and pregnancy outcomes involving an increased risk of miscarriage and stillbirths are observed. In this case report, we present a 28 years old pregnant woman with the diagnosis of TS. Due to hypergonadotrophic hypogonadism, she was proposed an in vitro fertilization (IVF) program with an oocyte donor from unrelated anonymous women. After the second transfer, implantation occurred. In the 24th week of gestation, gestational diabetes class 1 was diagnosed. In the 31st week of gestation, polyhydramnios was diagnosed, although other parameters were reassuring. Considering the polyhydramnios, along with the diagnosis of Turner syndrome in the mother, we decided to perform an elective cesarean section. Subsequently, a healthy term male was born. For most women with the diagnosis of TS, the only way to become pregnant is through oocyte donation. The aim of this work was to characterize the course of pregnancy in TS patient and review literature addressing this issue.

  14. Prevalence of Metabolic Syndrome and its influence on microvascular complications in the Indian population with Type 2 Diabetes Mellitus. Sankara Nethralaya Diabetic Retinopathy Epidemiology And Molecular Genetic Study (SN-DREAMS, report 14

    Directory of Open Access Journals (Sweden)

    Raman Rajiv

    2010-11-01

    Full Text Available Abstract Background The Metabolic syndrome (MS consists of central obesity, glucose intolerance, hyperinsulinemia, low high density lipoproteins, high triglycerides and hypertension. Different studies have observed that MS causes microvascular complications in patients with type 2 diabetes. The aim of the study was to find out the prevalence of MS in the Indian population with type 2 diabetes mellitus in relation to gender, duration of diabetes, and to evaluate the influence of MS and its individual components on microvascular complications such as diabetic retinopathy, diabetic nephropathy and diabetic neuropathy. Methods A population-based cross sectional survey was conducted with 1414 patients having type 2 diabetes mellitus. The International Diabetes Federation (IDF criteria were used to identify the metabolic syndrome. Diabetic retinopathy was graded using the stereoscopic digital fundus photography. Neuropathy was assessed by measuring the vibration perception threshold through a sensitometer. Nephropathy was diagnosed by the presence of microalbuminuria in the first morning urine sample. Results The age and gender adjusted prevalence of MS, using the IDF criteria, in the South Indian population was 73.3%. The prevalence was higher in women (83.3%, compared to men (65.3%. In subjects with diabetes mellitus, without and with MS, the prevalence of retinopathy was 21.3% and 16.9% (p = 0.057; prevalence of nephropathy was 20.5% and 18.0% (p = 0.296, and prevalence of neuropathy was17.2% and 19.4% (p = 0.353 respectively. Overall and in women, the clustering of MS components led to an increase in the prevalence of diabetic nephropathy. The prevalence of retinopathy and neuropathy in MS subjects, who had diabetes for Conclusions The association of MS with microangiopathies decreased with an increase in the duration of diabetes. MS behaved differently in men and women. It may need to be managed differently in the two groups.

  15. Genitourinary complications of systemic lupus erythematosus.

    Science.gov (United States)

    Meyers, K E; Pfieffer, S; Lu, T; Kaplan, B S

    2000-05-01

    A 14-year-old African-American girl was diagnosed with antiphospholipid-positive systemic lupus erythematosus (SLE) in July 1994. The course was complicated by nephrotic syndrome, sepsis, hemolytic anemia, acute renal failure, saphenous vein thrombosis, cutaneous vasculitis, mesenteric vasculitis, appendicitis, hemorrhagic cystitis, and avascular necrosis of the hips. In August 1997, she developed ovarian and fallopian tube complications secondary to SLE. Genitourinary complications of SLE, however, are uncommon, and ovarian vasculitis has not previously been reported as a complication of SLE. This report describes the course of an adolescent patient with SLE and focuses specifically on her genitourinary complications.

  16. Tuberculose pleural após uso de adalimumabe na doença de Crohn: relato de caso Pleural tuberculosis after using adalimumab in Crohn's disease: a case report

    Directory of Open Access Journals (Sweden)

    Rodrigo Rocha Batista

    2011-03-01

    Full Text Available O tratamento da doença de Crohn perineal é feito pela combinação da terapia medicamentosa e da cirúrgica. A terapia biológica é importante devido à eficácia clínica na indução e manutenção de remissão da doença. No entanto, devido à ação imunomoduladora e imunossupressora, o uso de biológicos como infliximabe e adalimumabe eleva o risco de infecções oportunistas. Relatou-se o caso de paciente feminino, 28 anos, diagnosticada com doença de Crohn perineal, em uso de azatioprina e adalimumabe. Há sete dias com tosse seca, febre vespertina e dispneia. Ao exame físico, febril, desidratada, diminuição do murmúrio vesicular nos campos médio e inferior do hemitoráx direito e cicatriz de fistulotomia anorretal sem sinais flogísticos. A radiografia de tórax mostrou derrame pleural em hemitórax direito, e a análise do líquido pleural constatou adenosina deaminase elevada, nível de glicose normal e citologia diferencial com 88% de monomorfonucleares. Foi estabelecido o diagnóstico de tuberculose pleural, e a paciente foi medicada com esquema tríplice (rifampicina, isoniazida e pirazinamida por seis meses associada à prednisona 40 mg/dia, por um mês, com posterior desmame do corticoide. Atualmente, encontra-se assintomática e em uso de ciprofloxina 1 g/dia para a doença de Crohn perineal.The Crohn's disease perineal treatment is made by the combination of drug and surgery therapy. The biological therapy is important due to clinical efficacy in inducing and maintaining disease remission. However, because of immunomodulating and immunosuppressive effects, the use of biological as infliximab and adalimumab increases the risk of opportunistic infections. We report a case of a 28-year-old, female patient, diagnosed with Crohn's perineal disease, taking azathioprine and adalimumab, complaining of dry cough, evening fever, and dyspnea for seven days. On physical examination, febrile, dehydrated, decreased, breath sounds in

  17. Interest of the scintigraphy PET {sup 18}F-F.D.G. for the evaluation of the early response to the treatment by adalimumab in the rheumatoid poly-arthritis; Interet de la scintigraphie TEP {sup 18}F-FDG pour l'evaluation de la reponse precoce au traitement par adalimumab dans la polyarthrite rhumatoide

    Energy Technology Data Exchange (ETDEWEB)

    Eder, V.; Courtehoux, M.; Baulieu, J.L. [Service de medecine nucleaire, CHU de Tours, (France); Mulleman, D.; Paintaud, G.; Goupille, P.; Ternant, D. [Service de rhumatologie, CHU de Tours, (France)

    2009-05-15

    The aim of this study was to evaluate the interest of the follow-up of the {sup 18}F-F.D.G. uptake during the treatment of the rheumatoid poly-arthritis by 40 mg during 2 weeks of adalimumab (an anti-TNF-alpha human monoclonal antibody), comparatively to the usual clinical and biological follow-up. The PET with {sup 18}F-F.D.G. is a promising technique to enlighten early the anti inflammatory pharmaco-dynamic effect of the adalimumab in the rheumatoid poly-arthritis. (N.C.)

  18. Neurological complications of chickenpox

    Directory of Open Access Journals (Sweden)

    Girija A

    2007-01-01

    Full Text Available Aim: To assess the neurological complications of chickenpox with prognosis. Background: The neurological complications occur in 0.03% of persons who get chickenpox. There is no universal vaccination against chicken pox in India. Most patients prefer alternate modalities of treatment. Hence these complications of chickenpox are likely to continue to occur. Study Design: A prospective study was conducted for 2 years (from March 2002 on the admitted cases with neurological complications after chickenpox (with rash or scar. Patients were investigated with CT/MRI, CSF study, EEG and nerve conduction studies and hematological workup. They were followed-up for 1 year and outcome assessed using modified Rankin scale. Results: The latency for the neurological complications was 4-32 days (mean: 16.32 days. There were 18 cases: 10 adults (64% and 8 children (36%. Cerebellar ataxia (normal CT/MRI was observed in 7 cases (32% (mean age: 6.85 years. One patient (6 years had acute right hemiparesis in the fifth week due to left capsular infarct. All these cases spontaneously recovered by 4 weeks. The age range of the adult patients was 13-47 years (mean: 27 years. The manifestations included cerebellar and pyramidal signs (n-4 with features of demyelination in MRI who recovered spontaneously or with methylprednisolone by 8 weeks. Patient with encephalitis recovered in 2 weeks with acyclovir. Guillain Barre syndrome of the demyelinating type (n-2 was treated with Intravenous immunoglobulin (IVIG and they had a slow recovery by a modified Rankin scale (mRs score of 3 and 2 at 6 months and 1 year, respectively. One case died after hemorrhage into the occipital infarct. There were two cases of asymmetrical neuropathy, one each of the seventh cranial and brachial neuritis. Conclusion: Spontaneous recovery occurs in post-chickenpox cerebellar ataxia. Rarely, serious complications can occur in adults. The demyelinating disorders, either of the central or peripheral

  19. Adalimumab, etanercept and ustekinumab for treating plaque psoriasis in children and young people: systematic review and economic evaluation.

    Science.gov (United States)

    Duarte, Ana; Mebrahtu, Teumzghi; Goncalves, Pedro Saramago; Harden, Melissa; Murphy, Ruth; Palmer, Stephen; Woolacott, Nerys; Rodgers, Mark; Rothery, Claire

    2017-11-01

    Psoriasis is a chronic inflammatory disease that predominantly affects the skin. Adalimumab (HUMIRA ® , AbbVie, Maidenhead, UK), etanercept (Enbrel ® , Pfizer, New York, NY, USA) and ustekinumab (STELARA ® , Janssen Biotech, Inc., Titusville, NJ, USA) are the three biological treatments currently licensed for psoriasis in children. To determine the clinical effectiveness and cost-effectiveness of adalimumab, etanercept and ustekinumab within their respective licensed indications for the treatment of plaque psoriasis in children and young people. Searches of the literature and regulatory sources, contact with European psoriasis registries, company submissions and clinical study reports from manufacturers, and previous National Institute for Health and Care Excellence (NICE) technology appraisal documentation. Included studies were summarised and subjected to detailed critical appraisal. A network meta-analysis incorporating adult data was developed to connect the effectiveness data in children and young people and populate a de novo decision-analytic model. The model estimated the cost-effectiveness of adalimumab, etanercept and ustekinumab compared with each other and with either methotrexate or best supportive care (BSC), depending on the position of the intervention in the management pathway. Of the 2386 non-duplicate records identified, nine studies (one randomised controlled trial for each drug plus six observational studies) were included in the review of clinical effectiveness and safety. Etanercept and ustekinumab resulted in significantly greater improvements in psoriasis symptoms than placebo at 12 weeks' follow-up. The magnitude and persistence of the effects beyond 12 weeks is less certain. Adalimumab resulted in significantly greater improvements in psoriasis symptoms than methotrexate for some but not all measures at 16 weeks. Quality-of-life benefits were inconsistent across different measures. There was limited evidence of excess short

  20. Comparative study of both versions of an immunoassay commercialized for therapeutic drug monitoring of adalimumab in rheumatoid arthritis.

