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1

Stevens-Johnson syndrome complicating adalimumab therapy in Crohn's disease  

PubMed Central

The anti-tumor necrosis factor (TNF)? medications demonstrate efficacy in the induction of remission and its maintenance in numerous chronic inflammatory conditions. With the increasing number of patients receiving anti-TNF? agents, however, less common adverse reactions will occur. Cutaneous eruptions complicating treatment with an anti-TNF? agent are not uncommon, occurring in around 20% of patients. Adalimumab, a fully humanized antibody against TNF?, may be expected to cause minimal immune-mediated skin reactions compared to the chimeric monoclonal antibody, infliximab. We, however, report a case of Stevens-Johnson syndrome that required hospitalization and cessation of adalimumab in a patient with Crohn’s disease (CD). In this case report, a 29-year-old male with colonic and perianal CD with associated erythema nodosum and large joint arthropathy developed severe mucositis, peripheral rash and desquamation, fevers and respiratory symptoms concomitant with a second dose of 40 mg adalimumab after a 2 mo break from adalimumab therapy. Skin biopsies of the abdominal wall confirmed erythema multiforme and the patient was on no other drugs and infective etiologies were excluded. The patient responded rapidly to IV hydrocortisone and was able to be commenced on infliximab without recurrence of the Stevens-Johnson syndrome. Desquamating skin reactions have now been described in three of the TNF? antagonists (infliximab, etanercept and adalimumab). These reactions can be serious and prescribers need to be aware of the potential mucocutaneous side effects of these agents, especially as Stevens-Johnson syndrome is associated with significant morbidity and mortality. PMID:19764100

Salama, Muna; Lawrance, Ian Craig

2009-01-01

2

Stevens Johnson Syndrome associated with Lamotrigine.  

PubMed

Stevens-Johnsons Syndrome (SJS) is an immune-complex-mediated hypersensitivity reaction and has been linked as an adverse side effects to many drugs. Lamotrigine, an anticonvulsive medication and also a commonly used mood stabiliser, can be associated with this adverse reaction. Although this has not been reported very commonly , SJS has high mortality and morbidity and requires careful attention as the use of Lamotrigine is increasing in clinical practice. We present a case where the patient developed Stevens - Johnson Syndrome three weeks after being started on Lamotrigine. The case is discussed for its relevance to the use of Lamotrigine which is currently prescribed very commonly in psychiatric practices. PMID:24550973

Parveen, Shama; Javed, M Afzal

2013-11-01

3

Stevens Johnson Syndrome associated with Lamotrigine  

PubMed Central

Stevens-Johnsons Syndrome (SJS) is an immune-complex-mediated hypersensitivity reaction and has been linked as an adverse side effects to many drugs. Lamotrigine, an anticonvulsive medication and also a commonly used mood stabiliser, can be associated with this adverse reaction. Although this has not been reported very commonly , SJS has high mortality and morbidity and requires careful attention as the use of Lamotrigine is increasing in clinical practice. We present a case where the patient developed Stevens - Johnson Syndrome three weeks after being started on Lamotrigine. The case is discussed for its relevance to the use of Lamotrigine which is currently prescribed very commonly in psychiatric practices. PMID:24550973

Parveen, Shama; Javed, M. Afzal

2013-01-01

4

[Stevens-Johnson syndrome after lamotrigine treatment].  

PubMed

We report a 18 years old female with Lennox-Gastaut syndrome under treatment with sodium valproate, carbamazepine and clonazepam. When seizures increased we stopped carbamazepine and introduced lamotrigine slowly. One month later the girl developed haemorrhagic erosions in mucoses and limbs with deterioration of her general state. Skin biopsy confirmed the diagnosis of erythema multiforme, the Stevens-Johnson's form. The immediate withdrawal of lamotrigine and treatment with antihystaminics and steroids was followed of a slowly favourable course with disappearance of symptomatology one month later. It's another case of Stevens-Johnson syndrome related to the introduction of lamotrigine in polytherapy. PMID:8556627

Campistol, J; Geli, M; Llistosella, E; Molins, J; Llobet, M

1995-01-01

5

The Steven Johnson syndrome. A case study.  

PubMed

Steven Johnson's Syndrome is a serious systemic disorder in which there are vesicobullous lesions involving the skin and mucous membranes. It can result as an immune response to an antigen or as a drug reaction. Most often it is considered as an allergic reaction. It is a self-limiting condition which responds to immediate management or may result in fluid loss, sepsis and death. PMID:10818901

Baby, S; Doris, S

1999-07-01

6

Imatinib-induced Stevens-Johnsons syndrome.  

PubMed

Imatinib mesylate is a tyrosine kinase inhibitor used widely as the first-line treatment for chronic myeloid leukaemia (CML). The side-effect profile of this drug includes fluid retention, muscle cramps, diarrhoea, myelosuppression and skin rashes. Of these, rashes of the type maculo-papular eruptions and oedema developed most commonly. The cutaneous adverse reactions other than maculo-papular eruptions are rare with imatinib. Severe and life-threatening cutaneous reactions can occur in 5% cases. Here, the author reports a case of newly diagnosed CML that developed Steven-Johnsons syndrome due to imatinib therapy. Patient responded and discharged successfully on withdrawal of the culminating drug. PMID:23349042

Jha, Praveen; Himanshu, D; Jain, Nirdesh; Singh, Ajay Kumar

2013-01-01

7

Steven-Johnson syndrome due to unknown drugs [corrected].  

PubMed

Steven-Johnson syndrome may be considered as a cytotoxic immune reaction to drugs, infections etc. This is a case report of Steven-Johnson syndrome due to an ayurvedic preparation which was used in the treatment of mental retardation in a young girl. PMID:22345890

Shivamurthy, Raghu Prasada M; Kallappa, Ravindra; Reddy, Shashikala G H; Rangappa, Druva Kumar B

2012-01-01

8

Acetaminophen induced Steven Johnson syndrome-toxic epidermal necrolysis overlap  

Microsoft Academic Search

Steven Johnson Syndrome and Toxic Epidermal Necrolysis are rare but severe form of hypersensitivity inflammatory reactions to multiple offending agents including drugs. Acetaminophen is extensively used due to its analgesic and anti-pyretic properties. It is rendered to be relatively safe, with hepatotoxicity considered to be the major adverse effect. However, very few cases of Steven Johnson Syndrome and Toxic Epidermal

Ali Khawaja; Ahmed Shahab; Syed Ather Hussain

2012-01-01

9

Phenytoin induced Stevens-Johnson syndrome exacerbated by cefepime.  

PubMed

Steven Johnson syndrome (SJS) is a rare drug induced mucocutaneous reaction. Here, we present an elaborate report of a 28-year-old female patient who developed Phenytoin induced SJS, which was exacerbated by cefepime. PMID:24250210

Prabhu, Varsha A; Doddapaneni, Sahiti; Thunga, Girish; Thiyagu, Rajakannan; Prabhu, M Mukyaprana; Naha, Kushal

2013-10-01

10

Late onset imatinib-induced Stevens-Johnson syndrome.  

PubMed

Imatinib, a BCR-ABL tyrosine kinase inhibitor, is known to cause mild to moderate cutaneous reactions in up to approximately 20% of patients. It rarely causes severe reactions, such as Stevens-Johnson syndrome, which can be life threatening. Typically, these rashes occur within two months of initiating therapy. We report a case of Stevens-Johnson syndrome induced by imatinib after several years of therapy, but later successfully was treated with nilotinib therapy. PMID:24399835

Bois, Evan; Holle, Lisa M; Farooq, Umar

2014-12-01

11

Stevens-Johnson syndrome associated with Mycoplasma pneumoniae infections.  

PubMed

The Stevens-Johnson syndrome is a multisystem inflammatory disorder associated with a widespread erythematous eruption that can result in death. Although usually considered a pediatric disease, this syndrome frequently affects adults. There are many etiologic associations including drugs and infections; however, the pathophysiology of the syndrome remains obscure. Treatment at present is symptomatic and supportive. Although frequently used, the beneficial role of corticosteroids in this syndrome remains to be proved. The case report describes a young woman who after treatment with several drugs developed the Stevens-Johnson syndrome in association with a Mycoplasma pneumoniae infection. We include a brief review of the literature with emphasis on the Stevens-Johnsons syndrome's association with M pneumoniae infections. Those caring for patients with skin disease should be aware of the association between such treatable infections and this syndrome. PMID:629550

Sontheimer, R D; Garibaldi, R A; Krueger, G G

1978-02-01

12

Incomplete Stevens-Johnson syndrome secondary to atypical pneumonia.  

PubMed

Steven-Johnson syndrome is a common condition characterised by erythematous target lesions on the skin and involvement of the oral mucosa, genitals and conjunctivae. It has been documented as one of the extra-pulmonary manifestations of Mycoplasma pneumoniae infection. Recently, there has been several documentation of an incomplete presentation of this syndrome - without the typical rash but with mucosal, conjunctival and genital involvement. Our case illustrates that the incomplete Steven-Johnson syndrome may present with oral mucosal and conjunctival involvement alone without skin or genital involvement. This important clinical diagnosis should not be missed due to its atypical presentation. Treatment of Steven-Johnson syndrome remains supportive along with treating the underlying infection if recognised. PMID:22679161

Ramasamy, Anantharaman; Patel, Chiraush; Conlon, Christopher

2011-01-01

13

Steven Johnson syndrome in a patient with Cushing's disease.  

PubMed

Cushing's syndrome is a pathological condition associated with excessive cortisol production, the commonest etiology being Cushing's disease. Corticosteroids in high doses have been used in the management of Steven Johnson Syndrome (SJS) with favourable outcome. We describe a patient with Cushing's disease who developed SJS, one week after taking sperulina a product from sea-weed while waiting for transphenoidal surgery. PMID:20527277

Mustafa, N; Periyasamy, P; Kamaruddin, N

2009-09-01

14

Intravenous immunoglobulin therapy for Stevens-Johnson syndrome.  

PubMed

Stevens-Johnson syndrome (SJS) is an acute mucocutaneous disorder that can be associated with considerable morbidity. Several previous reports, all involving either adults with acquired immunodeficiency syndrome or children, suggest that intravenous immunoglobulin may be an effective treatment for SJS. We report a case of SJS in an immunocompetent adult whose condition improved dramatically after therapy with intravenous immunoglobulin. PMID:11284525

Brett, A S; Philips, D; Lynn, A W

2001-03-01

15

Acetaminophen induced Steven Johnson syndrome-toxic epidermal necrolysis overlap.  

PubMed

Steven Johnson Syndrome and Toxic Epidermal Necrolysis are rare but severe form of hypersensitivity inflammatory reactions to multiple offending agents including drugs. Acetaminophen is extensively used due to its analgesic and anti-pyretic properties. It is rendered to be relatively safe, with hepatotoxicity considered to be the major adverse effect. However, very few cases of Steven Johnson Syndrome and Toxic Epidermal Necrolysis have been reported with acetaminophen usage in the past. We present the case of a 40 years old lady who developed an overlap of the two condition after taking several doses of acetaminophen for fever. She presented with widespread maculopapular rash, stinging in the eyes, oral mucosal ulcerations and high grade fever. She was successfully treated with corticosteroid therapy along with the supportive treatment. This case addresses the fact, that severe hypersensitivity reactions can occur with acetaminophen which can be potentially life threatening. PMID:22755330

Khawaja, Ali; Shahab, Ahmed; Hussain, Syed Ather

2012-05-01

16

Clinical profiles of Stevens-Johnson syndrome among Thai patients.  

PubMed

The objective of this study was to demonstrate the clinical profiles of Stevens-Johnson syndrome (SJS) in Thai patients, and to compare those clinical features between younger and older patients. Medical records of all patients with SJS who were admitted to Srinagarind Hospital Medical School, Khon Kaen, Thailand, from January 2002 to December 2014 were reviewed. Epidemiological features, etiologies, treatment and clinical outcomes were collected. There were 45 patients with SJS during the 10-year period. Females were the majority (57.8%) and the median age was 49 years. Hepatitis was the most frequent complication (67.5%). Phenytoin (15.6%), sulfonamide drugs (15.6%) and allopurinol (13.3%) were implicated as leading causes of SJS. Steroids were prescribed in 37 cases (82.2%). The mortality rate was 4.4%. Comparing older patients to younger patients, allopurinol appeared to be the main instigating drug to develop SJS with an odds ratio of 5.6 (95% confidence interval, 2.8-10.6). In conclusion, clinical features of Thai patients with SJS were similar to other reports. Allopurinol had the strongest association with SJS in older patients as compared to the younger ones. PMID:24815085

Limpawattana, Panita; Choonhakarn, Charoen; Kongbunkiat, Kannikar

2014-07-01

17

Acquired labial sinechiae and hydrocolpos secondary to Stevens-Johnson syndrome.  

PubMed

Stevens-Johnson syndrome frequently affects the genitalia. Vaginal problems have been recognized in female patients; however, reports about the problem and its treatment are rare. Labial sinechiae have not yet been reported as sequelae of Stevens-Johnson syndrome. Amenorrhea, cyclical abdominal pain, and a hypogastric mass in girls affected by Stevens-Johnson syndrome could indicate acquired vaginal obstruction. Extensive labial sinechiae in such patients can cause dysuria, urinary tract infection, and sexual dysfunction. After a diagnosis of Stevens-Johnson syndrome in girls, it is prudent to schedule a prepubertal genital examination to diagnose genital disease preemptively and avoid obstructed menstruation and future sexual problems. PMID:22921694

de Jesus, Lisieux Eyer; Dekermacher, Samuel; Manhães, Carolina R; Faria, Luciana M; Barros, Malena L

2012-10-01

18

Aceclofenac induced Stevens-Johnson/toxic epidermal necrolysis overlap syndrome.  

PubMed

The purpose of this paper is to report a rare occurrence of Stevens-Johnson/Toxic epidermal necrolysis (SJS/TEN) overlap syndrome after the use of aceclofenac. A 38 year old healthy adult male presented with rapidly evolving rash over face and upper body with ulceration of buccal mucosa and breathlessness after taking aceclofenac tablet. Naranjo score for this adverse drug event was six, thereby making it a probable adverse drug reaction. Despite aggressive fluid resuscitation and use of antihistamines and systemic steroids, patient's health rapidly worsened and died within six hours of presentation. Aceclofenac induced SJS/TEN overlap is an extremely rare clinical association previously reported only once in medical literature. To the best of our knowledge, this is the first case report of such an association in the Indian population. We are presenting this case to highlight the serious adverse reactions possible from a routinely prescribed drug. PMID:23662031

Ameen, Kaderthambi Hajamohideen Nooru; Pinninti, Rakesh; Jami, Swathi

2013-01-01

19

The ophthalmologic management of acute Stevens-Johnson syndrome.  

PubMed

Stevens-Johnson syndrome (SJS) and its more severe variant, toxic epidermal necrolysis (TEN), cause significant ocular surface damage. The majority of affected patients develop conjunctival inflammation during the acute phase of the disease. If intense, this inflammation yields permanent destruction of the normal mucosal tissue of the ocular surface and eyelids. Loss of the normal glandular structures leads to severe dry eye problems and vision loss. Medical treatments do little to arrest these problems, and repairing the damage after the acute phase is difficult, if not impossible. Recently, the application of cryopreserved amniotic membrane to the ocular surface during the acute phase of the disease has shown great promise in limiting the destructive inflammation and its long-term sequelae. A rationale for this treatment and a detailed description of the application procedure are provided, along with practical guidelines for the managing surgeon. PMID:18418506

Gregory, Darren G

2008-04-01

20

Mycoplasma pneumoniae and atypical Stevens-Johnson syndrome: a case series.  

PubMed

Mycoplasma pneumoniae is a common cause of community-acquired respiratory illness in the adolescent population. Stevens-Johnson syndrome is an extrapulmonary manifestation that has been associated with M. pneumoniae infections. Three adolescent males presented within a 1-month period with M. pneumoniae respiratory illnesses and severe mucositis but without the classic rash typical of Stevens-Johnson. Diagnosis was facilitated by the use of a polymerase chain reaction-based assay. This case series highlights the potential for M. pneumoniae-associated Stevens-Johnson syndrome to occur without rash and supports the use of polymerase chain reaction for early diagnosis. PMID:17353300

Ravin, Karen A; Rappaport, Lara D; Zuckerbraun, Noel S; Wadowsky, Robert M; Wald, Ellen R; Michaels, Marian M

2007-04-01

21

Oral Acetazolamide after Boston Keratoprosthesis in Stevens Johnson Syndrome  

PubMed Central

Background Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a rare but severe and sometimes fatal condition associated with exposure to medications; sulfamethoxazole is among the most common causes. We sought to address the safety of acetazolamide, a chemically related compound, in patients with prior SJS/TEN and glaucoma. A retrospective case series is described of patients at the Massachusetts Eye and Ear Infirmary who underwent keratoprosthesis surgery for corneal blindness from SJS/TEN, and later required oral acetazolamide for elevated intraocular pressure. Findings Over the last 10?years, 17 patients with SJS/TEN received a Boston keratoprosthesis. Of these, 11 developed elevated intraocular pressure that required administration of oral acetazolamide. One of 11 developed a mild allergic reaction, but no patient experienced a recurrence of SJS/TEN or any severe adverse reaction. Conclusion Although an increase in the rate of recurrent SJS/TEN due to oral acetazolamide would not necessarily be apparent after treating only 11 patients, in our series, acetazolamide administration was well tolerated without serious sequela. PMID:22546532

2012-01-01

22

[Stevens-Johnson syndrome in the literature and authors' own studies].  

PubMed

Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis (Lyell Syndrome) are severe, multisystem disease caused mainly by reaction to drugs. The clinical features include: changes on the skin and mucosa as well as lesions in the internal organs. There are no standards of treatment in this diesease group. There are ongoing trials in various groups of drugs. Apart from providing the appriopriate conditions and symptomatic treatment, immunoglobulins, cytostatics and plasmapheresis are used. The authors present the clinical course of a patient with Stevens-Johnsons Syndrome, probably caused by klaritromycin. The 13 y.o. boy was admitted to a Department of Paediatric Surgery specializing in treatment of burns. Immunoglobulin, cytostatics, antibacterial and antiviral drugs were used as well as topical medicines routinely applied in burns. The method proved to give good results. The treatment of Stevens-Johnsons Syndrome is difficult and expensive. Due to lack of standards, the treatment depends on one's own experience and up to date literature. PMID:19305034

Ch?ystowska, Marta; Pietruszka-Chmarra, Anna; Szafra?ski, Tomasz; Michalak, Jerzy

2008-01-01

23

Trans-ethnic study confirmed independent associations of HLA-A*02:06 and HLA-B*44:03 with cold medicine-related Stevens-Johnson syndrome with severe ocular surface complications  

PubMed Central

Stevens-Johnson syndrome (SJS) and its severe variant, toxic epidermal necrolysis (TEN), are acute inflammatory vesiculobullous reactions of the skin and mucous membranes. Cold medicines including non-steroidal anti-inflammatory drugs and multi-ingredient cold medications are reported to be important inciting drugs. Recently, we reported that cold medicine related SJS/TEN (CM-SJS/TEN) with severe mucosal involvement including severe ocular surface complications (SOC) is associated with HLA-A*02:06 and HLA-B*44:03 in the Japanese. In this study, to determine whether HLA-B*44:03 is a common risk factor for CM-SJS/TEN with SOC in different ethnic groups we used samples from Indian, Brazilian, and Korean patients with CM-SJS/TEN with SOC, and investigated the association between CM-SJS/TEN with SOC and HLA-B*44:03 and/or HLA-A*02:06. We found that HLA-B*44:03 was significantly associated with CM-SJS/TEN with SOC in the Indian and Brazilian but not the Korean population, and that HLA-A*02:06 might be weakly associated in the Korean- but not the Indian and Brazilian population. PMID:25099678

Ueta, Mayumi; Kannabiran, Chitra; Wakamatsu, Tais Hitomi; Kim, Mee Kum; Yoon, Kyung-Chul; Seo, Kyoung Yul; Joo, Choun-Ki; Sangwan, Virender; Rathi, Varsha; Basu, Sayan; Shamaila, Almas; Lee, Hyo Seok; Yoon, Sangchul; Sotozono, Chie; Gomes, Jose Alvaro Pereira; Tokunaga, Katsushi; Kinoshita, Shigeru

2014-01-01

24

Nevirapine-induced stevens johnson-syndrome and fulminant hepatic failure requiring liver transplantation.  

PubMed

We describe a case of nevirapine-induced Stevens-Johnson Syndrome (SJS) and fulminant hepatic failure (FHF) requiring liver transplantation. Five weeks prior to admission, a 57-year-old female with HIV infection had been switched to a nevirapine-based regimen of highly active antiretroviral therapy (HAART) with a CD4 cell count of 695/mm(3). Examination of the explanted native liver at initial transplantation revealed massive hepatic necrosis consistent with drug-induced liver injury. Primary graft nonfunction complicated the early postoperative course and liver retransplantation was required. On follow-up 2 years later, she remains in good health with an undetectable viral load on an efavirenz-based regimen of HAART. To our knowledge, this is the first report of successful liver transplantation following SJS and FHF. PMID:20642694

Jao, J; Sturdevant, M; del Rio Martin, J; Schiano, T; Fiel, M I; Huprikar, S

2010-07-01

25

Stevens Johnson syndrome after carbamazepine and SJS/TEN overlap syndrome after amoxicillin: case reports and a review  

PubMed Central

We present a case of Stevens Johnson syndrome in a child after carbamazepine application and Stevens Johnson/TEN overlap syndrome in an adult after amoxicillin application. On the basis of two reported cases we review the most commonly associated drugs, the postulated pathogenesis, clinical manifestation and management in these severe life-threatening diseases. We especially discuss the controversial systemic corticosteroid therapy. Topical care is also discussed. PMID:22371734

Gocki, Jacek; Placek, Waldemar; Zegarska, Barbara; Krause, Pawe?

2010-01-01

26

Drug-mediated rash: erythema multiforme versus Stevens-Johnson syndrome.  

PubMed

A 92-year-old woman presented with an acute onset generalised maculopapular rash with associated mucosal involvement, on a background of recent start of griseofulvin. The rash progressed rapidly over 2?days to involve most of her body, however, mucosal involvement was limited to her oral mucosa. Characteristic target lesions appeared at 72?h, and a diagnosis of erythema multiforme secondary to griseofulvin was made after further investigation and skin biopsy. The patient was monitored closely for progression of the rash and other indicators of more severe dermatological conditions such as Stevens-Johnson syndrome. She was managed symptomatically, with resolution of the rash in 4?weeks and full recovery to her premorbid level of functioning. This case details the diagnostic and management approach to erythema multiforme, a condition that warrants thorough consideration for the differential of Stevens-Johnson syndrome. PMID:25246464

Hidajat, Cassandra; Loi, Duncan

2014-01-01

27

Skin eruption with gabapentin in a patient with repeated AED-induced Stevens-Johnson's syndrome.  

PubMed

Skin eruptions have been reported with the use of all antiepileptic drugs and there is a significant risk of cross-reactivity between these agents in causing serious eruptions such as Stevens-Johnson's syndrome. Gabepentin is usually considered a safe agent for patients with a previous history of drug allergies and there have been no cases of skin eruption reported to the gabapentin post marketing surveillance. We report a patient who had severe Stevens-Johnson's syndrome induced by phenytoin and later by carbamazepine. Subsequent use of gabapentin also resulted in a skin eruption which was limited to the lower extremities but without systemic or mucosal involvement. This case suggests that patients with a strong history of drug-induced idiosyncratic reactions may experience such reactions to gabapentin as well. PMID:10051068

DeToledo, J C; Minagar, A; Lowe, M R; Ramsay, R E

1999-02-01

28

Steven's Johnson syndrome with toxic epidermal necrolysis due to thalidomide in a case of multiple myeloma  

PubMed Central

Thalidomide developed in 1954 for morning sickness had proven to be a teratogen and hence was withdrawn from market. Resurgence of thalidomide began as an immunomodulator when it was shown to be effective in the management of multiple myeloma and many conditions like erythema nodosum leprosum, graft versus host disease, recurrent aphthous ulcers etc. We report a case of Stevens Johnson syndrome-toxic epidermal necrolysis developing in an elderly male who was prescribed thalidomide after being diagnosed with multiple myeloma. PMID:25298592

Das, Anupam; Sil, Amrita; Mishra, Vivek; Das, Nilay Kanti

2014-01-01

29

Oral lesions associated with nevirapine-related Stevens Johnson syndrome: A report of four cases  

PubMed Central

Nevirapine is a non-nucleoside reverse transcriptase inhibitor, widely used in combination with other antiretroviral agents for treatment of HIV infection. Steven Johnson syndrome (SJS) is the major toxicity of nevirapine. We describe here four cases of SJS in HIV seropositive patients following nevirapine therapy. In all four cases cutaneous hypersensitivity reaction was seen with extreme oral lesions, three patients presented clinically with elevated liver enzymes and hepatitis, and two patients had ocular involvement. PMID:21731276

Balasundaram, S; Ranganathan, K; Umadevi, K; Gunaseelan, R; Kumaraswamy, N; Solomon, Sunithi; Devaleenol, Bella; Ambrose, Pradeep

2011-01-01

30

Oral lesions associated with nevirapine-related Stevens Johnson syndrome: A report of four cases.  

PubMed

Nevirapine is a non-nucleoside reverse transcriptase inhibitor, widely used in combination with other antiretroviral agents for treatment of HIV infection. Steven Johnson syndrome (SJS) is the major toxicity of nevirapine. We describe here four cases of SJS in HIV seropositive patients following nevirapine therapy. In all four cases cutaneous hypersensitivity reaction was seen with extreme oral lesions, three patients presented clinically with elevated liver enzymes and hepatitis, and two patients had ocular involvement. PMID:21731276

Balasundaram, S; Ranganathan, K; Umadevi, K; Gunaseelan, R; Kumaraswamy, N; Solomon, Sunithi; Devaleenol, Bella; Ambrose, Pradeep

2011-01-01

31

Evaluation of conjunctival bacterial flora in patients with Stevens-Johnson Syndrome  

PubMed Central

OBJECTIVE: To determine the conjunctival bacterial flora present in patients with Stevens-Johnson syndrome. METHODS: A prospective study of the conjunctival bacterial flora was performed in 41 eyes of 22 patients with Stevens-Johnson syndrome. The information gathered included the patient's sex and age, the duration of disease, the cause of Stevens-Johnson syndrome, and treatments. Scrapings of the inferior conjunctival fornix were performed in both eyes. Fourteen days before scraping, the patients were asked to interrupt all topical medication and start using 0.5% nonpreserved methylcellulose. The microbiological evaluation included microorganism identification and determination of antibiotic sensitivity. RESULTS: Of 22 patients (41 eyes), 14 (64%) were females, and eight (36%) were males. The mean age was 33.2 years, and the mean duration of disease was 15.6 years. Visual acuity ranged from light perception to 20/25 (1.57 logMar). The treatment received by most patients consisted of tear substitutes, topical antibiotics, and contact lenses. Bacterial identification was positive in 39 eyes (95%) and negative in two eyes (5%). Gram-positive cocci accounted for 55.5% of the microorganisms, whereas gram-positive bacilli and gram-negative bacilli accounted for 19% and 25.5%, respectively. Half of the patients (54%) had multiple bacterial species in their flora, and only one bacterial species was identified in the other half. Resistant bacteria were isolated from four eyes. The antibiotic sensitivity results for the Streptococcus group showed the lowest sensitivity and the highest microbial resistance identified. CONCLUSION: Patients with Stevens-Johnson syndrome have a diverse conjunctival flora that includes many pathogenic species. PMID:24626941

Frizon, Luciana; Araujo, Marilia Cavalcante; Andrade, Larissa; Yu, Maria Cecilia Zorat; Wakamatsu, Tais Hitomi; Hofling-Lima, Ana Luisa; Gomes, Jose Alvaro Pereira

2014-01-01

32

Rapid onset of Stevens-Johnson syndrome and toxic epidermal necrolysis after ingestion of acetaminophen.  

PubMed

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare, but life-threatening, severe cutaneous adverse reactions most frequently caused by exposure to drugs. Several reports have associated the use of acetaminophen with the risk of SJS or TEN. A typical interval from the beginning of drug therapy to the onset of an adverse reaction is 1-3 weeks. A 43-year-old woman and a 60-year-old man developed skin lesions within 3 days after administration of acetaminophen for a 3-day period. Rapid identification of the symptoms of SJS and TEN caused by ingestion of acetaminophen enabled prompt withdrawal of the culprit drug. After administration of intravenous immunoglobulin G, both patients recovered fully and were discharged. These two cases of rapidly developed SJS/TEN after ingestion of acetaminophen highlight the possibility that these complications can develop within only a few days following ingestion of over-the-counter medications such as acetaminophen. PMID:24527413

Kim, Eun-Jin; Lim, Hyun; Park, So Young; Kim, Sujeong; Yoon, Sun-Young; Bae, Yun-Jeong; Kwon, Hyouk-Soo; Cho, You Sook; Moon, Hee-Bom; Kim, Tae-Bum

2014-01-01

33

Combination of Steven-Johnson syndrome and neuroleptic malignant syndrome following carbamazepine therapy: a rare occurrence.  

PubMed

Stevens-Johnson syndrome (SJS) is a severe, episodic, acute mucocutaneous reaction that is most often elicited by drugs and occasionally by infections. The drugs commonly implicated as the cause of SJS are anticonvulsants, sulfonamides, non-steroidal anti-inflammatory drugs and antibiotics. Carbamazepine (CBZ) has been commonly implicated in SJS. Neuroleptic malignant syndrome (NMS) is a rare, life-threatening but potentially treatable condition. Among the neuroleptics, haloperidol (parenteral) is implicated as a most common drug for NMS. Though rare, association of NMS with CBZ and association of NMS with toxic epidermal necrolysis (TEN) in a single patient after administration of neuroleptics has been reported in the literature before. However, a combination of NMS and SJS in a single patient after administration of CBZ has not been reported so far. We present a patient with seizure who developed SJS and NMS following administration of CBZ. PMID:23761563

Sharma, Bhawna; Sannegowda, Raghavendra Bakki; Gandhi, Pankaj; Dubey, Parul; Panagariya, Ashok

2013-01-01

34

Review of Intravenous Immunoglobulin in the Treatment of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis  

PubMed Central

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare and serious cutaneous adverse reactions. There is controversy over the use of intravenous immunoglobulin (IVIG) in the treatment of SJS or TEN. The lack of randomized controlled trials to assess the benefits and risks of IVIG is due to its low prevalence and the high mortality rate associated with these cutaneous adverse reactions, especially in TEN. This article reviews published literature on case series that either supports or refutes the use of IVIG in the treatment of SJS or TEN. PMID:20967183

Momin, Saira B.

2009-01-01

35

Mycoplasma Pneumoniae Infections and Stevens-Johnson SyndromeReport of Eight Cases and Review of the Literature  

Microsoft Academic Search

On the basis of a literature review and eight cases of our own, we analyzed 37 cases of Mycoplasma pneumoniae (MP) infection and Stevens-Johnson syndrome (SJS). Our clinical and laboratory findings do not differ from those reported in the literature for MP infection with no exanthem or for SJS of various etiologies. Eighty percent of the children presented with symptoms

Maurice Levy; Neil H. Shear

1991-01-01

36

[Is intravenous immunoglobulin effective in toxic epidermal necrolysis and Stevens-Johnson syndrome?].  

PubMed

Toxic epidermal necrolysis and Stevens-Johnson syndrome are severe cutaneous adverse drug reactions. Intravenous immunoglobulin is described as a therapeutic option, however its use is still controversial. Using Epistemonikos database, which is maintained by screening over 20 databases, we identified six systematic reviews, including 39 primary studies. We combined the evidence using tables for summary of findings, following the GRADE approach, and concluded there is uncertainty about the effects of intravenous immunoglobulin because the certainty of the evidence is very low; it probably leads to important adverse effects; and has high cost. Intravenous immunoglobulin should not be used outside the context of a clinical trial, or only in cases where other treatments have failed and there are no resource constraints. PMID:25340656

Navajas, Lucas; Rada, Gabriel

2014-01-01

37

Trimethoprim-sulfamethoxazole-induced Steven Johnson syndrome in an HIV-infected patient.  

PubMed

Trimethoprim-sulfamethoxazole (TMP/SMX) is a widely prescribed antimicrobial for the management of several uncomplicated infections. It is commonly used for the treatment and prophylaxis of Pneumocystis jirovecii pneumonia (PCP) in the HIV-infected population. The adverse reaction to TMP/SMX is more frequent and severe in HIV-infected patients as compared to the general population. Here, we report a case of Stevens-Johnson syndrome (SJS) secondary to TMP/SMX. The patient had a generalized cutaneous reaction with involvement of the eyes, oral cavity, and genitals. He had elevated hepatic alanine aminotransferase and aspartate aminotransferase enzyme. TMP/SMX therapy was stopped and supportive treatment was started. His condition improved after eight days of stopping TMP/SMX therapy. PMID:23087524

Taqi, Syed Ahmed; Zaki, Syed Ahmed; Nilofer, Angadi Rajasab; Sami, Lateef Begum

2012-01-01

38

Using a claims database to investigate drug-induced Stevens-Johnson syndrome.  

PubMed

In order to explore a priori hypotheses about drug-induced Stevens-Johnson Syndrome (SJS), a case-control study was initiated using data from COMPASS, a computerized data base consisting of Medicaid claims data. The records of 3.8 million patients in five U.S. states were searched to identify patients with an inpatient diagnosis of ICD-9-CM code 695.1 (erythema multiforme-EM). Out of the total of 367 cases that were identified, primary medical records for 249 were sought and 128 (51.4 per cent) of these were obtained. The remainder could not be obtained because: in 36 (29.8 per cent) the hospital refused to provide medical records; in 33 (27.3 per cent) there were transcription errors; in 20 (16.5 per cent) the state could not translate the identification number, primarily because the patients lost Medicaid eligibility too long before our request; in 27 (22.3 per cent) the hospital could not locate the patient's record; and in 5 (4.1 per cent) there were other reasons. Of those with a medical record, 121 (94.5 per cent) had a skin diagnosis and 109 (85.2 per cent) had a diagnosis compatible with ICD-9-CM code 695.1 specified on their discharge summary. However, in 35 (27.3 per cent) an expert reviewer felt that the discharge diagnosis was incorrect. In 50 (39 per cent) the computer diagnosis was incorrect. Only 19 (14.8 per cent) were judged by the expert reviewer to truly have Stevens-Johnson Syndrome, and an additional 37 (28.9 per cent) were judged to have erythema multiforme minor. Thus, the computerized diagnosis agreed very well with the diagnoses specified on the discharge summary. However, EM is frequently misdiagnosed, ICD-9-CM code 695.1 contains multiple other diagnoses which are not EM, and much of hospitalized EM is EM minor. Thus, studies of SJS cannot be performed except in patients whose medical records are available. PMID:2057655

Strom, B L; Carson, J L; Halpern, A C; Schinnar, R; Snyder, E S; Stolley, P D; Shaw, M; Tilson, H H; Joseph, M; Dai, W S

1991-04-01

39

Effect of High-Dose Intravenous Immunoglobulin Therapy in Stevens-Johnson Syndrome: A Retrospective, Multicenter Study  

Microsoft Academic Search

Background:Stevens-Johnson syndrome (SJS) is a severe cutaneous drug reaction associated with considerable morbidity, possible transition to toxic epidermal necrolysis (TEN) and death in certain cases. Objective: To determine whether treatment with high-dose IVIG in SJS patients may improve outcome. Methods: Data from 12 patients (collected between January 1997 and November 2000 from7 university dermatology centers in Europe and North America)

Christa Prins; Carmela Vittorio; R. Steven Padilla; Thomas Hunziker; Peter Itin; John Förster; Eva-B. Bröcker; Jean-Hilaire Saurat; Lars E. French

2003-01-01

40

Clinical risk management of Stevens-Johnson syndrome/toxic epidermal necrolysis spectrum.  

PubMed

Clinical risk management concedes that risk is inherent to all health-care processes. Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but potentially life-threatening reactions to medications. Risk management should be considered prior to starting, during, and after therapy. Prior to starting therapy, risks that need to be assessed include any specific patient groups that may be at greater risk for the development of SJS/TEN. Gene testing is in place for Chinese and Thai patients who are going to be exposed to carbamazepine. During therapy, it is important to recognize SJS/TEN as a possible adverse drug reaction. Diagnostic criteria have changed, and more data exist on drugs with an increased risk. Although there is no standardized treatment for all patients with SJS/TEN, options that have been used include cyclosporine, corticosteroids, and intravenous immunoglobulin. Standards of care are usually defined locally, but new treatments, such as amniotic membrane support for ocular damage, may need to be considered. Good communication skills are needed to allow practitioners to show empathy and to provide disclosure. Risk management after a reaction includes skills in acknowledging bad outcomes or error; freedom to say "sorry" as defined by "apology laws," and knowing the rights provided by "Quality Assurance Conferences," where the information discussed is protected. In other words, the patient is best supported after an event like SJS/TEN if the practitioner is knowledgeable about optimal care standards and their legal rights and obligations. PMID:19845721

Knowles, Sandra; Shear, Neil H

2009-01-01

41

Stevens-Johnson syndrome / toxic epidermal necrolysis: an Asia-Pacific perspective  

PubMed Central

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe cutaneous adverse reactions (SCAR) to drugs which are associated with significant morbidity and mortality. High risk drugs in Asia are similar to those reported worldwide. Human leukocyte antigen (HLA)-related risk alleles for carbamazepine and allopurinol SCAR are unique to Asians. Although prognostic scoring systems like the SCORTEN have been used for more than a decade, pitfalls and caveats need to be recognized, in particular in patients with multiple medical co-morbidities and systemic features in SJS/TEN. In centres without a tertiary Burns Centre, SJS/TEN patients can still be managed successfully in general and dermatology wards with well-executed supportive/nursing care. Controversy remains regarding the effectiveness of immunomodulation in reducing SJS/TEN morbidity, mortality and hastening re-epithelialization. Despite paucity of robust evidence, intravenous immunoglobulins and ciclosporin remain the most commonly used modalities worldwide. Acute and long-term ocular effects are an important source of morbidity for which emerging ophthalmic therapies appear promising. Quality of life issues have now become an important outcome in patients with SJS/TEN as they often impact survivors' future attitudes towards pharmacotherapy. Even though pharmacogenetic testing for high-risk drugs appears to be the panacea for preventing carbamazepine- and allopurinol-induced SJS/TEN in ethnic Asians, many issues remain before health regulators in our region can conclusively determine whether testing should be made mandatory or highly recommended as standard of care. PMID:24260726

2013-01-01

42

Mucous membrane grafting for the post-Steven-Johnson syndrome symblepharon: a case report.  