    Science.gov (United States)

    Llinares-Tello, Francisca; Rosas, José; de la Torre, Inmaculada; Valor, Lara; Barber, Xavier; Senabre, José Miguel

    2014-01-01

    To describe the results of the comparative study between both versions of an immunoassay commercialized for therapeutic drug monitoring of adalimumab (ADA) in rheumatoid arthritis (AR). 140 samples of patients with RA treated with ADA 40mg every 14 days were analyzed by both versions of the test (V1 or previous and V2 or updated). A good correlation was obtained by both versions. In general V2 provides higher results of ADA's concentration than V1 and presents greater precision in the range of concentrations for clinical decisions, adjusting for the real concentration of the drug in blood. In addition, V2 allows for complete automation, which simplifies the analysis and reduces significantly the variability. ADA's monitoring with the updated version demonstrated to have technical significant advantages, constituting a more practical tool for therapeutic decisions in patients with RA. Copyright © 2013 Elsevier España, S.L. All rights reserved.

  1. A double-blind placebo-controlled randomized trial of adalimumab in the treatment of hidradenitis suppurativa

    DEFF Research Database (Denmark)

    Miller, I; Lynggaard, C D; Lophaven, S

    2011-01-01

    the outcomes were blinded to group assignment. The primary efficacy endpoints were changes in the HS scores (Sartorius and Hurley scoring systems). Secondary efficacy endpoints included changes in pain (visual analogue scale), days with lesions and Dermatology Life Quality Index, and evaluation of scarring......BACKGROUND: Hidradenitis suppurativa (HS) has an impact on patients' quality of life. Treatment of HS is generally unsatisfactory, thus new treatments are needed. OBJECTIVES: To test the efficacy of adalimumab in HS. METHODS: This was a prospective, randomized, double-blinded, placebo...... subcutaneously (s.c.) at baseline followed by 40 mg s.c. every other week for 12 weeks. Placebo-treated patients received identical-looking injections with no active ingredient. The medicine was dispensed in sequentially numbered computer-randomized containers. Participants, care givers and those assessing...

  2. Plasma MicroRNA Profiles in Patients with Early Rheumatoid Arthritis Responding to Adalimumab plus Methotrexate vs Methotrexate Alone

    DEFF Research Database (Denmark)

    Sode, Jacob; Krintel, Sophine B; Carlsen, Anting Liu

    2018-01-01

    OBJECTIVE: The aim was to identify plasma (i.e., cell-free) microRNA (miRNA) predicting antitumor necrosis and/or methotrexate (MTX) treatment response in patients enrolled in an investigator-initiated, prospective, double-blinded, placebo-controlled trial (The OPERA study, NCT00660647). METHODS......: We included 180 disease-modifying antirheumatic drug-naive patients with early rheumatoid arthritis (RA) randomized to adalimumab (ADA; n = 89) or placebo (n = 91) in combination with MTX. Plasma samples before and 3 months after treatment initiation were analyzed for 91 specific mi...... multivariate miRNA models were able to predict response to ADA treatment after 3 and 12 months, with 63% and 82% area under the ROC curves, respectively. CONCLUSION: We identified miR-27a-3p as a potential predictive biomarker of ACR/EULAR remission in patients with early RA treated with ADA in combination...

  3. Acute tubulointerstitial nephritis in an HLA-B27-positive patient with axial spondyloarthritis being treated with adalimumab.

    Science.gov (United States)

    Castro Corredor, David; Sánchez de la Nieta, María Dolores; de Lara Simón, Isabel María

    2017-06-14

    Antagonists of tumor necrosis factor-alpha (ATNF) are used for the treatment of multiple diseases such as psoriatic arthritis, Crohn's disease, ankylosing spondylitis and juvenile idiopathic arthritis, usually, when they are refractory to first-line treatment 1 . The use of ATNF has been associated with the induction of autoimmune diseases such as systemic lupus erythematosus-like disease, vasculitis, sarcoidosis-like diseases and, recently, acute granulomatous tubulointerstitial nephritis. We report a case of acute nongranulomatous tubulointerstitial nephritis in an HLA-B27-positive patient with axial spondyloarthritis and Crohn's disease being treated with adalimumab. Copyright © 2017 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.

  4. Sweet's syndrome complicated by Kikuchi's disease and hemophagocytic syndrome which presented with retinoic acid-inducible gene-I in both the skin epidermal basal layer and the cervical lymph nodes.

    Science.gov (United States)

    Koga, Tomohiro; Takano, Kanako; Horai, Yoshiro; Yamasaki, Satoshi; Nakamura, Hideki; Mizokami, Akinari; Ohshima, Koichi; Kawakami, Atsushi

    2013-01-01

    A 21-year-old man was admitted to our hospital with a fever, erythema, cervical lymphadenopathy and pancytopenia. A diagnosis of Sweet's syndrome (SS) with Kikuchi's disease (KD) and hemophagocytic syndrome (HPS) was made based on the results of a bone marrow aspiration along with the results from biopsy specimens of the brachial skin and a cervical lymph node. The expression of retinoic acid-inducible gene-I (RIG-I) in the skin epidermal basal layer as well as in a cervical lymph node was revealed through immunohistochemistry. He successfully entered remission through treatment with prednisolone. This findings of this case indicate that when SS with KD presents as HPS, it may suggest an association with an RIG-I-related innate immunity.

  5. A treat-to-target strategy with methotrexate and intra-articular triamcinolone with or without adalimumab effectively reduces MRI synovitis, osteitis and tenosynovitis and halts structural damage progression in early rheumatoid arthritis: results from the OPERA randomised controlled trial

    DEFF Research Database (Denmark)

    Axelsen, Mette Bjørndal; Eshed, Iris; Hørslev-Petersen, Kim

    2015-01-01

    To investigate whether a treat-to-target strategy with methotrexate and intra-articular glucocorticosteroid injections suppresses MRI inflammation and halts structural damage progression in patients with early rheumatoid arthritis (ERA), and whether adalimumab provides an additional effect....

  6. Chronic complications of inhalation injury: chest HRCT findings and a correlation with the pulmonary function test in reactive airway dysfunction syndrome

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    Song, Ki Hyeok; Lee, In Sun; Jung, Eun Hee; Ji, Young Gu; Lee, Young Seok [Dankook University Hospital, Cheonan (Korea, Republic of)

    2007-09-15

    To evaluate the HRCT findings and to correlate the findings with the results of a pulmonary function test (PFT) in patients with reactive airway dysfunction syndrome (RADS). On March 2003, a fire at a boarding house of primary school soccer players caused a multiple casualty disaster. After 8 months, nine boys that presented with chronic cough and dyspnea were treated, and were subjected to follow-up evaluations. Eight patients underwent a chest radiograph, HRCT, and a PET. Two patients with severe symptoms received extended follow-up after 1 year. Two radiologists retrospectively reviewed the chest radiographs and the follow-up HRCT scans. We correlated the HRCT findings with the results of the PET. Six patients with an inhalation injury were diagnosed with RADS. On the chest radiographs, eight patients showed no abnormal findings. On an HRCT scan, four patients showed abnormal findings. The abnormal findings were mosaic air trapping (n = 4), bronchial wall thickening (n = 1), and parenchymal consolidation (n = 1). In all four patients that showed abnormal findings in the HRCT scan, abnormal results of the PET were also seen. The two patients that received extended follow-up showed an improvement of the clinical symptoms, as seen by the PFT, and had a decreased extent and degree of mosaic air trapping, as seen on HRCT. An HRCT scan is an essential modality for the diagnosis and follow-up of patients with RADS. Both a full expiratory and inspiratory HRCT scan must be performed for an accurate diagnosis.

  7. False aneurysm of the interosseous artery and anterior interosseous syndrome - an unusual complication of penetrating injury of the forearm: a case report

    Directory of Open Access Journals (Sweden)

    Garavaglia Guido

    2009-12-01

    Full Text Available Abstract Background Palsies involving the anterior interosseous nerve (AIN comprise less than 1% of all upper extremity nerve palsies. Objectives This case highlights the potential vascular and neurological hazards of minimal penetrating injury of the proximal forearm and emphasizes the phenomenon of delayed presentation of vascular injuries following seemingly obscure penetrating wounds. Case Report We report a case of a 22-year-old male admitted for a minimal penetrating trauma of the proximal forearm that, some days later, developed an anterior interosseous syndrome. A Duplex study performed immediately after the trauma was normal. Further radiologic investigations i.e. a computer-tomographic-angiography (CTA revealed a false aneurysm of the proximal portion of the interosseous artery (IA. Endovascular management was proposed but a spontaneous rupture dictated surgical revision with simple excision. Complete neurological recovery was documented at 4 months postoperatively. Conclusions/Summary After every penetrating injury of the proximal forearm we propose routinely a detailed neurological and vascular status and a CTA if Duplex evaluation is negative.

  8. Abdominal complications in black and Indian children with nephrotic ...

    African Journals Online (AJOL)

    Abdominal complications were detected and investigated in 19 (10%) of 191 children with nephrotic syndrome who experienced 35 episodes of these complications. Fourteen children were Indian with steroid-responsive nephrotic syndrome, and 5 were black, of whom 4 had membranous nephropathy and 1 focal ...