PubMed

An 18-year-old woman was referred with late sequelae of chloroquine-induced Steven-Johnson syndrome. At the time of presentation, the symblepharon was involving the upper lids to almost the whole of the cornea, and part of the lower bulbar conjunctiva with the lower lid bilaterally. Other ocular examinations were not possible due to the symblepharon. B-scan ultrasonography revealed acoustically clear vitreous, normal chorioretinal thickness, and normal optic nerve head, with an attached retina. Conjunctivo-corneal adhesion released by superficial lamellar dissection of the cornea. Ocular surface reconstruction was carried out with a buccal mucous membrane. A bandage contact lens was placed over the cornea followed by the symblepharon ring to prevent further adhesion. The mucosal graft was well taken up along with corneal re-epithelization. Best corrected visual acuity of 20/120 in both sides after 1 month and 20/80 after 3 months was achieved and maintained till the 2.5-year follow-up. PMID:21586847

Das, Jayanta Kumar; Medhi, Jnanankar; Chakravarty, Ranjay; Soibam, Ronel

2011-01-01

43

Diagnosis, classification, and management of erythema multiforme and Stevens-Johnson syndrome  

PubMed Central

BACKGROUND—In adults, erythema multiforme (EM) is thought to be mainly related to herpes infection and Stevens-Johnson syndrome (SJS) to drug reactions.?AIMS—To investigate this hypothesis in children, and to review our experience in the management of these patients.?METHODS—A retrospective analysis of 77 paediatric cases of EM or SJS admitted to the Children's Hospital in Bordeaux between 1974and 1998.?RESULTS—Thirty five cases, inadequately documented or misdiagnosed mostly as urticarias or non-EM drug reactions were excluded. Among the remaining 42 patients (14 girls and 28 boys), 22 had EM (11EM minor and 11 EM major), 17 had SJS, and three had isolated mucous membrane involvement and were classified separately. Childhood EM was mostly related to herpes infection and SJS to infectious agents, especially Mycoplasma pneumoniae. Only two cases were firmly attributed to drugs (antibiotics). No patient died. EM and SJS sequelae were minor and steroids were of no overall benefit.?CONCLUSION—In paediatric practice EM is frequently misdiagnosed. The proposal that SJS is drug related in adults does not apply to children, and in our recruitment EM and SJS are mostly triggered by infectious agents. The course of both diseases, even though dramatic at onset, leads to low morbidity and mortality when appropriate symptomatic treatment is given.?? PMID:10999875

Leaute-Labreze, C; Lamireau, T; Chawki, D; Maleville, J; Taieb, A

2000-01-01

44

[Stevens-Johnson syndrome and toxic epidermal necrolysis--updates and innovations].  

PubMed

Stevens-Johnson Syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare severe cutaneous adverse reactions, usually induced by drugs. The reactions, which are characterized by extensive necrosis and detachment of the epidermis, followed by erosions of the skin and mucous membranes, are associated with high rates of mortality. There is growing evidence that SJS and TEN are a single disease with common causes and mechanisms. The present article summarizes recent updates and innovations related to the etiology, pathogenesis, genetic background, prognosis and treatment of these reactions. Among high-risk drugs associated with SJS/TEN, allopurinol is the most common cause of SJS/TEN in Europe and Israel. The prognosis of SJS/TEN can be predicted by a scoring system based on seven clinical and laboratory parameters. Founded on the genetic background of SJS/TEN, predictive tests can be used prior to starting high-risk medications. Treatment is still controversial, and further controlled studies are necessary. PMID:20684173

Halevy, Sima

2010-03-01

45

Modified tectonic keratoplasty with minimal corneal graft for corneal perforation in severe Stevens - Johnson syndrome: a case series study  

PubMed Central

Background Corneal perforation in severe Stevens-Johnson syndrome (SJS) presenting great therapeutic difficulties, the imperative corneal transplantation always result in graft failure and repeated recurrence of perforation. The aim of this study was to evaluate the effectiveness of a modified small tectonic keratoplasty (MSTK) with minimal corneal graft in the management of refractory corneal perforation in severe SJS. Methods Refractory corneal perforations in ten patients (10 eyes) with severe SJS were mended with a minimal corneal patch graft, under the guidance of anterior chamber optical coherence tomography, combined with conjunctival flap covering. The outcome measures included healing of the corneal perforation, survival of the corneal graft and conjunctival flap, relevant complications, and improvement in visual acuity. Results Corneal perforation healed, and global integrity was achieved in all eyes. No immune rejection or graft melting was detected. Retraction of conjunctival flap occurred in one eye, which was treated with additional procedure. Visual acuity improved in six eyes (60%), unchanged in three eyes (30%) and declined in one eye (10%). Conclusions The MSTK combined with conjunctival flap covering seems to be effective for refractory corneal perforation in severe SJS. PMID:25102918

2014-01-01

46

Stevens-Johnson Syndrome Associated with Drugs and Vaccines in Children: A Case-Control Study  

PubMed Central

Objective Stevens-Johnson Syndrome (SJS) is one of the most severe muco-cutaneous diseases and its occurrence is often attributed to drug use. The aim of the present study is to quantify the risk of SJS in association with drug and vaccine use in children. Methods A multicenter surveillance of children hospitalized through the emergency departments for acute conditions of interest is currently ongoing in Italy. Cases with a diagnosis of SJS were retrieved from all admissions. Parents were interviewed on child’s use of drugs and vaccines preceding the onset of symptoms that led to the hospitalization. We compared the use of drugs and vaccines in cases with the corresponding use in a control group of children hospitalized for acute neurological conditions. Results Twenty-nine children with a diagnosis of SJS and 1,362 with neurological disorders were hospitalized between 1st November 1999 and 31st October 2012. Cases were more frequently exposed to drugs (79% vs 58% in the control group; adjusted OR 2.4; 95% CI 1.0–6.1). Anticonvulsants presented the highest adjusted OR: 26.8 (95% CI 8.4–86.0). Significantly elevated risks were also estimated for antibiotics use (adjusted OR 3.3; 95% CI 1.5–7.2), corticosteroids (adjusted OR 4.2; 95% CI 1.8–9.9) and paracetamol (adjusted OR 3.2; 95% CI 1.5–6.9). No increased risk was estimated for vaccines (adjusted OR: 0.9; 95% CI 0.3–2.8). Discussion Our study provides additional evidence on the etiologic role of drugs and vaccines in the occurrence of SJS in children. PMID:23874553

Raucci, Umberto; Rossi, Rossella; Da Cas, Roberto; Rafaniello, Concita; Mores, Nadia; Bersani, Giulia; Reale, Antonino; Pirozzi, Nicola; Menniti-Ippolito, Francesca; Traversa, Giuseppe; in Drug and Children, Italian Multicenter Study Group for Vaccine Safety

2013-01-01

47

Stevens-Johnson syndrome and toxic epidermal necrolysis: efficacy of intravenous immunoglobulin and a review of treatment options.  

PubMed

Toxic epidermal necrolysis (TEN) is a rare, severe adverse drug reaction. Steven-Johnson syndrome (SJS) represents the milder end of the spectrum. The exact pathogenesis of TEN and SJS is still unknown and many drugs, including prednisolone, cyclosporin and intravenous immunoglobulin (IVIG), have been used in an attempt to halt the disease process. The use of IVIG in particular is controversial. We share our experience with the use of IVIG in six patients with TEN. We will also review the various proposed mechanisms underlying TEN, the mechanism of action of IVIG in TEN and summarise useful treatment options. PMID:19224081

Teo, L; Tay, Y K; Liu, T T; Kwok, C

2009-01-01

48

Oral lesions associated with Nevirapine-induced Stevens-Johnson syndrome and toxic epidermal necrolysis: A report of 10 cases.  

PubMed

Stevens-Johnson Syndrome (SJS) and toxic epidermal necrolysis (TEN) are closely related severe, acute mucocutaneous reactions usually caused by drugs. They are acute life-threatening conditions and cause widespread necrosis of the epithelium. There is persistence of a high risk of SJS or TEN in relation to human immunodeficiency virus (HIV) infection associated with exposure to nevirapine (NVP). In this article, we present nine cases of SJS and one case of TEN in HIV-seropositive individuals who developed cutaneous, oral, ocular and genital lesions while being treated with NVP. PMID:24574667

Reddy, Ramana Bv; Shekar, P Chandra; Chandra, K Lalith Prakash; Aravind, Rs

2013-09-01

49

Stevens-Johnson syndrome developing in a girl with systemic lupus erythematosus on high-dose corticosteroid therapy.  

PubMed

Stevens-Johnson syndrome (SJS) is a vesiculobullous disease of the skin and mucosa. This generalized hypersensitivity reaction is well known to occur in association with certain drugs, particularly sulfonamides, nonsteroidal anti-inflammatory agents (NSAIDs), and anticonvulsants. The disease is usually seen in children and young adults and is often treated with corticosteroids. We present a 9-year-old girl with lupus nephritis who developed cutaneous bullae and mucositis while being treated with intravenous methylprednisolone. The initial differential diagnosis included bullous lupus, but skin biopsy specimen findings supported a diagnosis of SJS. She was treated with intravenous immunoglobulin (IVIg). PMID:11860572

Samimi, Sarah S; Siegfried, Elaine

2002-01-01

50

Amniotic Membrane Transplantation as a New Therapy for the Acute Ocular Manifestations of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis  

PubMed Central

Stevens-Johnson syndrome and its more severe variant, toxic epidermal necrolysis, have relatively low overall incidence; however, this disease presents with high morbidity and mortality. The majority of patients develop ocular inflammation and ulceration at the acute stage. Due to the hidden nature of these ocular lesions and the concentration of effort toward life-threatening issues, current acute management has not devised a strategy to preclude blinding cicatricial complications. This review summarizes recent literature data, showing how sight-threatening corneal complications can progressively develop from cicatricial pathologies of lid margin, tarsus, and fornix at the chronic stage. It illustrates how such pathologies can be prevented with the early intervention of cryopreserved amniotic membrane transplantation to suppress inflammation and promote epithelial healing at the acute stage. Significant dry eye problems and photophobia can also be avoided with this intervention. This new therapeutic strategy can avert the catastrophic ophthalmic sequelae of this rare but devastating disease. PMID:19699503

Shay, Elizabeth; Kheirkhah, Ahmad; Liang, Lingyi; Sheha, Hossam; Gregory, Darren G.; Tseng, Scheffer C.G.

2010-01-01

51

Boston type 1 based keratoprosthesis (Auro Kpro) and its modification (LVP Kpro) in chronic Stevens Johnson syndrome.  

PubMed

Two young women with completely dry and keratinised eyes post-Stevens Johnson Syndrome (SJS) underwent unilateral oral mucous membrane grafts (MMGs) in preparation for modified osteo-odonto keratoprosthesis (MOOKP) implantation. In both cases, the mucosal graft was deemed to be too tight to accommodate the MOOKP implant. Instead of proceeding with MOOKP, the first patient underwent Auro Kpro (Boston type 1-based keratoprosthesis) implantation under the MMG, while the second patient underwent implantation of a modification of Auro Kpro with a longer optical stem (LVP Kpro) exposed through the MMG. Both patients maintained a visual acuity of 20/20, N6 at 15 months post-implantation. The first patient needed repeated mucosal trimming because of mucosal overgrowth; while in the second patient, mucosal overgrowth did not occur. This report highlights the innovative and successful use of Boston type 1-based keratoprosthesis (Auro Kpro) and its modification (LVP Kpro) in completely dry and keratinised post-SJS eyes. PMID:24663249

Basu, Sayan; Sureka, Shraddha; Shukla, Rashmi; Sangwan, Virender

2014-01-01

52

Bronchiolitis obliterans associated with Stevens-Johnson Syndrome: histopathological bronchial reconstruction of the whole lung and immunohistochemical study  

PubMed Central

This study presents an extremely rare case of constrictive bronchiolitis obliterans (BO) associated with Stevens-Johnson Syndrome (SJS) provides the morphological and immunohistochemical features using histopathological bronchial reconstruction technique. A 27-year-old female developed progressive dyspnea after SJS induced by taking amoxicillin at the age of 10. Finally, she died of exacerbation of type II respiratory failure after 17 years from clinically diagnosed as having BO. Macroscopic bronchial reconstruction of the whole lungs at autopsy showed the beginning of bronchial obliterations was in the 4th to 5th branches, numbering from each segmental bronchus. Once they were obliterated, the distal and proximal bronchi were dilated. Microscopic bronchial reconstruction demonstrated the localization of obliteration was mainly from small bronchi to membranous bronchioli with intermittent airway luminal narrowing or obliteration. Moreover, CD3-, CD20-, and CD68-positive cells were found in the BO lesions. CD34- and D2-40-positive cells were mainly distributed in the peribronchiolar lesions and bronchiolar lumens, respectively. SMA- and TGF-?-positive cells were seen in the fibrous tissue of BO lesions. The virtual slides The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1071703140102601. PMID:23919759

2013-01-01

53

Causes and Treatment Outcomes of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in 82 Adult Patients  

PubMed Central

Background/Aims Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are predominantly known as medication-induced diseases. However, at our institution, we have experienced more cases of non-drug-related SJS and TEN than expected. Therefore, we studied the difference between non-drug-related and drug-related SJS and TEN in terms of clinical characteristics and prognoses. Methods The etiologies, clinical characteristics, and treatment outcomes for 82 adult patients with SJS and TEN were retrospectively reviewed. Results A total of 71 patients (86.6%) were classified as having SJS, and the other 11 patients (13.4%) were classified as having TEN. Drug-related cases were more common (43, 52.4%) than non-drug-related cases (39, 47.6%). Anticonvulsants (12/82, 14.6%) and antibiotics (9/82, 11%) were the most common causative medications. Anemia (p = 0.017) and C-reactive protein of ? 5 mg/dL (p = 0.026) were more common in the drug-related cases than in the non-drug-related cases. Intravenous steroid therapy was used as the main treatment regimen (70/82, 85.4%). Of the 82 patients, 8 (9.8%) died during the clinical course. A univariate analysis for mortality showed statistical significance for the following: kidney function abnormality, pneumonia, hemoglobin of < 10 g/dL, and combined underlying diseases. In a multivariate analysis, only pneumonia was statistically significant (odds ratio, 25.79; p = 0.009). Conclusions Drugs were the most frequent cause of these diseases. However, non-drug-related causes also contributed to a significant proportion of cases. Physicians should keep this in mind when documenting patient history. In addition, early recognition and treatment may be important for better outcomes. PMID:22707893

Kim, Hye-In; Park, Ga-Young; Kwon, Eu-Gene; Kim, Hyo-Hoon; Jeong, Ju-Young; Chang, Hyun-Ha; Lee, Jong-Myung; Kim, Neung-Su

2012-01-01

54

Spontaneous Bilateral Corneal Perforation in Stevens- Johnsons Syndrome-A Challenge in Management  

PubMed Central

A 42-year-old man from Ghana presented with bilateral painful corneal perforations following ingestion of a sulphur-based antibiotic. Emergency bilateral penetrating keratoplasty was performed, with restoration of globe integrity. However, surgical complications arose such as non-healing epithelial defect, secondary infection, graft dehiscence, and mounting intraocular pressure. This case illustrates the challenges faced in managing corneal grafts in patients with already compromised ocular surfaces. PMID:23785259

Md Noh, Umi Kalthum; Then, Kong Yong

2013-01-01

55

Spontaneous bilateral corneal perforation in stevens- johnsons syndrome-a challenge in management.  

PubMed

A 42-year-old man from Ghana presented with bilateral painful corneal perforations following ingestion of a sulphur-based antibiotic. Emergency bilateral penetrating keratoplasty was performed, with restoration of globe integrity. However, surgical complications arose such as non-healing epithelial defect, secondary infection, graft dehiscence, and mounting intraocular pressure. This case illustrates the challenges faced in managing corneal grafts in patients with already compromised ocular surfaces. PMID:23785259

Md Noh, Umi Kalthum; Then, Kong Yong

2013-01-01

56

Elevation of Conjunctival Epithelial CD45INTCD11b+CD16+CD14? Neutrophils inOcular Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis  

PubMed Central

Purpose. Ocular complications related to Stevens-Johnson Syndrome (SJS)–Toxic Epidermal Necrolysis (TEN) may persist and progress after resolution of systemic disease. This is thought to be related in part to persistent ocular innate-immune signaling. In this study, our aim was to characterize infiltrative conjunctival cellular profiles during acute (<12 months) and chronic (>12 months) disease. Methods. Consecutive patients presenting with SJS-TEN over a 12-month period were followed for 1 year. Detailed clinical examination and conjunctival impression cell recovery was analyzed by flow cytometry for the presence of intraepithelial leukocytes and compared with healthy controls (n = 21). Results. Ten patients were recruited of whom six had acute disease and five were classified as TEN (SCORTEN = 1, n = 4). Conjunctival inflammation was graded as absent/mild in a total of nine patients; but despite this, evidence of fornix shrinkage was observed in nine subjects. This inversely correlated with disease duration (P < 0.05). A reduction in percentage of CD8??+ T cells compared with controls (80% vs. 57%; P < 0.01) was associated with a corresponding increase in the number/percentage of CD45INTCD11b+CD16+CD14? neutrophils (186 vs. 3.4, P < 0.01, 31% vs. 0.8%, P < 0.001). Neutrophils inversely correlated with disease duration (r = ?0.71, P = 0.03), yet there was no absolute change in the CD8??+ or neutrophil populations during the study period (P = 1.0). Conclusions. These data highlight that a neutrophilic infiltrate is present in mildly inflamed or clinically quiescent conjunctival mucosa in patients with ocular SJS-TEN, where neutrophil numbers inversely correlate with disease duration. Neutrophil persistence endorses the hypothesis of an unresolved innate-inflammatory process that might account for disease progression. PMID:23737478

Williams, Geraint P.; Tomlins, Paul J.; Denniston, Alastair K.; Southworth, H. Susan; Sreekantham, Sreekanth; Curnow, S. John; Rauz, Saaeha

2013-01-01

57

Retrospective Analysis of Corticosteroid Treatment in Stevens-Johnson Syndrome and/or Toxic Epidermal Necrolysis over a Period of 10 Years in Vajira Hospital, Navamindradhiraj University, Bangkok  

PubMed Central

Background. Stevens-Johnson syndrome (SJS) and/or toxic epidermal necrolysis (TEN) are uncommon and life-threatening drug reaction associated with a high morbidity and mortality. Objective. We studied SJS and/or TEN by conducting a retrospective analysis of 87 patients treated during a 10-year period. Methods. We conducted a retrospective review of the records of all patients with a diagnosis of SJS and/or TEN based on clinical features and histological confirmation of SJS and/or TEN was not available at the Department of Medicine, Vajira hospital, Bangkok, Thailand. The data were collected from two groups from 2003 to 2007 and 2008 to 2012. Results. A total of 87 cases of SJS and/or TEN were found, comprising 44 males and 43 females whose mean age was 46.5 years. The average length of stay was 17 days. Antibiotics, anticonvulsants, and allopurinol were the major culprit drugs in both groups. The mean SCORTEN on admission was 2.1 in first the group while 1.7 in second the group. From 2008 to 2012, thirty-nine patients (76.5%) were treated with corticosteroids while only eight patients (22.2%) were treated between 2003 and 2007. The mortality rate declined from 25% from the first group to 13.7% in the second group. Complications between first and second groups had no significant differences. Conclusions. Short-term corticosteroids may contribute to a reduced mortality rate in SJS and/or TEN without increasing secondary infection. Further well-designed studies are required to compare the effect of corticosteroids treatment for SJS and/or TEN. PMID:25024697

Prompongsa, Sirikarn

2014-01-01

58

Serum Levels of the Th1 Promoter IL12 and the Th2 Chemokine TARC Are Elevated in Erythema Multiforme and Stevens-Johnson Syndrome\\/Toxic Epidermal Necrolysis and Correlate with Soluble Fas Ligand Expression  

Microsoft Academic Search

Background: No data exist as to Th2 chemokines in erythema multiforme (EM) and Stevens-Johnson syndrome (SJS)\\/toxic epidermal necrolysis (TEN). Objective: To evaluate thymus- and activation-regulated chemokine (TARC), macrophage-derived chemokine (MDC) and regulated upon activation, normal T-lymphocyte-expressed and secreted chemokine (RANTES) expression in EM and SJS\\/TEN and to correlate with the serum levels of the Th1 promoter interleukin (IL)-12 and soluble

Pietro Quaglino; Marzia Caproni; Emiliano Antiga; Elena Del Bianco; Simona Osella-Abate; Paola Savoia; Alessandra Frezzolini; Donatella Schena; Angelo Marzano; Walter Volpi; Clara De Simone; Aurora Parodi; Paolo Fabbri; Maria Grazia Bernengo

2007-01-01

59

Association of HLA-B*5801 allele and allopurinol-induced stevens johnson syndrome and toxic epidermal necrolysis: a systematic review and meta-analysis  

PubMed Central

Background Despite some studies suggesting a possible association between human leukocyte antigen, HLA-B*5801 and allopurinol induced Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN), the evidence of association and its magnitude remain inconclusive. This study aims to systematically review and meta-analyze the association between HLA-B*5801 allele and allopurinol-induced SJS/TEN. Methods A comprehensive search was performed in databases including MEDLINE, Pre-MEDLINE, Cochrane Library, EMBASE, International Pharmaceutical Abstracts (IPA), CINAHL, PsychInfo, the WHO International, Clinical Trial Registry, and ClinicalTrial.gov from their inceptions to June 2011. Only studies investigating association between HLA-B*5801 with allopurinol-induced SJS/TEN were included. All studies were extracted by two independent authors. The primary analysis was the carrier frequency of HLA-B*5801 comparison between allopurinol-induced SJS/TEN cases and each comparative group. The pooled odds ratios were calculated using a random effect model. Results A total of 4 studies with 55 SJS/TEN cases and 678 matched-controls (allopurinol-tolerant control) was identified, while 5 studies with 69 SJS/TEN cases and 3378 population-controls (general population) were found. SJS/TEN cases were found to be significantly associated with HLA-B*5801 allele in both groups of studies with matched-control (OR 96.60, 95%CI 24.49-381.00, p < 0.001) and population-control (OR 79.28, 95%CI 41.51-151.35, p < 0.001). Subgroup analysis for Asian and Non-Asian population yielded similar findings. Conclusion We found a strong and significant association between HLA-B*5801 and allopurinol-induced SJS/TEN. Therefore, HLA-B*5801 allele screening may be considered in patients who will be treated with allopurinol. PMID:21906289

2011-01-01

60

Association of HLA-B*1502 and *1511 allele with antiepileptic drug-induced Stevens-Johnson syndrome in central China.  

PubMed

Previous studies have demonstrated a strong association between carbamazepine (CBZ)-induced Stevens-Johnson syndrome (SJS) and HLA-B*1502 in Han Chinese. Here, we extended the study of HLA-B*1502 susceptibility to two different antiepileptic drugs, oxcarbazepine (OXC) and phenobarbital (PB). In addition, we genotyped HLA-B*1511 in a case of CBZ-induced SJS with genotype negative for HLA-B*1502. The presence of HLA-B*1502 was determined using polymerase chain reaction with sequence-specific primers (PCR-SSP). Moreover, we genotyped HLA-B*1502 in 17 cases of antiepileptic drugs (AEDs)-induced cutaneous adverse drug reactions (cADRs), in comparison with AEDs-tolerant (n=32) and normal controls (n=38) in the central region of China. The data showed that HLA-B*1502 was positive in 5 of 6 cases of AEDs-induced SJS (4 CBZ, 1 OXC and 1 PB), which was significantly more frequent than AEDs-tolerant (2/32, 18 CBZ, 6 PB and 8 OXC) and normal controls (3/38). Compared with AEDs-tolerant and normal controls, the OR for patients carrying the HLA-B*1502 with AEDs-induced SJS was 6.25 (95% CI: 1.06-36.74) and 4.86 (95% CI: 1.01-23.47). The sensitivity and specificity of HLA-B*1502 for prediction of AEDs-induced SJS were 71.4%. The sensitivity and specificity of HLA-B*1502 for prediction of CBZ-induced SJS were 60% and 94%. HLA-B*1502 was not found in 11 children with maculopapular exanthema (MPE) (n=9) and hypersensitivity syndrome (HSS) (n=2). However, we also found one case of CBZ-induced SJS who was negative for HLA-B*1502 but carried HLA-B*1511. It was suggested that the association between the CBZ-induced SJS and HLA-B*1502 allele in Han Chinese children can extend to other aromatic AEDs including OXC and PB related SJS. HLA-B*1511 may be a risk factor for some patients with CBZ-induced SJS negative for HLA-B*1502. PMID:24496695

Sun, Dan; Yu, Chun-hua; Liu, Zhi-sheng; He, Xue-lian; Hu, Jia-sheng; Wu, Ge-fei; Mao, Bing; Wu, Shu-hua; Xiang, Hui-hui

2014-02-01

61

Effect of Age and Early Intervention with a Systemic Steroid, Intravenous Immunoglobulin or Amniotic Membrane Transplantation on the Ocular Outcomes of Patients with Stevens-Johnson Syndrome  

PubMed Central

Purpose This retrospective observational case series of fifty-one consecutive patients referred to the eye clinic with acute-stage Stevens-Johnson syndrome (SJS) or toxic epidermal necrolysis (TEN) from 1995 to 2011 examines the effect of early treatment with a systemic corticosteroid or intravenous immunoglobulin (IVIG) on the ocular outcomes in patients with SJS or TEN. Methods All patients were classified by age (?18 years vs. >18 years) and analyzed by treatment modality and early intervention with systemic corticosteroids (?5 days), IVIG (?6 days), or amniotic membrane graft transplantation (AMT) (?15 days). The main outcomes were best-corrected visual acuity (BCVA) in logarithm of the minimum angle of resolution (logMAR) and ocular involvement scores (OIS, 0-12), which were calculated based on the presence of superficial punctate keratitis, epithelial defect, conjunctivalization, neovascularization, corneal opacity, keratinization, hyperemia, symblepharon, trichiasis, mucocutaneous junction involvement, meibomian gland involvement, and punctal damage. Results The mean logMAR and OIS scores at the initial visit were not significantly different in the pediatric group (logMAR = 0.44, OIS = 2.76, n = 17) or the adult group (logMAR = 0.60, OIS = 2.21, n = 34). At the final follow-up, the logMAR and OIS had improved significantly in the adult group (p = 0.0002, p = 0.023, respectively), but not in the pediatric group. Early intervention with IVIG or corticosteroids significantly improved the mean BCVA and OIS in the adult group (p = 0.043 and p = 0.024, respectively for IVIG; p = 0.002 and p = 0.034, respectively for corticosteroid). AMT was found to be associated with a significantly improved BCVA or OIS in the late treatment group or the group with a better initial OIS (p = 0.043 and p = 0.043, respectively for BCVA; p = 0.042 and p = 0.041, respectively for OIS). Conclusions Our findings suggest that patients with SJS or TEN who are aged 18 years or less have poorer ocular outcomes than older patients and that early treatment with steroid or immunoglobulin therapy improves ocular outcomes. PMID:24082770

Kim, Kyeong Hwan; Park, Sung Wook; Wee, Won Ryang

2013-01-01

62

HLA-B*15:02 association with carbamazepine-induced Stevens-Johnson syndrome and toxic epidermal necrolysis in an Indian population: a pooled-data analysis and meta-analysis.  

PubMed

This study aimed to investigate the prevalence and association of HLA-B*15:02 with carbamazepine-induced Stevens-Johnson syndrome and toxic epidermal necrolysis (CBZ-SJS/TEN) in the Indian population in Malaysia, which mostly originated from Southern India. HLA-B alleles in five Indian case patients with CBZ-SJS/TEN and 52 CBZ-tolerant controls, and followed by a pooled sample of seven cases from two centers in Malaysia were analyzed. Positive association for HLA-B*15:02 with CBZ-SJS/TEN was detected in Indians (40% [2/5] vs. 3.8% [2/52], odds ratio [OR] 16.7, p = 0.0349), of which 80% (4/5) of the Indian patients originated from Southern India. A pooled sample of seven cases showed stronger association between HLA-B*15:02 and CBZ-SJS/TEN (57.1% [4/7] vs. 3.8% [2/52], OR 33.3, 95% confidence interval [CI] 4.25-162.21, p = 1.05 × 10(-3) ). Subsequent meta-analysis on Indians from Malaysia and India further demonstrated a significant and strong association between HLA-B*15:02 and CBZ-SJS/TEN (OR 38.54; 95% CI 6.83-217.34, p < 1.0 × 10(-4) ). Our study is the first on Indians predominantly from Southern India that demonstrated HLA-B*15:02 as a strong risk factor for CBZ-SJS/TEN despite a low population allele frequency. This stressed the importance of testing for HLA-B*15:02, irrespective of the ancestral background, including populations with low allele frequency. PMID:25266342

Khor, Amy Hui-Ping; Lim, Kheng-Seang; Tan, Chong-Tin; Wong, Su-Ming; Ng, Ching-Ching

2014-11-01

63

Where Good Ideas Come From: The Natural History of Innovation, Steven Johnson, Riverhead Hardcover, 2010.  

E-print Network

and the Neuroscience of Everyday Life, Steven Johnson, Scribner, 2004. Everything Bad is Good For You, Steven Johnson, Riverhead Books, 2005. Steven Johnson . [Mind Wide Open] ( ?) , ( , , , ) . . . [Everything Bad is Good For You] , TV , , , . . . 03/24/2013 2 #12;· On Human Nature, Edward O.Wilson, Harvard University Press

64

Innovation Squared: Comparison of Models by Tony Wagner and Steven Johnson  

ERIC Educational Resources Information Center

On the surface, Tony Wagner's model of innovation differs from Steven Johnson's. One explores the following: how might we develop a nation of innovators? The other offers seven patterns that mark environments for innovation. Drawing from triangulated data, both authors create regularities (not laws) that have new paradigm, scientific credibility.…

Fluellen, Jerry E., Jr.

2012-01-01

65

Severe idiosyncratic drug reaction (Lyells syndrome) after ingesting dihydroartemisinin.  

PubMed

Lyells syndrome also called Toxic epidermal necrolysis is the extreme form of idiosyncratic drug reaction that is called Steven Johnsons Syndrome: The condition results in an extensive loss of the skin with mucous membrane involvement. Lyells syndrome has been induced by many agents. The commonest agent in the literature being sulphonamides. However, in our search of the medical literature there was no report of dihydroarthemisinin as a cause of Lyells syndrome. We report three patients seen at two tertiary health institutions with Lyells syndrome after treatment for malaria with dihydroarthemisinin. This resulted from administration of dihydroarthemisinin with chloroquine in two patients and dihydroarthemisinin with Amodiaquine in one patient. The first patient was a seven year old child who developed 90% cutaneous involvement and died from hemorrhagic shock. The second was a 28 old female that developed a 76% body surface involvement and died from septicemia. The third patient was a pregnant 37 year old woman that developed 52% body involvement and died from septic shock. In these patients the earliest symptoms were not recognized and there was considerable delay before referral. In view of the recent WHO recommendation ofArthemisinin Combination Treatment (ACT) for malaria, we expect more cases of Steven Johnson Syndrome and Lyells syndrome from ACT treatment. The aim of this report is to raise the awareness of clinicians to this potentially fatal complication. PMID:19764682

Ugburo, A O; Ilombu, C A; Temiye, E O; Fadeyibi, I O; Akinolai, O I

2009-06-01

66

Chlorhexidine gluconate-impregnated central-line dressings and necrosis in complicated skin disorder patients.  

PubMed

Although chlorhexidine gluconate (CHG) disks have been shown to help reduce the incidence of central line-associated blood stream infections, their use can result in local skin necrosis. The effects of CHG disks on patients with complex skin pathology have not been studied. We report 6 cases of dermal necrosis associated with Biopatch (Ethicon Inc, Somerville, NJ) CHG disks in adults with complex skin pathology including those with Stevens-Johnson syndrome, toxic epidermal necrolysis syndrome, graft-versus-host disease, burns, and anasarca. All patients had a CHG disk placed at a central venous catheter insertion site. Age range was from 21 to 84 years. Discovery of the reaction ranged from 4 to 14 days after disk placement. Resultant skin erosions required 2 to 10 weeks to reepithelialize. Complicated skin disorder patients represent a rare subset of the critically ill who appear prone to CHG disk necrosis. Continuous contact of CHG under occlusive dressings is speculated to predispose Stevens-Johnson syndrome, toxic epidermal necrolysis syndrome, graft-versus-host disease, and burn patients to local chemical injury secondary to loss of the epithelial tissue barrier, decreased cohesion of the epidermal-dermal junction, and increased tissue permeability. In these patients, the risk of placing the CHG disk may present more risk than using alternative antimicrobial dressings. PMID:25035049

Wall, Jennifer B; Divito, Sherrie J; Talbot, Simon G

2014-12-01

67

Progeria syndrome with cardiac complications.  

PubMed

A case report of 6-year-old boy with progeria syndrome, with marked cardiac complications is presented. The boy had cardiorespiratory failure. Discoloured purpuric skin patches, alopecia, prominent forehead, protuberant eyes, flattened nasal cartilage, malformed mandible, hypodentition, and deformed rigid fingers and toes were observed on examination. The boy was unable to speak. A sclerotic systolic murmur was audible over the mitral and aortic areas. Chest x-rays showed cardiac enlargement and the electrocardiogram (ECG) showed giant peaked P waves (right atrial hypertrophy) and right ventricular hypertrophy. Atherosclerotic dilated ascending aorta, thickened sclerotic aortic, mitral, and tricuspid valves with increased echo texture, left and right atrial and right ventricular dilatation, reduced left ventricular cavity, and thickened speckled atrial and ventricular septa were observed on echocardiography. PMID:24601202

Ilyas, Saadia; Ilyas, Hajira; Hameed, Abdul; Ilyas, Muhammad

2013-09-01

68

[Managing complications in intraoperative floppy iris syndrome].  

PubMed

The intraoperative floppy iris syndrome (IFIS) describes an ophthalmologically relevant phenomenon which is observed after systemic intake of alpha blockers for treatment of benign prostate hyperplasia. This leads to an increase in intraoperative complications in cataract surgery characterized by a flaccid iris which billows in response to currents with a tendency to prolapse towards the area of surgery. This results in damage to the iris by the instruments used or posterior capsule rupture with loss of lens material. We describe the preoperative and intraoperative measures and techniques to deal with this challenging situation in order to minimize development of IFIS and reduce the complication rate. PMID:23338529

Handzel, D M; Rausch, S; Kälble, T; Briesen, S

2013-04-01

69

Neurosurgical management for complicated catastrophic antiphospholipid syndrome.  

PubMed

Antiphospholipid syndrome (APS) is an autoimmune condition involving arterial and venous thrombosis. An unusual APS variant, catastrophic antiphospholipid syndrome (CAPS), includes rapid multi-organ failure from widespread small vessel thrombosis. Central nervous system complications arise in one-third of CAPS patients. In rare cases, CAPS co-manifests with cerebellar hemorrhage presenting a neurosurgical emergency. We present a 65-year-old woman with CAPS-related cerebellar hematoma, co-morbid idiopathic thrombocytopenic purpura, deep vein thrombosis and altered mental status, with treatment complicated by thrombocytopenia. The patient suddenly deteriorated, secondary to a cerebellar subdural hematoma, and underwent decompression and excision of the hematoma. After recovery in the intensive care unit, she developed a new spontaneous epidural hematoma requiring additional surgery. Management of these patients is hematologically complex and often requires a multi-disciplinary team of physicians. This patient provides an important learning point for clinicians - consider CAPS when hemorrhage and thrombosis are present. PMID:24269552

Drazin, Doniel; Westley Phillips, H; Shirzadi, Ali; Drazin, Noam; Schievink, Wouter

2014-04-01

70

Use of Cross-linked Donor Corneas as Carriers for the Boston Keratoprosthesis  

ClinicalTrials.gov

Chemical Injuries; Unspecified Complication of Corneal Transplant; Autoimmune Diseases; Ocular Cicatricial Pemphigoid; Stevens Johnson Syndrome; Lupus Erythematosus, Systemic; Rheumatoid Arthritis; Other Autoimmune Diseases

2013-03-20

71

[Epileptic seizures complicated by Takotsubo syndrome].  