  9. Analysis of IRS-1-mediated phosphatidylinositol 3-kinase activation in the adipose tissue of polycystic ovary syndrome patients complicated with insulin resistance

    International Nuclear Information System (INIS)

    Chu Yongli; Qiu Hongyu; Sun Yongyu; Li Min; Li Hongfa

    2004-01-01

    Objective: To investigate the insulin receptor substance-1 (IRS-1)-mediated phosphatidylinositol-3 (PI-3) kinase activity in adipose tissue of polycystic ovary syndrome (PCOS) patients, and to explore molecular mechanisms of insulin resistance of PCOS. Methods: Blood and adipose tissue samples from patients with PCOS with insulin resistance (n=19), PCOS without insulin resistance (n=10) and controls (n=15) were collected. Serum luteinizing hormone (LH), follicle stimulating hormone (FSH), testosterone (T) were measured by chemiluminescence assay. Fasting insulin (FIN) was measured by radioimmunoassay. Fasting plasma glucose (FPG) was measured by oxidase assay. Insulin resistance index (IR) was calculated using homeostasis model assessment (HOMA) to analyze the relationship between these markers and insulin resistance. The tyrosine phosphorylation of IRS-1 was measured by immunoprecipitation and enhanced chemiluminescent immunoblotting technique. PI-3 kinase activity was detected by immunoprecipitation, thin-layer chromatography and gamma scintillation counting. The results were analyzed by statistical methods. Results: 1) The levels of serum LH, LH/FSH, T, FIN and HOMA-IR in PCOS without insulin resistance were significantly higher than those of control group (all P<0.05); the levels of serum LH, LH/FSH, T, FIN and HOMA-IR in PCOS with insulin resistance were significantly higher than those of PCOS without insulin resistance (all P<0.05). 2) The tyrosine phosphorylation analysis of IRS-1 showed that IRS-1 tyrosine phosphorylation was significantly decreased in PCOS with insulin resistance compared to that of PCOS without insulin resistance and control groups (P<0.01). 3) PI-3 kinase activity was significantly decreased (P<0.01) and negatively correlated with HOMA-IR. Conclusion: In consequence of the weaker signal caused by the change of upper stream signal molecule IRS-1 tyrosine phosphorylation, PI-3 kinase activity decreased, it affects the insulin signal

  10. Complications of Measles (Rubeola)

    Science.gov (United States)

    ... of Measles Signs and Symptoms Transmission Photos of Measles Complications Frequently Asked Questions Top Things Parents Need to ... of age are more likely to suffer from measles complications. Infographic Common Complications Common measles complications include ear ...

  11. Pneumonia por varicela associada com síndrome da angústia respiratória aguda: relato de dois casos Varicella pneumonia complicated with acute respiratory distress syndrome: two cases report

    Directory of Open Access Journals (Sweden)

    Marcelo Moreno

    2007-03-01

    Full Text Available JUSTIFICATIVA E OBJETIVOS: A varicela é uma doença exantemática causada pela infecção primária do vírus varicela zoster (VVZ. A pneumonia pelo VVZ complicada com a síndrome da angústia respiratória aguda (SARA é rara e associa-se a altas taxas de morbimortalidade. O objetivo deste estudo foi apresentar dois casos de pneumonia por varicela que evoluíram com SARA e outras disfunções orgânicas. RELATO DOS CASOS: Paciente de 15 anos, imunocomprometido com a síndrome da imunodeficiência adquirida (SIDA e uma paciente do sexo feminino imunocompetente, foram admitidos na UTI com quadro clínico de varicela, SARA, trombocitopenia e acidose graves. Além disso, disfunção cardiovascular e falência renal ocorreram no primeiro e segundo casos, respectivamente. Foram tratados com aciclovir além de ventilação mecânica protetora. CONCLUSÕES: Os dois casos de pneumonia por varicela, que apresentaram SARA e disfunções de múltiplos órgãos, obtiveram boa evolução clínica.BACKGROUNG AND OBJECTIVES: Varicella is an exantematic disease caused by varicella-zoster virus. Varicella pneumonia complicated with acute respiratory distress syndrome (ARDS is very rare in adults and is associated with high morbimortality. We report two cases of ARDS secondary to varicella-zoster virus pneumonia. CASES REPORT: We report two cases of ARDS and multiple organ dysfunction syndrome (MODS secondary to varicella-zoster virus pneumonia. A 15-year-old man with human immunodeficiency virus (HIV infection and a 29-year-old immunocompetent female were admitted in the ICU with primary varicella infection and pneumonia. Both cases progressed towards ARDS, severe thrombocytopenia and acidosis. In addition cardiovascular and renal failure occurred in the first and second patients, respectively. Treatment consisted of immediate administration of intravenous acyclovir and a lung-protective ventilation strategy. CONCLUSIONS: Both cases of varicella

  12. Blockade of Tumor Necrosis Factor-Alpha: A Role for Adalimumab in Neovascular Age-Related Macular Degeneration Refractory to Anti-Angiogenesis Therapy

    Directory of Open Access Journals (Sweden)

    Beatriz Fernández-Vega

    2016-03-01

    Full Text Available Aims: To report a case of wet age-related macular degeneration (wet-AMD refractory to intravitreal anti-vascular endothelial growth factor (anti-VEGF therapy in a patient who showed visual and anatomical improvement and stabilization after starting a subcutaneous treatment course with adalimumab, an anti-tumor necrosis factor-alpha (TNF-α drug, for concomitant Crohn's disease. Methods: Observational case report of a female patient. Ophthalmological evaluation was performed by slit lamp and ophthalmoscopy (posterior pole and anterior segment. Best-corrected visual acuity (BCVA was determined, and imaging was performed by fluorescein angiography, indocyanine green angiography, and optical coherence tomography (OCT. Intravitreal therapies used and treatment with anti-TNF-α were recorded. Results: A 64-year-old woman with wet-AMD was treated with fourteen intravitreal injections of ranibizumab (0.5 mg for a period of 40 months with intervals of 1-6 months. She initially showed a good visual and anatomical response to periodic anti-VEGF treatment but during check visits, anatomical and functional responses deteriorated. At the 40-month follow-up, the patient had developed Crohn's disease, and her rheumatologist started treatment with adalimumab (40 mg subcutaneously every 2 weeks. During the 25 months of treatment with adalimumab, the patient did not require any additional intravitreal anti-VEGF treatments because her BCVA, clinical, and OCT findings improved and remained stable. Conclusions: We described a case of a patient with wet-AMD refractory to anti-VEGF therapy, which clinically benefited from subcutaneous adalimumab therapy. Treatment with subcutaneous anti-TNF-α in combination with anti-VEGF therapy avoids the high cost and risks related to multiple intravitreal anti-VEGF injections with good functional and anatomic outcomes.

  13. Patient-reported outcomes from a phase 3 study of baricitinib versus placebo or adalimumab in rheumatoid arthritis: secondary analyses from the RA-BEAM study

    Science.gov (United States)

    Keystone, Edward C; Taylor, Peter C; Tanaka, Yoshiya; Gaich, Carol; DeLozier, Amy M; Dudek, Anna; Zamora, Jorge Velasco; Cobos, Jose Arturo Covarrubias; Rooney, Terence; de Bono, Stephanie; Arora, Vipin; Linetzky, Bruno; Weinblatt, Michael E

    2017-01-01

    Background To assess the effect of baricitinib on patient-reported outcomes (PROs) in patients with active rheumatoid arthritis and an inadequate response to methotrexate (MTX). Methods In this double-blind phase 3 study, patients were randomised 3:3:2 to placebo (n=488), baricitinib 4 mg once daily (n=487), or adalimumab 40 mg biweekly (n=330) with background MTX. PROs included the SF-36, EuroQol 5-D (EQ-5D) index scores and visual analogue scale, Functional Assessment of Chronic Illness Therapy-Fatigue (FACIT-F), Health Assessment Questionnaire-Disability Index (HAQ-DI), Patient’s Global Assessment of Disease Activity (PtGA), patient’s assessment of pain and Work Productivity and Activity Impairment Questionnaire-Rheumatoid Arthritis (WPAI-RA), and measures collected in electronic patient daily diaries: duration and severity of morning joint stiffness (MJS), Worst Ttiredness and Worst Joint Pain. The primary study endpoint was at week 12. Treatment comparisons were assessed with logistic regression for categorical measures or analysis of covariance for continuous variables. Results Compared with placebo and adalimumab, baricitinib showed statistically significant improvements (p≤0.05) in HAQ-DI, PtGA, pain, FACIT-F, SF-36 physical component score, EQ-5D index scores and WPAI-RA daily activity at week 12. Improvements were maintained for measures assessed to week 52. Statistically significant improvement in patient diary measures (MJS duration and severity), worst tiredness and worst joint pain were observed for baricitinib versus placebo and adalimumab at week 12 (p≤0.05). Conclusions Baricitinib provided significantly greater improvement in most PROs compared with placebo and adalimumab, including physical function MJS, pain, fatigue and quality of life. Improvement was maintained to the end of the study (week 52). Trial registration NCT01710358. PMID:28798049

  14. Gene Expression Analysis before and after Treatment with Adalimumab in Patients with Ankylosing Spondylitis Identifies Molecular Pathways Associated with Response to Therapy

    OpenAIRE

    Dolcino, Marzia; Tinazzi, Elisa; Pelosi, Andrea; Patuzzo, Giuseppe; Moretta, Francesca; Lunardi, Claudio; Puccetti, Antonio

    2017-01-01

    The etiology of Ankylosing spondylitis (AS) is still unknown and the identification of the involved molecular pathogenetic pathways is a current challenge in the study of the disease. Adalimumab (ADA), an anti-tumor necrosis factor (TNF)-alpha agent, is used in the treatment of AS. We aimed at identifying pathogenetic pathways modified by ADA in patients with a good response to the treatment. Gene expression analysis of Peripheral Blood Cells (PBC) from six responders and four not responder p...

  15. Patient-reported outcomes from a phase 3 study of baricitinib versus placebo or adalimumab in rheumatoid arthritis: secondary analyses from the RA-BEAM study.

    Science.gov (United States)

    Keystone, Edward C; Taylor, Peter C; Tanaka, Yoshiya; Gaich, Carol; DeLozier, Amy M; Dudek, Anna; Zamora, Jorge Velasco; Cobos, Jose Arturo Covarrubias; Rooney, Terence; Bono, Stephanie de; Arora, Vipin; Linetzky, Bruno; Weinblatt, Michael E

    2017-11-01

    To assess the effect of baricitinib on patient-reported outcomes (PROs) in patients with active rheumatoid arthritis and an inadequate response to methotrexate (MTX). In this double-blind phase 3 study, patients were randomised 3:3:2 to placebo (n=488), baricitinib 4 mg once daily (n=487), or adalimumab 40 mg biweekly (n=330) with background MTX. PROs included the SF-36, EuroQol 5-D (EQ-5D) index scores and visual analogue scale, Functional Assessment of Chronic Illness Therapy-Fatigue (FACIT-F), Health Assessment Questionnaire-Disability Index (HAQ-DI), Patient's Global Assessment of Disease Activity (PtGA), patient's assessment of pain and Work Productivity and Activity Impairment Questionnaire-Rheumatoid Arthritis (WPAI-RA), and measures collected in electronic patient daily diaries: duration and severity of morning joint stiffness (MJS), Worst Ttiredness and Worst Joint Pain. The primary study endpoint was at week 12. Treatment comparisons were assessed with logistic regression for categorical measures or analysis of covariance for continuous variables. Compared with placebo and adalimumab, baricitinib showed statistically significant improvements (p≤0.05) in HAQ-DI, PtGA, pain, FACIT-F, SF-36 physical component score, EQ-5D index scores and WPAI-RA daily activity at week 12. Improvements were maintained for measures assessed to week 52. Statistically significant improvement in patient diary measures (MJS duration and severity), worst tiredness and worst joint pain were observed for baricitinib versus placebo and adalimumab at week 12 (p≤0.05). Baricitinib provided significantly greater improvement in most PROs compared with placebo and adalimumab, including physical function MJS, pain, fatigue and quality of life. Improvement was maintained to the end of the study (week 52). NCT01710358. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless

  16. Adalimumab for orbital myositis in a patient with Crohn's disease who discontinued infliximab: a case report and review of the literature.