PubMed

INTRODUCTION. Takotsubo syndrome is a disorder characterised by a reversible ventricular dysfunction, angina-like precordial pain and electromyographic changes with no evidence of coronary obstruction in examinations performed by coronary catheterisation. It is triggered by stress and is frequent following bouts of epileptic seizures. We report the case of a patient who began with this cardiomyopathy following epileptic seizures suffered after one of her haemodialysis sessions. CASE REPORT. We report the case of a 55-year-old female on haemodialysis due to chronic renal failure, with epilepsy secondary to a residual lesion in the right frontoparietal area due to a haematoma that required surgical evacuation. After her haemodialysis session she suffered an attack of focal epilepsy with secondary generalisation and, some hours later, pain in the middle of her chest. Serial enzymes revealed increased levels of troponin I and, electrocardiographically, negative T waves were observed in precordial derivations (V2-V6). Coronary catheterisation was performed, with normal results, and alterations were noted in contractility, which were confirmed as being transient in a serial echocardiography study. All the previous data lead us to a suspected diagnosis of Takotsubo syndrome. CONCLUSIONS. Cardiac complications are one of the causes of morbidity and mortality in epilepsy, and Takotsubo syndrome is an example of them. The real incidence of this syndrome is unknown, but given its involvement in mortality caused by heart problems in epilepsy it is important to suspect it in the presence of cardiac dysfunction following epileptic seizures. PMID:25342054

Garea Garcia-Malvar, M J; Gonzalez-Silva, Y; Epureanu-Epureanu, V

2014-11-01

72

Vascular complications of thoracic outlet syndrome.  

PubMed

Vascular complications of thoracic outlet syndrome are uncommon but may result in significant long-term disability. This report documents a retrospective review of 17 such patients. Ten patients presented with acute onset of upper extremity swelling and axillosubclavian vein thrombosis. One patient presented with chronic, intermittent arm swelling and subclavian vein stenosis. Three patients presented with acute symptoms of upper extremity emboli, and three presented with chronic arm claudication. Cervical ribs were discovered in four patients with arterial symptoms and in no patients with venous symptoms. All ten patients with acute venous thrombosis underwent successful thrombolysis, with venous stenosis uncovered in 8. Thrombolysis was also performed for two patients with arterial emboli. All 17 patients underwent surgical decompression of the thoracic outlet, 16 via a supraclavicular approach and one via a transaxillary approach. One subclavian arteriotomy with endarterectomy and one resection of a subclavian artery aneurysm were performed at the time of decompression. Repeat venography after decompression demonstrated persistent venous stenosis in one patient that was treated with balloon angioplasty and stenting. After a mean of 22 months' follow-up, 12 patients had no residual symptoms, and 5 had experienced significant improvement of symptoms. In conclusion, a combined approach of thrombolysis and surgical decompression of the thoracic outlet provides a salutary outcome in a majority of patients. PMID:9322672

Hood, D B; Kuehne, J; Yellin, A E; Weaver, F A

1997-10-01

73

Tapia's syndrome -- a rare complication following cardiac surgery  

PubMed Central

Tapia's syndrome is a rare complication following cardiac surgery. It includes the extracranial involvement of the recurrent laryngeal nerve and the hypoglossal nerve and results in ipsilateral paralysis of the vocal cord and the tongue. It is usually a complication related to anaesthesia and positioning of the head of the patient during surgery. We describe this rare complication which occurred at our institute. A 49-year old man developed Tapia's syndrome after an uneventful coronary artery bypass surgery. He complained of dysphonia, hoarseness of voice and an inability to swallow soon after extubation. The syndrome resolved completely over the following weeks with no neurological deficit. PMID:22108947

Nalladaru, Zubin; Wessels, Andre; DuPreez, Leon

2012-01-01

74

Two Cases of Walker-Warburg Syndrome Complicated by Hydrocephalus  

Microsoft Academic Search

Walker-Warburg syndrome is a very rare autosomal recessive disorder with congenital muscular dystrophy, brain malformations on the basis of a neuronal migration defect and ocular abnormalities. We report our experience in treating two cases of Walker-Warburg syndrome complicated by hydrocephalus with shunting and endoscopic techniques.

M. Preuss; M. Heckmann; M. Stein; U. Nestler

2010-01-01

75

Pulmonary Hemorrhage Complicating a Typical Hemolytic-Uremic Syndrome  

Microsoft Academic Search

We describe a case of pulmonary bleeding and subsequent acute respiratory distress syndrome (ARDS) in a 20-month-old female suffering from a typical postdiarrheal hemolytic-uremic syndrome (HUS). Acute renal failure was treated early by peritoneal dialysis. It is of interest to underline that thrombocytopenia or any coagulative impairment was absent when this complication occurred, and spontaneous diuresis recovery was ongoing. All

M. Piastra; A. Ruggiero; A. Langer; E. Caresta; A. Chiaretti; S. Pulitanò; G. Polidori; R. Riccardi

2004-01-01

76

Superior Mesenteric Artery Syndrome: An Infrequent Complication of Scoliosis Surgery  

PubMed Central

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.

Akgul, Turgut; Bayraktar, Adem; Dikici, Fatih; Bal?k, Emre

2014-01-01

77

Toxic shock syndrome: A rare complication to enhanced external counterpulsation.  

PubMed

Enhanced external counterpulsation (EECP) is known to reduce angina pectoris in patients in whom revascularization is not possible. The therapy is associated with few adverse effects. A case with a previously unknown complication - toxic shock syndrome - that occurred twice in an EECP-treated patient is described. Toxic shock syndrome initially resembles the state of septic shock. Early recognition of the syndrome and initiation of therapy is of vital importance to prevent rapid progression and a possibly fatal outcome. Awareness of this condition among cardiologists offering EECP is essential. PMID:21165367

Jørgensen, Peter Godsk; Lindberg, Jens Aage; May, Ole

2010-12-01

78

Toxic shock syndrome: A rare complication to enhanced external counterpulsation  

PubMed Central

Enhanced external counterpulsation (EECP) is known to reduce angina pectoris in patients in whom revascularization is not possible. The therapy is associated with few adverse effects. A case with a previously unknown complication – toxic shock syndrome – that occurred twice in an EECP-treated patient is described. Toxic shock syndrome initially resembles the state of septic shock. Early recognition of the syndrome and initiation of therapy is of vital importance to prevent rapid progression and a possibly fatal outcome. Awareness of this condition among cardiologists offering EECP is essential. PMID:21165367

J?rgensen, Peter Godsk; Lindberg, Jens; May, Ole

2010-01-01

79

Guillain-Barre syndrome complicated by acute fatal rhabdomyolysis  

PubMed Central

Guillain-Barre syndrome (GBS) is a heterogenous group of peripheral-nerve disorders with similar clinical presentation characterized by acute, self-limited, progressive, bilateral and relatively symmetric ascending flaccid paralysis, which peaks in 2-4 weeks and then subsides. The usual complications, which occur in a patient of GBS are pneumonia, sepsis, pulmonary embolism, respiratory insufficiency and cardiac arrest. The clinical course of GBS complicated by acute rhabdomyolysis is extremely rare. We present the case of GBS with marked elevation in serum creatine kinase, serum myoglobin levels and persistent hyperkalemia as a result of associated acute rhabdomyolysis. PMID:24872655

Saxena, Amrish; Singh, Vineeta; Verma, Nitin

2014-01-01

80

Chronic graft-versus-host disease complicated by nephrotic syndrome  

Microsoft Academic Search

Chronic graft-versus-host disease (cGVHD) is one of the most frequent and serious complications of allogeneic hematopoietic stem cell transplantation (HSCT). Nephrotic syndrome (NS) is an uncommon and underrecognized manifestation of cGVHD. We report a patient who developed NS 18 months after allogeneic bone marrow transplantation. The onset of NS was accompanied by active manifestations of cGVHD, and immunosuppressants had not

Hsin-Hui Wang; An-Hang Yang; Ling-Yu Yang; Giun-Yi Hung; Jei-Wen Chang; Chun-Kai Wang; Tzong-Yann Lee; Ren-Bin Tang

2011-01-01

81

Economy class syndrome: still a recurrent complication of long journeys.  

PubMed

Economy class syndrome is a rare but still unavoidable complication of long haul flights, particularly in patients who carry various intrinsic risk factors. The tendency to affect even asymptomatic young people and the greater risk to fragment and propagate to the pulmonary circulation are the main characteristics of deep vein thrombosis of long-flight travelers. We report the clinical history of eight patients admitted to intensive care unit for confirmed or highly suspected economy class syndrome. Seven of them developed the syndrome within 72 h from a long return flight, one suffered from pulmonary embolism after a 12-h car trip. Two out of eight patients died, one because of extremely severe hemodynamic impairment, the other as a consequence of multiple organ failure caused by a concomitant myocardial infarction. Deep vein thrombosis and pulmonary embolism represent one of the main medical problems of air travel and cause almost 20% of deaths in people with no medical history. Although economy class syndrome occurs mostly in elderly, even the healthy young population can be affected and, in fact, three out of eight patients of our series were under 50 years of age. All our patients but one carried a well recognized risk factor for deep vein thrombosis. Clinical symptoms of deep vein thrombosis can sometimes be aspecific and confusing, so that a certain proportion of post-travel deep vein thrombosis, evolving favorably and not giving rise to pulmonary embolism, might effectively remain undiagnosed. Economy class syndrome is still quite difficult to deal with and controversial in terms of preventive strategies. PMID:17496687

Feltracco, Paolo; Barbieri, Stefania; Bertamini, Francesca; Michieletto, Elisa; Ori, Carlo

2007-04-01

82

Multiple Giant Gastrointestinal Diverticula Complicated by Perforated Jejunoileal Diverticulitis in Marfan Syndrome  

Microsoft Academic Search

Gastrointestinal complications of Marfan syndrome are rare. We report a case of multiple giant gastric, duodenal, small intestinal and colonic diverticula, complicated by perforated jejunoileal diverticulitis, in a patient with Marfan syndrome. Recovery followed resection of the entire involved small bowel. Whereas the association of colonic diverticula with Marfan syndrome has been sporadically reported, the presence of small intestinal diverticula

Oz Shapira; Eliezer Mavor; Dan Simon; Harry Rothstein; Reuven Pfeffermann

1992-01-01

83

[Psychiatric and psychological complications in obstructive sleep apnea syndrome].  

PubMed

It is estimated, that symptoms of obstructive sleep apnea syndrome (OSAS) affect 2 to 4% of the middle-aged population and their prevalence increases with age (over 50% people aged 65 and older suffer from OSAS). Among risk factors of OSAS we can distinguish: the male sex, race, overweight and obesity, thyroid hypofunction, age and alcohol abuse. Obstructive sleep apnea results in the absence (apnea) or reduction (hypopnea) of airflow lasting at least 10 s despite normal respiratory exertion. The apnea and hypopnea result in decreased oxygen saturation levels in the blood (hypoxemia). A number of consequences of OSAS can appear including: disruption of the sleep cycle, fragmentation of the sleep cycle, sleepiness, fatigue, headaches, cognitive impairments, irritability and mood disturbance, the higher risk of accidents at work and car accidents, the decrease of the quality of life and the higher risk of cardiovascular diseases. Besides various psychiatric and psychological complications presented in this review can occur in obstructive sleep apnea syndrome. PMID:21190150

Ga?ecki, Piotr; Florkowski, Antoni; Zboralski, Krzysztof; Pietras, Tadeusz; Szemraj, Janusz; Talarowska, Monika

2011-01-01

84

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

PubMed

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. PMID:25194187

Tack, Jan; Deloose, Eveline

2014-08-01

85

Cognitive deficits and associated neurological complications in individuals with Down's syndrome.  

PubMed

Improvements in medical interventions for people with Down's syndrome have led to a substantial increase in their longevity. Diagnosis and treatment of neurological complications are important in maintaining optimal cognitive functioning. The cognitive phenotype in Down's syndrome is characterised by impairments in morphosyntax, verbal short-term memory, and explicit long-term memory. However, visuospatial short-term memory, associative learning, and implicit long-term memory functions are preserved. Seizures are associated with cognitive decline and seem to cause additional decline in cognitive functioning, particularly in people with Down's syndrome and comorbid disorders such as autism. Vision and hearing disorders as well as hypothyroidism can negatively impact cognitive functioning in people with Down's syndrome. Dementia that resembles Alzheimer's disease is common in adults with Down's syndrome. Early-onset dementia in adults with Down's syndrome does not seem to be associated with atherosclerotic complications. PMID:20494326

Lott, Ira T; Dierssen, Mara

2010-06-01

86

A rare neurological complication of typhoid fever: Guillain-Barre' syndrome  

PubMed Central

Guillain-Barre’ syndrome is a rare complication of typhoid fever, and only a few such cases have been reported in the pediatric age group. We report a young boy with blood culture proven typhoid fever that developed this very rare neurological complication quite early in the course of the disease. Following treatment with intravenous antibiotics and intravenous immunoglobulin, he improved.

Kapoor, Kapil; Jain, Sumidha; Jajoo, Mamta; Talukdar, Bibek

2014-01-01

87

A rare neurological complication of typhoid fever: Guillain-Barre' syndrome.  

PubMed

Guillain-Barre' syndrome is a rare complication of typhoid fever, and only a few such cases have been reported in the pediatric age group. We report a young boy with blood culture proven typhoid fever that developed this very rare neurological complication quite early in the course of the disease. Following treatment with intravenous antibiotics and intravenous immunoglobulin, he improved. PMID:25250072

Kapoor, Kapil; Jain, Sumidha; Jajoo, Mamta; Talukdar, Bibek

2014-05-01

88

Churg-strauss syndrome.  

PubMed

Churg-Strauss syndrome (CSS) is a rare granulomatous necrotizing small vessel vasculitis characterized by the presence of asthma, sinusitis, and hypereosinophilia. The cause of this allergic angiitis and granulomatosis is unknown. Other common manifestations are pulmonary infiltrates, skin, gastrointestinal, and cardiovascular involvement. No data have been reported regarding the role of immune complexes or cell mediated mechanisms in this disease, although autoimmunity is evident with the presence hypergammaglobulinemia, increased levels of IgE and Antineutrophil cytoplasmic antibody (positive in 40%). We report the case of a 27-year-old lady presenting with painful swelling of predominantly lower limbs with extensive vesicles and ecchymotic patches and fever shortly after stopping systemic steroids taken for a prolonged duration (2002--2010). The aim of this case report is to point to the possibility of CSS in patients presenting with extensive skin lesions masquerading as Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis Syndrome (SJS/TENS). PMID:22345778

Ghosh, Subhasish; Bhattacharya, Maitreyee; Dhar, Sandipan

2011-11-01

89

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

PubMed

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. PMID:24081809

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

2014-10-01

90

A case of ocular hypertension complicated by SUNCT syndrome.  

PubMed

We report a 53-year-old woman with laser iridotomy (LI)-resistant angle-closure and conjunctival injection, which was thought to be the cause of ciliochoroidal effusion associated with short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) syndrome. LI had no effect on any of the symptoms except for intraocular pressure. The symptoms disappeared after a subsequent procedure for SUNCT syndrome. MRI of the left eye showed ciliochoroidal effusion at paroxysm and was normalized upon relief. PMID:21138147

Murakami, Junko; Kim, Akio; Sugiyama, Takeshi; Inoue, Keiji; Matsumoto, Chota; Shimomura, Yoshikazu

2010-01-01

91

Complications of Midgut Carcinoid Tumors and Carcinoid Syndrome  

Microsoft Academic Search

The carcinoid syndrome, associated with carcinoid tumors of the midgut, consists of symptoms such as diarrhea, flushing, wheezing and cardiovascular symptoms. This review focuses on these symptoms and discusses therapeutic options. The symptoms are caused by the secretion of biogenic amines, polypeptides and other factors of which serotonin is the most prominent. However, diarrhea is also due to factors such

Anouk N. A. van der Horst-Schrivers; A. N. Machteld Wymenga; Thera P. Links; Pax H. B. Willemse; Ido P. Kema; Elisabeth G. E. de Vries

2004-01-01

92

Pregnancy outcomes in women complicated by thalassemia syndrome at Maharaj Nakorn Chiang Mai Hospital  

Microsoft Academic Search

Objective  To determine the maternal and fetal outcomes of women complicated with thalassemia syndrome.\\u000a \\u000a \\u000a \\u000a Study design  Retrospective descriptive study.\\u000a \\u000a \\u000a \\u000a Materials and methods  The database of Maternal-Fetal Medicine unit and medical records, between January 2001 and April 2008, were reviewed to search\\u000a for pregnant women complicated with thalassemia syndrome and medical records were reviewed for patient’s baseline characteristics\\u000a and pregnancy outcomes. The inclusion criteria

Kuntharee Traisrisilp; Suchaya Luewan; Theera Tongsong

2009-01-01

93

[Endometrial cancer, estrogens and metabolic syndrome: scenario becomes more complicated].  

PubMed

Simultaneously with keeping one of the leading positions in the structure of oncogynecological morbidity, endometrial cancer (EC) presents for many decades 'the food for brains' of cancer endocrinologists. Step by step development of contemporary ideas and collecting the data on mechanisms of hormonal carcinogenesis, the role of excessive estrogenic stimulation and metabolic syndrome/insulin resistance as risk factors for EC, very probable gradual changing of the disease phenotype and significance of genetic damages (with PTEN-mutations as one of the examples), as a consequence point at potential perspectives in the usage of preventive and therapeutic approaches in this important area. PMID:25033674

Bershte?n, L M

2014-01-01

94

Respiratory complications of Ehlers-Danlos syndrome type IV.  

PubMed

We describe a case of Ehlers-Danlos syndrome (EDS) type IV in a male in early half in his twenties, who experienced recurrent and eventually fatal pulmonary hemorrhage. EDS type IV is a rare disorder of type III collagen synthesis that is characterized by unusual facies, thin translucent skin with a venous vascular pattern, easy bruising, and hypermobility of the small joints. Autopsy findings showed hypermobility of the joints and distensibility of the skin. Microscopically, the abdominal skin showed substantially decreased dermal thickness. Moreover, the reticular dermis showed fine collagen bundles and large interstitial spaces compared with the skin from a normal control that showed large collagen bundles. Individual elastic fibers were also thicker than those observed in the skin of a normal control. The thoracic aorta showed thin adventitia and a relative increase in elastic fibers. The parenchyma of both the lungs showed markedly diffuse hemorrhage with hemosiderin-laden alveolar macrophages or old thrombi and organized thrombi in the small bronchi. Furthermore, both sections of the lung showed multiple fibrous nodules containing benign metaplastic bone. Vascular wall disruption and tearing of the vessel walls in the lung parenchyma were also observed. We concluded that EDS type IV led to the patient's death because of pulmonary hemorrhage. Because this syndrome resulted in the patient's death from arterial and bowel rupture, it is important to consider EDS as a potential cause of sudden death. PMID:22940417

Hatake, Katsuhiko; Morimura, Yoshifumi; Kudo, Risa; Kawashima, Wataru; Kasuda, Shogo; Kuniyasu, Hiroki

2013-01-01

95

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

Microsoft Academic Search

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

S Ryan; C T Taylor; W T McNicholas

2009-01-01

96

Use of an allograft patch in repair of hypoplastic left heart syndrome may complicate future transplantation  

Microsoft Academic Search

Objective: Cryopreserved allograft tissue used in the Norwood procedure for infants with hypoplastic left heart syndrome (HLHS) has the potential to cause marked immunologic sensitization which may complicate potential future heart transplantation, if required. The purpose of this study was to assess the anti-HLA antibody response to allograft patches used in the initial repair of HLHS. Methods: A prospective cohort

Steven R. Meyer; Patricia M. Campbell; Jennifer M. Rutledge; Anne M. Halpin; Lois E. Hawkins; Jonathan R. T. Lakey; Ivan M. Rebeyka; David B. Ross

2010-01-01

97

Use of an allograft patch in repair of hypoplastic left heart syndrome may complicate future transplantation  

Microsoft Academic Search

Objective: Cryopreserved allograft tissue used in the Norwood procedure for infants with hypoplastic left heart syndrome (HLHS) has the potential to cause marked immunologic sensitization which may complicate potential future heart transplantation, if required. The purpose of this study was to assess the anti-HLA antibody response to allograft patches used in the initial repair of HLHS. Methods: A prospective cohort

Steven R. Meyer; Patricia M. Campbell; Jennifer M. Rutledge; Anne M. Halpin; Lois E. Hawkins; Jonathan R. T. Lakey; Ivan M. Rebeyka; David B. Ross

2005-01-01

98

Novel pharmacological strategies to prevent aortic complications in Marfan syndrome  

PubMed Central

The Marfan syndrome (MFS) is a systemic connective tissue disorder caused by mutations in the FBN1 gene. Recent molecular studies, most performed in mouse models, revealed that the MFS is more a developmental abnormality with broad and complex effects on the morphogenesis and function of multiple organ systems. FBN1 haploinsufficiency and dysregulated transforming growth factor-beta (TGF-?) signaling seem to be critical for clinical manifestations in MFS including aortic root dilatation. Aortic root aneurysm and aortic dissection represent the main causes of morbidity and mortality in MFS. Most importantly, TGF-? antagonism through angiotensin II type 1 receptor blockers (ARBs), for example losartan, has been shown to prevent and possibly reverse aortic root dilatation in a mouse model of MFS. A first human study on a small pediatric cohort confirmed those promising results in reducing the aortic root growth over a follow-up period of 12 to 47 months. So, a large multicenter trial has been set up and results should be available soon. Other therapeutic strategies which might be combined with losartan include traditional ?-blockade, doxycyclin and statins. Such management could offer the first potential for primary prevention of clinical manifestations in MFS. PMID:22783312

Matt, Peter; Eckstein, Friedrich

2011-01-01

99

Novel pharmacological strategies to prevent aortic complications in Marfan syndrome.  

PubMed

The Marfan syndrome (MFS) is a systemic connective tissue disorder caused by mutations in the FBN1 gene. Recent molecular studies, most performed in mouse models, revealed that the MFS is more a developmental abnormality with broad and complex effects on the morphogenesis and function of multiple organ systems. FBN1 haploinsufficiency and dysregulated transforming growth factor-beta (TGF-?) signaling seem to be critical for clinical manifestations in MFS including aortic root dilatation. Aortic root aneurysm and aortic dissection represent the main causes of morbidity and mortality in MFS. Most importantly, TGF-? antagonism through angiotensin II type 1 receptor blockers (ARBs), for example losartan, has been shown to prevent and possibly reverse aortic root dilatation in a mouse model of MFS. A first human study on a small pediatric cohort confirmed those promising results in reducing the aortic root growth over a follow-up period of 12 to 47 months. So, a large multicenter trial has been set up and results should be available soon. Other therapeutic strategies which might be combined with losartan include traditional ?-blockade, doxycyclin and statins. Such management could offer the first potential for primary prevention of clinical manifestations in MFS. PMID:22783312

Matt, Peter; Eckstein, Friedrich

2011-12-01

100

Lower extremity anterior compartment syndrome complicating bilateral mastectomy and immediate breast reconstruction: A case report and literature review  

PubMed Central

‘Well leg compartment syndrome’ refers to compartment syndrome occurring in a nontraumatic setting. This occurs most commonly in the lower limb during surgery performed with the patient in an anatomically vulnerable position. While this complication is well documented in the setting of orthopedic, urological and gynecological surgeries, it is an exceptionally rare complication in plastic surgery; only seven cases have been published on compartment syndrome complicating an operation performed on a supine patient. A case involving a 56-year-old woman who developed an anterior compartment syndrome of her right lower leg following a bilateral mastectomy with immediate breast reconstruction is presented. A detailed literature review is also included. PMID:23730157

Tashakkor, A Yashar; Macadam, Sheina A

2012-01-01

101

Mayer-Rokitansky-Kuster-Hauser syndrome: complications, diagnosis and possible treatment options: a review.  

PubMed

Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a disorder in women that presents as Mullerian agenesis. These patients present internal genitalia abnormalities that include the absence of a uterus and the upper two-thirds of the vagina. In this review, current diagnostic methods, accompanying complications (congenital and psychological) and non-surgical and surgical treatments are summarized. Ultrasound and MRI have been the most documented options in MRKH syndrome diagnosis. Many women with MRKH syndrome have renal, skeletal, hearing or cardiac congenital anomalies and increased levels of psychological distress. Non-surgical interventions can be used to create a sexually functional neovagina through vaginal dilation, and surgical interventions provide alternate methods of creating a neovagina. Additionally, vaginal tissue engineering and gene therapy might provide more effective approaches in solving MRKH syndrome. PMID:24948340

Bombard, David S; Mousa, Shaker A

2014-09-01

102

Nodding syndrome in Ugandan children--clinical features, brain imaging and complications: a case series  

PubMed Central

Objectives Nodding syndrome is a devastating neurological disorder of uncertain aetiology affecting children in Africa. There is no diagnostic test, and risk factors and symptoms that would allow early diagnosis are poorly documented. This study aimed to describe the clinical, electrophysiological and brain imaging (MRI) features and complications of nodding syndrome in Ugandan children. Design Case series. Participants 22 children with nodding syndrome brought to Mulago National Referral Hospital for assessment. Outcome measures Clinical features, physical and functional disabilities, EEG and brain MRI findings and a staging system with a progressive development of symptoms and complications. Results The median age of symptom onset was 6 (range 4–10) years and median duration of symptoms was 8.5 (range 2–11) years. 16 of 22 families reported multiple affected children. Physical manifestations and complications included stunting, wasting, lip changes and gross physical deformities. The bone age was delayed by 2 (range 1–6) years. There was peripheral muscle wasting and progressive generalised wasting. Four children had nodding as the only seizure type; 18 in addition had myoclonic, absence and/or generalised tonic–clonic seizures developing 1–3?years after the onset of illness. Psychiatric manifestations included wandering, aggression, depression and disordered perception. Cognitive assessment in three children demonstrated profound impairment. The EEG was abnormal in all, suggesting symptomatic generalised epilepsy in the majority. There were different degrees of cortical and cerebellar atrophy on brain MRI, but no hippocampal changes. Five stages with worsening physical, EEG and brain imaging features were identified: a prodrome, the development of head nodding and cognitive decline, other seizure types, multiple complications and severe disability. Conclusions Nodding syndrome is a neurological disorder that may be characterised as probably symptomatic generalised epilepsy. Clinical manifestations and complications develop in stages which might be useful in defining treatment and rehabilitation. Studies of risk factors, pathogenesis, management and outcome are urgently needed. PMID:23645924

Idro, Richard; Opoka, Robert Opika; Aanyu, Hellen T; Kakooza-Mwesige, Angelina; Piloya-Were, Theresa; Namusoke, Hanifa; Musoke, Sarah Bonita; Nalugya, Joyce; Bangirana, Paul; Mwaka, Amos Deogratius; White, Steven; Chong, Kling; Atai-Omoruto, Anne D; Mworozi, Edison; Nankunda, Jolly; Kiguli, Sarah; Aceng, Jane Ruth; Tumwine, James K

2013-01-01

103

Calciphylaxis as a Catastrophic Complication in a Patient with POEMS Syndrome  

PubMed Central

Calciphylaxis is a vascular calcification-cutaneous necrosis syndrome, usually seen in patients with end-stage renal disease and secondary hyperparathyroidism. We report a 57-year-old polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome patient complicated with extensive skin ulcers due to calciphylaxis. He first noted a painful cutaneous ulcer on his left thigh, and then skin lesions rapidly worsened, resulting in multiple intractable ulcers with gangrene on his legs and trunk in a few months. Serum vascular endothelial growth factor (VEGF) was markedly elevated. Biopsy samples from his skin ulcers showed the deposition of calcium in the medial layer of cutaneous vessels, this finding being compatible with calciphylaxis. This is the second reported case with POEMS syndrome complicated with calciphylaxis. Both patients had no evidence of renal failure, hyperparathyroidism, or clotting disorders. The pathogenic link between POEMS syndrome and calciphylaxis is still unclear, but VEGF is known to regulate vascular calcification, in cooperation with bone morphogenetic proteins. Further, corticosteroid and several proinflammatory cytokines activate nuclear factor-?B pathway, known as the final common pathway leading to vascular calcification. Taken together, we consider that POEMS syndrome can be an independent risk condition for calciphylaxis. PMID:20847836

Hineno, Akiyo; Kinoshita, Tomomi; Kinoshita, Michiaki; Arakura, Fuyuko; Naito, Ko-suke; Shimojima, Yasuhiro; Matsuda, Masayuki; Yoshida, Kunihiro; Ikeda, Shu-ichi

2009-01-01

104

HELLP Syndrome Complicated by Subcapsular Hematoma of Liver: A Case Report and Review of the Literature  

PubMed Central

Subcapsular liver hematoma (SLH) is a rare complication of severe preeclampsia and HELLP syndrome. These patients must be followed up in intensive care unit for advanced medical support with infused fluid, replacement of blood products, and treatment of underlying disorders. There are a lot of therapeutic options varying from conservative management to surgical treatment including hepatic resection, hepatic artery ligation, and liver transplantation. In this report we aimed to present a 26-year-old woman with SLH secondary to HELLP syndrome. PMID:24804129

Karateke, Atilla; Silfeler, Dilek; Karateke, Faruk; Kurt, Raziye; Guler, Ayse; Kartal, Ismail

2014-01-01

105

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

PubMed

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. PMID:22852104

Cernea, Daniela; Dragoescu, Alice; Novac, Marius

2012-01-01

106

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

PubMed Central

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. PMID:22852104

Cernea, Daniela; Dragoescu, Alice; Novac, Marius

2012-01-01

107

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

PubMed Central

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 ?-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-? 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

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

2011-01-01

108

Compartment syndrome of the thigh complicating surgical treatment of ipsilateral femur and ankle fractures  

NASA Technical Reports Server (NTRS)

A 26-year-old man presented with ipsilateral femur and ankle fractures. The patient was treated with interlocking nail of his femur fracture, followed by open reduction and internal fixation of his ankle fracture under tourniquet control. Postoperatively, the patient developed compartment syndrome of his thigh with elevated pressures, requiring decompressive fasciotomies. This case illustrates the possible complication of treating a femur fracture with intramedullary nailing and then immediately applying a tourniquet to treat an ipsilateral extremity fracture. Because of the complication with this patient, we feel the procedure should be staged, or a tourniquet should be avoided if possible.

Moore, M. R.; Garfin, S. R.; Hargens, A. R.

1987-01-01

109

A Rare Case of Neonatal Complicated Appendicitis in a Child with Patau's Syndrome  

PubMed Central

Neonatal appendicitis is a rare condition with high mortality rate. Signs and symptoms are often nonspecific, imaging modalities are not always diagnostic, and preoperative diagnosis is difficult with subsequent delay and complications. Its pathophysiology may be different from appendicitis in older children and comorbidities can be found. We report a case of a female neonate with Patau's syndrome, intestinal malrotation, and Fallot tetralogy in whom perforated appendix, probably occurring during fetal period due to vascular insufficiency, was found at laparotomy. PMID:25276460

Pastore, Valentina; Bartoli, Fabio

2014-01-01

110

Henoch–Schönlein purpura nephritis complicated by reversible posterior leukoencephalopathy syndrome  

Microsoft Academic Search

We report a young female patient with Henoch–Schönlein purpura (HSP) nephritis complicated by reversible posterior leukoencephalopathy\\u000a syndrome (RPLS). The patient suddenly showed generalized seizures and cortical blindness with severe hypertension due to renal\\u000a insufficiency approximately 1 year after cessation of corticosteroid treatment for HSP nephritis. Magnetic resonance imaging\\u000a (MRI) demonstrated bilateral abnormal signals mainly in the cerebellum and white matter of

Daimei Sasayama; Yasuhiro Shimojima; Takahisa Gono; Kazuma Kaneko; Masayuki Matsuda; Shu-ichi Ikeda

2007-01-01

111

Anaesthesia-related haemodynamic complications in Williams syndrome patients: a review of one institution's experience.  

PubMed

Williams syndrome is a genetic disorder associated with cardiac pathology, including supravalvular aortic stenosis and coronary artery stenosis. Sudden cardiac death has been reported in the perioperative period and attributed to cardiovascular pathology. In this retrospective audit, case note and anaesthetic records were reviewed for all confirmed Williams syndrome patients who had received an anaesthetic in our institution between July 1974 and November 2009. There were a total of 108 anaesthetics administered in 29 patients. Twelve of the anaesthetics (11.1%) were associated with cardiac complications including cardiac arrest in two cases (1.85%). Of the two cardiac arrests, one patient died within the first 24 hours postanaesthetic and the other patient survived, giving an overall mortality of 0.9% (3.4%). We conclude that Williams syndrome confers a significant anaesthetic risk, which should be recognised and considered by clinicians planning procedures requiring general anaesthesia. PMID:25233176

Olsen, M; Fahy, C J; Costi, D A; Kelly, A J; Burgoyne, L L

2014-09-01

112

Wound complications after cleft repair in children with Van der Woude syndrome.  

PubMed

Van der Woude syndrome (VWS; OMIM 119300) is an autosomal-dominant condition associated with clefts of the lip and/or palate and lower lip pits and is caused by mutations in interferon regulatory factor 6 (IRF6). The standard of practice for children born with cleft lip/palate is surgical repair, which requires proper wound healing. We tested the hypothesis that children with VWS are more likely to have wound complications after cleft repair than children with nonsyndromic cleft lip/palate (NSCLP). Furthermore, we hypothesized that children with VWS have more surgical procedures. A retrospective, case-controlled study was performed. Seventeen children with VWS and 68 matched controls with NSCLP were scored for the presence of wound complications after cleft repair, for the severity of complications, and for number of surgeries from age 0 to 10. Of the 17 children with VWS, 8 had wound complications. Of 68 controls, 13 had wound complications (P = 0.02). Of 8 wound complications in the VWS group, 6 were major, whereas of 13 complications in the control group, 9 were major (P = 0.04). Most wound complications were fistulae and occurred in isolated cleft palate and bilateral cleft lip. The mean number of surgeries in the VWS group was 3.0 compared with 2.8 in the control group (P = 0.67). Our studies suggest that children with VWS have an increased risk for wound complications after cleft repair compared with children with NSCLP. Furthermore, these data support a role for IRF6 in wound healing. PMID:20856020

Jones, Jodi L P; Canady, John W; Brookes, James T; Wehby, George L; L'Heureux, Jamie; Schutte, Brian C; Murray, Jeffrey C; Dunnwald, Martine

2010-09-01

113

Down syndrome and postoperative complications after paediatric cardiac surgery: a propensity-matched analysis  

PubMed Central

OBJECTIVES The incidence of congenital heart disease is ?50%, mostly related to endocardial cushion defects. The aim of our study was to investigate the postoperative complications that occur after paediatric cardiac surgery. METHODS Our perioperative data were analysed in paediatric patients with Down syndrome undergoing cardiac surgery. We retrospectively analysed the data from 2063 consecutive paediatric patients between January 2003 and December 2008. After excluding the patients who died or had missing data, the analysed database (before propensity matching) contained 129 Down patients and 1667 non-Down patients. After propensity matching, the study population comprised 222 patients and 111 patients had Down syndrome. RESULTS Before propensity matching, the occurrences of low output syndrome (21.2 vs 32.6%, P = 0.003), pulmonary complication (14 vs 28.7%, P < 0.001) and severe infection (11.9 vs 22.5%, P = 0.001) were higher in the Down group. Down patients were more likely to have prolonged mechanical ventilation [median (interquartile range) 22 (9–72) h vs 49 (24–117) h, P = 0.007]. The total intensive care unit length of stay [6.9 (4.2–12.4) days vs 8.3 (5.3–13.2) days, P = 0.04] and the total hospital length of stay [17.3 (13.3–23.2) days vs 18.3 (15.1–23.6) days, P = 0.05] of the Down patients were also longer. Mortality was similar in the two groups before (3.58 vs 3.88%, P = 0.86) and after (5.4 vs 4.5%, P = 1.00) propensity matching. After propensity matching, there was no difference in the occurrence of adverse events. CONCLUSIONS After propensity matching Down syndrome was not associated with increased mortality or complication rate following congenital cardiac surgery. PMID:23832837

Toth, Roland; Szanto, Peter; Prodan, Zsolt; Lex, Daniel J; Sapi, Erzsebet; Szatmari, Andras; Gal, Janos; Szanto, Tamas; Szekely, Andrea

2013-01-01

114

Complications of Carotid Blowout Syndrome in Patients with Head and Neck Cancers Treated by Covered Stents  

PubMed Central

Summary The purpose of this study was to improve clinical assessment of carotid-blowout syndrome (CBS) in patients with head-and-neck cancers and with covered stents by evaluating immediate and delayed complications of reconstructive management. Eleven such patients were treated with self-expandable covered stents. We evaluated immediate and delayed complications by assessing clinical and imaging findings. Technical success and immediate hemostasis were achieved in all patients. Immediate complications were noted in four patients (36.4%), including thromboembolism in three patients and, in one patient, dissection of the carotid artery and type III endoleak by the overlapped self-expandable stent causing rebleeding. Delayed complications were noted in eight patients (72.7%), including six episodes of rebleeding in five patients, distal marginal stenosis in five patients, and delayed carotid thrombosis in three patients (one with brain abscess formation). We suggest close follow-up of the patients and aggressive re-intervention of their complications to improve outcomes. PMID:20557798

Chang, Feng-Chi; Luo, Chao-Bao; Lirng, Jiing-Feng; Guo, Wan-Yuo; Wu, Hsiu-Mei; Teng, Michael Mu Huo; Chang, Cheng-Yen

2008-01-01

115

Adipose tissue mitochondrial dysfunction triggers a lipodystrophic syndrome with insulin resistance, hepatosteatosis, and cardiovascular complications.  