    Science.gov (United States)

    Verma, Sanam; Kroeker, Karen I; Fedorak, Richard N

    2013-04-04

    Orbital myositis is a rare extra-intestinal manifestation of inflammatory bowel disease. Seventeen cases of Crohn's disease associated orbital myositis and 3 cases of ulcerative colitis associated orbital myositis have been reported in the published literature since 1970. We report the use of adalimumab (Abbott, Canada, Inc.) for orbital myositis in a patient with Crohn's disease who discontinued infliximab (Janssen, Canada, Inc.) and review of the published literature. A 35 year-old male with a 7-year history of Crohn's disease was treated with an ileocolonic resection and re-anastomosis followed by infliximab which maintained full endoscopic and clinical remission for four years. After stopping the infliximab for infusion-related reactions he presented with 3-day history of severe right eye pain, pain with ocular movement, proptosis, and conjunctival injection. He had no intestinal symptoms and endoscopic assessment revealed no active luminal disease. CT of the orbit revealed an enlarged right medial rectus muscle with tendonous involvement and a diagnosis of orbital myositis was made. Treatment with 80 mg per day prednisone with tapering dose and adalimumab, induction and maintenance, resulted in rapid resolution of the orbital myositis and ocular symptoms with no recurrences on follow-up at 10 months. The current case demonstrates a rare extraintestinal manifestation of Crohn's disease, orbital myositis, and its temporal relationship to the discontinuance of infliximab therapy and its successful treatment, without recurrence with tapering prednisone and adalimumab.

  17. Pilot study of the safety and effect of adalimumab on pain, physical function, and musculoskeletal disease in mucopolysaccharidosis types I and II

    Directory of Open Access Journals (Sweden)

    Lynda E. Polgreen

    2017-03-01

    Full Text Available Mucopolysaccharidosis I and II are lysosomal storage disorders that, despite treatment with hematopoietic cell transplantation (HCT and/or enzyme replacement therapy (ERT, continue to cause significant skeletal abnormalities leading to pain, stiffness, physical dysfunction, and short stature. Tumor necrosis factor – alpha (TNF-α is elevated in individuals with MPS I and II and associated with pain and physical dysfunction. Therefore, we evaluated the safety and effects of the TNF-α inhibitor adalimumab in patients with MPS I and II in a 32-week, randomized, double blind, placebo-controlled, crossover study of adalimumab at a dose of 20 mg (weight 15–5° in seven of eight joints in Subject #1 and five of eight joints in Subject #2 (range 7.0° to 52.8°. There was no change in the PPQ, 6MWT, or hand dynamometer. Data from this small pilot study suggest that treatment with adalimumab is safe, tolerable, and may improve ROM, physical function, and possibly pain, in children with MPS I or II. However, additional clinical trials are needed before this therapy should be recommended as part of clinical care.

  18. Rare Complications with Monochorionic Twins: Ultrasonography and Pathology Correlations

    Energy Technology Data Exchange (ETDEWEB)

    Song, Mi Jin [Cheil General Hospital and Women' s Healthcare Center, Kwandong University College of Medicine, Seoul (Korea, Republic of)

    2009-12-15

    We reviewed the sonographic features of rare complications with monochorionic twins and we correlated these features with the pathologic findings. The complications, including the twin reversed arterial perfusion sequence, the twin embolization syndrome, the conjoined twins and umbilical cord entanglement. A better understanding of these complications can aid making an accurate prenatal diagnosis and predicting the fetal outcome

  19. Adalimumabe no tratamento da artrite reumatoide: uma revisão sistemática e metanálise de ensaios clínicos randomizados

    Directory of Open Access Journals (Sweden)

    Marina Amaral de Ávila Machado

    2013-10-01

    Full Text Available Desde a descoberta do papel do fator de necrose tumoral no processo fisiopatológico da artrite reumatoide, cinco medicamentos bloqueadores dessa citocina têm sido empregados como opção terapêutica. Para avaliar a eficácia e a segurança do adalimumabe no tratamento da artrite reumatoide foi conduzida uma revisão sistemática com metanálise de ensaios clínicos controlados e randomizados. Foi realizada busca de estudos relevantes nas bases de dados Medline (via PubMed e LILACS em junho de 2011. A seleção dos estudos, coleta e análise de dados foram realizadas de forma pareada e independente por dois revisores e por um terceiro revisor em casos de discordância. A metanálise foi conduzida no software Review Manager® 5.1 usando o modelo de efeitos aleatórios. Onze artigos referentes ao adalimumabe foram incluídos e contemplaram nove estudos com 3461 pacientes. Dez estudos mostraram baixo risco de viés quanto ao cegamento dos participantes e pessoal e cegamento de avaliação de resultados. Os pacientes que receberam tratamento da associação de adalimumabe e metotrexato apresentam melhores resultados de eficácia e menor progressão radiográfica quando comparados ao grupo placebo + metotrexato em 24 a 104 semanas. Os pacientes que utilizaram adalimumabe em monoterapia apresentaram melhores resultados de eficácia em relação ao placebo em 24 e 26 semanas. Os resultados das metanálises de eventos adversos não foram estatisticamente significantes, exceto para reações no local de aplicação, na qual favoreceu o grupo controle. A eficácia do adalimumabe foi demonstrada em monoterapia e associado a algum MMCD, porém as evidências para o uso combinado são mais robustas.

  20. Efficacy of adalimumab in patients with crohn's disease and failure to infliximab therapy: a clinical series Eficacia de Adalimumab en pacientes con enfermedad de Crohn y fracaso previo a la terapia con Infliximab: resultados de una serie clínica

    Directory of Open Access Journals (Sweden)

    Patricia Cordero-Ruiz

    2011-06-01

    Full Text Available Background: adalimumab, a human anti-TNF, is an effective induction and maintenance therapy for patients with moderate to severe Crohn's disease. It seems to be effective in patients with resistance to infliximab, too, though the experience is more limited. Aim: to evaluate the efficacy of adalimumab, in patients with Crohn's disease (CD and failure to previous treatment with infliximab. B twenty-five patients with CD and failure to previous treatment with infliximab were enrolled; they were treated with 160/80 (24 patients and 80/40 (1 patient induction doses. We analyze clinical response to treatment with adalimumab by the Crohn's disease Activity Index (CDAI and plasma concentration of C-reactive protein (CRP, steroid sparing and complete fistula closure at week 48. Results: eighteen out of twenty-five patients (72% achieved clinical remission (CDAI score Introducción: adalimumab, un anti-TNF humano, ha demostrado ser efectivo en la inducción y tratamiento de mantenimiento de la enfermedad de Crohn moderada-grave. Existe menos experiencia, pero este fármaco parece también eficaz en los pacientes con pérdida de respuesta o intolerancia al infliximab. Objetivo: evaluar la eficacia de adalimumab durante un año, en nuestra serie de pacientes con enfermedad de Crohn (EC y fracaso en el tratamiento previo con infliximab. Métodos: se incluyen 25 pacientes con enfermedad de Crohn y fracaso previo a la terapia con infliximab, que son tratados con adalimumab. Se utilizaron dosis de inducción de 160/80 mg en 24 pacientes y dosis de 80/40 en un paciente. Analizamos la respuesta clínica al tratamiento con adalimumab mediante el Índice de actividad de la enfermedad de Crohn (CDAI y las concentraciones plasmáticas de proteína C reactiva (PCR, el cese de la corticoterapia y el cierre completo de las fistulas en la semana 48. Resultados: dieciocho de veinticinco pacientes (72% alcanzan la remisión clínica (CDAI < 150 en la semana 24 y 15

  1. Severe neurological complication following adjustable gastric banding.

    Science.gov (United States)

    Martines, G; Musa, N; Aquilino, F; Capuano, P

    2018-01-01

    In the last years with the increase of bariatric surgery, first of all as a result of new indications, a rise in the incidence of nutrient-related complications has been observed. Currently little is known about the impact of post-bariatric malnutrition and neurological complications. Wernicke's encephalopathy is a severe neurological syndrome which occurs as a result of thiamine deficiency. Wernicke-Korsakoff syndrome must be considered a serious neurological complication of bariatric surgery with significant morbidity and mortality, with rapidly progressing neurological symptoms, and must be treated immediately. We report the case of a 35 years-old male patient, affected by morbid obesity, anxious-depressive syndrome and alcohol use disorder, who after adjustable gastric banding implanted in another hospital developed a severe malnutrition and neurological syndrome. The patient showed poor adherence to the follow-up and to the dietary indications and after all, we needed to place a PEG for enteral nutrition in order to resolve the malnutrition condition and the neurological syndrome. Our experience emphasizes that preoperative selection and assessment of a patient's nutritional status according to guidelines, is required to identify potential problems, and that bariatric surgeons or physicians caring for patient who have undergone bariatric surgery should be familiar with the constellation of nutritional and neurological disorder that may occur after surgery. We want to remark the importance of preoperative selection of the patients, the follow-up and the cooperation between patient and physician in order to obtain the best result and avoid severe complications.