PubMed

Mitochondrial dysfunction in adipose tissue occurs in obesity, type 2 diabetes, and some forms of lipodystrophy, but whether this dysfunction contributes to or is the result of these disorders is unknown. To investigate the physiological consequences of severe mitochondrial impairment in adipose tissue, we generated mice deficient in mitochondrial transcription factor A (TFAM) in adipocytes by using mice carrying adiponectin-Cre and TFAM floxed alleles. These adiponectin TFAM-knockout (adipo-TFAM-KO) mice had a 75-81% reduction in TFAM in the subcutaneous and intra-abdominal white adipose tissue (WAT) and interscapular brown adipose tissue (BAT), causing decreased expression and enzymatic activity of proteins in complexes I, III, and IV of the electron transport chain (ETC). This mitochondrial dysfunction led to adipocyte death and inflammation in WAT and a whitening of BAT. As a result, adipo-TFAM-KO mice were resistant to weight gain, but exhibited insulin resistance on both normal chow and high-fat diets. These lipodystrophic mice also developed hypertension, cardiac hypertrophy, and cardiac dysfunction. Thus, isolated mitochondrial dysfunction in adipose tissue can lead a syndrome of lipodystrophy with metabolic syndrome and cardiovascular complications.-Vernochet, C., Damilano, F., Mourier, A., Bezy, O., Mori, M. A., Smyth, G., Rosenzweig, A., Larsson, N.-G., Kahn, C. R. Adipose tissue mitochondrial dysfunction triggers a lipodystrophic syndrome with insulin resistance, hepatosteatosis, and cardiovascular complications. PMID:25005176

Vernochet, Cecile; Damilano, Federico; Mourier, Arnaud; Bezy, Olivier; Mori, Marcelo A; Smyth, Graham; Rosenzweig, Anthony; Larsson, Nils-Göran; Kahn, C Ronald

2014-10-01

116

Vascular Ehlers-Danlos syndrome: pathophysiology, diagnosis, and prevention and treatment of its complications.  

PubMed

The Ehlers-Danlos syndrome consists of a group of inherited connective tissue disorders caused by defects in the synthesis of collagen. The vascular type 4 form of Ehlers-Danlos syndrome (VEDS) is associated with serious vascular complications in young adults, such as the spontaneous rupture of large-caliber and medium-caliber arteries, often without true aneurysm formation or dissection. VEDS is inherited as an autosomal dominant trait that is caused by mutations in the COL3A1 gene. It affects the synthesis and structure of the pro a1 (III) chain of collagen type III, which causes vascular wall weakness. The diagnosis of VEDS is made from major and minor clinical criteria and can be confirmed by abnormalities in procollagen production and molecular genetic testing. Recently, the results of a study using the b-blocker celiprolol demonstrated a reduction in vascular complications of VEDS. The mechanisms of benefit may be related to a reduction in vascular hemodynamic stress with exercise and/or through a reduction in transforming growth factor-b. Inhibitors of the renin-angiotensin system may also be beneficial in VEDS. Surgery may be beneficial in treating the complications of VEDS. PMID:22143279

Beridze, Natalia; Frishman, William H

2012-01-01

117

Organophosphate intermediate syndrome with neurological complications of extrapyramidal symptoms in clinical practice.  

PubMed

Organophosphates (OPs) are ubiquitous in the world as domestic and industrial agricultural insecticides. Intentional poisoning as suicides attempts are clinical phenomena seen in emergency departments and clinics in agricultural areas. Intermediate syndrome with the neurological complication of extra pyramidal symptoms following acute OP ingestion may occur in pediatric and adult cases. While death is the most serious consequence of toxic OP doses, low levels of exposure and nonfatal doses may disrupt the neurobehavioral development of fetuses and children in addition to bring linked to testicular cancer and male and female infertility. These are disturbing. Chronic and acute toxicity from OPs are barriers to the health of our present and future generations. Symptoms and treatment of acute and chronic OP exposure are briefly referenced with inclusion of the intermediate syndrome. Suggestions for local and systemic reduction of the acute and long term consequences of OP ingestion are opined. PMID:25002781

Detweiler, Mark B

2014-07-01

118

Augmentation as a treatment complication of restless legs syndrome: concept and management.  

PubMed

Augmentation constitutes the main complication of long-term dopaminergic treatment in restless legs syndrome (RLS). Although this condition was first described in 1996, and is characterized by an overall increase in severity of RLS symptoms (including earlier onset of symptoms during the day, faster onset of symptoms when at rest, expansion to the upper limbs and trunk, and shorter duration of the treatment effect), precise diagnostic criteria were not established until 2003. These criteria have recently been updated to form a new definition of augmentation based on multicentric studies. The present article reviews our current knowledge on clinical diagnosis, evaluation, pathophysiology, and treatment recommendations for this condition. PMID:17580331

García-Borreguero, Diego; Allen, Richard P; Benes, Heike; Earley, Christopher; Happe, Svenja; Högl, Birgit; Kohnen, Ralf; Paulus, Walter; Rye, David; Winkelmann, Juliane

2007-01-01

119

Life-Threatening Complication During Percutaneous Ablation of Adrenal Gland Metastasis: Takotsubo Syndrome  

SciTech Connect

A rare life-threatening complication during percutaneous cryoablation of an adrenal gland metastasis from a lung carcinoma is reported. The patient presented hypertensive crisis at the beginning of the thawing cycle, followed by electrocardiographic change which necessitated interruption of the procedure and his transfer to the intensive care unit with suspicion of heart infarct. There was a slight increase in cardiac enzyme levels, and ventricular angiography demonstrated transient hypokinesis-dyskinesis of the mid left ventricular segments without apical involvement, while the coronary arteries showed no significant stenosis on coronarography. These findings led to the diagnosis of Takotsubo cardiomyopathy left ventricular dysfunction syndrome. This is the first case of Takotsubo cardiomyopathy occurring as a complication during percutaneous ablation of an adrenal gland tumor.

Tsoumakidou, Georgia, E-mail: gtsoumakidou@yahoo.com; Buy, Xavier, E-mail: Xavier.buy@chru-strasbourg.f [University Hospital of Strasbourg, Department of Non Vascular Interventional Radiology (France); Zickler, Pierre, E-mail: pierre.zickler@chru-strasbourg.f [University Hospital of Strasbourg, Department of Anaesthesiology (France); Zupan, Michel, E-mail: Michel.zupan@chru-strasbourg.fr; Douchet, Marie-Pierre, E-mail: Marie.pierre-douchet@chru-strasbourg.f [University Hospital of Strasbourg, Department of Cardiology (France); Gangi, Afshin, E-mail: gangi@rad6.u-strasbg.f [University Hospital of Strasbourg, Department of Non Vascular Interventional Radiology (France)

2010-06-15

120

Advances in the epidemiology, pathogenesis, and management of Cushing's syndrome complications.  

PubMed

Cushing's syndrome (CS) is a clinical condition resulting from chronic exposure to glucocorticoid excess. As a consequence, hypercortisolism contributes significantly to the early development of systemic disorders by direct and/or indirect effects. Complications such as obesity, hypertension, diabetes, dyslipidemia, and hypercoagulability cause premature atherosclerosis and increase cardiovascular mortality. Impairment of the skeletal system is a relevant cause of morbidity and disability in these patients especially due to the high prevalence of vertebral fractures. In addition, muscle weakness, emotional lability, depression, and impairment of quality of life are very common. Clinical management of these patients is complex and should be particularly careful in identifying global cardiovascular risks and aim at controlling all complications. Although the primary goal in the prevention and treatment of complications is the correction of hypercortisolism, treatment does not completely eliminate these comorbidities. Given that cardiovascular risk and fracture risk can persist after cure, early detection of each morbidity could prevent the development of irreversible damage. In this review we present the various complications of CS and their pathogenetic mechanisms. We also suggest the clinical management of these patients based on our extensive clinical experience and on the available literature. PMID:22652826

Arnaldi, G; Mancini, T; Tirabassi, G; Trementino, L; Boscaro, M

2012-04-01

121

Shiga toxin-associated hemolytic uremic syndrome complicated by intestinal perforation in a child with typical hemolytic uremic syndrome  

PubMed Central

Hemolytic uremic syndrome (HUS) is one of the most common causes of acute renal failure in childhood and is primarily diagnosed in up to 4.5% of children who undergo chronic renal replacement therapy. Escherichia coli serotype O157:H7 is the predominant bacterial strain identified in patients with HUS; more than 100 types of Shiga toxin-producing enterohemorrhagic E. coli (EHEC) subtypes have also been isolated. The typical HUS manifestations are microangiopathic hemolytic anemia, thrombocytopenia, and renal insufficiency. In typical HUS cases, more serious EHEC manifestations include severe hemorrhagic colitis, bowel necrosis and perforation, rectal prolapse, peritonitis, and intussusceptions. Colonic perforation, which has an incidence of 1%-2%, can be a fatal complication. In this study, we report a typical Shiga toxin-associated HUS case complicated by small intestinal perforation with refractory peritonitis that was possibly because of ischemic enteritis. Although the degree of renal damage is the main concern in HUS, extrarenal complications should also be considered in severe cases, as presented in our case. PMID:24678335

Chang, Hye Jin; Kim, Hwa Young; Choi, Jae Hong; Choi, Hyun Jin; Ko, Jae Sung; Ha, Il Soo; Cheong, Hae Il; Choi, Yong

2014-01-01

122

Shiga toxin-associated hemolytic uremic syndrome complicated by intestinal perforation in a child with typical hemolytic uremic syndrome.  

PubMed

Hemolytic uremic syndrome (HUS) is one of the most common causes of acute renal failure in childhood and is primarily diagnosed in up to 4.5% of children who undergo chronic renal replacement therapy. Escherichia coli serotype O157:H7 is the predominant bacterial strain identified in patients with HUS; more than 100 types of Shiga toxin-producing enterohemorrhagic E. coli (EHEC) subtypes have also been isolated. The typical HUS manifestations are microangiopathic hemolytic anemia, thrombocytopenia, and renal insufficiency. In typical HUS cases, more serious EHEC manifestations include severe hemorrhagic colitis, bowel necrosis and perforation, rectal prolapse, peritonitis, and intussusceptions. Colonic perforation, which has an incidence of 1%-2%, can be a fatal complication. In this study, we report a typical Shiga toxin-associated HUS case complicated by small intestinal perforation with refractory peritonitis that was possibly because of ischemic enteritis. Although the degree of renal damage is the main concern in HUS, extrarenal complications should also be considered in severe cases, as presented in our case. PMID:24678335

Chang, Hye Jin; Kim, Hwa Young; Choi, Jae Hong; Choi, Hyun Jin; Ko, Jae Sung; Ha, Il Soo; Cheong, Hae Il; Choi, Yong; Kang, Hee Gyung

2014-02-01

123

Neurovascular complications of ovarian hyperstimulation syndrome (OHSS): from pathophysiology to recent treatment options.  

PubMed

Ovarian hyperstimulation syndrome (OHSS) is a severe iatrogenic complication of ovulation induction, which has a very serious impact on the patient's health, as it is often associated with a high morbidity and mortality risk. Indeed, patients classified as having severe OHSS presented with liquid imbalance signs (such as rapid weight gain, tense ascites, respiratory difficulty and progressive oliguria), which are related to the fluid shift from the intravascular space to third space compartments subsequent to an increased capillary permeability. In this way, cardiovascular system findings include decreased intravascular volume, decreased blood pressure, decreased central venous perfusion, and compensatory increased heart rate and cardiac output with arterial vasodilation might be found concomitantly. Notwithstanding that venous thromboembolic phenomena are a possible complication in advanced phases of OHSS, arterial ischemia involving the cerebral circulation is a rare but recently reported problem. The pathogenesis of thromboembolism in OHSS is not fully understood, even though hemoconcentration and blood hyperviscosity seem to play a role in developing thrombotic changes into both venous and arterial system. Interestingly, the presence of cardiac abnormalities in combination with inherited or acquired hypercoagulable state seems to increase the risk of cerebral infarct in these subjects, as recently shown by our group. This review is aimed at investigating the pathomechanism and the management of neurovascular complications related to OHSS, including new treatment options. PMID:24902709

Calabro, Rocco S; Gervasi, Giuseppe; Leo, Antonino; De Luca, Rosaria; Balletta, Tina; Casella, Carmela; Triolo, Onofrio; Bramanti, Placido

2014-01-01

124

Abdominal compartment syndrome – the prevention and treatment of possible lethal complications following hip arthroscopy: a case report  

PubMed Central

Introduction Intra-abdominal hypertension and abdominal compartment syndrome have been increasingly recognized as a hip arthroscopy complication over the past decade. In the absence of consensus definitions and treatment guidelines, the diagnosis and management of intra-abdominal hypertension and abdominal compartment syndrome remains variable from institution to institution. Case presentation We report the occurrence of the extravasation of fluid into the abdomen during arthroscopic treatment of femoroacetabular impingement combined with resection of trochanteric bursa and our management of the condition in a 55-year old Caucasian woman. Conclusions We present an algorithm of treatment of abdominal compartment syndrome, as a hip arthroscopy complication, according to the consensus definitions and recommendations of the World Society of the Abdominal Compartment Syndrome. In the algorithm options, we have included paracentesis and percutaneous catheter decompression as the main point of treatment. Our algorithm will have a broader clinical impact on orthopedic surgery, anesthesiology and emergency medicine. PMID:25394557

2014-01-01

125

Posterior reversible encephalopathy syndrome in a renal allograft recipient: A complication of immunosuppression?  

PubMed

Posterior reversible encephalopathy syndrome (PRES) is an uncommon post-renal transplant complication. We report a 16-year-old boy who had an acute cellular rejection immediate post-transplant and was given intravenous methylprednisolone along with an increase in tacrolimus dose. He was diagnosed to have PRES based on clinical and radiological features within 6 h of intensified immunosuppression. This is an unusual case report of successfully managing PRES with continuation of the intensified immunosuppression as warranted by the clinical situation, along with aggressive blood pressure control. After 6 weeks, magnetic resonance imaging showed complete resolution of lesions. He has good graft function and no residual neurological deficits while on small doses of three antihypertensives, 12 months after transplantation. PMID:23716922

Alexander, S; David, V G; Varughese, S; Tamilarasi, V; Jacob, C K

2013-03-01

126

Feeding Complications in Hypoplastic Left Heart Syndrome After the Norwood Procedure: A Systematic Review of the Literature  

Microsoft Academic Search

Gastrointestinal and feeding complications after the Norwood procedure in infants with hypoplastic left heart syndrome increases\\u000a morbidity and mortality. These problems are the result of intraoperative challenges, shunt-dependent physiology, and the absence\\u000a of best-practice guidelines. In response, a systematic review of feeding-related complications and management strategies was\\u000a performed. A literature search from 1950 to March 2010 identified 21 primary research

Jessica R. Golbus; Brandon M. Wojcik; John R. Charpie; Jennifer C. Hirsch

2011-01-01

127

A case of Clostridium difficile infection complicated by acute respiratory distress syndrome treated with fecal microbiota transplantation.  

PubMed

Acute respiratory distress syndrome is a life-threatening disorder caused mainly by pneumonia. Clostridium difficile infection (CDI) is a common nosocomial diarrheal disease. Disruption of normal intestinal flora by antibiotics is the main risk factor for CDI. The use of broad-spectrum antibiotics for serious medical conditions can make it difficult to treat CDI complicated by acute respiratory distress syndrome. Fecal microbiota transplantation is a highly effective treatment in patients with refractory CDI. Here we report on a patient with refractory CDI and acute respiratory distress syndrome caused by pneumonia who was treated with fecal microbiota transplantation. PMID:25253977

Kim, Ji Eun; Gweon, Tae-Geun; Yeo, Chang Dong; Cho, Young-Seok; Kim, Gi Jun; Kim, Jae Young; Kim, Jong Wook; Kim, Hyunho; Lee, Hye Won; Lim, Taeseok; Ham, Hyoju; Oh, Hyun Jin; Lee, Yeongbok; Byeon, Jaeho; Park, Sung Soo

2014-09-21

128

A case of Clostridium difficile infection complicated by acute respiratory distress syndrome treated with fecal microbiota transplantation  

PubMed Central

Acute respiratory distress syndrome is a life-threatening disorder caused mainly by pneumonia. Clostridium difficile infection (CDI) is a common nosocomial diarrheal disease. Disruption of normal intestinal flora by antibiotics is the main risk factor for CDI. The use of broad-spectrum antibiotics for serious medical conditions can make it difficult to treat CDI complicated by acute respiratory distress syndrome. Fecal microbiota transplantation is a highly effective treatment in patients with refractory CDI. Here we report on a patient with refractory CDI and acute respiratory distress syndrome caused by pneumonia who was treated with fecal microbiota transplantation. PMID:25253977

Kim, Ji Eun; Gweon, Tae-Geun; Yeo, Chang Dong; Cho, Young-Seok; Kim, Gi Jun; Kim, Jae Young; Kim, Jong Wook; Kim, Hyunho; Lee, Hye Won; Lim, Taeseok; Ham, Hyoju; Oh, Hyun Jin; Lee, Yeongbok; Byeon, Jaeho; Park, Sung Soo

2014-01-01

129

Cushing's syndrome in pregnancy with a severe maternal complication: a case report.  

PubMed

Cushing's syndrome (CS) in pregnancy may be confused with a complication of pregnancy, such as pre-eclampsia or gestational diabetes. We managed a case of CS in pregnancy that was considered to be severe pre-eclampsia due to uncontrolled hypertension. The fetus was delivered via emergency cesarean section at 31 weeks' gestation because of severe pre-eclampsia and pulmonary edema. The parturient was admitted to the intensive care unit for severe maternal complications, including pulmonary hemorrhage, acute renal failure, disseminated intravascular coagulopathy, and congestive heart failure. A spine magnetic resonance image and 99m-technetium whole-body scan obtained postpartum showed multiple thoracolumbar spine compression fractures (Deleted; t-2,5,8,10,11, and -12; and L-1,2,3,4, and -5), multiple rib fractures, and a left iliac bone fracture due to osteoporosis. As a result of diagnosing CS after delivery, an adrenal cortical adenoma of the right adrenal gland was demonstrated and a laparoscopic adrenalectomy was successfully performed. PMID:21159041

Choi, Won Jun; Jung, Tae Sik; Paik, Won Young

2011-02-01

130

FKBP14-related Ehlers-Danlos syndrome: expansion of the phenotype to include vascular complications.  

PubMed

Biallelic mutations in FKBP14 cause a recessive form of Ehlers-Danlos syndrome (EDS) characterized by progressive kyphoscoliosis, myopathy, and hearing loss. To date, four children and one adult with this condition have been reported. We recently identified a 42-year-old man with severe kyphoscoliosis, restrictive/obstructive lung disease, short stature, mild hearing loss, decreased muscle mass, and a dissection of the celiac artery at age 41. He also had complete occlusion of the superior mesenteric artery with compensatory flow through an enlarged and tortuous inferior mesenteric artery. He was homozygous for a previously identified FKBP14 mutation, c.362dupC, p.(Glu122Argfs*7). He had no mutations in COL3A1, ACTA2, TGFBR1, TGFBR2, or SMAD3. The FKBP14 mutations in our patient occurred on the same haplotype as others with this same mutation. Although one family member in a previous report was thought to have early vascular complications, it could not be confirmed that she had biallelic mutations in FKBP14. This report expands the phenotype of FKBP14-related EDS to include risk for vascular complications and also raises the question of whether the shared haplotype represents a risk allele or founder mutation. PMID:24677762

Murray, Mitzi L; Yang, Margaret; Fauth, Christine; Byers, Peter H

2014-07-01

131

Vitreous phenotype: a key diagnostic sign in Stickler syndrome types 1 and 2 complicated by double heterozygosity.  

PubMed

We describe the clinical findings in two patients with double heterozygosity, both involving Stickler syndrome. In case 1, the proposita had Albright hereditary osteodystrophy which was inherited from her mother and type 1 Stickler syndrome which was a new mutation. The combination of manifestations from the two syndromes had resulted in initial diagnostic confusion. Diagnosis of the latter syndrome was made only following ophthalmic examination which documented the presence of a membranous vitreous anomaly characteristic of type 1 Stickler syndrome. Subsequent confirmation was achieved by mutation analysis of the COL2A1 gene. The propositus in case 2 inherited Treacher Collins syndrome paternally and type 2 Stickler syndrome maternally. The overlap of facial anomalies may have resulted in a more severe phenotype for the patient. The diagnosis of Stickler syndrome in the propositus was confirmed initially by vitreous assessment and later by demonstration of mutation in the COL11A1 gene. These two patients highlight the key role of vitreous examination and vitreoretinal phenotyping in the differential diagnosis of Stickler syndrome and its subtypes in cases where the clinical picture is complicated by double heterozygosity. PMID:17318849

Ang, Alan; Ung, Tsiang; Puvanachandra, Narman; Wilson, Louise; Howard, Frances; Ryalls, Michael; Richards, Allan; Meredith, Sarah; Laidlaw, Maureen; Poulson, Arabella; Scott, John; Snead, Martin

2007-03-15

132

Characterization of vascular complications in experimental model of fructose-induced metabolic syndrome.  

PubMed

Abstract Vascular dysfunction is an important complication associated with metabolic syndrome (MS). Here we fully characterized vascular complications in a rat model of fructose-induced MS. MS was induced by adding fructose (10%) to drinking water to male Wistar rats of 6 weeks age. Blood pressure (BP) and isolated aorta responses phenylephrine (PE), KCl, acetylcholine (ACh), and sodium nitroprusside (SNP) were recorded after 6, 9, and 12 weeks of fructose administration. In addition, serum levels of glucose, insulin, uric acid, tumor necrosis factor ? (TNF?), lipids, advanced glycation end products (AGEs), and arginase activity were determined. Furthermore, aortic reactive oxygen species (ROS) generation, hemeoxygenase-1 expression, and collagen deposition were examined. Fructose administration resulted in a significant hyperinslinemia after 6 weeks which continued for 12 weeks. It was also associated with a significant increase in BP after 6 weeks which was stable for 12 weeks. Aorta isolated from MS animals showed exaggerated contractility to PE and KCl and impaired relaxation to ACh compared with control after 6 weeks which were clearer at 12 weeks of fructose administration. In addition, MS animals showed significant increases in serum levels of lipids, uric acid, AGEs, TNF?, and arginase enzyme activity after 12 weeks of fructose administration. Furthermore, aortae isolated from MS animals were characterized by increased ROS generation and collagen deposition. In conclusion, adding fructose (10%) to drinking water produces a model of MS with vascular complications after 12 weeks that are characterized by insulin resistance, hypertension, disturbed vascular reactivity and structure, hyperuricemia, dyslipidemia, and low-grade inflammation. PMID:25046175

El-Bassossy, Hany M; Dsokey, Nora; Fahmy, Ahmed

2014-12-01

133

Hermansky-Pudlak Syndrome Complicated by Pulmonary Fibrosis: Radiologic-Pathologic Correlation and Review of Pulmonary Complications  

PubMed Central

Hermansky–Pudlak syndrome (HPS) is a rare autosomal recessive disorder characterized by oculocutaneous hypopigmentation, platelet dysfunction, and in many cases, life-threatening pulmonary fibrosis. We report the clinical course, imaging, and postmortem findings of a 38-year-old female with HPS-related progressive pulmonary fibrosis, highlighting the role of imaging in assessment of disease severity and prognosis. PMID:25379352

Kelil, Tatiana; Shen, Jeanne; O'Neill, Ailbhe C; Howard, Stephanie A

2014-01-01

134

[Acute colonic pseudo-obstruction (Ogilvie's syndrome) - a rare complication after ablation mammae and direct reconstruction with a free TRAM flap].  

PubMed

We report a case of a 62 year old woman who presented with Ogilvie's syndrome as a complication of mastectomy with free TRAM flap reconstruction due to angiosarcoma of the right breast. In the acute postoperative period, the patient expired as a result of Ogilvie's syndrome related complications. Several assumptions regarding the causes of Ogilvie's Syndrome as well as current theories about aetiology, diagnosis and therapy are discussed in this case presentation. PMID:25162245

Rauer, T; Sproedt, J; Gelpke, H; Jandali, A R

2014-08-01

135

A possible role of polycystic ovary syndrome for pregnancy complications in women with psoriasis.  

PubMed

Postmarketing Phase IV Psoriasis is a common, chronic, relapsing immune-mediated inflammatory disease (IMID) of the skin. IMIDs are multifactorial diseases characterized by common molecular pathways leading to a systemic inflammation. Patients with an IMID are also at higher risk of developing co-morbidities, such as adverse pregnancy outcomes, than the general population. A higher rate of pregnancy complications have been seen in inflammatory bowel disease and rheumatoid arthritis. The data for psoriasis are inconsistent but it appears that women with moderate-to-severe psoriasis may also have an increased risk of poor pregnancy outcomes. The cause of this association is unknown, although it may be related to elevated proinflammatory cytokines such as IL-6 and TNF-?, the high prevalence of comorbidities and other unhealthy behaviours, or the high prevalence of polycystic ovary syndrome (PCOS). In a recent study, PCOS prevalence in a psoriatic cohort (n?=?51) was higher than in non-psoriatic women (n?=?102) (47% versus 11%), and women with PCOS and psoriasis had a greater probability of insulin resistance, hyperinsulinaemia, and dyslipidaemia as well as a more severe skin condition, than those with psoriasis alone. Further studies are necessary to clarify the impact of psoriasis on pregnancy and in particular if these effects are mediated by concomitant PCOS. PMID:25381981

De Simone, Clara; Caldarola, Giacomo; Corbeddu, Marialuisa; Moro, Francesca; Tropea, Anna; Moretta, Gaia; Apa, Rosanna

2014-11-01

136

A review of the literature on three extraintestinal complications of ulcerative colitis: an ulcerative colitis flare complicated by Budd-Chiari syndrome, cerebral venous thrombosis and idiopathic thrombocytopenia.  

PubMed

Extraintestinal manifestations are well described and recognized in association with ulcerative colitis. Immunologically mediated and thrombotic events are among the more rare manifestations associated with flares. These manifestations include Budd-Chiari syndrome, idiopathic thrombocytopenia, and cerebral venous thrombosis. A 22 year-old male with a three-year history of ulcerative colitis presented with worsening hematochezia, fatigue, headache and upper respiratory symptoms. Laboratory evaluation demonstrated a platelet count of 24 x 10(9)/L (normal baseline platelet count noted 3 months prior) and hemoglobin of 8.6 x 10(9)/L. Imaging demonstrated hepatic venous thrombosis consistent with Budd-Chiari syndrome and cerebral venous thrombosis. Based on peripheral smear analysis and eventual marked response to steroids, a diagnosis of idiopathic thrombocytopenia was made. He was started on prednisone 40mg daily with brisk improvement in both his ulcerative colitis flare and his platelet count increasing above 100 x 10(9)L. He was therapeutically anticoagulated for the cerebral venous thrombosis. He continued to do well and was discharged on therapeutic enoxaparin and a 40 mg prednisone taper without recurrent flare or idiopathic thrombocytopenia two weeks post-hospitalization. To our knowledge, this is the first report of all three known but rare complications diagnosed concurrently in the same patient. This review examines three extraintestinal complications of ulcerative colitis, including the presentation, diagnosis, and treatment. PMID:24261025

Jaqua, Nathan T; Stratton, Amy; Yaccobe, Lior; Tahir, Usman; Kenny, Patrick; Kerns, Tamie

2013-09-01

137

Mucosal lesions may be a minor complication of SAPHO syndrome: a study of 11 Japanese patients with SAPHO syndrome  

Microsoft Academic Search

Since the term synovitis–acne–pustulosis–hyperostosis–osteitis (SAPHO) syndrome was proposed by Chamot et al. (Rev Rhum Mal\\u000a Osteoartic 54:187–196, 1987), clinical reviews concerning this syndrome have been mainly reported from Europe. We carried out a retrospective analysis\\u000a of 11 Japanese patients with SAPHO syndrome, and reviewed the clinical features of our series in comparison with those in\\u000a a European large case study.

Hiroki Yabe; Hisaji Ohshima; Yoji Takano; Takahiro Koyanagi; Hiroshi Usui; Kensuke Ochi; Michiya Kihara; Yukio Horiuchi

2010-01-01

138

Long-term blood product transfusion support for patients with myelodysplastic syndromes (MDS): cost analysis and complications  

Microsoft Academic Search

Patients with myelodysplastic syndromes (MDS) frequently become dependent on blood transfusions. We analyzed the total transfusion support required, and its complications and cost, following the diagnosis of MDS (total period=79.7 patient-years) in 50 patients followed at the Minneapolis VA Medical Center. From diagnosis of MDS to transformation to AML or death (the MDS phase), 41 patients (82%) required transfusions. The

Pankaj Gupta; Suzanne C. LeRoy; Sharon D. Luikart; Anne Bateman; Vicki A. Morrison

1999-01-01

139

Granulomatous skin lesions complicating Varicella infection in a patient with Rothmund-Thomson syndrome and immune deficiency: case report  

Microsoft Academic Search

Rothmund-Thomson syndrome (RTS)(OMIM 268400) is a rare autosomal recessive genodermatosis characterized by poikiloderma, small stature, skeletal and dental abnormalities, cataract and an increased risk of cancer. It is caused by mutations in RECQL4 at 8q24. Immune deficiency is not described as a classical feature of the disease. Here we report the appearance of granulomatous skin lesions complicating primary Varicella Zoster

Lien De Somer; Carine Wouters; Marie-Anne Morren; Rita De Vos; Joost Van Den Oord; Koenraad Devriendt; Isabelle Meyts

2010-01-01

140

Post-operative respiratory complications after palatoplasty in a 19-month-old female with Cornelia de Lange syndrome--a case report.  

PubMed

Cornelia De Lange syndrome is a rare genetically heterogeneous and sporadic syndrome, with an estimated prevalence of 1 in 10,000 to 30,000. The disorder may present many complications during anesthesia due to cardiac, gastrointestinal and airway anomalies. We report a case of an ex premature toddler presenting for repair of a cleft palate. Postoperatively she had respiratory distress, successfully treated by the anesthetic care team Causes for the complication are discussed. PMID:22428500

Ingram, Brooke; Frost, Elizabeth A M

2011-10-01

141

[A patient with Fisher syndrome and pharyngeal-cervical-brachial variant of Guillain-Barré syndrome having a complication of SIADH].  

PubMed

A 69-year-old woman complained of diplopia and truncal titubation after upper respiratory infection. She presented with mydriasis and external opthalmoplegia of bilateral eyes, ataxia, hyporeflexia and cervical-brachial muscle weakness. The protein abnormally increased (49 mg/dl) in the cerebrospinal fluid, and the serum anti-GQ1b and anti-GT1a IgG antibodies were positive. The blood sodium level was 128 mmol/l indicating hyponatremia. She had low plasma osmolarity (251 mOsm/kg), high urine osmolarity (357 mOsm/kg) and high urine sodium level (129 mmol/l), while the blood level of antidiuretic hormone was not able to be measured. She was diagnosed to have Fisher syndrome (FS), pharyngeal-cervical-brachial variant of Guillain-Barré syndrome (PCB) and syndrome of inappropriate secretion of antidiuretic hormone (SIADH). The hyponatremia improved with hyperosmotic saline infusion and restriction of water intake. Intravenous immunoglobulin therapy (IVIg) was effective only for ataxia, but the other symptoms mostly remained unchanged for a month. The serum anti-GQ1b IgG antibody was still positive even after one month. We performed high-dose intravenous steroid-pulse therapy. Then the mydriasis, external opthalmoplegia and cervical-brachial muscle weakness were immediately improved. This was a rare case of FS and PCB complicated with SIADH. IVIg, not steroid therapy, is generally chosen for FS since FS is considered as a variant of Guillain-Barré syndrome and steroid is not effective for Guillain-Barré syndrome as was proven by double-blind study. We suppose that the combined therapy of IVIg and steroid would be effective in patients with complicated symptoms and multiple antibodies. PMID:23603545

Murakami, Takenobu; Yoshihara, Akioh; Kikuchi, Saeko; Yasuda, Megumi; Hoshi, Akihiko; Ugawa, Yoshikazu

2013-01-01

142

Classic Bartter syndrome complicated with profound growth hormone deficiency: a case report  

PubMed Central

Introduction Classic Bartter syndrome is a salt-wasting tubulopathy caused by mutations in the CLCNKB (chloride channel Kb) gene. Although growth hormone deficiency has been suggested as a cause for persistent growth failure in patients with classic Bartter syndrome, in our opinion the diagnoses of growth hormone deficiency has been unconvincing in some reports. Moreover, Gitelman syndrome seems to have been confused with Bartter syndrome in some cases in the literature. In the present work, we describe a new case with CLCNKB gene mutations and review the reported cases of classic Bartter syndrome associated with growth hormone deficiency. Case presentation Our patient was a Japanese boy diagnosed as having classic Bartter syndrome at eight months of age. The diagnosis of Bartter syndrome was confirmed by CLCNKB gene analysis, which revealed compound heterozygous mutations with deletion of exons 1 to 3 (derived from his mother) and ?L130 (derived from his father). His medical therapy consisted of potassium (K), sodium chloride, spironolactone, and anti-inflammatory agents; this regime was started at eight months of age. Our patient was very short (131.1cm, -4.9 standard deviation) at 14.3 years and showed profoundly impaired growth hormone responses to pharmacological stimulants: 0.15?g/L to insulin-induced hypoglycemia and 0.39?g/L to arginine. His growth response to growth hormone therapy was excellent. Conclusions The present case strengthens the association between classic Bartter syndrome and growth hormone deficiency. We propose that growth hormone status should be considered while treating children with classic Bartter syndrome. PMID:24377430

2013-01-01

143

Acute abdominal compartment syndrome as a complication of Holmium laser enucleation of the prostate: a case report  

PubMed Central

Background In 1996, Holmium laser enucleation of the prostate was introduced and has been shown to be safe and highly effective. Case presentation We report a case of a rare complication that resulted in intra-abdominal compartment syndrome with prolonged intubation and intensive care, involving an 74-year-old male after holmium laser enucleation of prostate, with a massive irrigant fluid leakage into the retroperitoneal space. The elevated abdominal pressure was reduced by forced diuresis. The tracheal tube was removed 18 hours after the patient’s transfer to the ICU. The patient was discharged to home one week after the operation. Conclusion In rare cases when no obvious ruptures of the prostate capsule or the bladder occur during laser enucleation of prostate, knowledge regarding possible emersion of massive amounts of irrigant fluid into the retroperitoneal space leading to intra-abdominal compartment syndrome aids in the diagnosis and subsequent successful therapy of intra-abdominal hypertension. PMID:24910537

2014-01-01

144

Renal amyloidosis and renal failure - a novel complication of the SAPHO syndrome  

Microsoft Academic Search

The acronym 'SAPHO' denotes the combination of urine was unremarkable. Treatment with retinoids Synostosis - Acne - Pustulosis - Hyperostosis - (TigasonA 2?25 mg\\/day) was started. Osteitis; this rare syndrome is characterized by the In the fall of 1994, the patient was admitted to our occurrence of dermatoses with formation of sterile hospital for the first time. He complained of

R. Valentin; K. F. Gurtler; A. Schaker

1997-01-01

145

Effects of Tailored and Manualized Hypnotic Inductions for Complicated Irritable Bowel Syndrome Patients  

Microsoft Academic Search

This modest clinical pilot study was intended to provide preliminary data on the effects of hypnotic inductions tailored to an irritable bowel syndrome patient at each session compared to Palsson’s manualized protocol. Patients (N = 8) who had not previously responded to any form of treatment were assigned randomly to either a tailored or manualized induction condition. Other than pretesting

Arreed Barabasz; Marianne Barabasz

2006-01-01

146

Occam Paradox? A Variation of Tapia Syndrome and an Unreported Complication of Guidewire-Assisted Pedicle Screw Insertion  

PubMed Central

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. PMID:24436711

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

2013-01-01

147

Vasoplegic syndrome after off-pump coronary artery bypass surgery: an unusual complication.  

PubMed

We report the case of a 65-year-old man who developed norepinephrine-resistant vasoplegic syndrome after elective off-pump coronary artery bypass surgery (OPCAB). The failure of norepinephrine to improve the patient's hemodynamics prompted us to start treatment with vasopressin; within 30 minutes, the hemodynamics began to improve. After 12 hours, the patient was stable enough to be weaned from the vasopressin. He was discharged from the hospital on the 10th postoperative day. To our knowledge, ours is the 1st report of vasopressin use for vasodilatory shock after OPCAB in the English-language medical literature. Herein, we discuss the pathophysiology and management of vasoplegic syndrome--which is controversial--with special emphasis on the use of vasopressin in this situation. PMID:15745296

Raja, Shahzad G; Dreyfus, Gilles D

2004-01-01

148

Behavioural abnormalities in children with nephrotic syndrome--an underappreciated complication of a standard treatment?  

PubMed

Behaviour and psychosocial adjustment are impaired in children with steroid-sensitive idiopathic nephrotic syndrome (SSNS). Both illness-related variables and family climate play a role. Steroid treatment-both short- and long-term-is an important contributor among other determinants. The exact mechanisms by which steroids lead to behavioural alterations in humans is unclear. Optimizing the benefit/risk ratio of steroid treatment in children with SSNS is a constant goal. PMID:20573807

Neuhaus, Thomas J; Langlois, Valerie; Licht, Christoph

2010-08-01

149

Zika virus infection complicated by Guillain-Barre syndrome--case report, French Polynesia, December 2013.  