  2. Abdominal CT findings of delayed postoperative complications

    Energy Technology Data Exchange (ETDEWEB)

    Zissin, R.; Osadchy, A. [Sapir Medical Center, Dept. of Diagnostic Imaging, Kfar Saba (Israel)]. E-mail: zisinrivka@clalit.org.il; Gayer, G. [Assaf Harofe Medical Center, Dept. of Diagnostic Imaging, Zrifin (Israel)

    2007-10-15

    Despite progress in surgical techniques and modern medical treatment, postoperative complications occur not infrequently and vary according to type of surgery, clinical setting, and time elapsed since surgery. In general, they can be divided into early and delayed complications. Delayed postoperative complications can be classified as specific and nonspecific. The common nonspecific delayed complications are incisional hernia and postoperative bowel obstruction. Bowel obstruction can be further categorized as obstruction related to benign or neoplastic etiology, the latter occurring in oncology patients in whom the primary surgery was related to an underlying abdominal neoplasm. Gossypiboma is another, fortunately rare, postoperative complication. Specific complications appear after specific operations and include the following: Splenosis - following splenectomy. Retained gallstones and spilled gallstones - following cholecystectomy, mainly laparoscopic. Dropped appendicolith and stump appendicitis - following appendectomy, mainly laparoscopic. Obturation obstruction by a bezoar - following gastric surgery. Afferent loop syndrome (ALS) - following Bilroth II gastrectomy. (author)

  3. Relapsing polychondritis associated with psoriasis vulgaris successfully treated with adalimumab: A case report with published work review.

    Science.gov (United States)

    Matsuo, Haruka; Asahina, Akihiko; Fukuda, Takeshi; Umezawa, Yoshinori; Nakagawa, Hidemi

    2017-07-01

    Relapsing polychondritis (RP) is a rare autoimmune-mediated disease characterized by inflammation involving cartilaginous tissues. We report here a case of RP in a 38-year-old Japanese man with 13-year duration of psoriasis vulgaris treated with topical steroids and vitamin D 3 . The patient presented with tender swelling and erythema of both auricles, and the antibody to type II collagen was detected. The biopsy specimen revealed a dense mixed cell infiltration over the auricular cartilage. We reviewed eight cases with the association of RP and psoriasis, and in all cases the clinical course of psoriasis did not correlate with that of RP. The severity of RP was mild in the majority of cases, and our case was unique in that the patient had no joint symptoms. Adalimumab treatment was effective for both RP and psoriasis. Fat-suppressed contrast-enhanced magnetic resonance imaging was beneficial, not only to demonstrate subclinical inflammation in the nasal septum, but also to subjectively assess the improvement of RP. © 2017 Japanese Dermatological Association.

  4. [Metastatic prostate cancer complicated with chronic disseminated intravascular coagulopathy causing acute renal failure, mimicking thrombotic thrombocytopenic purpura and hemolytic uremic syndrome: pathomechanism, differential diagnosis and therapy related to a case].

    Science.gov (United States)

    Deme, Dániel; Ragán, Márton; Kalmár, Katalin; Kovács, Lajos; Varga, Erzsébet; Varga, Tünde; Rakonczai, Ervin

    2010-12-01

    in the peripheral blood), normal INR, elevated lactate dehydrogenase (LDH) and ARF suggested TTP-HUS. Hemodialysis and six plasmaferesis (PF) were carried out. After the fifth PF, skin manifestations of thrombotic microangiopathy occurred on the feet. Clotting analysis revealed elevated D-dimer (>5 μg/mL), normal fibrinogen (3.2 g/L), a slightly raised INR (1.36) and activated partial prothrombin time (APTT) (45.8 sec), normal reticulocyte (57 G/L) and a slightly low platelet count (123 G/L), which proved to be chronic DIC. Therapeutic dose of low-molecular-weight heparin (LMWH) was started. Elevated prostate-specific antigen (PSA) (109.6 ng/mL) suggested prostatic carcinoma. Prostate biopsy revealed adenocarcinoma (Gleason: 4+4 for left lobe and 3+3 for right lobe). Elevated alkaline phosphatase suggested metastases in the bone, which were confirmed by bone scintigraphy. Combined androgen blockade (CAB) was started. After three months follow-up our patient's status is satisfactory. PSA is in the normal range (4.6 ng/mL). Thrombocytopenia of uncertain origin with normal or raised INR, APTT, elevated D-dimer, normal fibrinogen and reticulocyte count prove the diagnosis of chronic DIC. This process warrants searching for metastatic neoplasia. Due to the relatively low serum levels of circulating procoagulant factors (e.g. tissue factor), therapeutic dose of LMWH can be used with good efficiency in chronic DIC with low risk of bleeding. Severe DIC as a complication of metastatic prostate cancer can be treated by androgen deprivation therapy (ADT) or CAB in combination with ketokonazole and concomitant use of supportive treatment. Deme D, Ragán M, Kovács L, Kalmár K, Varga E, Varga T, Rakonczai E. Metastatic prostate cancer complicated with chronic disseminated intravascular coagulopathy causing acute renal failure mimicking thrombotic thrombocytopenic purpura and hemolytic uremic syndrome: pathomechanism, differential diagnosis and therapy related to a case.

  5. Empty Sella Syndrome complicating meningitis | Ogunrin | Nigerian ...

    African Journals Online (AJOL)

    A.O. Ogunrin, V. Ajiduah, R. Onyemekeihia, M.T. Shokunbi. Full Text: EMAIL FREE FULL TEXT EMAIL FREE FULL TEXT · DOWNLOAD FULL TEXT DOWNLOAD FULL TEXT · AJOL African Journals Online. HOW TO USE AJOL... for Researchers · for Librarians · for Authors · FAQ's · More about AJOL · AJOL's Partners ...

  6. Acquired immunodeficiency syndrome and its ocular complications

    Directory of Open Access Journals (Sweden)

    Rao Narsing

    1994-01-01

    Full Text Available Human immunodeficiency virus infection is the first major pandemic of the 20th century. At present, almost 10 million people are known to be infected with this virus, and it is estimated that by the year 2000, approximately 40 million people will be infected. Transmission of this deadly infection is predominantly by sexual contact. Individuals infected with this virus pass through several predictable stages with progressive decrease in circulating CD4+ T cells. During the advanced stage, these patients develop various opportunistic infections or malignancies, or both. It is this advanced stage that was first recognized as AIDS, which has a 100% mortality rate. The opportunistic organisms that can involve the eye in patients with AIDS include cytomegalovirus, herpes zoster, Toxoplasma gondii, Mycobacterium tuberculosis, Cryptococcus neoformans, Mycobacterium avium-intracellulare, Pneumocystis carinii, Histoplasma capsulatum, Candida, and others. Intraocular lesions from these agents often represent disseminated infections. Visual morbidity occurs secondary to retinitis due to cytomegalovirus, herpes zoster, or Toxoplasma gondii. Anti-viral agents such as ganciclovir or foscarnet are effective against cytomegalovirus infection. The role of the ophthalmologist in the diagnosis and management of AIDS is becoming increasingly important. Not only does the eye reflect systemic disease, but ocular involvement may often precede systemic manifestations. In the AIDS patient, the ophthalmologist thus has an opportunity to make not only a slight-saving, but also life-saving diagnosis of disseminated opportunistic infections.

  7. FREY SYNDROME COMPLICATING PAROTIDECTOMY: A CASE ...

    African Journals Online (AJOL)

    Ademu

    No features of facial nerve palsy, no history of drug ingestion like coprozamole, steroids likely to cause parotid swelling, no past history of systemic illnesses like liver diseases, diabetic mellitus or malnutrition. No past history of trauma or irradiation to the region. There was no history suggestive of metastases to the lungs, ...

  8. Complications of MR vaccination in Hamedan

    Directory of Open Access Journals (Sweden)

    fatemeh Eghbalian

    2007-01-01

    Full Text Available Background: Complications of national program vaccination have great importance, because widespread use of vaccines and expanded age range of vaccinees increases the percentage of side effects. Present study was performed for evaluating the complications of national measles and rubella vaccination in Hamedan province. Materials and methods: This cross-sectional descriptive study was done from December sixth to January fifth 2003 and two months after termination of vaccination program. The study was performed by filling complication forms that were distributed in the vaccination centers. The data was analyzed by SPSS software. Results: A total of 827468 persons had been vaccinated in Hamedan, 260 out of them showed different complications. They had occurred mostly in 10 to 14 year old age group and had reported mainly by vaccination teams. 61.41 % of complications had been occurred in the first 24 hour after vaccination. 86 % of them had reactions to vaccination. 91 % of cases were outpatient. The complications with incidence rate more than were urticaria, fever, head ache, vomiting, lymphadenopathy, cough, skin rash, myalgia, pharyngitis, arthritis, rhinitis, restlessness and occular complications in decreasing order. The complications with incidence rate less than were sever dyspnea, seizure, diarrhea, paresthesia, anaphylaxis, encephalitis, thrombocytopenia, and Gillan – Barre syndrome. Conclusion: Mild complications were more frequent than severe ones. Most of the complications were related to reaction to vaccine, we didn’t have any program fault. Therefore we suggest a potent and flexible care system for reacting to side effects.

  9. Det hepatopulmonale syndrome

    DEFF Research Database (Denmark)

    Eibye, Simone; Christensen, Erik

    2016-01-01

    The hepatopulmonary syndrome (HPS) - a complication of liver disease - seems to be underdiagnosed, probably because of decreased awareness. HPS consists of the triade liver disease, intrapulmonary vascular dilatation and as a consequence arterial hypoxaemia. No medical therapy has proven effective...

  10. Ehlers-Danlos' Syndrom

    DEFF Research Database (Denmark)

    Leganger, Julie; Søborg, Marie-Louise Kulas; Farholt, Stense

    2016-01-01

    Ehlers-Danlos syndrome (EDS) comprises a group of diseases characterized by connective tissue fragility. The clinical symptoms primarily involve the skin, joints, blood vessels and internal organs. Diagnosing EDS is complicated because of the clinical variability, imprecise diagnostic criteria...

  11. A review of complications of odontogenic infections.

    Science.gov (United States)

    Bali, Rishi Kumar; Sharma, Parveen; Gaba, Shivani; Kaur, Avneet; Ghanghas, Priya

    2015-01-01

    Life-threatening infections of odontogenic or upper airway origin may extend to potential spaces formed by fascial planes of the lower head and upper cervical area. Complications include airway obstruction, mediastinitis, necrotizing fascitis, cavernous sinus thrombosis, sepsis, thoracic empyema, Lemierre's syndrome, cerebral abscess, orbital abscess, and osteomyelitis. The incidence of these "space infections" has been greatly reduced by modern antibiotic therapy. However, serious morbidity and even fatalities continue to occur. This study reviews complications of odontogenic infections. The search done was based on PubMed and Google Scholar, and an extensive published work search was undertaken. Advanced MEDLINE search was performed using the terms "odontogenic infections," "complications," and "risk factors."