PubMed

Zika fever, considered as an emerging disease of arboviral origin, because of its expanding geographic area, is known as a benign infection usually presenting as an influenza-like illness with cutaneous rash. So far, Zika virus infection has never led to hospitalisation. We describe the first case of Guillain-Barré syndrome (GBS) occurring immediately after a Zika virus infection, during the current Zika and type 1 and 3 dengue fever co-epidemics in French Polynesia. PMID:24626205

Oehler, E; Watrin, L; Larre, P; Leparc-Goffart, I; Lastere, S; Valour, F; Baudouin, L; Mallet, Hp; Musso, D; Ghawche, F

2014-01-01

150

Coxiella burnetii causing haemophagocytic syndrome: a rare complication of an unusual pathogen  

Microsoft Academic Search

We describe an unusual presentation of Q fever with associated haemophagocytic syndrome, confirmed by bone marrow aspirate,\\u000a Q fever polymerase chain reaction (PCR) and serological testing. Clinical recovery was observed after the commencement of\\u000a doxycycline with normalisation of the patient’s full blood count and serum biochemistry. Serial monitoring of the Q fever\\u000a serology revealed the subsequent development of sustained high

P. Harris; R. Dixit; R. Norton

151

Ischemic Ileal Perforation in the Donor of Monochorionic Twins Complicated by Twin-Twin Transfusion Syndrome  

Microsoft Academic Search

Twin-twin transfusion syndrome (TTTS) carries a significant risk of perinatal morbidity and mortality. Serial amnioreduction and fetoscopic laser photocoagulation have decreased perinatal mortality, thereby shifting attention towards short-term and long-term morbidity. Most morbidity occurs within the central nervous system. Ischemic lesions, however, such as congenital skin loss and limb necrosis may occur anywhere in the vasculature of recipient twins. Ischemia

Kiyomi Tsukimori; Yasuo Yumoto; Kouji Masumoto; Tomoaki Taguchi; Haruhiko Kondo; Katsuo Sueishi; Norio Wake

2009-01-01

152

[Nebido in the treatment of hypogonadism syndrome and its complications in men].  

PubMed

The article presents original experience with use of undecanoate (nebido, BayerHealthcare Pharmaceuticals, Germany) in androgenic testosteron replacement therapy in males with hypogonadism. Prospective studies of nebido efficacy were made in males with vein-occlusive erectile dysfunction (n = 20), chronic pelvic pain syndrome (n = 77), metabolic syndrome (n = 170). Retrospective studies assessed efficacy of nebido monotherapy in patients with erectile dysfunction and hypogonadism (n = 34), hematological and urological safety of the drug (n = 40). Laboratory monitoring was performed in all the studies according to ISSAM recommendations. The patients were not included in contraindications to androgenic therapy. Nebido treatment significantly improved libido and erectile function, efficacy of phosphodiesterase of type 5 inhibiors used in moderate and severe erectile dysfunction. Depressive, asthenic, pain symptoms declined in males with chronic pelvic pain. Body fat reduced in metabolic syndrome with alleviation of its other components. Insignificant rise of hemoglobin level and packed cell volume was observed in some patients while a PSA level increase was clinically significant in 10% patients who had initial PSA > 2.5 ng/ml and acromegalia. Also, nebido depressed production of gonadotropins and spermatogenesis. Thus, nebido is highly effective in sexual dysfunction and other somatic disorders caused by hypogonadism. Nebido does not induce severe side effects, but clinically significant rise of PSA level requires treatment discontinuation and more careful urological examination. In view of nebido ability to suppress spermatogenesis, the drug should not be used in reproductively active men. PMID:22448484

Dedov, I I; Kurbatov, D G; Rozhivanov, R V; Lepetukhin, A E; Dubski?, S A; Goncharov, N P

2011-01-01

153

Twin pregnancy with HELLP syndrome complicated with acute renal failure for emergency cesarean section: An unusual case and its anesthetic management  

PubMed Central

Acute renal failure is not common in pregnancy. However, the incidence rises when pregnancy is complicated with Hemolysis, Elevated Liver enzymes, Low Platelets (HELLP) syndrome, which itself is a rare occurrence. We had an unusual case of HELLP syndrome in twin pregnancy with deranged renal profile for emergency cesarean section. We report the case, its anesthetic management for emergency cesarean section, and perioperative supportive treatment for acute renal failure.

Ghodki, Poonam S.; Singh, Noopur D.; Patil, Kalyani N.

2013-01-01

154

[A patient with Parkinson's disease complicated by hypothyroidism who developed malignant syndrome after discontinuation of etizolam].  

PubMed

A 59-year-old man, who was diagnosed as having Parkinson's disease and depression seven years ago and was on oral antiparkinsonian agents, antianxiety agents, and antidepressants, developed a high fever, disturbed consciousness, and marked muscle rigidity after discontinuation of etizolam and amitriptyline. He was admitted to a nearby hospital. Hypothyroidism had been noted two months before admission. Marked muscle rigidity and increased serum CK were observed. Since discontinuation of benzodiazepine has been known to rarely trigger a neuroleptic malignant syndrome (NMS), he was diagnosed as having NMS. After receiving dantrolene and bromocriptine, these symptoms temporarily improved but he again developed consciousness disturbance, and convulsive seizures associated with an elevated serum CK. He was transferred to our hospital. On admission, the CK level was normal at 168 IU/l, while free T4 was 0.6 ng/dl (normal range, 0.9-2.3) and TSH was 108.7 mU/ml (normal range, 0.2-4.2) in serum, indicating the presence of primary hypothyroidism. As an increase in thyroid hormone dosage improved the thyroid function to normal level, his disturbed consciousness and muscle rigidity gradually improved. Convulsive seizure and recurrence of NMS in a short interval are unusual in neuroleptic malignant syndrome. In this patient, hypothyroidism may have contributed to the development of malignant syndrome through metabolic changes of the central dopaminergic system, and discontinuation of etizolam, a kind of benzodiazepine, may have triggered NMS, since there has not been reported that discontinuation of antidepressants including amitriptyline triggers NMS. PMID:12424963

Kawajiri, Masakazu; Ohyagi, Yasumasa; Furuya, Hirokazu; Araki, Takehisa; Inoue, Naohide; Esaki, Shigemitsu; Yamada, Takeshi; Kira, Junichi

2002-02-01

155

Glue embolus complicating the endovascular treatment of a patient with Loeys-Dietz syndrome.  

PubMed

A 34-year-old [corrected] woman was diagnosed with Loeys-Dietz syndrome. Five months later, the patient presented with a symptomatic 2.6-cm subclavian pseudoaneurysm. Staged endovascular treatment was initiated with left vertebral artery embolization, followed by sac ablation and stent graft exclusion. The pseudoaneurysm cavity was filled with n-butylcyanoacrylate ("glue") via a microcatheter. Despite balloon occlusion of the pseudoaneurysm orifice, a small amount of glue debris embolized to the brachial artery, necessitating a vein bypass. In this case, distal embolization of glue may have been avoided by leaving a microcatheter in the aneurysm sac for glue injection after first deploying the stent graft. PMID:20655686

Marine, Leopoldo; Gupta, Rishi; Gornik, Heather L; Kashyap, Vikram S

2010-11-01

156

[Coronary-subclavian steal syndrome, a complication following surgical revascularization of myocardium].  

PubMed

Coronary-subclavian steal syndrome (CSS) is defined as a reversal of flow in a previously constructed internal mammary artery coronary conduit, producing myocardial ischemia. The most often cause is a proximal subclavian artery stenosis or closure. For the first time was CSS described in 1974 and initially was believed to be rare. However, today is the internal mammary artery to the left anterior descending coronary artery used in cardiosurgery as a standard and we can see increasing documentation of this phenomenon. A case report is presented and possibilities of management are discussed in the article. PMID:23256834

Gloger, V

2012-11-01

157

A Case of Vivax Malaria Complicated by Adult Respiratory Distress Syndrome and Successful Management with Extracorporeal Membrane Oxygenation  

PubMed Central

Complicated malaria is mainly caused by Plasmodium falciparum, but, increasingly, Plasmodium vivax is also being reported as a cause. Since the reemergence of indigenous vivax malaria in 1993, cases of severe malaria have been steadily reported in Korea. Herein, we report a case of vivax malaria complicated by adult respiratory distress syndrome (ARDS) that was successfully managed with extracorporeal membrane oxygenation (ECMO). A 59-year-old man presented at our hospital with fever and abdominal pain, which had persisted for 10 days. On admission, the patient had impaired consciousness, shock, hypoxia and haziness in both lungs, jaundice, thrombocytopenia and disseminated intravascular coagulation, metabolic acidosis, and acute kidney injury. A peripheral blood smear and a rapid diagnostic test verified P. vivax mono-infection. Ten hours after admission, hypoxia became more severe, despite providing maximal ventilatory support. The administration of antimalarial agents, ECMO, and continuous venovenous hemofiltration resulted in an improvement of his vital signs and laboratory findings. He was discharged from the hospital 7 weeks later, without any sequelae. PMID:24327781

Lee, Hyun-Jung; Baek, Ji-Hyeon; Chae, Myoung-Hun; Joo, Hoyeon; Chung, Moon-Hyun; Park, Yun-Kyu; Kim, Joung-Teak

2013-01-01

158

Effects of tailored and manualized hypnotic inductions for complicated irritable bowel syndrome patients.  

PubMed

This modest clinical pilot study was intended to provide preliminary data on the effects of hypnotic inductions tailored to an irritable bowel syndrome patient at each session compared to Palsson's manualized protocol. Patients (N = 8) who had not previously responded to any form of treatment were assigned randomly to either a tailored or manualized induction condition. Other than pretesting for hypnotizability, the procedure followed for the manualized group (n = 4) was exactly as prescribed by O. Palsson (1998). The identical procedure was used for the other 4 patients except that the inductions were individualized. All 8 patients showed favorable responses to treatment immediately posttreatment and at 10-month follow-up. Only the tailored group showed no incapacitating pain at posttreatment but greater emotional stress than the manualized group. The tailored group continued to improve and showed better results than the manualized group at 10 months, and the posttreatment emotional distress was significantly attenuated. PMID:16316886

Barabasz, Arreed; Barabasz, Marianne

2006-01-01

159

Development of a complicated pain syndrome following cyanide poisoning in a U.S. soldier.  

PubMed

A majority of modern war wounds are caused by blasts and high-energy ballistics. Extremity injuries predominate since modern body armor does not protect these areas due to mobility limitations. A less known and more insidious mechanism of enemy attack among our soldiers involves treachery by the local populace posing as noncombatants. One such recent event involved the contamination of tobacco with cyanide (CN). We describe a case of a soldier with CN intoxication due to ingestion of tobacco purchased from a local merchant. The soldier developed a complex neuropathic pain syndrome and was successfully treated with an inpatient high-dose intravenous ketamine infusion in combination with continuous peripheral nerve blockade. PMID:20446507

Lenart, Mark; Buckenmaier, Chester C; Kim, Moon J; Plunkett, Anthony R

2010-04-01

160

Bartter syndrome type 3 in an elderly complicated with adrenocorticotropin-deficiency.  

PubMed

Bartter syndrome (BS) is a disorder with normotensive hypokalemic alkalosis and hyperreninemic hyperaldosteronemia. BS affects infants or early childhood. Patients with BS type 3 harbor mutation in CLCNKB, Cl channel Kb. Gitelman syndrome (GS) is a disorder in childhood, with mutation in SLC12A3. Isolated adrenocorticotropin deficiency (IAD) causes secondary adrenal insufficiency. Neither elderly cases, nor cases with IAD were previously reported in BS. A 72-year-old man was admitted with acute adrenal crisis. He had been treated for IAD for 19 years. He had no trouble during perinatal period, delivery, and growth. After the recovery from adrenal crisis, laboratory tests revealed hypokalemia; 3.0 mEq/L (normal: 3.5-4.5), impaired renal function: eGFR; 37.6 mL/min/1.73m(2), normomagnesemia; 2.1 mg/dL (1.7-2.3), hyperreninemia; 59.4 ng/mL/h (0.2-2.7), hyperaldosteronemia; 23.5 ng/dL (3.0-15.9), and normal urinary ratio of calcium/creatinine. In diuretic tests, he showed a fine response to furosemide, and a mild response to thiazide. In genetic tests, no mutation of SLC12A3 was found and homozygous mutation: c.1830 G>A in CLCNKB was shown. Thus he was diagnosed as BS type 3. Current case presented with unusual features as BS type 3, 1) his late and mild clinical manifestation suggested GS rather than BS, 2) laboratory data and diuretics tests did not show typical features as BS, and 3) IAD and chronic renal failure altered electrolyte metabolism. In conclusion, current case implies that BS type 3 should be considered even in elderly cases with normotensive hypokalemia, and highlights importance of endocrinological and genetic examinations. PMID:24965226

Tamagawa, Eri; Inaba, Hidefumi; Ota, Takayuki; Ariyasu, Hiroyuki; Kawashima, Hiromichi; Wakasaki, Hisao; Furuta, Hiroto; Nishi, Masahiro; Nakao, Taisei; Kaito, Hiroshi; Iijima, Kazumoto; Nakanishi, Koichi; Yoshikawa, Norishige; Akamizu, Takashi

2014-09-29

161

Epoxyeicosatrienoic acids and heme oxygenase-1 interaction attenuates diabetes and metabolic syndrome complications  

PubMed Central

MSCs are considered to be the natural precursors to adipocyte development through the process of adipogenesis. A link has been established between decreased protective effects of EETs or HO-1 and their interaction in metabolic syndrome. Decreases in HO-1 or EET were associated with an increase in adipocyte stem cell differentiation and increased levels of inflammatory cytokines. EET agonist (AKR-I-27-28) inhibited MSC-derived adipocytes and decreased the levels of inflammatory cytokines. We further describe the role of CYP-epoxygenase expression, HO expression, and circulating cytokine levels in an obese mouse, ob/ob?/? mouse model. Ex vivo measurements of EET expression within MSCs derived from ob/ob?/? showed decreased levels of EETs that were increased by HO induction. This review demonstrates that suppression of HO and EET systems exist in MSCs prior to the development of adipocyte dysfunction. Further, adipocyte dysfunction can be ameliorated by induction of HO-1 and CYP-epoxygenase, i.e. EET. PMID:22100745

Burgess, Angela; Vanella, Luca; Bellner, Lars; Schwartzman, Michal L.; Abraham, Nader G.

2011-01-01

162

[Severe neurological and obstetrical complications in a patient with antiphospholipid syndrome].  

PubMed

Antiphospholipd syndrome (APS) is a disease characterised by venous and arterial thrombosis or recurrent foetal loss, which are associated with antiphospholipid antibodies or/and lupus anticoagulant. Clinical symptoms ofAPS are assocciated with presence of noninflammatory thrombosis ocluding arteries or venes. Symptoms of APS are often very dramatic what can be illustrated by the presented case of 40 year patient. At the age of 25 after a miscarriage the patient developed tetraparesis and motor aphasia in the course of thrombosis in central nervous system vessels. In physical examination besides neurological symptoms reticular livedo was found on the trunk and limbs. Serological tests have revealed presence of high titre of IgG and IgM anticardiolipin antibodies and lupus anticoagulant (LA). CT examination revealed hypodensic foci in left parietal lobe, and abnormal EEG findings were observed in fronto-temporal leads of left hemisphera of brain. At that point the patient did not meet the criteria of connective tissue diseases, including lupus. The diagnosis of primary APS was suggested. The patient received anti-aggregation treatment and also immunosuppressive drugs (azathioprine and prednison) due to progression of neurological manifestations. 3 years later, the second pregnancy ended in the 27th week with intrauterine fetal death. During the 3rd pregnancy, 2 years afterwards, the patient was treated with heparin, aspirin and intravenous immunoglobulin. The pregnancy finished with a successful delivery at term, the newborn was in good condition. During the following pregnancy the symptoms of preeclampsia occurred at 36/37th week but the newborn was delivered in a good condition after a caesarean section. At the age of 36 patient developed ischemic brain stroke with left-side hemiparesis inspite of anti-aggregation and immunosupressive (prednison and azathioprine) treatment. At that time homogenic type antinuclear antibodies (ANA) 1:2560, anti-beta2-glycoprotein antibodies (beta2-GPI), aCL antibodies in medium titer and thrombocytopenia were found. The patient was treated with heparin, cyclophosphamide and methyloprednisolon intravenously. During rehabilitation process gradual improvement of cognitive functions, speech and motorical functions was observed. Recently high titre of ANA and recurent thrombocytopenia < 100,000/mm3 were present. Maybe during the further follow-up, 14 years after the first symptoms of APS, the patient will develop full blown symptoms of SLE. PMID:17195361

Bobrowska-Snarska, Danuta; Ostanek, Lidia; Nesterowicz, Barbara; Brzosko, Marek

2006-05-01

163

Pregnancy Complications: HELLP Syndrome  

MedlinePLUS

... pregnancy or suffer abdominal trauma. I had a miscarriage. How long should I wait to try again? ... or treatments to discover the cause of the miscarriage, it's usually OK for you to become pregnant ...

164

Anesthesia & Down Syndrome  

MedlinePLUS

... Health Care » Associated Conditions » Anesthesia & Down Syndrome Anesthesia & Down Syndrome Complications of anesthesia (sedation during surgery) occur in ... histories are complicated. Why Would an Individual With Down Syndrome Need A nesthesia? 40-60% of infants born ...

165

Monitoring serum IL-18 levels is useful for treatment of a patient with systemic juvenile idiopathic arthritis complicated by macrophage activation syndrome  

PubMed Central

Systemic juvenile idiopathic arthritis (sJIA) is a systemic inflammatory disease characterized by arthritis, spiking fever and a skin rash that is frequently complicated by macrophage activation syndrome (MAS), a life-threatening disorder. We report a 22-month-old girl with sJIA who developed severe MAS but was successfully treated with corticosteroids, cyclosporin A, and non-steroidal anti-inflammatory drugs by monitoring serum IL-18 levels. IL-18 is an extremely useful cytokine for monitoring the activity of sJIA and MAS, and serum IL-18 can be used as an indicator for the effectiveness of treatment and the decision to discontinue therapy. PMID:21749729

2011-01-01

166

Clinical review: Bleeding - a notable complication of treatment in patients with acute coronary syndromes: incidence, predictors, classification, impact on prognosis, and management  

PubMed Central

This article focuses on the incidence, predictors, classification, impact on prognosis, and management of bleeding associated with the treatment of acute coronary syndrome. The issue of bleeding complications is related to the continual improvement of ischemic heart disease treatment, which involves mainly (a) the widespread use of coronary angiography, (b) developments in percutaneous coronary interventions, and (c) the introduction of new antithrombotics. Bleeding has become an important health and economic problem and has an incidence of 2.0% to 17%. Bleeding significantly influences both the short- and long-term prognoses. If a group of patients at higher risk of bleeding complications can be identified according to known risk factors and a risk scoring system can be developed, we may focus more on preventive measures that should help us to reduce the incidence of bleeding. PMID:24093465

2013-01-01

167

Pacemaker twiddler's syndrome (rotation of the pacemaker around the electrode cable, a rare complication of pacemaker therapy).  

PubMed

Rotation of the pacemaker generator around the electrode cable (i.e. twiddler's syndrome) was observed by the authors in six cases during the implantation of 4250 pacemakers. Twiddler's syndrome developed in three cases following implantation and in three cases after the replacement of the pacemaker. As a result of the rotation of the device, displacement of the electrode occurred in all cases. The factors predisposing to rotation of the device were as follows: (i) a loose, dilated pocket in 5 cases; (ii) seroma formation around the device in 2 cases; (iii) manipulation with the pacemaker in one case. For treating twiddler's syndrome, reimplantation was performed, fashioning a small and tight pocket for the device and fixing it by transfixing sutures. After reimplantation, the patients became complaint free, no recurrences occurred. PMID:2596244

Solti, F; Moravcsik, E; Rényi-Vámos, F; Szabó, Z

1989-01-01

168

Laparoscopic treatment of celiac axis compression syndrome (CACS) and hiatal hernia: Case report with bleeding complications and review?  

PubMed Central

INTRODUCTION Median arcuate ligament (MAL) malposition is a rare cause of celiac axis compression syndrome (CACS) or Dunbar syndrome. PRESENTATION OF CASE A 26-year-old female presented with severe postprandial epigastric pain, weight loss, heartburn and regurgitation unresponsive to medical therapy. CT angiography and duplex ultrasound demonstrated the MAL crossing anterior to the celiac artery (CA). Reconstructions demonstrated CA compression, while the superior mesenteric artery (SMA) was normal. The MAL was laparoscopically divided, releasing the celiac axis. A concomitant Nissen fundoplication was performed. At 3-months follow-up, the CT-scan demonstrated no evidence of CACS with complete symptom resolution. DISCUSSION Dunbar's syndrome can be treated with endovascular surgery, laparoscopic MAL division or vascular surgery.Six anatomical and morphologic variations of aortic and esophageal hiatus are described. The result of the analysis of these anatomical data leads to the conclusion that hiatus hernia, Dunbar's syndrome and GERD have a common etiopathogenesis and physiopathology. CONCLUSION Laparoscopic treatment is useful and feasible in centers with experience in majorlaparoscopic surgery with reduced invasiveness, better cosmetic effect and shorter postoperative course. PMID:23973901

di Libero, Lorenzo; Varricchio, Antonio; Tartaglia, Ernesto; Iazzetta, Igino; Tartaglia, Alberto; Bernardo, Antonella; Bernardo, Rosanna; Triscino, Giovangiuseppe; Conte, Domenico Lo

2013-01-01

169

Metformin reduces pregnancy complications without affecting androgen levels in pregnant polycystic ovary syndrome women: results of a randomized study  

Microsoft Academic Search

BACKGROUND: Investigation of a possible effect of metformin on androgen levels in pregnant women with poly- cystic ovary syndrome (PCOS). METHODS: A prospective, randomized, double-blind, placebo-controlled pilot study was conducted. Forty pregnant women with PCOS received diet and lifestyle counselling and were random- ized to either metformin 850 mg twice daily or placebo. Primary outcome measures were changes in serum

E. Vanky; R. Heimstad; K. J. Fougner; P. Romundstad; S. M. Carlsen

2004-01-01

170

[Papillary fibroelastoma of the left ventricular outflow tract complicated with acute coronary syndrome;report of a case].  

PubMed

We experienced a case of papillary fibroelastoma (PFE) that was incidentally diagnosed along with acute coronary syndrome( ACS). An 83-year-old female with paralysis of the left leg was diagnosed with acute coronary syndrome(ACS) based on an increased level of cardiac troponin I and ST elevation in the chest lead on electrocardiogram(ECG). On an echocardiogram, the patient was found to have a mobile mass that was likely to be a cardiac tumor. Coronary angiography showed 99% stenosis in the middle left anterior descending coronary artery. We performed emergent coronary aortic bypass graft(CABG) and excision of the tumor. The tumor was attached to the left ventricular outflow tract wall beneath the right coronary cusp with a sea anemone appearance. The histopathological findings revealed a papillary fibroelastoma. The patient's postoperative course was uneventful. PMID:25201373

Yamane, Yoshitaka; Hiraoka, Toshifumi; Morimoto, Hironobu; Mukai, Shogo

2014-09-01

171

Late onset ulcerative colitis complicating a patient with Budd-Chiari syndrome: a case report and review of the literature.  

PubMed

We report a case of a 33-year-old female patient with Budd-Chiari syndrome because of polycythemia vera. A transjugular intrahepatic portal-systemic shunt was performed because of refractory ascites 7 months after diagnosis. She had a stable hepatic function receiving anticoagulants until 3 years later when she presented with bloody diarrheas, liver function deterioration with prolonged prothrombin time and hypoalbuminemia, encephalopathy, and ascites. Colonoscopy revealed ulcerative pancolitis and the patient was treated with corticosteroids and antibiotics. Hepatic function was stabilized in parallel to controlling ulcerative colitis and the patient is in good health until now receiving maintenance therapy for ulcerative colitis and anticoagulants for Budd-Chiari syndrome. PMID:19060635

Vassiliadis, Themistoklis; Mpoumponaris, Alexander; Giouleme, Olga; Hatzidakis, Adam; Patsiaoura, Kalliopi; Zezos, Petros; Vakalopoulou, Sofia; Kargiotis, Konstantinos; Gkisakis, Dimitrios; Katsinelos, Panagiotis; Evgenidis, Nikolaos

2009-01-01

172

Sjögren's Syndrome  

MedlinePLUS

... syndrome sometimes develops as a complication of another autoimmune disorder. Symptoms vary in type and intensity, but many ... syndrome is not known, but it is an autoimmune disorder. People with this disease have abnormal proteins in ...

173

The efficacy of therapeutic plasmapheresis for the treatment of fatal hemophagocytic syndrome: two case reports.  

PubMed

A potentially fatal hemophagocytic syndrome (HPS) has been noted in patients with reactive HPS. We describe 2 patients with reactive HPS treated with a regimen of therapeutic plasmapheresis and evaluate the efficacy of plasmapheresis for fatal HPS. Case 1 was a 31 year-old woman who had been treated for systemic lupus erythematosus (SLE) with corticosteroid hormones and immunosuppressants. She presented with persistent leukopenia and thrombocytopenia with spiking fever. She had an elevated level of serum ferritin, liver dysfunction, coagulopathy, and plasma inflammatory cytokines. Her bone marrow smear disclosed numerous hemophagocytosis of histiocytes. She was administered therapeutic plasmapheresis with total plasma exchange by fresh frozen plasma. There was an immediate and prominent decrease of cytokines, and she completely recovered. Case 2 was a 34 year-old woman who had been receiving high doses of corticosteroids and plasmapheresis for severe Stevens-Johnson's syndrome. After 18 months, she presented with physical and laboratory findings resembling lupus-like conditions and was administered high doses of corticosteroids and immunosuppressants. Human parvovirus B19 infection was detected by IgM and IgG antibodies and viral DNA from a bone marrow sample; moreover, a bone marrow smear disclosed findings of HPS. Repeated therapeutic plasmapheresis was effective for improving her symptoms and laboratory abnormalities; however, she suffered from septic methicilline resistant staphylococcus aureus infection and finally died of a brain hemorrhage resulting from disseminated intravascular coagulation (DIC). PMID:10227760

Matsumoto, Y; Naniwa, D; Banno, S; Sugiura, Y

1998-11-01

174

Neurologic complications of carcinoid.  

PubMed

We reviewed the records of all patients treated for carcinoid tumors at Memorial Sloan-Kettering Cancer Center from 1974 through 1984. Of 219 patients, 90 developed metastatic complications, and of these, 36 developed neurologic complications. Metastases, the most common neurologic complication, included epidural spinal cord compression (14 patients), intracranial metastases (13 patients), leptomeningeal metastases (1 patient), and peripheral nerve lesions (5 patients). Nonmetastatic complications were hepatic encephalopathy (six patients), herpes zoster infection (two patients), cerebral infarction due to septic emboli (one patient), superior sagittal sinus thrombosis (one patient), and carcinoid myopathy (one patient). The carcinoid syndrome was seen in eight patients (4%). Response of neurologic metastases to conventional radiation therapy was usually favorable. We conclude that (1) the frequency and type of neurologic complications associated with carcinoid tumors are similar to those seen with other systemic cancers; (2) CNS metastases are relatively common in patients with metastatic carcinoid (29%); and (3) the carcinoid syndrome is less common than CNS metastasis. PMID:3703281

Patchell, R A; Posner, J B

1986-06-01

175

In-hospital complications and 1-year outcome of acute coronary syndrome in patients with hypertension: findings from the 2nd Gulf Registry of Acute Cardiac Events.  

PubMed

Using data from the 2nd Gulf Registry of Acute Coronary Events (Gulf RACE-2) in 2008-09 we investigated the in-hospital complications and 1-year outcome of acute coronary syndrome (ACS) in patients with systemic hypertension from 6 Gulf countries. Of 7847 consecutive patients admitted with ACS, 3746 (47.7%) had hypertension. Hypertension was more prevalent in women, in Arabs than non-Arabs and in older age groups. Patients with hypertension were more likely than those without hypertension to present with dyspnoea and advanced Killip class. Among hypertensive patients, the mortality rate was higher only among those admitted with ST-elevation myocardial infarction. After adjustment for baseline variables, hypertension was an independent predictive factor for heart failure (OR = 1.31) and stroke (OR = 2.47). here were no significant differences in mortality in hypertensive ACS patients when stratified by sex, age or ethnicity. PMID:23057382

Ali, W M; Al Habib, K F; Hersi, A; Asaad, N; Sulaiman, K; Al-Shiek-Ali, A; Al Suwaidi, J

2012-09-01

176

Fulminant systemic capillary leak syndrome due to C1 inhibitor deficiency complicating acute dermatomyositis: a case report  

PubMed Central

Introduction Dermatomyositis is a chronic inflammatory disorder characterized by muscular and dermatologic symptoms with variable internal organ involvement. This is the first report on a patient with acute dermatomyositis and fulminant systemic capillary leak syndrome. Case presentation A 69-year-old Caucasian woman with chronic dermatomyositis presented with clinical signs of severe hypovolemic shock and pronounced hemoconcentration (hematocrit, 69%). Her colloid osmotic pressure was 4.6mmHg. Following a bolus dose of prednisolone (500mg), fluid resuscitation was initiated. During volume loading, anasarca and acute respiratory distress rapidly developed. Echocardiography revealed an underfilled, hypokinetic, diastolic dysfunctional left ventricle with pericardial effusion but no signs of tamponade. Despite continued fluid resuscitation and high-dosed catecholamine therapy, the patient died from refractory shock 12 hours after intensive care unit admission. A laboratory analysis of her complement system suggested the presence of C1 inhibitor deficiency as the cause for systemic capillary leakage. The post-mortem examination revealed bilateral pleural, pericardial and peritoneal effusions as well as left ventricular hypertrophy with patchy myocardial fibrosis. Different patterns of endomysial/perimysial lymphocytic infiltrations adjacent to degenerated cardiomyocytes in her myocardium and necrotic muscle fibers in her right psoas major muscle were found in the histological examination. Conclusions This case report indicates that acute exacerbation of chronic dermatomyositis can result in a fulminant systemic capillary leak syndrome with intense hemoconcentration, hypovolemic shock and acute heart failure. In the presented patient, the cause for diffuse capillary leakage was most probably acquired angioedema, a condition that has been associated with both lymphoproliferative and autoimmunologic disorders. PMID:24467750

2014-01-01

177

Vaginoperineal Fistula as a Complication of Perianal Surgery in a Patient with Sj?gren's Syndrome: A Case Report  

PubMed Central

Forty-seven-year-old woman with Sjögren's syndrome had been operated on because of transsphincteric perianal fistula secondary to perianal abscess. Vaginal wall injury occurred during the course of the operation and injured tissue was repaired primarily. Three months later, patient suffered from the recurrence of perianal fistula symptoms and fistulectomy was performed once again under antibiotic suppression. Several months later, perineal discharge continued, and, therefore, patient was admitted to the hospital for the third time and a fistulotomy was performed. Two months after the third operation, patient was admitted with leukorrhea and a perineovaginal fistula was detected. This time, not only her surgical problem but also her immune system disorder was considered in the preoperative workup. Then, patient was hospitalized for the fourth time and “fistulectomy/perineoplasty” was performed successfully. We believe that patients with autoimmune disorders with or without medical treatment may have healing problems during the course of surgical processes and therefore such medical problems must be taken into consideration by the surgeons. PMID:25295212

Beksac, Kemal; Turgal, Mert; Aran, Omer; Beksac, M. Sinan

2014-01-01

178

Neurological Complications of Transplantation  

PubMed Central

Hematopoietic cell transplantation (HCT) is the preferred treatment for an expanding range of neoplastic and nonmalignant conditions. Increasing numbers of solid organ transplantations (SOTs) add an additional population of immunosuppressed patients with multiple potential neurological problems. While the spectrum of neurological complications varies with conditioning procedure and hematopoietic cell or solid organ source, major neurological complications occur with all transplantation procedures. This 2 part review emphasizes a practical consultative approach to central and peripheral nervous system problems related to HCT or SOT with clinical and neuroimaging examples from the authors’ institutional experience with the following conditions: the diversity of manifestations of common infections such as varicella zoster virus, Aspergillus, and progressive multifocal leukoencephalopathy (PML), drug therapy-related complications, stroke mechanisms, the spectrum of graft versus host disease (GVHD), and neurologically important syndromes of immune reconstitution inflammatory syndrome (IRIS), posterior reversible encephalopathy syndrome (PRES), and posttransplantation lymphoproliferative disorder (PTLD). These complications preferentially occur at specific intervals after HCT and SOT, and neurological consultants must recognize an extensive spectrum of syndromes in order to effect timely diagnosis and expedite appropriate treatment. PMID:23983885

Pruitt, Amy A.; Graus, Francesc; Rosenfeld, Myrna R.

2013-01-01

179

Pregnancy Complications  

MedlinePLUS

... Emergency Preparedness and Response Pregnant Women and Flu Pregnancy Complications On this Page Before Pregnancy During Pregnancy ... to decrease the risk of pregnancy complications. Before Pregnancy Make sure to talk to your doctor about ...

180

LASIK Complications  

Microsoft Academic Search

Laser in situ keratomileusis (LASIK) is a rapidly evolving ophthalmic surgical procedure. Several anatomic and refractive complications have been identified. Anatomic complications include corneal flap abnormalities, epithelial ingrowth, and corneal ectasia. Refractive complications include unexpected refractive outcomes, irregular astigmatism, decentration, visual aberrations, and loss of vision. Infectious keratitis, dry eyes, and diffuse lamellar keratitis may also occur following LASIK. By

Samir A Melki; Dimitri T Azar

2001-01-01

181

Gastrointestinal complications of immunodeficiency syndromes.  

PubMed

Patients with B cell deficiency have a high incidence of prolonged Giardia lamblia infection of the gastrointestinal tract that causes symptoms of malabsorption with villus flattening. The changes are reversible with therapy directed against Giardia. There is a high incidence of pernicious anaemia in patients with agammaglobulinaemia. Those with abnormal B lymphocytes tend to develop lymphoid nodular hyperplasia. Gastrointestinal disease is rare in boys with X-linked agammaglobulinaemia when compared with adults with the 'acquired' or common variable form of the disease. T cell deficiency results in intractable diarrhoea and monilial infection of the gastrointestinal tract. PMID:346324

Katz, A J; Rosen, F S

182

[Neurological complications in uremia].  

PubMed

Neurological complications due to the uremic state or hemodialysis, contribute to the important cause of mortality in patients with uremia. Despite continuous advances in uremic treatment, many neurological complications of uremia, like uremic encephalopathy, peripheral neuropathy and myopathy fail to fully respond to hemodialysis. Moreover, hemodialysis or kidney transplantation may even induce neurological complications. Hemodialysis can directly or indirectly be associated with Wernicke's encephalopathy, dialytic dementia, dysequilibrium syndrome, cerebrovascular accidents, osmotic myelinolysis and mononeuropathy. Renal transplantation can give rise to rejection encephalopathy and acute femoral neuropathy. The use of immunosuppressive drugs after renal transplantation can cause reversible posterior leukoencephalopathy encephalopathy. The clinical, pathophysiological and therapeutical aspects of central nervous system, peripheral nervous system and myopathy complications in uremia are reviewed. PMID:18686653

Fong, Chin-Shih

2008-06-01

183

Vascular complications (splenic and hepatic artery aneurysms) in the occipital horn syndrome: report of a patient and review of the literature  

Microsoft Academic Search

We report an 18-year-old boy with occipital horn syndrome who developed aneurysms of the splenic and hepatic arteries. Occipital\\u000a horn syndrome, also called X-linked cutis laxa or Ehlers-Danlos syndrome (EDS) type IX, is characterised by a skeletal dysplasia\\u000a which includes occipital horns, broad clavicles, deformed radii, ulnae and humeri, narrow rib cage, undercalcified long bones\\u000a and coxa valga. Distinctive features

Hans-Joachim Mentzel; Jörg Seidel; Susanna Vogt; Lothar Vogt; Werner A. Kaiser

1999-01-01

184

[Type 2 diabetes complications].  

PubMed

People with type 2 diabetes are at increased risk of many complications, which are mainly due to complex and interconnected mechanisms such as hyperglycemia, insulino-resistance, low-grade inflammation and accelerated atherogenesis. Cardi-cerebrovascular disease are frequently associated to type 2 diabetes and may become life threatening, particularly coronaropathy, stroke and heart failure. Their clinical picture are sometimes atypical and silencious for a long time. Type 2 diabetes must be considered as an independent cardiovascular risk factor. Nephropathy is frequent in type 2 diabetes but has a mixed origin. Now it is the highest cause of end-stage renal disease. Better metabolic and blood pressure control and an improved management of microalbuminuria are able to slowdown the course of the disease. Retinopathy which is paradoxically slightly progressive must however be screened and treated in these rather old patients which are globally at high ophthalmologic risk. Diabetic foot is a severe complication secondary to microangiopathy, microangiopathy and neuropathy. It may be considered as a super-complication of several complications. Its screening must be done on a routine basis. Some cancer may be considered as an emerging complication of type 2 diabetes as well as cognitive decline, sleep apnea syndrome, mood disorders and bone metabolism impairments. Most of the type 2 diabetes complications may be prevented by a strategy combining a systematic screening and multi-interventional therapies. PMID:23528336

Schlienger, Jean-Louis

2013-05-01

185

Arterial thoracic outlet syndrome  

Microsoft Academic Search

Thoracic outlet syndrome (TOS) is a well described upper extremity disorder comprising neurovascular complications caused by thoracic outlet compression. By far neurogenic thoracic outlet syndrome is the most common manifesta- tion of this disorder. Arterial complications of thoracic outlet compression are much rarer, accounting for less than 5% of all operations performed for TOS. Major arterial degenerative and thromboembolic complications

Gerald M. Patton

186

Complicated grief in late life  

PubMed Central

Complicated grief (CG) is a syndrome that affects 10% to 20% of grievers regardless of age, although proportionally more will face the death of loved ones in late life, CG is characterized by preoccupying and disabling symptoms that can persist for decades such as an inability to accept the death, intense yearning or avoidance, frequent reveries, deep sadness, crying, somatic distress, social withdrawal, and suicidal ideation. This syndrome is distinct from major depression and post-traumatic stress disorder, but CG maybe comorbid with each. This communication will focus on the impact of CG in late life (over age 60) and will include a case vignette for illustrating complicated grief therapy. PMID:22754292

Miller, Mark D.