  12. Ulnar nerve entrapment complicating radial head excision

    Directory of Open Access Journals (Sweden)

    Kevin Parfait Bienvenu Bouhelo-Pam

    Full Text Available Introduction: Several mechanisms are involved in ischemia or mechanical compression of ulnar nerve at the elbow. Presentation of case: We hereby present the case of a road accident victim, who received a radial head excision for an isolated fracture of the radial head and complicated by onset of cubital tunnel syndrome. This outcome could be the consequence of an iatrogenic valgus of the elbow due to excision of the radial head. Hitherto the surgical treatment of choice it is gradually been abandoned due to development of radial head implant arthroplasty. However, this management option is still being performed in some rural centers with low resources. Discussion: The radial head plays an important role in the stability of the elbow and his iatrogenic deformity can be complicated by cubital tunnel syndrome. Conclusion: An ulnar nerve release was performed with favorable outcome. Keywords: Cubital tunnel syndrome, Peripheral nerve palsy, Radial head excision, Elbow valgus

  13. Clinical remission in patients with active psoriatic arthritis treated with adalimumab and correlations in joint and skin manifestations.

    Science.gov (United States)

    Van den Bosch, Filip; Kavanaugh, Arthur; Kron, Martina; Kupper, Hartmut; Mease, Philip J

    2015-06-01

    Adalimumab (ADA) was evaluated for its efficacy in patients with moderate to severely active psoriatic arthritis (PsA) and for the presence of correlations in disease change variables. Patients with inadequate response to standard PsA therapy were given 40 mg of ADA every other week for up to 12 weeks or 20 weeks. Outcome variables encompassed tender joint count (TJC), swollen joint count (SJC), physician's global assessment (PGA) of psoriasis, Health Assessment Questionnaire (HAQ), patient's global assessment (PtGA) of disease activity and pain, C-reactive protein, as well as composite measures of disease activity. Patients with inactive skin disease symptoms at baseline were excluded from the remission analyses. Of 268 patients with active baseline joint and skin disease and data available at Week 12 following open-label ADA therapy, 73 achieved joint remission (27.2%, TJC ≤ 1 + SJC ≤ 1) and 144 achieved skin remission criteria (53.7%, PGA = clear/almost clear). Simultaneous joint and skin remission criteria were achieved in 16.0% and 24.8% of patients at weeks 12 and 20, respectively. In patients who did not achieve skin and/or joint remission, 12-week ADA treatment improved mean clinical and functional scores. Joint remission was more frequently associated with achieving clinically relevant outcomes including HAQ, PtGA disease activity, and PtGA pain compared to skin remission. No correlation between improvement in skin and joint disease was observed. ADA was effective in achieving strict criteria for remission in joint or skin disease in many patients with active PsA within 12 weeks and sustained through 20 weeks. (NCT00235885).

  14. Treatment efficacy and methotrexate-related toxicity in patients with rheumatoid arthritis receiving methotrexate in combination with adalimumab.

    Science.gov (United States)

    Burmester, Gerd R; Kaeley, Gurjit S; Kavanaugh, Arthur F; Gabay, Cem; MacCarter, Daryl K; Nash, Peter; Takeuchi, Tsutomu; Goss, Sandra L; Rodila, Ramona; Chen, Kun; Kupper, Hartmut; Kalabic, Jasmina

    2017-01-01

    Treatment of rheumatoid arthritis (RA) with a combination of methotrexate (MTX)+adalimumab (ADA) is more effective than ADA monotherapy. We assessed the toxicity of different doses of MTX and treatment efficacy of ADA+MTX in two trials. Data originated from CONCERTO, in patients with early RA initiating ADA+ 2.5, 5, 10 or 20 mg/week MTX for 26 weeks; and MUSICA, in patients with an inadequate response to MTX initiating ADA+ 7.5 or 20 mg/week MTX for 24 weeks. Efficacy was assessed by the American College of Rheumatology 50 (ACR50). Patient-reported MTX-related toxicity information was collected at each visit on 18 prespecified MTX-related adverse events (AE) in the MTX label. In CONCERTO, ACR50 rates increased over time, ranging from 54% to 68% at week 26, while AE rates remained steady, ranging from 2.4% to 17.8% at week 26. Of 395 patients, 113 (28.6%) reported 345 MTX-related AEs, including one serious AE (SAE, excessive fatigue and/or malaise); 10 AEs (in two patients) led to study discontinuation. In MUSICA, ACR50 rates increased over time, and were 32.3% and 37.5% at week 24, while MTX-related AE rates remained steady and were 6.5% at week 24. Of 309 patients, 71 (23%) reported 185 MTX-related AEs, including 5 SAEs (four infections and one fever/chills); six AEs (in four patients) led to study discontinuation. In patients with RA initiating ADA+MTX combination, treatment efficacy was achieved and increased throughout both trials, while rates of MTX-related AEs remained steady. MTX-related AEs were observed in up to 30% of patients and most were mild. MTX was discontinued by 0.5%-1.3% of patients. MUSICA (NCT01185288), CONCERTO (NCT01185301), Post results.

  15. Usefulness of a rapid faecal calprotectin test to predict relapse in Crohn's disease patients on maintenance treatment with adalimumab.

    Science.gov (United States)

    Ferreiro-Iglesias, Rocio; Barreiro-de Acosta, Manuel; Lorenzo-Gonzalez, Aurelio; Dominguez-Muñoz, Juan Enrique

    2016-01-01

    Predicting relapse in Crohn's disease (CD) patients by measuring non-invasive biomarkers could allow for early changes of treatment. Data are scarce regarding the utility of monitoring calprotectin to predict relapse. The aim of the study was to evaluate the predictive value of a rapid test of faecal calprotectin (FC) to predict for flares in CD patients on maintenance treatment with adalimumab (ADA). A prospective, observational cohort study was designed. Inclusion criteria were CD patients in clinical remission on a standard dose of ADA therapy. Fresh FC was measured using a rapid test. Thirty patients were included (median age 38 years, 56.7% female). After the 4 months follow-up, 70.0% patients remained in clinical remission and 30.0% had a relapse. FC concentration at inclusion was significantly higher in those patients who relapsed during the follow-up (625 μg/g) compared to those who stayed in remission (45 μg/g). The optimal cut-off for FC to predict relapse was 204 μg/g. The area under the receiver-operating characteristic curve was 0.968. Sensitivity, specificity, positive, and negative predictive value of FC to predict relapse were 100%, 85.7%, 74.1%, and 100%, respectively. In CD patients on ADA maintenance therapy, FC levels measured with a rapid test allow relapse over the following months to be predicted with high accuracy. Low FC levels exclude relapse within at least 4 months after testing, whereas high levels are associated with relapse in three out of every four patients.

  16. Comparative effectiveness and survival of infliximab, adalimumab, and etanercept for rheumatoid arthritis patients in the Hellenic Registry of Biologics: Low rates of remission and 5-year drug survival.

    Science.gov (United States)

    Flouri, Irini; Markatseli, Theodora E; Voulgari, Paraskevi V; Boki, Kyriaki A; Papadopoulos, Ioannis; Settas, Loukas; Zisopoulos, Dimitrios; Skopouli, Fotini N; Iliopoulos, Alexios; Bertsias, George K; Geborek, Pierre; Drosos, Alexandros A; Boumpas, Dimitrios T; Sidiropoulos, Prodromos

    2014-02-01

    To compare effectiveness, drug survival, and safety between infliximab, adalimumab, and etanercept, in a nationwide cohort of rheumatoid arthritis (RA) patients. This study is a prospective cohort study of 1208 active RA patients. Effectiveness, drug survival, and serious adverse events during entire follow-up (median 2.9 years) were monitored. EULAR and CDAI responses were comparable between the three agents (EULAR good/moderate responses at 12 months ranged 76-79%). At 12 months, 15-23% achieved remission. For adalimumab and etanercept, adjusted hazard rate (HR) for EULAR/ACR remission (reference: infliximab) was 2.7 and 2.1 (95% confidence interval was 1.7-4.1 and 1.3-3.4, respectively); males (HR 1.6; 1.1-2.4), use of glucocorticoids (HR 2.0; 1.3-3.0), and swollen joint count >7 (HR 0.36; 0.24-0.55) were independent predictors. Five-year drug survival was 31%, 43%, and 49% for infliximab, adalimumab, and etanercept, respectively (p = 0.010). Infliximab was associated with significantly more withdrawals due to adverse events. Disease activity, CRP, and use of glucocorticoids predicted efficacy-related drug survival; age, use of methotrexate, and prior DMARDs failures predicted safety-related survival. Risk for serious infections was lower with adalimumab (odds ratio [OR] 0.62; 0.38-1.00) or etanercept (OR 0.39; 0.21-0.72) than infliximab, independent of the effects of age (OR 1.65; 1.37-2.00 per 10 years), tender joint count >10 (OR 1.86; 1.21-2.86), and glucocorticoids >35mg/week (OR 1.83; 1.12-2.99). Response rates were comparable among anti-TNF agents. Overall, 5-year drug survival was below 50%, with infliximab demonstrating increased safety-related discontinuations. Remission rates are low in clinical practice. Strategies to increase effectiveness and long-term survival of anti-TNF agents in RA are needed. Copyright © 2014 Elsevier Inc. All rights reserved.

  17. Do Newborns Have More Complications When Mom Has Asthma?

    Science.gov (United States)

    ... earlier. Maternal asthma also increased the risk for neonatal intensive care unit (NICU) admission; jaundice; and breathing complications including respiratory distress syndrome, transient tachypnea of the newborn, and asphyxia. These findings were present even if babies were ...

  18. Ehlers-Danlos' syndrom

    DEFF Research Database (Denmark)

    Leganger, Julie; Søborg, Marie-Louise Kulas; Farholt, Stense

    2016-01-01

    Ehlers-Danlos syndrome Ehlers-Danlos syndrome (EDS) comprises a group of diseases characterized by connective tissue fragility. The clinical symptoms primarily involve the skin, joints, blood vessels and internal organs. Diagnosing EDS is complicated because of the clinical variability, imprecise...

  19. Dressler Syndrome

    Directory of Open Access Journals (Sweden)

    Erkan Ceylan

    2016-02-01

    Full Text Available Dressler Syndrome (DS is a febrile illness secondary to an inflammatory reaction involving the pleura and pericardium. It is more common in patients who have undergone surgery that involves opening the pericardium. However, DS has also been described following myocardial infarction and as an unusual complication after percutaneous procedures such as coronary stent implantation, after implantation of epicardial pacemaker leads and transvenous pacemaker leads, and following blunt trauma, stab wounds, and heart puncture. Pericardial effusions often accompany the syndrome and may develop into early or late postoperative cardiac tamponade and even recurrent cardiac tamponade. The syndrome is also characterized by pericardial or pleuritic pain, pleural effusions, pneumonitis, and abnormal ECG and radiography findings.

  20. Cirrhotic Multiorgan Syndrome

    DEFF Research Database (Denmark)

    Møller, Søren; Bendtsen, Flemming

    2015-01-01

    complications such as hepatorenal syndrome. In patients with chronic organ dysfunction, various precipitating events may induce an acute-on-chronic renal failure and acute-on-chronic liver failure that negatively affect the prognosis. Future research on the pathophysiologic mechanisms of the complications...

  1. Neuroacanthocytosis Syndromes

    Directory of Open Access Journals (Sweden)

    Walker Ruth H

    2011-10-01

    . Differential diagnoses include Huntington disease and other causes of progressive hyperkinetic movement disorders. There are no curative therapies for NA syndromes. Regular cardiologic studies and avoidance of transfusion complications are mandatory in McLeod syndrome. The hyperkinetic movement disorder may be treated as in Huntington disease. Other symptoms including psychiatric manifestations should be managed in a symptom-oriented manner. NA syndromes have a relentlessly progressive course usually over two to three decades.

  2. Complications and Deaths - State

    Data.gov (United States)

    U.S. Department of Health & Human Services — Complications and deaths - state data. This data set includes state-level data for the hip/knee complication measure, the Agency for Healthcare Research and Quality...

  3. Complications and Deaths - National

    Data.gov (United States)

    U.S. Department of Health & Human Services — Complications and deaths - national data. This data set includes national-level data for the hip/knee complication measure, the Agency for Healthcare Research and...

  4. Complications and Treatments

    Science.gov (United States)

    ... Possible Complications Links to Other Websites People with sickle cell disease (SCD) start to have signs of the disease during the first year of life, usually around 5 months of age. Symptoms and complications of SCD are different for each ...

  5. Pregnancy Complications: Shoulder Dystocia

    Science.gov (United States)

    ... X Home > Complications & Loss > Pregnancy complications > Shoulder dystocia Shoulder dystocia Now playing: E-mail to a friend Please ... some women more likely than others to have shoulder dystocia? A pregnant woman may be at risk for ...

  6. Complications and Deaths - Hospital

    Data.gov (United States)

    U.S. Department of Health & Human Services — Complications and deaths - provider data. This data set includes provider data for the hip/knee complication measure, the Agency for Healthcare Research and Quality...

  7. Pregnancy Complications: Anemia

    Science.gov (United States)

    ... online community Home > Complications & Loss > Pregnancy complications > Anemia Anemia E-mail to a friend Please fill in ... anemia at a prenatal care visit . What causes anemia? Usually, a woman becomes anemic (has anemia) because ...

  8. [Economic impact of etanercept and adalimumab biosimilars on hospitals scale covered by PharmAlp'Ain, a hospitals grouping of orders for health products].

    Science.gov (United States)

    Berreur, B; Guerin, F; Christophe, B; Limido, G; Paubel, P

    2018-01-01

    To evaluate the economic impact of future prescriptions of etanercept and adalimumab biosimilars at the territorial scale covered by PharmAlp'Ain, a hospitals grouping of orders for health products. Determination of the number and status of patients (naive or in continuation of treatment) from the National Database "Datamart de Consommation Inter-Régimes" of health insurance, concerned by a dispensation in a pharmacy of etanercept or adalimumab in 2015. Calculation of potential savings in case of biosimilar requirements according to 3 hypotheses: 63% (rate observed in a previous study) of initiations are treated with biosimiliaries and the others by princeps (H 1 ); all initiations under biosimilars and continuation therapy with the princeps (H 2 ) or all patients are treated with biosimilars (H 3 ). The annual savings are estimated at 237,000 € with the H 1 hypothesis. In the case of H 2 , the expected savings would be 376,200 € per year. In the case of H 3 , savings for the community could reach almost 1,282,800 € per year. The arrival of biosimilars allows significant savings for medicines market. According to the French recommendations in 2016, the expected savings are between the H 1 and H 2 hypothesis. The rate of penetration of biosimilars depends on many factors such as the involvement of health professionals, patient adherence, or health authority recommendations. Copyright © 2017 Académie Nationale de Pharmacie. Published by Elsevier Masson SAS. All rights reserved.

  9. Complications Arising in Twin Pregnancy: Findings of Prenatal Ultrasonography

    Science.gov (United States)

    Kim, Jeong-Ah; Lee, Young Ho; Song, Mi Jin; Min, Jee-Yeon; Lee, Hak Jong; Han, Byoung Hee; Lee, Kyung-Sang; Cho, Byung Jae; Chun, Yi-Kyeong

    2003-01-01

    Multifetal gestations are high-risk pregnancies involving higher perinatal morbidity and mortality, and are subject to unique complications including twin oligohydramnios-polyhydramnios sequence, twin-to-twin transfusion syndrome, acardiac twins, conjoined twins, co-twin demise, and heterotopic pregnancies. The purpose of this study is to describe the prenatal ultrasonographic and pathologic findings of these complications. PMID:12679635

  10. Pfeiffer syndrome

    Directory of Open Access Journals (Sweden)

    Fryns Jean-Pierre

    2006-06-01

    Full Text Available Abstract Pfeiffer syndrome is a rare autosomal dominantly inherited disorder that associates craniosynostosis, broad and deviated thumbs and big toes, and partial syndactyly on hands and feet. Hydrocephaly may be found occasionally, along with severe ocular proptosis, ankylosed elbows, abnormal viscera, and slow development. Based on the severity of the phenotype, Pfeiffer syndrome is divided into three clinical subtypes. Type 1 "classic" Pfeiffer syndrome involves individuals with mild manifestations including brachycephaly, midface hypoplasia and finger and toe abnormalities; it is associated with normal intelligence and generally good outcome. Type 2 consists of cloverleaf skull, extreme proptosis, finger and toe abnormalities, elbow ankylosis or synostosis, developmental delay and neurological complications. Type 3 is similar to type 2 but without a cloverleaf skull. Clinical overlap between the three types may occur. Pfeiffer syndrome affects about 1 in 100,000 individuals. The disorder can be caused by mutations in the fibroblast growth factor receptor genes FGFR-1 or FGFR-2. Pfeiffer syndrome can be diagnosed prenatally by sonography showing craniosynostosis, hypertelorism with proptosis, and broad thumb, or molecularly if it concerns a recurrence and the causative mutation was found. Molecular genetic testing is important to confirm the diagnosis. Management includes multiple-staged surgery of craniosynostosis. Midfacial surgery is performed to reduce the exophthalmos and the midfacial hypoplasia.

  11. Evaluación económica de la monoterapia con tocilizumab frente a adalimumab en artritis reumatoide

    Directory of Open Access Journals (Sweden)

    Federico Navarro Sarabia

    2013-01-01

    Full Text Available Fundamento: Tocilizumab (TCZ fue superior a adalimumab (ADA en monoterapia en la reducción de los signos y síntomas de la artritis reumatoide del adulto (AR en pacientes intolerantes o con respuesta inadecuada a metotrexato (MTX. El objetivo del estudio fue analizar el costeefectividad de TCZ vs ADA en estos pacientes. Métodos: Evaluación económica del coste por respuesta o remisión con TCZ vs ADAa partir del estudioADACTA(horizonte temporal: 24 semanas. Criterios de respuesta clínica ACR o de remisión de la enfermedad, índice DAS28. Ámbito: Sistema Nacional de Salud. Los costes incluidos (adquisición, administración y monitorización de los medicamentos en € de 2012 se obtuvieron de fuentes españolas. Se efectuaron análisis de sensibilidad simples univariantes. Resultados: Las tasas de respuestaACR20,ACR50 yACR70 con TCZ y ADA se obtuvieron en el 65% y 49,4% (p <0,01; 47,2% y 27,8% (p <0,01; y en el 32,5% y 17,9% (p <0,01 de los pacientes, respectivamente. La remisión DAS28 se produjo en el 39,9% y 10,5%, respectivamente (p <0,0001. El coste por respuesta fue menor con TCZ que con ADA (ACR20: 8.105 y 11.553 €; ACR50: 11.162 y 20.529 €; ACR70: 16.211 y 31.882 € respectivamente. El coste de la remisión DAS28 fue de 13.204 € y 54.352 € respectivamente. En todos los escenarios el tratamiento con TCZ tuvo mayor eficacia y menores costes que con ADA . Conclusiones: Según este análisis, en España la monoterapia con TCZ es una estrategia eficiente frente a ADA para el tratamiento de los pacientes con AR intolerantes o con respuesta inadecuada a MTX.

  12. Changes in Ultrasonographic Vascularity Upon Initiation of Adalimumab Combination Therapy in Rheumatoid Arthritis Patients With an Inadequate Response to Methotrexate.

    Science.gov (United States)

    Kaeley, Gurjit S; Nishio, Midori J; Goyal, Janak R; MacCarter, Daryl K; Wells, Alvin F; Chen, Su; Kupper, Hartmut; Kalabic, Jasmina

    2016-11-01

    To assess joint disease activity by ultrasound (US) in patients with rheumatoid arthritis (RA) initiating treatment with adalimumab (ADA) plus methotrexate (MTX). Data for this post hoc analysis originated from the MUSICA trial (ClinicalTrials.gov identifier: NCT01185288), which evaluated the efficacy of initiating ADA (40 mg every other week) plus 7.5 or 20 mg/week MTX in 309 patients with RA with an inadequate response to MTX. Synovial vascularization over 24 weeks was assessed bilaterally at metacarpophalangeal joint 2 (MCP2), MCP3, MCP5, metatarsophalangeal joint 5, and the wrists by power Doppler US (PDUS). A semiquantitative 4-grade scale was used. Disease activity was assessed using the Disease Activity Score in 28 joints using the C-reactive protein level (DAS28-CRP) and Simplified Disease Activity Index (SDAI). The correlation between continuous variables was assessed using Pearson's correlation coefficient. After 24 weeks of treatment with ADA plus MTX, rapid improvements in the mean synovial vascularity score were observed; the greatest improvements were in MCP2 (-0.5), MCP3 (-0.4), and the wrist (-0.4). At week 24, patients with the lowest DAS28-CRP ( 0.9). Synovial vascularity scores correlated poorly with DAS28, swollen joint count in 66 joints (SJC66), SJC28, tender joint count in 68 joints (TJC68), TJC28, Clinical Disease Activity Index (CDAI), SDAI, physician's global assessment, patient's global assessment of pain, and disease duration (ρ < 0.2). Thirty-two (70%) of 46 patients with a DAS28-CRP of <2.6, and 11 (58%) of 19 patients with an SDAI indicating remission had at least 1 joint with a synovial vascularity score of ≥1. PDUS detects changes in synovial vascularity in RA patients treated with ADA plus MTX, and residual synovial vascularity in patients in whom clinical disease control has been achieved. © 2016 The Authors. Arthritis & Rheumatology published by Wiley Periodicals, Inc. on behalf of the American College of Rheumatology.