2012-01-01

187

[Diabetes and cardiovascular complications].  

PubMed

The prevalence of obesity and diabetes is increasing dramatically. Currently, 800,000 patients are suffering from diabetes mellitus in Austria. Chronic hyperglycemia results in micro- and macrovascular complications, which reduce life expectancy up to 8 years. Furthermore, diabetes is among the most important risk factors for premature atherosclerosis and coronary artery disease. The incidence of coronary artery disease in diabetics is relatively high with about 146 cases per 10,000 patient years. Apart, it could be demonstrated that the presence of diabetes mellitus worsens the prognosis after an acute coronary syndrome. Considering ischemic stroke, the situation is nearly the same, as it is known that diabetes mellitus increases the risk for ischemic stroke events up to 5 times. Beside the macrovascular complications, microvascular complications like diabetic retinopathy, diabetic nephropathy and diabetic neuropathy also play a critical role. Retinopathy can be detected in nearly every patient after a diabetes duration of 20 years. Diabetic nephropathy, which is a major complication of diabetes mellitus, accounts for 19% of end stage renal disease. Microalbuminuria, which is an early marker of diabetic nephropathy, can be found in 30% of the patients after 10 years of diabetes. Due to the severity of the diabetic complications an early intensified antidiabetic treatment is highly important for the prevention of micro- and macrovascular events. PMID:20229155

Resl, Michael; Clodi, Martin

2010-01-01

188

Treatment of complications of parotid gland surgery  

PubMed Central

Summary Although several reports in the literature have documented the surgical technique, and the oncological outcome achieved with parotidectomy, only a few articles have described the complications of parotid gland surgery and their management. Several complications have been reported in parotid surgery. We re-classified the complications of parotidectomy in intra-operative and post-operative (early and late). The commonest complications after parotidectomy are temporary or permanent facial palsy and Frey’s syndrome. PMID:16450773

Marchese-Ragona, R; De Filippis, C; Marioni, G; Staffieri, A

2005-01-01

189

The Evaluation of Pain Complicating Primary Disabling Disease  

Microsoft Academic Search

Pain syndromes complicate many diseases that cause disability. Not uncom- monly, more than one pain syndrome may follow a disease process: The literature describes somatic incisional pain, neurogenic phantom pain, and neurogenic neu- roma pain following amputation (1). Precisely defined, as well as generalized, pain syndromes exist: For example, thalamic, or central pain syndrome, charac- terized by burning hemibody pain

Donna Marie Bloodworth

190

Pulmonary complications of hemoglobinopathies.  

PubMed

Hemoglobinopathies are diseases caused by genetic mutations that result in abnormal, dysfunctional hemoglobin molecules or lower levels of normal hemoglobin molecules. The most common hemoglobinopathies are sickle cell disease (SCD) and the thalassemias. In SCD, an abnormal hemoglobin alters the erythrocyte, causing a chronic hemolytic anemia, which can lead to pulmonary parenchymal damage and impaired vascular function. Pulmonary complications of SCD include the acute chest syndrome (ACS), reactive airways disease, pulmonary hypertension (PH), and pulmonary fibrosis. Episodes of ACS and the development of PH both increase the risk of death in patients with SCD. Both ? and ? thalassemia are characterized by impaired production of hemoglobin subunits, and severity of disease varies widely. Although screening studies suggest that PH is a common complication for patients with thalassemia, its impact on survival is unknown. Understanding the pathogenesis, diagnostic options, and prevention and treatment strategies for such complications is critical for clinicians who care for these patients. In this review, we discuss the mechanisms and clinical presentation of pulmonary complications associated with hemoglobinopathies, with a focus on recent advances in pathogenesis and treatment. PMID:20923801

Vij, Rekha; Machado, Roberto F

2010-10-01

191

[Acute hepatic vascular complications].  

PubMed

Acute hepatic vascular complications are rare. Acute portal vein thrombosis (PVT) and the Budd-Chiari syndrome (BSC) are the leading causes. Coagulopathy and local factors are present in up to 80% of cases. Diagnosis is established by colour-coded Doppler sonography, contrast-enhanced computed tomography or magnetic resonance imaging. Patients with acute PVT present with abdominal pain and disturbed intestinal motility. In the absence of cirrhosis anticoagulation with heparin is established followed by oral anticoagulation. In severe cases, surgical thrombectomy or transjugular thrombolysis with stent shunt may be necessary. Acute or fulminant BCS may require emergency liver transplantation or a transjugular intrahepatic portosystemic stent shunt, if patients present with acute liver failure. Milder cases receive anticoagulation for thrombolysis of occluded hepatic veins. Sinusoidal obstruction syndrome (SOS) is diagnosed after total body irradiation or chemotherapy, the term SOS replacing the former veno-occlusive disease. The treatment of congenital vascular malformations, complications in the setting of OLTX as well as patients with hepatic involvement of hereditary hemorrhagic telangiectasia requires significant expertise in a multidisciplinary approach. PMID:21667100

Ochs, A

2011-07-01

192

Paroxysmal Nocturnal Hemoglobinuria Following Alemtuzumab Immunosuppressive Therapy for Myelodysplastic Syndrome and Complicated by Recurrent Life-Threatening Thrombosis Despite Anticoagulation: Successful Intervention with Eculizumab and Fondaparinux  

PubMed Central

The pathogenesis of paroxysmal nocturnal hemoglobinuria (PNH) is not fully understood. We report a patient with myelodysplastic syndrome who developed symptomatic PNH following treatment with alemtuzumab. A small PNH clone, identified prior to alemtuzumab, expanded resulting in hemolytic anemia and recurrent CNS thromboses despite anticoagulation. Remission was achieved with eculizumab and fondaparinux therapy. Alemtuzumab has been associated with the development of glycosylphosphotidylinositol negative cells, but its clinical significance has been unclear. Our case emphasizes its potential clinical importance. Future studies are necessary to expand our understanding of this rare disease entity and improve its management. PMID:19836075

Cheng, Kit L.; Brody, Judith; Warshall, Craig E.; Sloand, Elaine M.; Allen, Steven L.

2009-01-01

193

Osteo-odonto-keratoprosthesis - a maxillofacial perspective.  

PubMed

The OOKP (osteo-odonto-keratoprosthesis) is the treatment of choice for conditions like Stevens-Johnson syndrome, ocular cicatricial pemphigoid, trachoma, multiple failed grafts and chemical burns which are not amenable to penetrating keratoplasty. The OOKP is an autograft which replaces the cornea with a polymethacrylate cylinder mounted on a tooth-bone complex. The aim of this paper was to retrospectively analyze the records in 26 patients undergoing OOKP surgery between 2007 and 2011. The paper describes our experience with the procedure, with emphasis on its oral and maxillofacial aspects and management of associated complications. The aetiology of blindness in 23 patients was Stevens-Johnson's syndrome and chemical burns in three. Twenty-two patients had their maxillary canines, two had mandibular canines and two had maxillary first premolars as the choice of donor tooth. An oroantral fistula developed in four patients. One patient needed to undergo a surgical procedure for closure of the same. Roots of adjacent teeth were exposed in 12 patients. Twenty-four patients underwent both stages of the procedure with 19 being visually rehabilitated successfully. There was no improvement in the vision of four patients. One patient was lost to follow-up. Two patients have yet to undergo Stage 2. PMID:22425501

Narayanan, Vinod; Nirvikalpa, Natarajan; Rao, Srinivas K

2012-12-01

194

Abernethy malformation type II with nephrotic syndrome and other multisystemic presentation: an illustrative case for understanding pathogenesis of extrahepatic complication of congenital portosystemic shunt.  

PubMed

Abernethy malformation, an extrahepatic congenital portosystemic shunt, is more often diagnosed based on associated cardiac or pulmonary malformation. Although predominately a pediatric diagnosis, "late diagnoses" in adulthood have been reported especially in type II malformations that involve only a partial shunt of portal circulation directly into the inferior vena cava. Aside from the cardiac-related presentation, Abernethy malformation is also associated with multiple liver nodules, either benign or malignant, and pulmonary hypertension. In this report, we present immunoglobulin A glomerulonephritis with nephrotic syndrome as a hitherto unrecognized manifestation of this malformation outside the pediatric population, in a patient who also had pulmonary hypertension and multiple liver tumors. We also propose a pathogenetic basis for this multisystemic presentation that includes release into the systemic circulation of unfiltered bacteria, vasoactive substances, and immunoglobulin A-antigen complexes. PMID:23245671

Schaeffer, David F; Laiq, Simin; Jang, Hyun-Jung; John, Rohan; Adeyi, Oyedele A

2013-03-01

195

Endovascular treatment combined with stratified surgery is effective in the management of venous thoracic outlet syndrome complications: a long term ultrasound follow-up study in patients with thrombotic events due to venous thoracic outlet syndrome  

Microsoft Academic Search

Thoracic outlet syndrome (TOS) is caused by compression of peripheral nerves and vascular structures along their course through\\u000a the upper thoracic aperture to the axilla. The aim of our study was to analyze long-term outcomes of different treatments\\u000a stratified by symptom severity. We performed a retrospective analysis of a cohort of 73 consecutive patients treated at our\\u000a institution presenting with

Lenka Skalicka; Jean-Claude Lubanda; Simon Jirat; Petr Varejka; Stanislav Beran; Ondrej Dostal; Pavel Prochazka; Vratislav Mrazek; Ales Linhart

196

A review of important skin disorders occurring in the posttransplantation patient.  

PubMed

Hematopoietic stem cell transplantation continues to be the mainstay of treatment for many hematologic dyscrasias and malignancies, including acute leukemias, lymphomas, and aplastic anemia. There can be significant complications, however, and often these complications are manifested in the skin as an eruption. Common among these are acute and chronic graft-versus-host disease, erythema multiforme, Stevens-Johnson syndrome/toxic epidermal necrolysis, eruption of lymphocyte recovery, staphylococcal scalded skin syndrome, morbiliform drug eruptions, infections, and toxic erythema of chemotherapy. These entities can show significant clinical and histopathologic overlap, yet accurate distinctions among them are critical to initiating appropriate clinical interventions. In this review, we will discuss the key clinical and histopathologic findings in each entity as well as appropriate differential diagnostic entities. PMID:25105934

Sundram, Uma

2014-09-01

197

Pregnancy Complications: Gonorrhea  

MedlinePLUS

... in the community. Home > Pregnancy > Pregnancy Complications > Gonorrhea Pregnancy complications Pregnancy complications may need special medical care. ... the United States. Can gonorrhea cause complications during pregnancy and for your baby? Yes. Gonorrhea can lead ...

198

Pregnancy Complications: Preeclampsia  

MedlinePLUS

... in the community. Home > Pregnancy > Pregnancy Complications > Preeclampsia Pregnancy complications Pregnancy complications may need special medical care. ... health care provider right away. What health and pregnancy complications can preeclampsia cause? Without treatment, preeclampsia can ...

199

SEVERE NEUROLOGICAL COMPLICATIONS OF CHICKENPOX Report of four cases  

Microsoft Academic Search

Neurological complications caused by chickenpox are estimated as approximately 0.01%- 0.03%. Frequent complications related to central nerve system involvement are cerebellar ataxia and encephalitis, and rare complications are transverse myelitis, aseptic meningitis, Guillian- Barré syndrome, meningoencephalitis, ventriculitis, optic neuritis, post-hepatic neuralgia, herpes zoster ophalmicus, delayed controlateral hemiparesis, peripheral motor neuropathy, cerebral angitis, Reye syndrome and facial paralysis. In present study,

Cahide Yilmaz; Hüseyin Çaksen

200

Neurological Complications of Solid Organ Transplantation  

PubMed Central

Solid organ transplantation (SOT) is the preferred treatment for an expanding range of conditions whose successful therapy has produced a growing population of chronically immunosuppressed patients with potential neurological problems. While the spectrum of neurological complications varies with the type of organ transplanted, the indication for the procedure, and the intensity of long-term required immunosuppression, major neurological complications occur with all SOT types. The second part of this 2-part article on transplantation neurology reviews central and peripheral nervous system problems associated with SOT with clinical and neuroimaging examples from the authors’ institutional experience. Particular emphasis is given to conditions acquired from the donated organ or tissue, problems specific to types of organs transplanted and drug therapy-related complications likely to be encountered by hospitalists. Neurologically important syndromes such as immune reconstitution inflammatory syndrome (IRIS), posterior reversible encephalopathy syndrome (PRES), and posttransplantation lymphoproliferative disorder (PTLD) are readdressed in the context of SOT. PMID:24167649

Pruitt, Amy A.; Graus, Francesc; Rosenfeld, Myrna R.

2013-01-01

201

Treatment of toxic epidermal necrolysis with intravenous immunoglobulin: a series of three cases.  

PubMed

Stevens-Johnson's syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening dermatoses, that lead to keratinocyte apoptosis induced by interactions between Fas (cell death receptor) and soluble Fas-ligand, present in serum of Stevens-Johnson's syndrome / toxic epidermal necrolysis patients. Anti-Fas antibodies in intravenous immunoglobulin (IVIG) would block the apoptosis cascade. Three cases of toxic epidermal necrolysis occurred in one male and two female patients, after use of allopurinol, leprosy multidrug therapy concomitant with dipyrone, and diclofenac. The cases were treated with intravenous immunoglobulin 2-3 mg/kg and prednisone 20-50 mg/day. The interruption of new lesions outbreak and reepithelization were extremely fast after the use of intravenous immunoglobulin, without adverse effects. Controlled studies are needed to confirm the efficacy of intravenous immunoglobulin in Stevens-Johnson's syndrome / toxic epidermal necrolysis, but the results seem promising. PMID:22714769

Comparin, Cristiane; Hans Filho, Günter; Takita, Luiz Carlos; Costa, Nayara de Castro Wiziack; Nascimento, Roberta Ayres Ferreira do; Nanni, Lidiane de Oliveira Costa

2012-01-01

202

Pregnancy Complications: Genital Herpes  

MedlinePLUS

... Home > Pregnancy > Pregnancy Complications > Genital herpes and pregnancy Pregnancy complications Pregnancy complications may need special medical care. ... been added to your dashboard . Genital herpes and pregnancy Genital herpes is a sexually transmitted disease (also ...

203

Pregnancy Complications: Chlamydia  

MedlinePLUS

... in the community. Home > Pregnancy > Pregnancy Complications > Chlamydia Pregnancy complications Pregnancy complications may need special medical care. ... younger than 25. Can chlamydia cause problems during pregnancy? Yes. If you get it before or during ...

204

Pregnancy Complications: Syphilis  

MedlinePLUS

... in the community. Home > Pregnancy > Pregnancy Complications > Syphilis Pregnancy complications Pregnancy complications may need special medical care. ... syphilis each year. Can syphilis cause problems during pregnancy and for your baby? Yes. You can pass ...

205

[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].  

PubMed

Disseminated intravascular coagulopathy (DIC) is characterized as activation of the clotting system resulting in fibrin thrombi, gradually diminishing levels of clotting factors with increased risk of bleeding. Basically two types of DIC are distinguished: (1) chronic (compensated) - with alteration of laboratory values and (2) acute (non-compensated) - with severe clinical manifestations: bleeding, shock, acute renal failure (ARF), transient focal neurologic deficit, delirium or coma. Chronic DIC related to metastatic neoplasia is caused by pancreatic, gastric or prostatic carcinoma in most of the cases. Incidence rate of DIC is 13-30% in prostate cancer, among those only 0.4-1.65% of patients had clinical signs and symptoms of DIC. In other words, chronic DIC is developed in one of eight patients with prostate cancer. DIC is considered as a poor prognostic factor in prostatic carcinoma. The similar clinical and laboratory findings of TTP-HUS (thrombotic thrombocytopenic purpura - hemolytic uremic syndrome) and DIC makes it difficult to differentiate between them. A 71 years old male patient with known chronic obstructive pulmonary disease, benign prostatic hyperplasia, significant carotid artery stenosis, gastric ulcer and alcoholic liver disease was admitted to another hospital with melena. Gastroscopy revealed intact gastric mucosa and actually non-bleeding duodenal ulcer covered by clots. Laboratory results showed hyperkalemia, elevated kidney function tests, indirect hyperbilirubinemia, increased liver function tests, leukocytosis, anemia, thrombocytopenia and elevated international normalized ratio (INR). He was treated with saline infusions, four units of red blood cells and one unit of fresh frozen plasma transfusions. Four days later he was transported to our Institution with ARF. Physical examination revealed dyspnoe, petechiae, hemoptoe, oliguria, chest-wall pain and aggressive behavior. Thrombocytopenia, signs of MAHA (fragmentocytes and helmet cells 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 ?g/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. PMID:21163766

Deme, Dániel; Ragán, Márton; Kalmár, Katalin; Kovács, Lajos; Varga, Erzsébet; Varga, Tünde; Rakonczai, Ervin

2010-12-01

206

Sipple syndrome and pregnancy.  

PubMed

Sipple syndrome is an unusual entity during pregnancy. To our knowledge, this is the second case report of a pregnant patient with signs of Sipple syndrome. During pregnancy, this syndrome may present unusual complications, which require informed physician expertise for appropriate management. Our patient had a strong family history for Sipple syndrome, which is considered to be transmitted as an autosomal dominant. Thus, other family members, including the patient's offspring must be assessed frequently during their lifetime for any of the developing manifestations of this entity. Genetic counseling should be included in the management of all patients with Sipple syndrome. PMID:946212

Harris, R E; Gilstrap, L C; Brekken, A L

1976-03-15

207

Hepatorenal Syndrome  

PubMed Central

Hepatorenal syndrome (HRS) is a serious complication of liver cirrhosis with critically poor prognosis. The pathophysiological hallmark is severe renal vasoconstriction, resulting from complex changes in splanchnic and general circulations as well as systemic and renal vasoconstrictors and vasodilators. Rapid diagnosis and management are important, since recent treatment modalities including vasoconstrictor therapy can improve short-term outcome and buy time for liver transplantation, which can result in complete recovery. PMID:17603637

Ng, Charles KF; Chan, Michael HM; Tai, Morris HL; Lam, Christopher WK

2007-01-01

208

Neuroacanthocytosis Syndromes  

PubMed Central

Neuroacanthocytosis (NA) syndromes are a group of genetically defined diseases characterized by the association of red blood cell acanthocytosis and progressive degeneration of the basal ganglia. NA syndromes are exceptionally rare with an estimated prevalence of less than 1 to 5 per 1'000'000 inhabitants for each disorder. The core NA syndromes include autosomal recessive chorea-acanthocytosis and X-linked McLeod syndrome which have a Huntington´s disease-like phenotype consisting of a choreatic movement disorder, psychiatric manifestations and cognitive decline, and additional multi-system features including myopathy and axonal neuropathy. In addition, cardiomyopathy may occur in McLeod syndrome. Acanthocytes are also found in a proportion of patients with autosomal dominant Huntington's disease-like 2, autosomal recessive pantothenate kinase-associated neurodegeneration and several inherited disorders of lipoprotein metabolism, namely abetalipoproteinemia (Bassen-Kornzweig syndrome) and hypobetalipoproteinemia leading to vitamin E malabsorption. The latter disorders are characterized by a peripheral neuropathy and sensory ataxia due to dorsal column degeneration, but movement disorders and cognitive impairment are not present. NA syndromes are caused by disease-specific genetic mutations. The mechanism by which these mutations cause neurodegeneration is not known. The association of the acanthocytic membrane abnormality with selective degeneration of the basal ganglia, however, suggests a common pathogenetic pathway. Laboratory tests include blood smears to detect acanthocytosis and determination of serum creatine kinase. Cerebral magnetic resonance imaging may demonstrate striatal atrophy. Kell and Kx blood group antigens are reduced or absent in McLeod syndrome. Western blot for chorein demonstrates absence of this protein in red blood cells of chorea-acanthocytosis patients. Specific genetic testing is possible in all NA syndromes. 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. PMID:22027213

2011-01-01

209

Pregnancy Complications/Health Problems Complication Explanation  

E-print Network

1 Pregnancy Complications/Health Problems Complication Explanation Preterm Labor Labor that starts Premature Rupture of Membranes (PROM) Amniotic fluid bag surrounding baby ruptures or leaks before labor in pregnancy and goes away after delivery. Preeclampsia (hypertension) Pregnancy induced high blood pressure

Stromswold, Karin

210

Pfeiffer syndrome.  

PubMed

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. PMID:16740155

Vogels, Annick; Fryns, Jean-Pierre

2006-01-01

211

Complications of Diabetes and Their Implications for Service Providers.  

ERIC Educational Resources Information Center

This article presents information on the complications of both Type I and Type II diabetes and the implications for the rehabilitation of persons with diabetes and visual impairment. Topics covered include retinopathy, cataracts, glaucoma, peripheral neuropathy, carpal tunnel syndrome, diabetic hand syndrome, neuropathy of the autonomic nervous…

Ponchillia, S. V.

1993-01-01

212

Neurologic complications of craniovertebral dislocation.  

PubMed

Craniovertebral dislocation is uncommon, but its diagnosis is important taking into account the potential severity of the neurologic complications. A number of causes are known; the most common are Down syndrome, rheumatoid arthritis, Paget's disease, other metabolic bone diseases, and craniocervical trauma. Down's syndrome is a relatively common clinical condition but craniovertebral subluxation is only observed in a small percentage of patients. About half of all cervical spine injuries affect the atlanto-occipital region and C2 vertebra. In rheumatoid arthritis, craniocervical dislocation occurs in up to 40% of patients with severe disease. In Paget's disease, involvement of the craniovertebral region occurs in about 30% of all cases. The clinical neurologic syndrome is characterized by local pain, features of upper spinal cord and medullary compression, positive Lhermitte phenomenon, syncope associated with neck flexion, vertebral artery obstruction or dissection leading to stroke, and asymmetrical lower cranial nerve palsies. Neuroimaging is essential to confirm the clinical diagnosis and to categorize severity. The treatment of this disorder is usually surgical, but traction and external immobilization is relevant in some cases. Specific conditions may require additional treatments such as radiotherapy, antibiotics, or chemotherapy. PMID:24365311

de Carvalho, Mamede; Swash, Michael

2014-01-01

213

Complications of nephropathia epidemica: three cases.  

PubMed

Haemorrhagic fever with renal syndrome (HFRS) in Scandinavia is called nephropathia epidemica (NE), and is caused by the Puumala-virus, which belongs to the Hanta-virus genus. The clinical course of NE is mostly benign, complications are uncommon, and deaths are rarely observed. We report the cases of three patients who developed serious complications in the course of NE caused by the Puumala-virus. One patient died within 24 h after admission, another developed progressive neuromuscular dysfunction (Guillain-Barré syndrome) which required auxiliary ventilation for several weeks before a slow recovery, and a third patient developed empty sella syndrome with pituitary gland insufficiency. In the first two cases the diagnosis of NE was confirmed by a rapid avidity assay for IgG antibody against Puumala-virus. In the third case the clinical course, and demonstration of NE immunity 16 years later, suggested that NE might have caused the hypopituitarism. Some patients with NE caused by the Puumala-virus may require intensive-care treatment, and the development of late complications such as empty sella syndrome and hypopituitarism should be taken into consideration. PMID:1353521

Forslund, T; Saltevo, J; Anttinen, J; Auvinen, S; Brummer-Korvenkontio, M; Korhonen, A; Poutiainen, M

1992-07-01

214

Gastrointestinal complications of diabetes mellitus  

PubMed Central

Diabetes mellitus affects virtually every organ system in the body and the degree of organ involvement depends on the duration and severity of the disease, and other co-morbidities. Gastrointestinal (GI) involvement can present with esophageal dysmotility, gastro-esophageal reflux disease (GERD), gastroparesis, enteropathy, non alcoholic fatty liver disease (NAFLD) and glycogenic hepatopathy. Severity of GERD is inversely related to glycemic control and management is with prokinetics and proton pump inhibitors. Diabetic gastroparesis manifests as early satiety, bloating, vomiting, abdominal pain and erratic glycemic control. Gastric emptying scintigraphy is considered the gold standard test for diagnosis. Management includes dietary modifications, maintaining euglycemia, prokinetics, endoscopic and surgical treatments. Diabetic enteropathy is also common and management involves glycemic control and symptomatic measures. NAFLD is considered a hepatic manifestation of metabolic syndrome and treatment is mainly lifestyle measures, with diabetes and dyslipidemia management when coexistent. Glycogenic hepatopathy is a manifestation of poorly controlled type 1 diabetes and is managed by prompt insulin treatment. Though GI complications of diabetes are relatively common, awareness about its manifestations and treatment options are low among physicians. Optimal management of GI complications is important for appropriate metabolic control of diabetes and improvement in quality of life of the patient. This review is an update on the GI complications of diabetes, their pathophysiology, diagnostic evaluation and management. PMID:23772273

Krishnan, Babu; Babu, Shithu; Walker, Jessica; Walker, Adrian B; Pappachan, Joseph M

2013-01-01

215

Pulmonary complications after esophagectomy  

Microsoft Academic Search

Background. Pulmonary complications are common in patients who have undergone esophagectomy. There are no good predictive variables for these complications. In addition, the role that preoperative treatment with chemotherapy and radiation may play in postoperative complications remains unclear.Methods. We performed a retrospective review of all patients who underwent esophagectomy by a single surgeon at our institution over a 6-year period.

Christopher E Avendano; Patrick A Flume; Gerard A Silvestri; Lydia B King; Carolyn E Reed

2002-01-01

216

Ultrasound guided selective cervical nerve root block and superficial cervical plexus block for surgeries on the clavicle  

PubMed Central

We report the anaesthetic management of two cases involving surgeries on the clavicle, performed under superficial cervical plexus block and selective C5 nerve root block under ultrasound (US) guidance, along with general anaesthesia. Regional analgesia for clavicular surgeries is challenging. Our patients also had significant comorbidities necessitating individualised approach. The first patient had a history of emphysema, obesity, and was allergic to morphine and hydromorphone. The second patient had clavicular arthritis and pain due to previous surgeries. He had a history of smoking, Stevens-Johnson syndrome, along with daily marijuana and prescription opioid use. Both patients had an effective regional block and required minimal supplementation of analgesia, both being discharged on the same day. Interscalene block with its associated risks and complications may not be suitable for every patient. This report highlights the importance of selective regional blockade and also the use of US guidance for an effective and safe block. PMID:25024480

Shanthanna, Harsha

2014-01-01

217

Review of endophthalmitis following Boston keratoprosthesis type 1.  

PubMed

Endophthalmitis remains one of the most damaging and challenging complications following Boston keratoprosthesis type 1 (KPro) surgery. The authors reviewed the literature from 2001 onward to identify cases of endophthalmitis following KPro surgery and present an additional case of endophthalmitis in a patient with Stevens Johnson syndrome. The prevalence of endophthalmitis between 2001 and 2011 was 5.4%. Gram-positive bacteria are the most common agents responsible for endophthalmitis in this patient population while gram-negative bacteria and fungi are emerging pathogens. Risk factors for endophthalmitis include preoperative diagnosis of cicatricial disease and postoperative infectious keratitis, glaucoma drainage device erosion and non-compliance with antibiotic prophylaxis. Additional studies on the prevention and treatment of endophthalmitis are required to improve the overall prognosis of these patients. PMID:22493035

Robert, Marie-Claude; Moussally, Krystel; Harissi-Dagher, Mona

2012-06-01

218

Risks and complications in rhinoplasty  

PubMed Central

Rhinoplasty is regarded to be associated with many risks as the expectations of patient and physician are not always corresponding. Besides of postoperative deformities many other risks and complications have to be considered. Reduction-rhinoplasty e.g. can cause breathing disturbances which are reported in 70% of all revision-rhinoplasty-patients. One has to be aware however that scars and loss of mucosal-sensation can also give the feeling of a “blocked nose”. The main risks of autogenous transplants are dislocation and resorption, while alloplasts can cause infection and extrusion. In this respect silicone implants can have a complication rate between 5-20%. Less complications are reported with other materials like Gore-Tex. Complications of skin and soft tissues can be atrophy, fibrosis, numbness, cysts originating from displaced mucosa or subcutaneous granulomas caused by ointment material. Postoperative swelling depends mainly on the osteotomy technique. Percutaneous osteotomies cause less trauma, but may result in visible scars. Infections are rare but sometimes life-threatening (toxic-shock-syndrome). The risk is higher, when sinus surgery and rhinoplasty are combined. Osteotomies can also cause injuries of the orbital region. Necrosis of eye-lids by infections and blindness by central artery occlusion are known. There are reports on various other risks like rhinoliquorrhea, brain damage, fistulas between sinus-cavernosus and carotid artery, aneurysms and thrombosis of the cavernous sinus. Discoloration of incisors are possible by damage of vessels and nerves. Rhinoplasty can also become a court-case in dissatisfied patients, a situation that may be called a “typical complication of rhinoplasty”. It can be avoided by proper patient selection and consideration of psychological disturbances. Postoperative deformities are considered as main risks of rhinoplasty, causing revision surgery in 5% to 15% of the cases. The analysis of postoperative deformities allowes the identification of specific risks. The most frequent postoperative deformity is the “pollybeak” when a deep naso-frontal angle, cartilaginous hump and reduced tip projection are present preoperatively. The pollybeak is the indication in about 50% of all revision rhinoplasties. Other frequent postoperative deformities are a pendant and wide nasal tip, retractions of the columella base or irregularities of the nasal dorsum. These deformities are very often combined and caused by a loss of septal support. This is why the stability of the caudal septum in septorhinoplasty is the key for a predictable result. Maintaining the position of the tip and the columella is one of the main issues to avoid typical postoperative deformities. The risks for rhinoplasty-complications can be reduced with increasing experience. A prerequisite is continuing education and an earnest distinction between complication and mistake. PMID:22073084

Rettinger, Gerhard

2008-01-01

219

Cirrhosis and its complications: Evidence based treatment  

PubMed Central

Cirrhosis results from progressive fibrosis and is the final outcome of all chronic liver disease. It is among the ten leading causes of death in United States. Cirrhosis can result in portal hypertension and/or hepatic dysfunction. Both of these either alone or in combination can lead to many complications, including ascites, varices, hepatic encephalopathy, hepatocellular carcinoma, hepatopulmonary syndrome, and coagulation disorders. Cirrhosis and its complications not only impair quality of life but also decrease survival. Managing patients with cirrhosis can be a challenge and requires an organized and systematic approach. Increasing physicians’ knowledge about prevention and treatment of these potential complications is important to improve patient outcomes. A literature search of the published data was performed to provide a comprehensive review regarding the management of cirrhosis and its complications. PMID:24833875

Nusrat, Salman; Khan, Muhammad S; Fazili, Javid; Madhoun, Mohammad F

2014-01-01

220

Transoesophageal Echocardiography Related Complications  

PubMed Central

Summary The application of transesophageal echocardiography (TEE) has been continuously increasing over past several decades. It is usually considered a very safe diagnostic and monitoring device. Though the complications are rare, but these complications must be known to the operators performing TEE. The goal of this article is to encapsulate the potential complications associated with TEE. The complications are primarily related to gastrointestinal, cardiovascular and respiratory systems along with some miscellaneous problems related to probe insertion, drugs and inexperience of the operator. Strategies for the prevention of these complications are also analyzed in order to avoid the risk. PMID:20640107

Mathur, S K; Singh, Pooja

2009-01-01

221

A nager acrofacial dysostosis syndrome patient with severe respiratory distress syndrome (RDS).  

PubMed

Nager acrofacial dysostosis is characterized by radial defect and the facial manifestations like Treacher-Collins syndrome. There are few reports about this syndrome in Japan. We report a typical Japanese Nager syndrome patient. He was complicated with respiratory distress syndrome. PMID:12503193

Kondoh, T; Ito, M; Ariyama, A; Shitijoh, T; Matsumoto, T; Tsuji, Y

1997-09-01

222

Pulmonary Complications of Sickle Cell Disease  

PubMed Central

Sickle cell disease (SCD) is a common monogenetic disorder with high associated morbidity and mortality. The pulmonary complications of SCD are of particular importance, as acute chest syndrome and pulmonary hypertension have the highest associated mortality rates within this population. This article reviews the pathophysiology, diagnosis, and treatment of clinically significant pulmonary manifestations of SCD, including acute chest syndrome, asthma, and pulmonary hypertension in adult and pediatric patients. Clinicians should be vigilant in screening and treating such comorbidities to improve patient outcomes. PMID:22447965

Miller, Andrew C.

2012-01-01

223

Early respiratory complications after liver transplantation.  

PubMed

The poor clinical conditions associated with end-stage cirrhosis, pre-existing pulmonary abnormalities, and high comorbidity rates in patients with high Model for End-Stage Liver Disease scores are all well-recognized factors that increase the risk of pulmonary complications after orthotopic liver transplantation (OLT) surgery. Many intraoperative and postoperative events, such as fluid overload, massive transfusion of blood products, hemodynamic instability, unexpected coagulation abnormalities, renal dysfunction, and serious adverse effects of reperfusion syndrome, are other factors that predispose an individual to postoperative respiratory disorders. Despite advances in surgical techniques and anesthesiological management, the lung may still suffer throughout the perioperative period from various types of injury and ventilatory impairment, with different clinical outcomes. Pulmonary complications after OLT can be classified as infectious or non-infectious. Pleural effusion, atelectasis, pulmonary edema, respiratory distress syndrome, and pneumonia may contribute considerably to early morbidity and mortality in liver transplant patients. It is of paramount importance to accurately identify lung disorders because infectious pulmonary complications warrant speedy and aggressive treatment to prevent diffuse lung injury and the risk of evolution into multisystem organ failure. This review discusses the most common perioperative factors that predispose an individual to postoperative pulmonary complications and these complications' early clinical manifestations after OLT and influence on patient outcome. PMID:24409054

Feltracco, Paolo; Carollo, Cristiana; Barbieri, Stefania; Pettenuzzo, Tommaso; Ori, Carlo

2013-12-28

224

Early respiratory complications after liver transplantation  

PubMed Central

The poor clinical conditions associated with end-stage cirrhosis, pre-existing pulmonary abnormalities, and high comorbidity rates in patients with high Model for End-Stage Liver Disease scores are all well-recognized factors that increase the risk of pulmonary complications after orthotopic liver transplantation (OLT) surgery. Many intraoperative and postoperative events, such as fluid overload, massive transfusion of blood products, hemodynamic instability, unexpected coagulation abnormalities, renal dysfunction, and serious adverse effects of reperfusion syndrome, are other factors that predispose an individual to postoperative respiratory disorders. Despite advances in surgical techniques and anesthesiological management, the lung may still suffer throughout the perioperative period from various types of injury and ventilatory impairment, with different clinical outcomes. Pulmonary complications after OLT can be classified as infectious or non-infectious. Pleural effusion, atelectasis, pulmonary edema, respiratory distress syndrome, and pneumonia may contribute considerably to early morbidity and mortality in liver transplant patients. It is of paramount importance to accurately identify lung disorders because infectious pulmonary complications warrant speedy and aggressive treatment to prevent diffuse lung injury and the risk of evolution into multisystem organ failure. This review discusses the most common perioperative factors that predispose an individual to postoperative pulmonary complications and these complications’ early clinical manifestations after OLT and influence on patient outcome. PMID:24409054

Feltracco, Paolo; Carollo, Cristiana; Barbieri, Stefania; Pettenuzzo, Tommaso; Ori, Carlo

2013-01-01

225

Bloom syndrome.  

PubMed

Bloom Syndrome (BS, MIM #210900) is an autosomal recessive genetic disorder caused by a mutation in the BLM gene, which codes for the DNA repair enzyme RecQL3 helicase. Without proper DNA repair mechanisms, abnormal DNA exchange takes place between sister chromatids and results in genetic instability that may lead to cancer, especially lymphoma and acute myelogenous leukemia, lower and upper gastrointestinal tract neoplasias, cutaneous tumors, and neoplasias in the genitalia and urinary tract. BS patients are usually of Ashkenazi Jewish descent and exhibit narrow facial features, elongated limbs, and several dermatologic complications including photosensitivity, poikiloderma, and telangiectatic erythema. The most concerning manifestation of BS is multiple malignancies, which require frequent screenings and strict vigilance by the physician. Therefore, distinguishing between BS and other dermatologic syndromes of similar presentation such as Rothmund-Thomson Syndrome, Erythropoietic Protoporphyria, and Cockayne Syndrome is paramount to disease management and to prolonging life. BS can be diagnosed through a variety of DNA sequencing methods, and genetic testing is available for high-risk populations. This review consolidates several sources on BS sequelae and aims to suggest the importance of differentiating BS from other dermatologic conditions. This paper also elucidates the recently discovered BRAFT and FANCM protein complexes that link BS and Fanconi anemia. PMID:24602044

Arora, Harleen; Chacon, Anna H; Choudhary, Sonal; McLeod, Michael P; Meshkov, Lauren; Nouri, Keyvan; Izakovic, Jan

2014-07-01

226

[Autoinflammatory syndromes].  

PubMed

Autoinflammatory syndromes are characterised by recurrent or persistent inflammation with no increase in the antibody titers or antigen-specific T lymphocytes, and absence of infection. Initially, they included the hereditary periodic fever syndromes, a group of innate immune system monogenic diseases characterised by recurrent febrile episodes, with different characteristics, duration and interval, accompanied by other symptoms. Secondary amyloidosis is a complication in this group. The advances in the last few years has led to the identification of susceptible genes, new proteins, and characterising mechanisms and pathogenic routes that have led to an improvement in the diagnosis and establishing more effective treatments. Among these routes, are the changes in the inflammasome components, a group of cytoplasmic proteins that regulate the production of several inflammatory response mediators. The initial group of monogenic autoinflammatory diseases have increased in the last few years, due to including several polygenic hereditary diseases. PMID:21596180

Antón, Jordi

2011-01-01

227

Hepatopulmonary syndrome.  

PubMed

The hepatopulmonary syndrome (HPS) is a pulmonary complication of cirrhosis and/or portal hypertension whereby patients develop hypoxemia as a result of alterations in pulmonary microvascular tone and architecture. HPS occurs in up to 30% of patients with cirrhosis. Although the degree of hypoxemia does not reliably correlate with the severity of liver disease, patients with HPS have a higher mortality than do patients with cirrhosis without the disorder. There has been progress into defining the mechanisms that lead to hypoxemia in HPS, but to date there are no therapeutic options for HPS aside from liver transplantation. PMID:24679503

Koch, David G; Fallon, Michael B

2014-05-01

228

Tattoo-associated complications.  

PubMed

Tattoo rates in the United States have been rising in recent years, with an expected concomitant rise in tattoo-associated complications. Tattoo complications range from cutaneous localized and generalized inflammatory eruptions, to local bacterial or viral infections, and finally to infectious endocarditis and hepatitis. Many complications may be avoided with proper counseling prior to tattoo placement, especially in high risk individuals. It is important for physicians to be able to recognize and diagnose complications from tattoos to avoid morbidity and possible mortality. PMID:23287600

Juhas, Elizabeth; English, Joseph C

2013-04-01

229

Organ and Metabolic Complications: Lipids\\/Atherosclerosis  

Microsoft Academic Search

The incidence of and mortality from atherosclerotic complications, particulary coronary atherosclerosis, is strikingly increased\\u000a in dialysed patients. One of the factors involved is dyslipoproteinemia. The main cause of dyslipoproteinemia is reduced activity\\u000a of enzymes catabolising lipoproteins (hepatic triglyceride lipase, lipoprotein lipase), but additional abnormalities clearly\\u000a contribute. Specific patterns of dyslipoproteinemia are present in patients with the nephrotic syndrome, diabetes mellitus

Eberhard Ritz; Michal Nowicki; Andrzej Wiecek

230

Vascular complications of osteochondromas  

Microsoft Academic Search

Purpose: Osteochondromas are the most common benign tumor of the bone. They are sometimes responsible for vascular complications involving either veins or arteries, principally around the knee.Methods: We report six cases of such complications. An extensive review of literature through a computerized research was performed.Results: We found 97 cases that were previously reported in the English literature giving sufficient details

Marc-Antoine Vasseur; Oliver Fabre

2000-01-01

231

Hodgkin's Lymphoma: A Review of Neurologic Complications.  

PubMed

Hodgkin's lymphoma is a hematolymphoid neoplasm, primarily of B cell lineage, that has unique histologic, immunophenotypic, and clinical features. Neurologic complications of Hodgkin's Lymphoma can be separated into those that result directly from the disease, indirectly from the disease, or from its treatment. Direct neurologic dysfunction from Hodgkin's Lymphoma results from metastatic intracranial spinal disease, epidural metastases causing spinal cord/cauda equina compression, leptomeningeal metastases, or intradural intramedullary spinal cord metastases. Indirect neurologic dysfunction may be caused by paraneoplastic disorders (such as paraneoplastic cerebellar degeneration or limbic encephalitis) and primary angiitis of the central nervous system. Hodgkin's lymphoma treatment typically includes chemotherapy or radiotherapy with potential treatment-related complications affecting the nervous system. Neurologic complications resulting from mantle-field radiotherapy include the "dropped head syndrome," acute brachial plexopathy, and transient ischemic attacks/cerebral infarcts. Chemotherapy for Hodgkin's lymphoma may cause cerebral infarction (due to emboli from anthracycline-induced cardiomyopathy) and peripheral neuropathy. PMID:20975772

Grimm, Sean; Chamberlain, Marc

2011-01-01

232

Encephalitis complicating smallpox vaccination.  

PubMed

A smallpox vaccination program has been initiated. The vaccine is a live virus that was used in the last century. Postvaccinal encephalitis is a complication of this vaccine. The clinical presentation, course, neuroimaging findings, and spinal fluid abnormalities are similar to a disorder that physicians are familiar with, acute disseminated encephalomyelitis. This complication can be prevented with the administration of antivaccinia gamma globulin at the time of vaccination. Antivaccinia gamma globulin is not efficacious once this complication occurs. Intravenous methylprednisolone is the recommended therapy, although intravenous immunoglobulin and plasmapheresis should be investigated in the treatment of postvaccinal encephalitis. PMID:12873847

Miravalle, Augusto; Roos, Karen L

2003-07-01

233

Complications of Circumcision  

PubMed Central

In the United States, circumcision is a commonly performed procedure. It is a relatively safe procedure with a low overall complication rate. Most complications are minor and can be managed easily. Though uncommon, complications of circumcision do represent a significant percentage of cases seen by pediatric urologists. Often they require surgical correction that results in a significant cost to the health care system. Severe complications are quite rare, but death has been reported as a result in some cases. A thorough and complete preoperative evaluation, focusing on bleeding history and birth history, is imperative. Proper selection of patients based on age and anatomic considerations as well as proper sterile surgical technique are critical to prevent future circumcision-related adverse events. PMID:22235177

Krill, Aaron J.; Palmer, Lane S.; Palmer, Jeffrey S.

2011-01-01

234

Pregnancy Complications: Shoulder Dystocia  

MedlinePLUS

... In the NICU Home after the NICU Your premature baby Birth Defects Loss and grief Pregnancy loss Newborn loss ... and the baby do well and have no permanent damage. But there may be some complications. For ...

235

Systemic Amyloidosis Complicating Dermatoses.  

National Technical Information Service (NTIS)

Certain dermatoses may be complicated by systemic amyloidosis. The underlying disorders in eight of 100 patients with secondary systemic amyloidosis studied at autopsy were: hidradenitis suppurativa, stasis ulcers, psoriatic arthritis, basal cell carcinom...

M. H. Brownstein, E. B. Helwig

1970-01-01

236

Tetanus: Symptoms and Complications  

MedlinePLUS

... of the muscles of the jaw, or "lockjaw". Tetanus symptoms include: Headache Jaw cramping Sudden, involuntary muscle ... sweating High blood pressure and fast heart rate Tetanus complications include: Uncontrolled/involuntary muscular contraction of the ...

237

Chickenpox (Varicella) Complications  

MedlinePLUS

... People at High Risk for Complications Interpreting Lab Tests Assessing Immunity Managing People at Risk for Severe Varicella Preventing Varicella in Healthcare Settings Monitoring the Impact of Varicella Vaccination Conducting Varicella Surveillance Laboratory Testing ...

238

Complications of circumcision  

Microsoft Academic Search

Circumcision remains the most common operation performed on males. Although, not technically difficult, it is accompanied\\u000a by a rate of morbidity and can result in complications ranging from trivial to tragic. The reported incidence of complications\\u000a varies from 0.1% to 35% the most common being infection, bleeding and failure to remove the appropriate amount of foreskin.\\u000a Forty patients suffering from

O. Latifoglu; R. Yavuzer; S. Ünal; A. Sari; S. Çenetoglu; N. K. Baran

1999-01-01

239

Cerebrovascular complications of pregnancy and the postpartum period.  

PubMed

Cerebrovascular complications of pregnancy, though uncommon, threaten women with severe morbidity or death, and they are the main causes of major long-term disability associated with pregnancy. In this review, we discuss the epidemiology, pathophysiology, presentation and diagnosis, and management and outcomes of ischemic and hemorrhagic stroke and cerebral venous thrombosis. We also discuss the posterior reversible encephalopathy syndrome, the reversible cerebral vasoconstriction syndrome including postpartum cerebral angiopathy, and their relationship as overlapping manifestations of pre-eclampsia-eclampsia. PMID:25239155

Razmara, Ali; Bakhadirov, Khamid; Batra, Ayush; Feske, Steven K

2014-10-01

240

Asymptomatic moyamoya syndrome, atlantoaxial subluxation and basal ganglia calcification in a child with Down syndrome  

PubMed Central

Down syndrome, the most common chromosomal abnormality, may be associated with various neurologic complications such as moyamoya syndrome, cervical spinal cord compression due to atlantoaxial subluxation, and basal ganglia damage, as well as epileptic seizures and stroke. Many cases of Down syndrome accompanied by isolated neurologic manifestations have been reported in children; however, Down syndrome with multiple neurologic conditions is rare. Here, we have reported a case of Down syndrome in a 10-year-old girl who presented with asymptomatic moyamoya syndrome, atlantoaxial subluxation with spinal cord compression, and basal ganglia calcification. To the best of our knowledge, this is the first report of Down syndrome, in a child, which was accompanied by these 3 neurologic complications simultaneously. As seen in this case, patients with Down syndrome may have neurologic conditions without any obvious neurologic symptoms; hence, patients with Down syndrome should be carefully examined for the presence of neurologic conditions. PMID:24416050

Lee, Kun-Soo; Weon, Young Cheol

2013-01-01

241

[Complications in the evolution of haemangiomas and vascular malformations].  

PubMed

The differentiation of haemangiomas and vascular malformations is histological, clinical and prognostic. Although the majority of haemangiomas evolve towards spontaneous resolution, as many as 10% of cases can develop complications with ulceration, pain and haemorrhaging. Besides, the localisation of haemangiomas in the head and neck, next to vital structures, can compromise their functions. Hence, compression of the airway might be a vital emergency. Periorbital haemangiomas can give rise to amblyopia due to sensory deprivation or due to a restrictive strabismus. Lumbosacral haemangiomas must be studied with Nuclear Magnetic Resonance because of their frequent association with alterations in the midline at the level of the spine, anus, genitals or kidneys. Amongst visceral haemangiomas, hepatic haemangiomas are the most serious due to their association with congestive cardiac insufficiency. The association of extensive facial haemangiomas with anomalies of the central nervous system, vascular, cardiac, ocular and sternal anomalies, is denominated PHACE syndrome and is frequently complicated by mental deficiency, convulsions or ictus. Vascular malformations of trigeminal localisation are associated in up to 15% of cases with glaucoma or choroidal or leptomeningeal haemangiomas (Sturge-Weber syndrome). Combined vascular malformations localised in the extremities can become complicated with thrombophlebitis, regional osteolysis and even distant thromboembolisms (Klippel-Treneaunay Syndrome). On the other hand, there is a coagulopathy due to consumption (Kassabach-Merrit Syndrome) that can complicate some vascular tumours such as the Kaposiform haemangioendothelioma and the tufted angioma. Finally, the complications of the treatments employed are reviewed. PMID:15148512

Belzunce, A; Casellas, M

2004-01-01

242

Tourette Syndrome  

MedlinePLUS

NINDS Tourette Syndrome Information Page Condensed from Tourette Syndrome Fact Sheet Table of Contents (click to jump to sections) ... Trials Organizations Additional resources from MedlinePlus What is Tourette Syndrome? Tourette syndrome (TS) is a neurological disorder ...

243

Fanconi syndrome  

MedlinePLUS

De Toni-Fanconi syndrome ... Fanconi syndrome can be caused by faulty genes, or it may result later in life due to kidney damage. Sometimes the cause of Fanconi syndrome is unknown. Common causes of Fanconi syndrome in ...

244

Cerebellar involvement of Griscelli syndrome type 2.  

PubMed

Griscelli syndrome type 2 is characterised by partial albinism and primary immunodeficiency. We present a case of a 3-year-old girl diagnosed with cerebellar involvement of Griscelli syndrome type 2. Neurological complications may accompany Griscelli syndrome, however, to the best of my knowledge there are only a few case reports of cerebellar involvement of Griscelli syndrome type 2 in the literature. PMID:25315806

I?ikay, Sedat

2014-01-01

245

Complications of hysterectomy.  

PubMed

Hysterectomy is the most common gynecologic procedure performed in the United States, with more than 600,000 procedures performed each year. Complications of hysterectomy vary based on route of surgery and surgical technique. The objective of this article is to review risk factors associated with specific types of complications associated with benign hysterectomy, methods to prevent and recognize complications, and appropriate management of complications. The most common complications of hysterectomy can be categorized as infectious, venous thromboembolic, genitourinary (GU) and gastrointestinal (GI) tract injury, bleeding, nerve injury, and vaginal cuff dehiscence. Infectious complications after hysterectomy are most common, ranging from 10.5% for abdominal hysterectomy to 13.0% for vaginal hysterectomy and 9.0% for laparoscopic hysterectomy. Venous thromboembolism is less common, ranging from a clinical diagnosis rate of 1% to events detected by more sensitive laboratory methods of up to 12%. Injury to the GU tract is estimated to occur at a rate of 1-2% for all major gynecologic surgeries, with 75% of these injuries occurring during hysterectomy. Injury to the GI tract after hysterectomy is less common, with a range of 0.1-1%. Bleeding complications after hysterectomy also are rare, with a median range of estimated blood loss of 238-660.5 mL for abdominal hysterectomy, 156-568 mL for laparoscopic hysterectomy, and 215-287 mL for vaginal hysterectomy, with transfusion only being more likely after laparoscopic compared to vaginal hysterectomy (odds ratio 2.07, confidence interval 1.12-3.81). Neuropathy after hysterectomy is a rare but significant event, with a rate of 0.2-2% after major pelvic surgery. Vaginal cuff dehiscence is estimated at a rate of 0.39%, and it is more common after total laparoscopic hysterectomy (1.35%) compared with laparoscopic-assisted vaginal hysterectomy (0.28%), total abdominal hysterectomy (0.15%), and total vaginal hysterectomy (0.08%). With an emphasis on optimizing surgical technique, recognition of surgical complications, and timely management, we aim to minimize risk for women undergoing hysterectomy. PMID:23635631

Clarke-Pearson, Daniel L; Geller, Elizabeth J

2013-03-01

246

Complicated necrotizing otitis externa.  

PubMed

Necrotizing (malignant) otitis externa (NOE) is a rare and invasive infection originating in the external acoustic meatus seen most commonly in diabetes and other immunocompromised states. After a protracted course, disease can smolder and extend into the mastoid, skull base, dural sinuses, and intracranially. We present a case of NOE complicated by mastoiditis, dural sinus thrombosis, and Bezold's abscess in an uncontrolled diabetic presenting with a prolonged course of facial nerve palsy. We stress the importance of maintaining a high index of clinical suspicion for NOE in diabetic patients and offering timely, aggressive treatment to mitigate its complications. PMID:23927998

Nawas, Mariam T; Daruwalla, Vistasp J; Spirer, David; Micco, Alan G; Nemeth, Alexander J

2013-01-01

247

[Complications of experimental lung transplantations].  

PubMed

Experimental lung transplantations are burdened with a very high rate of complications even is there is a strict consideration of sterile precautions. These complications occur predominantly directly in connection with organ rejection. Extrathoracic complications are rare and predominantly attributed to arterial embolism. All untreated receivers of lung had thoracic complications in autopsy. Only 5% of immune suppressively treated receivers were free of complications. Most frequent complications are pleuropulmonary infections. Weighty complications are haemorrhagic infarcts of the transplant and insufficiency of the bronchus. Only without complications can survived about years the chronic rejection. PMID:7025481

Engelmann, C; Kaltwasser, K; Danzmann, E; Riesner, R; Schimmack, L; Reutgen, H; Voigt, H

1981-01-01

248

Myhre syndrome.  

PubMed

Myhre syndrome (MS) is a developmental disorder characterized by typical facial dysmorphism, thickened skin, joint limitation and muscular pseudohypertrophy. Other features include brachydactyly, short stature, intellectual deficiency with behavioral problems and deafness. We identified SMAD4 as the gene responsible for MS. The identification of SMAD4 mutations in Laryngotracheal stenosis, Arthropathy, Prognathism and Short stature (LAPS) cases supports that LAPS and MS are a unique entity. The long-term follow up of patients shows that these conditions are progressive with life threatening complications. All mutations are de novo and changing in the majority of cases Ile500, located in the MH2 domain involved in transcriptional activation. We further showed an impairment of the transcriptional regulation via TGF? target genes in patient fibroblasts. Finally, the absence of SMAD4 mutations in three MS cases may support genetic heterogeneity. PMID:24580733

Le Goff, C; Michot, C; Cormier-Daire, V

2014-06-01

249

[Marfan syndrome].  

PubMed

Marfan's syndrome is a monogenetic disease with an autosomal dominant transmission generally accompanied by type I fibrillin abnormality. This widely-distributed molecule participates in the structure of connective tissues so that any aberration may result in disease of many systems: skeletal morphology, dislocation of the lens, neurological or cutaneous signs and dilatation of the aorta predisposing to dissection, mitral valve prolapse being a common association. The diagnosis, clinical because of the size of the culprit gene and the multiplicity of the possible mutations, is sometimes difficult, and diagnostic criteria have been proposed. It is important to make the diagnosis because treatment is based on the restriction of violent exercise, betablocker therapy and regular echocardiographic monitoring of the ascending aorta, the region at highest risk of dilatation and dissection. A family enquiry is essential to make the diagnosis before the onset of complications in pauci-symptomatic patients (great intra-familial variability). Pregnancy poses special problems in these patients. PMID:14694784

Jondeau, G; Boileau, C; Chevallier, B; Delorme, G; Digne, F; Guiti, C; Milleron, O; de SaintJean, M; Le Parc, J M; Moura, B

2003-11-01

250

Tattoos: dermatological complications  

Microsoft Academic Search

From the Eskimo in Greenland to the tribes in Polynesia­the whole world knows the art of tattoo. Despite their wide popularity the relation between the skin diseases and the tattooed pictures aren't studied in depth. With the appearance of professional tattoo studios, the risk of infectious complications was reduced. Simultaneously, on a global scale there has been an increase in

Jana Kazandjieva; Nikolai Tsankov

2007-01-01

251

Treatment of complicated grief  

PubMed Central

Following the death of a loved one, a small group of grievers develop an abnormal grieving style, termed complicated or prolonged grief. In the effort to establish complicated grief as a disorder in DSM and ICD, several attempts have been made over the past two decades to establish symptom criteria for this form of grieving. Complicated grief is different from depression and PTSD yet often comorbid with other psychological disorders. Meta-analyses of grief interventions show small to medium effect sizes, with only few studies yielding large effect sizes. In this article, an integrative cognitive behavioral treatment manual for complicated grief disorder (CG-CBT) of 25 individual sessions is described. Three treatment phases, each entailing several treatment strategies, allow patients to stabilize, explore, and confront the most painful aspects of the loss, and finally to integrate and transform their grief. Core aspects are cognitive restructuring and confrontation. Special attention is given to practical exercises. This article includes the case report of a woman whose daughter committed suicide. PMID:22893810

Rosner, Rita; Pfoh, Gabriele; Kotoucova, Michaela

2011-01-01

252

Hypoglycemia: The neglected complication  

PubMed Central

Hypoglycemia is an important complication of glucose-lowering therapy in patients with diabetes mellitus. Attempts made at intensive glycemic control invariably increases the risk of hypoglycemia. A six-fold increase in deaths due to diabetes has been attributed to patients experiencing severe hypoglycemia in comparison to those not experiencing severe hypoglycemia Repeated episodes of hypoglycemia can lead to impairment of the counter-regulatory system with the potential for development of hypoglycemia unawareness. The short- and long-term complications of diabetes related hypoglycemia include precipitation of acute cerebrovascular disease, myocardial infarction, neurocognitive dysfunction, retinal cell death and loss of vision in addition to health-related quality of life issues pertaining to sleep, driving, employment, recreational activities involving exercise and travel. There is an urgent need to examine the clinical spectrum and burden of hypoglycemia so that adequate control measures can be implemented against this neglected life-threatening complication. Early recognition of hypoglycemia risk factors, self-monitoring of blood glucose, selection of appropriate treatment regimens with minimal or no risk of hypoglycemia and appropriate educational programs for healthcare professionals and patients with diabetes are the major ways forward to maintain good glycemic control, minimize the risk of hypoglycemia and thereby prevent long-term complications. PMID:24083163

Kalra, Sanjay; Mukherjee, Jagat Jyoti; Venkataraman, Subramanium; Bantwal, Ganapathi; Shaikh, Shehla; Saboo, Banshi; Das, Ashok Kumar; Ramachandran, Ambady

2013-01-01

253

Complications of sphenoid sinusitis  

Microsoft Academic Search

Regarding the skull base, no structure is as centrally located as the sphenoid sinus. It sits at the junction of the anterior and middle cranial fossae, and is surrounded by vital structures. Although relatively rare, complications of the inflammatory and infectious conditions of sphenoid sinusitis are the result of direct extension of disease to the surrounding tissues, or a propagated

Andrew Goldman

2003-01-01

254

Pulmonary Complications in Patients with Severe Brain Injury  

PubMed Central

Pulmonary complications are prevalent in the critically ill neurological population. Respiratory failure, pneumonia, acute lung injury and the acute respiratory distress syndrome (ALI/ARDS), pulmonary edema, pulmonary contusions and pneumo/hemothorax, and pulmonary embolism are frequently encountered in the setting of severe brain injury. Direct brain injury, depressed level of consciousness and inability to protect the airway, disruption of natural defense barriers, decreased mobility, and secondary neurological insults inherent to severe brain injury are the main cause of pulmonary complications in critically ill neurological patients. Prevention strategies and current and future therapies need to be implemented to avoid and treat the development of these life-threatening medical complications. PMID:23133746

Lee, Kiwon; Rincon, Fred

2012-01-01

255

Intrauterine Fetal Resuscitation following Complications of Closed Fetal Surgery  

Microsoft Academic Search

Background: With advances in prenatal diagnosis and surgical technique more fetal interventions are being performed. There are limited data about the etiology, incidence, management, or long-term outcomes of complications associated with these procedures. Case: A gravida had a fetus diagnosed with posterior urethral valve syndrome. During placement of a peritoneal-amniotic shunt, profound fetal bradycardia was noted. Using closed fetal surgical

Steven S. Brumfield; M. Bardett Fausett; Melissa H. Fries

2005-01-01

256

Neurologic complications of cardiac tests and procedures.  

PubMed

Arterial or central venous vascular access is the cornerstone of invasive cardiac diagnosis, monitoring, and therapeutics. Although procedural safety has significantly improved with protocols perfected over decades of use, their prevalence renders even the uncommon neurologic complication clinically relevant. Serious peripheral nerve complications result from direct or indirect nerve injuries in the setting of a hematoma or compartment syndrome. Functional outcome is dependent upon prompt diagnosis and early treatment, so proceduralists should be aware of the relevant anatomy and early signs of nerve injury. Ischemic stroke is the most common central nervous system complication of diagnostic and therapeutic cardiac catheterization, and is presumed to be due to embolization of atherosclerotic plaque or thrombus dislodged during guiding catheter manipulation, platelet-fibrin thrombus that forms on the catheters, or air that appears during catheter flushing. Acute neurologic deterioration after thrombolysis for acute myocardial infarction should be presumed to be an intracranial hemorrhage until proven otherwise. The ideal angiography suite of the future is patientcentric and multipurpose, coordinating diagnostic and therapeutic strategies for multivascular disease, allowing for multispecialty collaboration, and, in the event of a neurologic complication of a cardiac procedure, facilitating the various treating physicians to converge efficiently upon the patient. PMID:24365287

Sila, Cathy

2014-01-01

257

Blepharoplasty complications: prevention and management.  

PubMed

This article presents common and rare complications following blepharoplasty, with discussion of avoidance of these complications through presurgical planning and review. Management of the complications is provided, with surgical details supported by images and advice for the best approaches. The complications discussed include hemorrhage, infection, corneal abrasion, ptosis, lacrimal gland injury, and residual excess skin. PMID:23186771

Whipple, Katherine M; Lim, Lee Hooi; Korn, Bobby S; Kikkawa, Don O

2013-01-01

258

Non-infectious pulmonary complications after bone marrow transplantation  

PubMed Central

Bone marrow transplantation (BMT) is a successful and recognised treatment option for patients with a number of haematological and non-haematological malignant and non-malignant conditions. Pulmonary complications both infectious and non-infectious are common after BMT. Multiple factors are thought to contribute to pulmonary complications, including the type and duration of immunological defects produced by the underlying disease and treatment, the development of graft-versus-host disease (GVHD), and the conditioning regimens employed. These complications are classified as early or late, depending on whether they occur before or after 100 days from transplantation. Early non-infectious pulmonary complications typically include pulmonary oedema, upper airway complications, diffuse alveolar haemorrhage, cytolytic thrombi, and pleural effusion. Bronchiolitis obliterans, veno-occlusive disease, and secondary malignancies occur late after BMT. Idiopathic pneumonia syndrome, GVHD, and radiation induced lung injury can occur in early or late period after BMT. PMID:12151565

Khurshid, I; Anderson, L

2002-01-01

259

Fistulas complicating diverticulitis  

Microsoft Academic Search

This study was undertaken to assess the appropriate management of patients with diverticulitis complicated by fistula formation.\\u000a A retrospective chart review was conducted on patients with symptoms of a fistula who presented between 1975 to 1995. There\\u000a were 42 patients (32 women, 76%; 10 men, 24%) who ranged in age from 46 to 89 years (mean 69.8±9.8). Six patients had

C.-A. Vasilevsky; P. Belliveau; J. L. Trudel; B. L. Stein; P. H. Gordon

1998-01-01

260

Neurologic Complications of Sarcoma  

Microsoft Academic Search

Sarcomas are a heterogeneous group of tumors that rarely involve the nervous system. Neurologic effects of sarcoma are more\\u000a often due to tumors outside of the central nervous system. However, as long-term survival rates in childhood sarcoma improves,\\u000a reports of late neurologic complications have increased. With recent advances in treating local sarcomas with targeted molecular\\u000a therapies, the incidence of late

Santosh Kesari; Lara J. Kunschner

261

Toe tourniquet syndrome.  

PubMed

Toe tourniquet syndrome refers to external, mechanical, circumferential constriction of the toes. We report a series of 4 infants with toe tourniquet syndrome from Saudi Arabia who presented during wintertime with very similar symptoms (approximately 48 hours of inconsolable crying and irritability), similar involved region (toes), and similar constricting agent (hairs). Immediate removal of the hair fibers was carried out in all patients, fortunately followed by fast healing with no signs of tissue necrosis. The prompt diagnosis and treatment of the condition were vital in attaining the good outcome and preventing ischemic complications.  PMID:25129188

Kamal, Naglaa M; Khan, Ubaid U; Mirza, Shazia J; Al-Malki, Talal A

2014-08-01

262

Hypocomplementemic Urticarial Vasculitis Syndrome  

PubMed Central

Hypocomplementemic urticarial vasculitis syndrome, as opposed to urticarial vasculitis or urticarial vasculitis syndrome, is a rare disease process where the exact pathophysiology remains unknown. This article discusses the case of a 34-year-old Hispanic man with an ongoing history of chronic urticaria comprising episodes induced by low ambient temperatures, emotional stress, and spontaneous occurrences. This article serves as a consolidated reference for specialists to comprehensively review the plethora of systemic manifestations that may accompany urticarial vasculitis and highlights new systemic complications reported in association with this disease which are also observed in this case. PMID:22328958

Christensen, Jim; McCarty, Morgan

2012-01-01

263

Jacobsen syndrome  

PubMed Central

Jacobsen syndrome is a MCA/MR contiguous gene syndrome caused by partial deletion of the long arm of chromosome 11. To date, over 200 cases have been reported. The prevalence has been estimated at 1/100,000 births, with a female/male ratio 2:1. The most common clinical features include pre- and postnatal physical growth retardation, psychomotor retardation, and characteristic facial dysmorphism (skull deformities, hypertelorism, ptosis, coloboma, downslanting palpebral fissures, epicanthal folds, broad nasal bridge, short nose, v-shaped mouth, small ears, low set posteriorly rotated ears). Abnormal platelet function, thrombocytopenia or pancytopenia are usually present at birth. Patients commonly have malformations of the heart, kidney, gastrointestinal tract, genitalia, central nervous system and skeleton. Ocular, hearing, immunological and hormonal problems may be also present. The deletion size ranges from ~7 to 20 Mb, with the proximal breakpoint within or telomeric to subband 11q23.3 and the deletion extending usually to the telomere. The deletion is de novo in 85% of reported cases, and in 15% of cases it results from an unbalanced segregation of a familial balanced translocation or from other chromosome rearrangements. In a minority of cases the breakpoint is at the FRA11B fragile site. Diagnosis is based on clinical findings (intellectual deficit, facial dysmorphic features and thrombocytopenia) and confirmed by cytogenetics analysis. Differential diagnoses include Turner and Noonan syndromes, and acquired thrombocytopenia due to sepsis. Prenatal diagnosis of 11q deletion is possible by amniocentesis or chorionic villus sampling and cytogenetic analysis. Management is multi-disciplinary and requires evaluation by general pediatrician, pediatric cardiologist, neurologist, ophthalmologist. Auditory tests, blood tests, endocrine and immunological assessment and follow-up should be offered to all patients. Cardiac malformations can be very severe and require heart surgery in the neonatal period. Newborns with Jacobsen syndrome may have difficulties in feeding and tube feeding may be necessary. Special attention should be devoted due to hematological problems. About 20% of children die during the first two years of life, most commonly related to complications from congenital heart disease, and less commonly from bleeding. For patients who survive the neonatal period and infancy, the life expectancy remains unknown. PMID:19267933

Mattina, Teresa; Perrotta, Concetta Simona; Grossfeld, Paul

2009-01-01

264

Ectopic Cushing syndrome secondary to metastatic medullary thyroid cancer in a child with multiple endocrine neoplasia syndrome type 2B: clues to early diagnosis of the paraneoplastic syndromes.  

PubMed

Abstract We describe a 13-year-old male with multiple endocrine neoplasia syndrome type 2B with medullary thyroid carcinoma who was diagnosed with ectopic adrenocorticotropin-dependent Cushing syndrome. This report highlights the importance of monitoring for paraneoplastic syndrome in MEN and clues to the diagnosis of this complication provided by growth patterns. PMID:24859505

Singer, Kanakadurga; Heiniger, Nicholas; Thomas, Inas; Worden, Francis P; Menon, Ram K; Chen, Ming

2014-09-20

265

Williams syndrome  

MedlinePLUS

Williams-Beuren syndrome ... Williams syndrome is a rare condition caused by missing a copy of several genes. Parents may not have ... history of the condition. However, a person with Williams syndrome has a 50% chance of passing the disorder ...

266

Down Syndrome  

MedlinePLUS

... Information Clinical Trials Resources and Publications En Español Down Syndrome: Condition Information Skip sharing on social media links Share this: Page Content What is Down syndrome? Down syndrome describes a set of cognitive and ...

267

Dravet Syndrome  

MedlinePLUS

NINDS Dravet Syndrome Information Page Synonym(s): Severe Myoclonic Epilepsy of Infancy (SMEI) Table of Contents (click to ... Dravet Syndrome? Dravet syndrome, also called severe myoclonic epilepsy of infancy (SMEI), is a severe form of ...

268

Piriformis Syndrome  

MedlinePLUS

... Awards Enhancing Diversity Find People About NINDS NINDS Piriformis Syndrome Information Page Table of Contents (click to ... is being done? Clinical Trials Organizations What is Piriformis Syndrome? Piriformis syndrome is a rare neuromuscular disorder ...

269

[Complications of hemorrhoids].  

PubMed

The most common and serious complications of haemorrhoids include perianal thrombosis and incarcerated prolapsed internal haemorrhoids with subsequent thrombosis. They are characterised by severe pain in the perianal region possibly with bleeding. In a short history of the perianal thrombosis, acute surgical incision or excision is indicated, which can result in rapid relief of the painful symptoms. In incarcerated prolapsed internal haemorrhoids, emergency haemorrhoidectomy may also be indicated. Segmental haemorrhoidectomy in the most affected quadrants followed by further elective surgery for haemorrhoids in the next stage is preferred. PMID:24881480

Slauf, P; Antoš, F; Marx, J

2014-04-01

270

Infantile hydrocephalus and the slit ventricle syndrome in early infancy  

Microsoft Academic Search

Slit ventricle syndrome is well known as a complication in the treatment of hydrocephalus by shunting. It is generally considered to be a chronic (but not acute) complication, occurring years after the shunt procedure; there has been no report of this syndrome occurring before 1 year of age. The authors present infantile cases that developed a severe form of this

Shizuo Oi; Satoshi Matsumoto

1987-01-01

271

Beckwith-Wiedemann Syndrome: Historical, Clinicopathological, and Etiopathogenetic Perspectives  

Microsoft Academic Search

Macroglossia, prenatal or postnatal overgrowth, and abdominal wall defects (omphalocele, umbilical hernia, or diastasis recti) permit early recognition of Beckwith-Wiedemann syndrome. Complications include neonatal hypoglycemia and an increased risk for Wilms tumor, adrenal cortical carcinoma, hepatoblastoma, rhabdomyosarcoma, and neuroblastoma, among others. Perinatal mortality can result from complications of prematurity, pronounced macroglossia, and rarely cardiomyopathy. The molecular basis of Beckwith-Wiedemann syndrome

M. Michael Cohen

2005-01-01

272

Tobacco-alcohol amblyopia: A rare complication of prolonged alcohol abuse  

PubMed Central

Tobacco-alcohol amblyopia is rare, however, extremely disabling complication seen in patient with alcohol dependence syndrome, which if not addressed properly and at the right time may lead to persisting deficits. We here report a patient of alcohol-dependence syndrome who presented with significant diminution of vision bilaterally in the background of excess alcohol consumption. PMID:22969186

Prakash, Jyoti; Ryali, VSSR; Srivastava, K.; Bhat, P. S.; Shashikumar, R.; Singal, A.

2011-01-01

273

Gastrointestinal complications of diabetes.  

PubMed

Gastrointestinal complications of diabetes include gastroparesis, intestinal enteropathy (which can cause diarrhea, constipation, and fecal incontinence), and nonalcoholic fatty liver disease. Patients with gastroparesis may present with early satiety, nausea, vomiting, bloating, postprandial fullness, or upper abdominal pain. The diagnosis of diabetic gastroparesis is made when other causes are excluded and postprandial gastric stasis is confirmed by gastric emptying scintigraphy. Whenever possible, patients should discontinue medications that exacerbate gastric dysmotility; control blood glucose levels; increase the liquid content of their diet; eat smaller meals more often; discontinue the use of tobacco products; and reduce the intake of insoluble dietary fiber, foods high in fat, and alcohol. Prokinetic agents (e.g., metoclopramide, erythromycin) may be helpful in controlling symptoms of gastroparesis. Treatment of diabetes-related constipation and diarrhea is aimed at supportive measures and symptom control. Nonalcoholic fatty liver disease is common in persons who are obese and who have diabetes. In persons with diabetes who have elevated hepatic transaminase levels, it is important to search for other causes of liver disease, including hepatitis and hemochromatosis. Gradual weight loss, control of blood glucose levels, and use of medications (e.g., pioglitazone, metformin) may normalize hepatic transaminase levels, but the clinical benefit of aggressively treating nonalcoholic fatty liver disease is unknown. Controlling blood glucose levels is important for managing most gastrointestinal complications. PMID:18619079

Shakil, Amer; Church, Robert J; Rao, Shobha S

2008-06-15

274

Osteomyelitis complicating Streptococcus milleri endocarditis.  

PubMed Central

A patient with osteomyelitis of the spine complicating bacterial endocarditis due to Streptococcus milleri is discussed. To our knowledge, this is the first time this organism has been associated with this complication. Images Figure 1 PMID:2385559

Barham, N. J.; Flint, E. J.; Mifsud, R. P.

1990-01-01

275

Postoperative Complications of Thyroid Cancer in a Single Center Experience  

PubMed Central

The aim of this study was to investigate the complications following surgical treatment of thyroid cancer and the association between the extent of surgery and complication rates. A total of 2,636 patients who underwent surgery due to thyroid cancer were retrospectively reviewed to identify surgical complications. Complication rates were assessed according to the extent of surgery, which was classified as follows; less-than-total thyroidectomy with central compartment node dissection (CCND) (Group I, n=636), total thyroidectomy with CCND (Group II, n=1,390), total thyroidectomy plus ipsilateral neck dissection (Group III, n=513), and total thyroidectomy plus bilateral neck dissection (Group IV, n=97). The most common surgical complication was symptomatic hypoparathyroidism, of which 28.4% of cases were transient and 0.3% permanent. The other surgical complications included vocal cord palsy (0.7% transient, and 0.2% permanent), hematoma (0.5%), seroma (4.7%), chyle fistula (1.8%), and Horner's syndrome (0.2%). The complication rates increased significantly with increasing the extent of surgery from Group I to Group IV. The more extensive surgery makes more complications, such as hypoparathyroidism, seroma, and others. PMID:20357995

Lee, Yong Sang; Nam, Kee-Hyun; Chung, Woong Youn; Park, Cheong Soo

2010-01-01

276

Renal abscess in Papillion-Lefèvre syndrome.  