  13. Successful treatment of PASH syndrome with infliximab, cyclosporine and dapsone.

    Science.gov (United States)

    Staub, J; Pfannschmidt, N; Strohal, R; Braun-Falco, M; Lohse, P; Goerdt, S; Leverkus, M

    2015-11-01

    The group of autoinflammatory syndromes associated with Pyoderma gangrenosum, Acne, and Suppurative Hidradenitis are poorly defined and difficult to control with currently available treatment modalities. We describe a patient with PASH syndrome and report about the successful multimodal treatment with infliximab, cyclosporine, and dapsone. A review of the available literature to date about this group of autoinflammatory diseases was performed. We performed genetic analysis for PSTPIP1 mutations associated with PAPA syndrome. A 22-year-old woman presented to our department with pyoderma gangrenosum, concomitant acne, and suppurative hidradenitis. She had previously been treated unsuccessfully with etanercept, adalimumab, fumaric acid and the IL-1 receptor antagonist (IL-1RA) anakinra without prolonged remission. Treatment with intravenous infliximab in combination with cyclosporine and dapsone lead to sudden and prolonged improvement of the clinical symptoms that we classified as PASH syndrome. We review the literature about this group of diseases and report the third case of PASH syndrome to date. PASH syndrome and associated diseases should be considered whenever hidradenitis suppurativa is found in association with pyoderma gangrenosum. We provide a systematic overview about PASH syndrome and suggest a novel multimodal therapeutic regimen beyond isolated inhibition of TNF or IL-1. © 2014 European Academy of Dermatology and Venereology.

  14. Erosive progression is minimal, but erosion healing rare, in rheumatoid arthritis patients treated with adalimumab. A 1 year investigator-initiated follow-up study using high-resolution computed tomography as the primary outcome measure

    DEFF Research Database (Denmark)

    Møller Døhn, Uffe; Boonen, Annelies; Hetland, Merete Lund

    2008-01-01

    -grade radiographic erosions in the wrist or metacarpophalangeal (MCP) joints in the same (index) hand, initiated adalimumab 40 mg sc. eow. Thirty-five patients completed the study (median age 61 years (range 19-86), disease duration 8 years (0-36)). CT of index wrist and MCP2-5 and radiographs of hands and forefeet...

  15. Extended complications of urethroplasty

    Directory of Open Access Journals (Sweden)

    Hosam S. Al-Qudah

    2005-08-01

    Full Text Available INTRODUCTION: An extensive study of complications following urethroplasty has never been published. We present 60 urethroplasty patients who were specifically questioned to determine every possible early and late complication. MATERIALS AND METHODS: Retrospective chart review of urethroplasty patients between August 2000 and March 2004. An "open format" questioning style allowed maximal patient reporting of all complications, no matter how minor. RESULTS: 60 patients underwent 62 urethroplasties (24 anterior anastomotic, 19 buccal mucosal and 10 fasciocutaneous, 9 posterior anastomotic with mean follow-up of 29 months. Early complications occurred in 40%, but only 3% were major (rectal injury and urosepsis. Early minor complications included scrotal swelling, scrotal ecchymosis and urinary urgency. Late complications occurred in 48%, but only 18% were significant (erectile dysfunction, chordee and fistula. Late minor complications included a feeling of wound tightness, scrotal numbness and urine spraying. Fasciocutaneous urethroplasty caused the most significant complications, and buccal mucus urethroplasty the least, while also resulting in the lowest recurrence rate (0%. CONCLUSIONS: Serious complications after urethroplasty (3% early and 18% late appear similar to those reported elsewhere, but minor bothersome complications appear to occur in much higher numbers than previously published (39% early and 40% late. While all the early complications were resolved and most (97% were minor, less than half of the late complications were resolved, although most (82% were minor. These complication rates should be considered when counseling urethroplasty patients, and generally tend to support the use of buccal mucosal onlay urethroplasty as it had the lowest rate of serious side effects.

  16. Neurological complications of Zika virus infection.

    Science.gov (United States)

    Carod-Artal, Francisco Javier

    2018-04-26

    Zika virus (ZIKV) disease is a vector-borne infectious disease transmitted by Aedes mosquitoes. Recently, ZIKV has caused outbreaks in most American countries. Areas covered: Publications about neurological complications of ZIKV infection retrieved from pubmed searchers were reviewed, and reference lists and relevant articles from review articles were also examined. Vertical/intrauterine transmission leads to congenital infection and causes microcephaly and congenital ZIKV syndrome. ZIKV preferentially infects human neural progenitor cells and triggers cell apoptosis. ZIKV RNA has been identified in foetal brain tissue and brains of microcephalic infants who died; amniotic fluid and placentas of pregnant mothers; and umbilical cord, cerebro-spinal fluid and meninges of newborns. The increase in the number of Guillain-Barre syndrome (GBS) cases during the ZIKV outbreak in the Americas provides epidemiological evidence for the link between ZIKV infection and GBS. Less frequently reported ZIKV neurological complications include encephalitis/meningoencephalitis, acute disseminated encephalomyelitis, myelitis, cerebrovascular complications (ischemic infarction; vasculopathy), seizures and encephalopathy, sensory polyneuropathy and sensory neuronopathy. Analysis of GBS incidence could serve as an epidemiological 'marker' or sentinel for ZIKV disease and other neurological complications associated to ZIKV. Expert commentary: An expanding spectrum of neurological complications associated with ZIKV infection is being recognised.

  17. Polyposis syndromes: pediatric implications.

    Science.gov (United States)

    Hyer, W

    2001-10-01

    The diagnosis of a polyposis syndrome, such as juvenile polyposis, Peutz-Jeghers syndrome, and familial adenomatous polyposis, requires knowledge of the site, number, and histologic type of the polyps and an appreciation of relevant family history. Children and adolescents with polyposis syndromes are faced with not only the immediate complications of the polyps, such as intussusception or bleeding, but also the extraintestinal manifestations and the long-term risk for malignancy. This article reviews the diagnosis, clinical management, surveillance, and surgical options for children with polyposis syndromes and discusses genetics and appropriate screening programs.

  18. Complications of allogeneic hematopoietic stem cell transplantation.

    Science.gov (United States)

    Arnaout, Karim; Patel, Nihar; Jain, Maneesh; El-Amm, Joelle; Amro, Farah; Tabbara, Imad A

    2014-08-01

    Infection, graft-versus-host disease (GVHD), and to a lesser extent sinusoidal obstructive syndrome (SOS) represent the major causes of morbidity and mortality in patients undergoing allogeneic hematopoietic stem cell transplantation (AHSCT). During the last decade, progress in prevention and treatment of these complications led to improvement in the outcome of these patients. Despite the fact that nonmyeloablative regimens have been increasingly used in elderly patients and in patients with co-morbidities, the nonrelapse related mortality remains a challenge and long-term follow-up is required. The objective of this manuscript is to provide an updated concise review of the complications of AHSCT and of the available treatment interventions.

  19. Correlation between Complicated Diverticulitis and Visceral Fat

    Science.gov (United States)

    Jeong, Jong Heon; Kim, Jin Ok; Tae, Hye Jin; Jung, Suk Hyun; Lee, Kang Nyeong; Jun, Dae Won; Lee, Oh Young; Yoon, Byung Chul; Choi, Ho Soon; Hahm, Joon Soo; Song, Soon Young

    2011-01-01

    The aim of this study was to examine the relationship of complications related to diverticulitis and visceral obesity. The study was based on a retrospective case note review conducted at the Hanyang University Hospital. Patients were diagnosed with diverticulitis based on clinical symptoms and abdominal computed tomography (CT) findings and divided into two groups: those admitted with complicated diverticulitis and those with a simple diverticulitis episode. We compared the body mass index (BMI) and degree of visceral obesity, measured by abdominal CT. The study included 140 patients, 87 (62.1%) were simple diverticulitis and 53 (37.9%) were complicated diverticulitis. In the complicated diverticulitis group, 9 (6.4%) cases were recurrent, 29 (20.7%) were perforation or abscess patients, and 28 (20%) were patients with systemic inflammatory response syndrome (SIRS). Of the SIRS patients, 13 were involved in other complication groups. When comparing in the two groups, the complicated diverticulitis group had a significantly higher visceral fat area (128.57 cm2 vs 102.80 cm2, P = 0.032) and a higher ratio of visceral fat area/subcutaneous fat area (0.997 vs 0.799, P = 0.014). Visceral obesity is significantly associated with complications of diverticulitis. PMID:22022188

  20. Correlation between complicated diverticulitis and visceral fat.

    Science.gov (United States)

    Jeong, Jong Heon; Lee, Hang Lak; Kim, Jin Ok; Tae, Hye Jin; Jung, Suk Hyun; Lee, Kang Nyeong; Jun, Dae Won; Lee, Oh Young; Yoon, Byung Chul; Choi, Ho Soon; Hahm, Joon Soo; Song, Soon Young

    2011-10-01

    The aim of this study was to examine the relationship of complications related to diverticulitis and visceral obesity. The study was based on a retrospective case note review conducted at the Hanyang University Hospital. Patients were diagnosed with diverticulitis based on clinical symptoms and abdominal computed tomography (CT) findings and divided into two groups: those admitted with complicated diverticulitis and those with a simple diverticulitis episode. We compared the body mass index (BMI) and degree of visceral obesity, measured by abdominal CT. The study included 140 patients, 87 (62.1%) were simple diverticulitis and 53 (37.9%) were complicated diverticulitis. In the complicated diverticulitis group, 9 (6.4%) cases were recurrent, 29 (20.7%) were perforation or abscess patients, and 28 (20%) were patients with systemic inflammatory response syndrome (SIRS). Of the SIRS patients, 13 were involved in other complication groups. When comparing in the two groups, the complicated diverticulitis group had a significantly higher visceral fat area (128.57 cm(2) vs 102.80 cm(2), P = 0.032) and a higher ratio of visceral fat area/subcutaneous fat area (0.997 vs 0.799, P = 0.014). Visceral obesity is significantly associated with complications of diverticulitis.