PubMed

A 5-year-old female with Papillon-Lefèvre syndrome presented with renal mass. A radiological diagnosis of malignancy was made; however, partial nephrectomy revealed granulomatous disease indicative of chronic infection. Although liver abscess is an emerging complication in patient with Papillon-Lefèvre syndrome, this case represents the first renal abscess described in such patients. PMID:21594717

Morgan, Robert David; Hannon, Edward; Lakhoo, Kokila

2011-12-01

277

Ehlers-Danlos syndrome type IV  

Microsoft Academic Search

Ehlers-Danlos syndrome type IV, the vascular type of Ehlers-Danlos syndromes (EDS), is an inherited connective tissue disorder defined by characteristic facial features (acrogeria) in most patients, translucent skin with highly visible subcutaneous vessels on the trunk and lower back, easy bruising, and severe arterial, digestive and uterine complications, which are rarely, if at all, observed in the other forms of

Dominique P Germain

2007-01-01

278

Surgical complications following cochlear implantation in adults based on a proposed reporting consensus  

PubMed Central

Conclusion: The rate of severe complications was low and cochlear implantation is a relatively safe procedure. Standardization is crucial when reporting on cochlear implant complications to ensure comparability between studies. A consensus on the reporting of complications proposed by a Danish team of researchers was applied, evaluated and found beneficial. Objectives: To report the surgical complications following cochlear implantation at our centre, applying and evaluating a proposed reporting consensus. Methods: A retrospective file review of 308 consecutive adult implantations in 269 patients between 1994 and 2010 at Odense University Hospital was performed. Results: The three most common major complications were wound infection (1.6%), permanent chorda tympani syndrome (1.6%) and electrode migration/misplacement/accidental removal (1.3%). Permanent facial nerve paresis occurred following one implantation (0.3%). Transient chorda tympani syndrome (30.8%), vertigo/dizziness (29.5%) and tinnitus (4.9%) were the most frequent minor complications. PMID:23768013

Faber, Christian Emil

2013-01-01

279

Complications of neuroimaging.  

PubMed

Modern imaging techniques with computed tomography (CT) and magnetic resonance imaging (MRI) have revolutionized neuroimaging. While eliminating the risks of invasive procedures, new risks must now be considered before ordering neuroimaging. Advanced imaging techniques with CT may pose a risk of significant radiation exposure. Contrast may pose a risk in patients with pre-existing renal failure. MR is associated with risks related to the static magnetic field, to risks associated with the magnetic field gradients, and to risk from contrast media. Neurointervenional techniques allow for nonoperative treatment of a variety of intracranial and spinal pathologies, but with associated risks of embolization, radiation exposure, bleeding complications at the access site and a potential for contrast-related nephropathy. PMID:24365444

Rosenblum, Jordan D; Pasternak, Olga; Mitchell, Myrosia M

2014-01-01

280

Developmental and Degenerative Features in a Complicated Spastic Paraplegia  

PubMed Central

Objective We sought to explore the genetic and molecular causes of Troyer syndrome, one of several complicated hereditary spastic paraplegias (HSPs). Troyer syndrome had been thought to be restricted to the Amish; however, we identified 2 Omani families with HSP, short stature, dysarthria and developmental delay—core features of Troyer syndrome—and a novel mutation in the SPG20 gene, which is also mutated in the Amish. In addition, we analyzed SPG20 expression throughout development to infer how disruption of this gene might generate the constellation of developmental and degenerative Troyer syndrome phenotypes. Methods Clinical characterization of 2 non-Amish families with Troyer syndrome was followed by linkage and sequencing analysis. Quantitative polymerase chain reaction and in situ hybridization analysis of SPG20 expression were carried out in embryonic and adult human and mouse tissue. Results Two Omani families carrying a novel SPG20 mutation displayed clinical features remarkably similar to the Amish patients with Troyer syndrome. SPG20 mRNA is expressed broadly but at low relative levels in the adult brain; however, it is robustly and specifically expressed in the limbs, face, and brain during early morphogenesis. Interpretation Null mutations in SPG20 cause Troyer syndrome, a specific clinical entity with developmental and degenerative features. Maximal expression of SPG20 in the limb buds and forebrain during embryogenesis may explain the developmental origin of the skeletal and cognitive defects observed in this disorder. ANN NEUROL 2010;67:516–525 PMID:20437587

Manzini, M Chiara; Rajab, Anna; Maynard, Thomas M; Mochida, Ganeshwaran H; Tan, Wen-Hann; Nasir, Ramzi; Hill, R Sean; Gleason, Danielle; Al Saffar, Muna; Partlow, Jennifer N; Barry, Brenda J; Vernon, Mike; LaMantia, Anthony-Samuel; Walsh, Christopher A

2010-01-01

281

Thrombotic Thrombocytopenic Purpura-Haemolytic Uremic Syndrome and pregnancy  

PubMed Central

Thrombotic Thrombocytopenic Purpura-Haemolytic Uremic Syndrome (TTP-HUS) is a rare pregnancy and postpartum complication that may simulate the more common obstetric complications, preeclampsia and the syndrome of haemolysis, elevated liver functions tests, low platelets (HELLP). We describe a 26 years old patient who presented with peri-partum TTP-HUSand was initially treated as a case of HELLP syndrome without any improvement. A brief review of the current TTP-HUS treatment options in pregnancy is also presented.

Mwita, Julius Chacha; Vento, Sandro; Benti, Tadele

2014-01-01

282

[Gastric carcinoma--rates and management of surgical treatment complications].  

PubMed

The authors present a study on rates and management of complications of gastric carcinoma surgeries. During a five-year period, a total of 149 patients with gastric carcinomas were operated in the Ist Surgical Clinic (Charles University Faculty Hospital). Radical resections were performed in 121 subjects. In 7 subjects, upper pole resections were performed. 21 subjects underwent paliative therapy or surgical exploration. In the authors' study group, no serious perioperative complications were recorded. The following postoperative complications were observed: fistulas in esophagojejunal anastomosis (8x), duodenal stub fistula (lx), subphrenic abscess (2x), adhesive ileus (1x). In two subjects, esophagojejunal stricture was diagnosed during the late postoperative period. Incisional hernia was diagnosed in two subjects. Two subjects exited- the first one from respiratory failure with ARDS syndrome in esophagojejunal dehiscence, the second subject died of hepatorenal failure in liver cirrhosis. Complications cannot be excluded in any surgical procedure. Should they occur, their timely diagnosis and adequate treatment is required. PMID:20514913

Vrba, R; Neoral, C; Aujeský, R

2010-03-01

283

Gastrointestinal complications of oncologic therapy.  

PubMed

Gastrointestinal complications are common in patients undergoing various forms of cancer treatment, including chemotherapy, radiation therapy, and molecular-targeted therapies. Many of these complications are life-threatening and require prompt diagnosis and treatment. Complications of oncologic therapy can occur in the esophagus (esophagitis, strictures, bacterial, viral and fungal infections), upper gastrointestinal tract (mucositis, bleeding, nausea and vomiting), colon (diarrhea, graft-versus-host disease, colitis and constipation), liver (drug hepatotoxicity and graft-versus-host disease), and pancreas (pancreatitis). Treatment of the different gastrointestinal complications should be tailored to the individual patient and based on the underlying pathophysiology of the complication. PMID:18941434

Davila, Marta; Bresalier, Robert S

2008-12-01

284

Joint hypermobility syndrome pain.  

PubMed

Joint hypermobility syndrome (JHS) was initially defined as the occurrence of musculoskeletal symptoms in the presence of joint laxity and hypermobility in otherwise healthy individuals. It is now perceived as a commonly overlooked, underdiagnosed, multifaceted, and multisystemic heritable disorder of connective tissue (HDCT), which shares many of the phenotypic features of other HDCTs such as Marfan syndrome and Ehlers-Danlos syndrome. Whereas the additional flexibility can confer benefits in terms of mobility and agility, adverse effects of tissue laxity and fragility can give rise to clinical consequences that resonate far beyond the confines of the musculoskeletal system. There is hardly a clinical specialty to be found that is not touched in one way or another by JHS. Over the past decade, it has become evident that of all the complications that may arise in JHS, chronic pain is arguably the most menacing and difficult to treat. PMID:19889283

Grahame, Rodney

2009-12-01

285

Pleural disease and acquired immune deficiency syndrome  

Microsoft Academic Search

Patients with acquired immune deficiency syndrome (AIDS) do not frequently have pleural complications. However, pneumothorax is a troublesome complication of patients with AIDS. At some medical centres, more than 50% of patients with spontaneous pneumothorax have AIDS. Most patients with sponta- neous pneumothorax and AIDS have Pneumocystis carinii infection and necrotic subpleural blebs. The pneumothoraces in these patients usually cannot

R. W. Light; H. Hamm

1997-01-01

286

Goldenhar's syndrome.  

PubMed

We present a report on 16 patients with Goldenhar's syndrome. The criteria we required for the diagnosis of Goldenhar's syndrome consisted of an eye abnormality (lipoma, lipodermoid, epibulbar dermoid, or upper eyelid coloboma) associated with ear, mandibular, or vertebral anomalies (two of the three). Although Treacher Collins' syndrome can be easily differentiated from Goldenhar's syndrome, the differences between Goldenhar's syndrome and hemifacial microsomia are more difficult to delineate. PMID:626178

Feingold, M; Baum, J

1978-02-01

287

Complications and Monitoring – Guidelines on Parenteral Nutrition, Chapter 11  

PubMed Central

Compared to enteral or hypocaloric oral nutrition, the use of PN (parenteral nutrition) is not associated with increased mortality, overall frequency of complications, or longer length of hospital stay (LOS). The risk of PN complications (e.g. refeeding-syndrome, hyperglycaemia, bone demineralisation, catheter infections) can be minimised by carefully monitoring patients and the use of nutrition support teams particularly during long-term PN. Occuring complications are e.g. the refeeding-syndrome in patients suffering from severe malnutrition with the initiation of refeeding or metabolic, hypertriglyceridemia, hyperglycaemia, osteomalacia and osteoporosis, and hepatic complications including fatty liver, non-alcoholic fatty liver disease, cholestasis, cholecystitis, and cholelithiasis. Efficient monitoring in all types of PN can result in reduced PN-associated complications and reduced costs. Water and electrolyte balance, blood sugar, and cardiovascular function should regularly be monitored during PN. Regular checks of serum electrolytes and triglycerides as well as additional monitoring measures are necessary in patients with altered renal function, electrolyte-free substrate intake, lipid infusions, and in intensive care patients. The metabolic monitoring of patients under long-term PN should be carried out according to standardised procedures. Monitoring metabolic determinants of bone metabolism is particularly important in patients receiving long-term PN. Markers of intermediary, electrolyte and trace element metabolism require regular checks. PMID:20049074

Hartl, W. H.; Jauch, K. W.; Parhofer, K.; Rittler, P.

2009-01-01

288

Genital herpes complicating pregnancy.  

PubMed

Approximately 22% of pregnant women are infected with herpes simplex virus (HSV)-2, and 2% of women will acquire HSV during pregnancy. Remarkably, up to 90% of these women are undiagnosed because they are asymptomatic or have subtle symptoms attributed to other vulvovaginal disorders. Diagnosis of genital herpes relies on laboratory confirmation with culture or polymerase chain reaction assay of genital lesions and type-specific glycoprotein G-based serologic testing. Neonatal herpes is the most severe complication of genital HSV infection and is caused by contact with infected genital secretions at the time of labor. Maternal acquisition of HSV in the third trimester of pregnancy carries the highest risk of neonatal transmission. Despite advances in the diagnosis and treatment of neonatal herpes, little change in the incidence or serious sequelae from this infection has occurred. As such, prevention of the initial neonatal infection is critically important. Obstetricians are in a unique position to prevent vertical HSV transmission by identifying women with genital lesions at the time of labor for cesarean delivery, prescribing antiviral suppressive therapy as appropriate, and avoiding unnecessary invasive intrapartum procedures in women with genital herpes. Enhanced prevention strategies include identification of women at risk for HSV acquisition during pregnancy by testing women and possibly their partners for HSV antibodies and providing counseling to prevent transmission to women in late pregnancy. PMID:16199646

Brown, Zane A; Gardella, Carolyn; Wald, Anna; Morrow, Rhoda Ashley; Corey, Lawrence

2005-10-01

289

Ureteral Cannulation as a Complication of Urethral Catheterization  

PubMed Central

Urinary catheterization is a common procedure, particularly among patients with neurogenic bladder secondary to spinal cord injury. Urethral catheterization is associated with the well-recognized complications of catheter-associated urinary tract infections and limited genitourinary trauma. Unintentional ureteral cannulation represents a rare complication of urethral catheterization and has been previously described in only eight cases within the literature. We describe two cases of aberrant ureteral cannulation involving two patients with quadriplegia. These cases along with prior reports identify the spastic, insensate bladder and altered pelvic sensorium found in upper motor neuron syndromes as major risk factors for ureteral cannulation with a urinary catheter.

Greenlund, Andrew C.

2014-01-01

290

High dose intravenous immunoglobulin may be complicated by myocardial infarction  

PubMed Central

Intravenous immunoglobulin [IVIg] is useful for treating several clinical conditions and is largely considered safe, without major adverse events. Here we report a case of acute ST elevation myocardial infarction associated with high dose IVIg administration in a previously healthy 69-year-old male patient of Guillain Barre syndrome. The case is being reported to emphasize the need for treating physicians to be aware of thrombotic complications associated with IVIg. The thrombotic complications associated with IVIg are reviewed in brief, and the measures to reduce them are discussed. PMID:24872657

Vinod, Kolar Vishwanath; Kumar, Mritunjai; Nisar, Kare Kadavath

2014-01-01

291

Neuroleptic Malignant Syndrome: A Review  

PubMed Central

There has recently been interest in neuroleptic malignant syndrome (NMS) as the disorder has been better characterized. Nms is still poorly recognized, however, by most physicians. Hyperpyrexia, rigidity, altered consciousness, autonomic instability, and enzymatic evidence of muscle breakdown after drug intake are the cardinal features. The authors of this article review the proposed pathogenesis, complications, and treatment of NMS. PMID:21248945

O'Brien, Richard A.; Young, G. Bryan

1989-01-01

292

Gastrointestinal complications of oncologic therapy  

Microsoft Academic Search

Gastrointestinal complications are common in patients undergoing various forms of cancer treatment, including chemotherapy, radiation therapy, and molecular-targeted therapies. Many of these complications are life-threatening and require prompt diagnosis and treatment. Complications of oncologic therapy can occur in the esophagus (esophagitis, strictures, bacterial, viral and fungal infections), upper gastrointestinal tract (mucositis, bleeding, nausea and vomiting), colon (diarrhea, graft–versus–host disease, colitis

Marta Davila; Robert S Bresalier

2008-01-01

293

Complications of shoulder instability surgery.  

PubMed

Complications of shoulder instability surgery may results from errors made during the preoperative, intraoperative, or postoperative periods. Some complications are preventable whereas the risk of others can be reduced. A few complications remain unpreventable. Two sources of error in the preoperative period that can lead to complications are an incorrect diagnosis and failure to address a patient's expectations of treatment. These errors and their subsequent complications are preventable. Preventing complications during the intraoperative period begins with proper patient positioning and a thorough knowledge of shoulder anatomy. Understanding the indications and limitations of the various stabilization procedures, as well as applying proper surgical technique, is essential to avoid a surgical misadventure. Complications recognized in the postoperative period include recurrent instability, limitation of motion, inability to return to the previous level of sport, problems related to hardware, pain, development of osteoarthritis, and neurovascular injuries. Infection and hematoma formation may also occur; both need to be recognized and treated early to maximize outcome. A protocol for treating each complication that may occur often is helpful. Knowledge of the complications that can arise is paramount to preventing their occurrence. This knowledge comes through experience, study, and continued research. PMID:8582009

Wall, M S; Warren, R F

1995-10-01

294

[Münchausen syndrome by proxy].  

PubMed

Münchausen syndrome by proxy (also known as factitious disorder by proxy) is a psychiatric disorder which consists of fabricating or inducing illness in a child, usually by his mother. The motivation for the perpetrator's behavior is receivng satisfaction from the investigations and treatments that the child receives and from the medical environment, as part of a unique mental disturbance. External incentives for the behavior (such as economic gain) are absent. During the last few years about 20 cases of Münchausen syndrome by proxy were diagnosed in our ward. We describe a few less typical cases of patients with Münchausen syndrome by proxy. In all cases the offender, the mother, had only secondary education and no medical background. The socioeconomic background was variable. Recognition of the syndrome and a high index of clinical suspicion are needed in order to diagnose the disorder. Suspicious signs and symptoms include prolonged and illogical course of disease, odd complications, exacerbation that appear just before discharge from the hospital and symptoms that occur only in the presence of a specific care giver. Münchausen syndrome by proxy is not very rare. It appears in all ethnic groups, socioeconomic status and educational backgrounds. PMID:24416819

Scheuerman, Oded; Grinbaum, Iris; Garty, Ben Zion

2013-11-01

295

Complicated diverticulitis following renal transplantation  

Microsoft Academic Search

PURPOSE: Colonic perforations in renal transplant recipients have historically been associated with mortality rates as high as 50 to 100 percent. However, these previous series generally predate the use of cyclosporine-based immuno-suppressive protocols. METHODS: We retrospectively reviewed all patients who had undergone renal transplant from our institution and who developed complicated diverticulitis. Complicated diverticulitis was defined as diverticulitis involving free

Eric D. Lederman; David J. Conti; Neil Lempert; T. Paul Singh; Edward C. Lee

1998-01-01

296

Neurological complications of pertussis inoculation  

Microsoft Academic Search

Findings are presented in 36 children, seen in the past 11 years, who are believed to have suffered from neurological complications of pertussis inoculation (given as triple vaccine). The clustering of complications in the first 24 hours after inoculation suggests a causal rather than a coincidental relation. Possible contributory factors were present in one-third of patients studied and support the

M. Kulenkampff; J. S. Schwartzman; J. Wilson

1974-01-01

297

Clinical complications of osseointegrated implants  

Microsoft Academic Search

Statement of problem. There is no comprehensive review of the literature that identifies the complications reported in clinical dental implant studies. Purpose. This article attempted to determine the types of complications that have been reported and to provide data regarding their frequency. Methods. All available clinical studies from 1981 to 1997, published in English or with English abstract, that presented

Charles J. Goodacre; Joseph Y. K. Kan; Kitichai Rungcharassaeng

1999-01-01

298

Tourette Syndrome  

MedlinePLUS

If you have Tourette syndrome, you make unusual movements or sounds, called tics. You have little or no control over them. Common tics ... words, spin, or, rarely, blurt out swear words. Tourette syndrome is a disorder of the nervous system. ...

299

Klinefelter syndrome  

MedlinePLUS

47 X-X-Y syndrome ... have two XX chromosomes. Boys normally have an X and a Y chromosome. Klinefelter syndrome is when ... boy is born with at least one extra X chromosome. Usually, this occurs due to one extra ...

300

Sjogren's Syndrome  

MedlinePLUS

... the prognosis? What research is being done? Clinical Trials Organizations Additional resources from MedlinePlus What is Sjögren's Syndrome? Sjögren's syndrome is an autoimmune disorder in which immune cells attack and destroy the glands that produce ...

301

Paraneoplastic Syndromes  

MedlinePLUS

... the prognosis? What research is being done? Clinical Trials Organizations What are Paraneoplastic Syndromes? Paraneoplastic syndromes are a group of rare disorders that are triggered by an abnormal immune system response to a cancerous tumor known as ...

302

Asperger Syndrome  

MedlinePLUS

... and symptoms of Asperger syndrome is given the diagnosis of Autism Spectrum Disorder (ASD). If a person was diagnosed with Asperger syndrome before May 2013, his or her diagnosis stays the same, but can be considered ASD ...

303

Asperger Syndrome  

MedlinePLUS

... is Asperger syndrome? Asperger syndrome (AS) is an autism spectrum disorder (ASD), one of a distinct group ... and stereotyped patterns of behavior. Other ASDs include autistic disorder, childhood disintegrative disorder, and pervasive developmental disorder not ...

304

[Nerve compression syndromes of the upper extremity].  

PubMed

The nerve compression syndromes of the upper extremity, the common carpal and cubital tunnel syndromes are described, but also the less common entrapments such as thoracic outlet syndrome,suprascapular-, interosseus-posterior- and anterior entrapments and the Loge-de-Guyon and Wartenberg syndromes are mentioned. Besides history, symptoms and physical examination electrodiagnostic and imaging are necessary for establishing the correct diagnosis. The conservative treatment is less successful than the operative therapy. Surgical procedures and techniques are discussed as well as complications. The prognosis is in most cases good--provided that indication and technique were correct. PMID:20936778

Assmus, H; Martini, A-K

2010-09-01

305

Combined Pulmonary Fibrosis and Emphysema Syndrome  

PubMed Central

There is increasing clinical, radiologic, and pathologic recognition of the coexistence of emphysema and pulmonary fibrosis in the same patient, resulting in a clinical syndrome known as combined pulmonary fibrosis and emphysema (CPFE) that is characterized by dyspnea, upper-lobe emphysema, lower-lobe fibrosis, and abnormalities of gas exchange. This syndrome frequently is complicated by pulmonary hypertension, acute lung injury, and lung cancer. The CPFE syndrome typically occurs in male smokers, and the mortality associated with this condition, especially if pulmonary hypertension is present, is significant. In this review, we explore the current state of the literature and discuss etiologic factors and clinical characteristics of the CPFE syndrome. PMID:22215830

Rounds, Sharon I. S.

2012-01-01

306

Outcome after implantable cardioverter-defibrillator in patients with Brugada syndrome: the Gulf Brugada syndrome registry  

Microsoft Academic Search

Background and ObjectiveAmong patients with Brugada syndrome (BS) and aborted cardiac arrest, syncope, or inducible ventricular fibrillation at electrophysiologic study (EPS), the only currently recommended therapy is an implantable cardioverter-defibrillator (ICD), but these are not without complications. We assessed the total number of shocks (appropriate and inappropriate) and complications related to ICD in patients with BS.

Amin Daoulah; Alawi A. Alsheikh-Ali; Ali H. Ocheltree; Sara Ocheltree; Salem Al-Kaabi; Majed Malik; Abdul-Karim Al-Habib; Adel Hamed; Najib Al-Rawahi; Ali Al-Sayegh; Saad Al-Hasaniah; Eijaz Ul-Haq

307

The ABC's of stroke complications.  

PubMed

Stroke is the third leading cause of death in the United States, with more than 140,000 deaths per year. Complications related to stroke resulting in morbidity and mortality are very common and may result from cerebral and extracerebral causes. Cerebral causes include cerebral edema, hemorrhagic conversion of an ischemic infarct, and progression of penumbra to infarction. Extracerebral complications include deep vein thrombosis and pulmonary embolism, urinary tract infection, and aspiration. Many of these complications are largely preventable and often tracked as "quality metrics" in institutions with a stroke center designation. The focus of the article is primarily on common poststroke complications, such as aspiration, DVT, decubitus ulcers, seizures, and urinary catheter infections. Knowledge about potential poststroke complications is critical to earlier diagnosis, proper preventive strategies, and management. PMID:21207342

Freeman, W David; Dawson, Steven B; Flemming, Kelly D

2010-11-01

308

Pregnancy complicated by multiple endocrine neoplasia type IIA (Sipple's syndrome)  

Microsoft Academic Search

A patient with preexisting multiple endocrine neoplasia type IIA had normal 24-hour urinary metanephrine and vanillylmandelic acid excretions before and during pregnancy. After a benign prenatal course, the patient had a term spontaneous vaginal delivery. Multiple endocrine neoplasia type IIA antedating pregnancy may be associated with a normal obstetric outcome in the absence of a pheochromocytoma. (Am J Obstet Gynecol

Joseph R. Wax; M EGGLESTONJR; Katherine E. Teague

1997-01-01

309

Velocardiofacial Syndrome  

ERIC Educational Resources Information Center

Velocardiofacial syndrome (VCFS), also known as DiGeorge, conotruncal anomaly face, and Cayler syndromes, is caused by a microdeletion in the long arm of Chromosome 22. We review the history of the syndrome from the first clinical reports almost half a century ago to the current intriguing molecular findings associating genes from the…

Gothelf, Doron; Frisch, Amos; Michaelovsky, Elena; Weizman, Abraham; Shprintzen, Robert J.

2009-01-01

310

Metabolic Syndrome  

MedlinePLUS

... metabolic syndrome fact sheet hOW is the metAbOlic syndrOme treAted? Increasing physical activity and losing weight are the best ways to begin to manage your condition. Medications can also treat risk factors such as ... factors for the metabolic syndrome, talk with your doctor. Your doctor can run ...

311

Aase syndrome  

MedlinePLUS

Aase-Smith syndrome; Hypoplastic anemia/Triphalangeal thumb syndrome ... Jones KL, ed. Aase syndrome. In: Smith's Recognizable Patterns Of Human Malformation. 6th ed. Saunders. 2005. Clinton C, Gazda HT. Diamond-Blackfan Anemia. 2009 Jun 25 [Updated 2013 Jul ...

312

Down syndrome  

MedlinePLUS

Down syndrome is a genetic condition in which a person has 47 chromosomes instead of the usual 46. ... In most cases, Down syndrome occurs when there is an extra copy of chromosome 21. This form of Down syndrome is called Trisomy 21. ...

313

[Unusual complication of noninvasive ventilation: The œsogastric pneumatosis associated with a subcutaneous emphysema].  

PubMed

The non-invasive ventilation (NIV) is a technique of ventilator support to avoid endotracheal intubation and its potential complications. However, it has some complications that are usually harmless to type of erythema and/or cutaneous ulcerations, mouth or nose dryness, conjunctival irritation and rarely lesions of barotrauma, volotrauma or gastric insufflation with nausea and vomiting. We report the case of a patient who had an unusual complication of NIV: sub mucosa gastro-esophageal pneumatosis associated with subcutaneous emphysema occurring on the second day after one hepatectomy which was settled but complicated with a postoperative pulmonary aspiration syndrome. PMID:24646777

Bakkali, H; Aissa, I; Massou, S; Wartiti, L; Abouelalaa, K; Balkhi, H; Drissi Kamili, N

2014-08-01

314

Biochemistry of HELLP syndrome.  

PubMed

The HELLP syndrome is a serious complication of pregnancy characterized by hemolysis (H), elevated liver (EL) enzymes, and low platelet (LP) count that occurs in 0.2-0.6% of all pregnancies and in 10-20% of cases with severe preeclampsia and frequently leads to adverse maternal and perinatal outcome. The exact pathobiology of HELLP syndrome has not been clearly defined. As it is considered a form or a complication of severe preeclampsia, it likely has its origin in aberrant placental development and function resulting in ischemia-producing oxidative stress. However, there is still a debate on whether HELLP must be considered a severe form of preeclampsia or a separate disease entity. It can be described as a placenta-induced disease, as is preeclampsia itself, but with a more acute and predominant inflammatory process typically targeting the liver and with a greater activation of the coagulation system. This occurs during a disordered immunologic process and may be due to a genetic predisposition. In this review, we discuss the main biochemical characteristics of HELLP syndrome, particularly focusing on molecular aspects of placental involvement and maternal systemic responses. PMID:21404915

Benedetto, Chiara; Marozio, Luca; Tancredi, Annalisa; Picardo, Elisa; Nardolillo, Paola; Tavella, Anna Maria; Salton, Loredana

2011-01-01

315

Hypermobility syndromes from the clinician's perspective: an overview.  

PubMed

Symptomatic generalized hypermobility is a frequent occurring condition among patients referred to the rheumatologist or other medical specialist. In a subset of patients, a further classifying diagnosis of a specific syndrome can (and should) be made, based on pattern recognition and knowledge of the spectrum of hypermobility syndromes. Diagnostic clues are the patient's and family history and signs at physical examination, including skin abnormalities. It is especially important to recognize hypermobility syndromes with potentially life threatening complications. Genetic testing is only available for some syndromes; is only indicated if there is a reasonable pretest probability regarding a specific syndrome, especially if this syndrome can have life-threatening complications. The therapy is for the major part of syndromes only symptomatic; key features of management are education and physical exercises; joint surgery is to be avoided. PMID:24861097

Jacobs, J W; da Silva, José António

2014-01-01

316

Sj?gren's syndrome associated with antiphospholipid syndrome and fetal myocardial echogenicity: case report  

PubMed Central

Summary Introduction Sjögren’s syndrome is a rare systemic autoimmune disorder associated with pregnancy (0.3–0.6%). The typical occurrence of anti-Ro/SSA and anti-La/SSB autoantibodies in the maternal serum can modify the perinatal outcome: neonatal lupus and congenital heart block are the most common fetal complications. Case we report a case of pregnancy complicated by a secondary form of SS associated with antiphospholipid syndrome and fetal myocardial echogenicity. Conclusion in conclusion, increased attention must be paid to pregnancies associated with autoimmune disorders, since careful ultrasonographic and clinical monitoring and preventive treatment with corticosteroids could minimize severe and common fetal complications. PMID:23991277

Giacobbe, Annamaria; Grasso, Roberta; Foti, Grazia; Interdonato, Maria Lieta; Mancuso, Alfredo

2013-01-01

317

Eosinophilic infiltrate in a patient with severe Legionella pneumonia as a levofloxacin-related complication: a case report  

Microsoft Academic Search

INTRODUCTION: Legionella pneumonia can appear with different levels of severity and it can often present with complications such as acute respiratory distress syndrome. CASE PRESENTATION: We report the case of a 44-year-old Caucasian man with Legionella pneumonia with successive development of severe acute respiratory distress syndrome. During his stay in intensive care the clinical and radiological situation of the previously

Nicola Facciolongo; Francesco Menzella; Claudia Castagnetti; Alberto Cavazza; Roberto Piro; Cristiano Carbonelli; Luigi Zucchi

2010-01-01

318

Allo-limbal transplantation in patients with limbal stem cell deficiency  

Microsoft Academic Search

AIMTo report the outcome of a series of patients with stem cell deficiency who underwent allo-limbal transplantation and to describe a technique for this procedure.METHODSSix consecutive patients underwent allo-limbal stem cell transplantation. The primary diagnosis included alkali burn (n=2), trachoma (n=1), chronic rosacea blepharitis and keratoconjunctivitis (n=1), aniridia (n=1), and Stevens–Johnson syndrome (n=1). The limbal rim consisted of peripheral cornea

Harminder S Dua; Augusto Azuara-Blanco; DENNIS S C LAM; M. L SLOPER

1999-01-01

319

Fluconazole induced toxic epidermal necrolysis: a case report  

PubMed Central

Drug induced toxic epidermal necrolysis and Stevens Johnson syndrome are more commonly associated with medications such as sulfonamides, penicillin, anticonvulsants, oxicam non-steroidal anti-inflammatory drugs, allopurinol and corticosteroids. Isolated instances secondary to drugs outside of the aforementioned classes have also been reported. We report a case of probable toxic epidermal necrolysis induced by fluconazole in a 52 year old woman. PMID:20062708

2009-01-01

320

[Toxic epidermal necrolysis after treatment with lamotrigine].  

PubMed

A case of toxic epidermal necrolysis following treatment with lamotrigine is presented. A 20 year old male suffering from epilepsy was treated with lamotrigine in addition to valproic acid. After three weeks he developed cutaneous manifestations of Steven-Johnson's syndrome followed by toxic epidermal necrolysis, mucosal lesions and liver symptoms. He was treated with systemic corticosteroids, antibiotics and intravenous fluid, and recovered after a few weeks. PMID:9825684

Zachariae, C O; Fogh, K

1998-11-01

321

[The clinico-laboratory characteristics and diagnosis of acute stomatogenic sepsis].  

PubMed

Thirty-four patients with acute stomatogenic sepsis developing in grave ulcerative necrotic stomatitis (including that in Stevens-Johnson's and Lyell's syndromes) were examined. Homeostasis parameters were shifted in these patients. To facilitate timely diagnosis of acute stomatogenic sepsis, the authors offer a differential diagnostic table. Patients with grave forms of stomatitis are recommended to be referred for examination and treatment to specialized dentistry hospitals in order to early diagnose the disease and prevent the development of acute sepsis. PMID:8754538

El'kova, N L; Korchinova, L D; Kobtseva, T M

1996-01-01

322

Complications of unilateral weight bearing.  

PubMed

The most common and significant complication of excessive unilateral weight bearing in adult horses is support limb laminitis. Young horses with unilateral lameness problems develop support limb laminitis infrequently compared with adult horses. PMID:19203705

Baxter, Gary M; Morrison, Scott

2008-12-01

323

Analysis of Complications Following Decompressive Craniectomy for Traumatic Brain Injury  

PubMed Central

Objective Adequate management of increased intracranial pressure (ICP) is critical in patients with traumatic brain injury (TBI), and decompressive craniectomy is widely used to treat refractory increased ICP. The authors reviewed and analyzed complications following decompressive craniectomy for the management of TBI. Methods A total of 89 consecutive patients who underwent decompressive craniectomy for TBI between February 2004 and February 2009 were reviewed retrospectively. Incidence rates of complications secondary to decompressive craniectomy were determined, and analyses were performed to identify clinical factors associated with the development of complications and the poor outcome. Results Complications secondary to decompressive craniectomy occurred in 48 of the 89 (53.9%) patients. Furthermore, these complications occurred in a sequential fashion at specific times after surgical intervention; cerebral contusion expansion (2.2 ± 1.2 days), newly appearing subdural or epidural hematoma contralateral to the craniectomy defect (1.5 ± 0.9 days), epilepsy (2.7 ± 1.5 days), cerebrospinal fluid leakage through the scalp incision (7.0 ± 4.2 days), and external cerebral herniation (5.5 ± 3.3 days). Subdural effusion (10.8 ± 5.2 days) and postoperative infection (9.8 ± 3.1 days) developed between one and four weeks postoperatively. Trephined and post-traumatic hydrocephalus syndromes developed after one month postoperatively (at 79.5 ± 23.6 and 49.2 ± 14.1 days, respectively). Conclusion A poor GCS score (? 8) and an age of ? 65 were found to be related to the occurrence of one of the above-mentioned complications. These results should help neurosurgeons anticipate these complications, to adopt management strategies that reduce the risks of complications, and to improve clinical outcomes. PMID:21082053

Ban, Seung Pil; Yang, Hee-Jin; Chung, Yeong Seob; Lee, Sang Hyung; Han, Dae Hee

2010-01-01

324

The Schnitzler syndrome  

PubMed Central

The Schnitzler syndrome is a rare and underdiagnosed entity which is considered today as being a paradigm of an acquired/late onset auto-inflammatory disease. It associates a chronic urticarial skin rash, corresponding from the clinico-pathological viewpoint to a neutrophilic urticarial dermatosis, a monoclonal IgM component and at least 2 of the following signs: fever, joint and/or bone pain, enlarged lymph nodes, spleen and/or liver, increased ESR, increased neutrophil count, abnormal bone imaging findings. It is a chronic disease with only one known case of spontaneous remission. Except of the severe alteration of quality of life related mainly to the rash, fever and pain, complications include severe inflammatory anemia and AA amyloidosis. About 20% of patients will develop a lymphoproliferative disorder, mainly Waldenström disease and lymphoma, a percentage close to other patients with IgM MGUS. It was exceedingly difficult to treat patients with this syndrome until the IL-1 receptor antagonist anakinra became available. Anakinra allows a complete control of all signs within hours after the first injection, but patients need continuous treatment with daily injections. In many aspects, the Schnitzler syndrome resembles the genetically determined auto-inflammatory syndromes involving activating mutations of the NLRP3 inflammasome. This latter point and its consequences will be addressed. PMID:21143856

2010-01-01

325

Oral complications in radiation therapy.  

PubMed

The dentist is integral to managing care for patients with head and neck cancer. Oral complications may manifest during and/or following radiation therapy and can last for the lifetime of the individual. This article reviews a number of these complications as well as the resources available to dentists for managing care of radiation oncology patients and improving their quality of life. PMID:15055655

Harrison, Jody S; Dale, Robert A; Haveman, Carl W; Redding, Spencer W

2003-01-01

326

Growing Teratoma Syndrome  

PubMed Central

Growing teratoma syndrome (GTS) is a rare clinical entity, which presents with enlarging teratomas masses of the retroperitoneum or other locations, occurring during or after systemic chemotherapy for the treatment of nonseminomatous germ cell of the testis (NSGCT), with normalised tumour markers. Awareness of this syndrome is necessary in order to prevent unnecessary chemotherapy and allow optimal management. Prognosis is excellent after the excision of these tumors, but surgery has to be as complete as possible. Surgical resection of bulky GTS lesions is technically challenging; intraoperative complications may occur; that is, why the treatment must not be delayed. Our experience in the surgical management of these lesions is reviewed in the following work. PMID:25197607

Scavuzzo, Anna; Santana Ríos, Zael Arturo; Noverón, Nancy Reynoso; Jimenez Ríos, Miguel Angel

2014-01-01

327

Neurologic Complications in Percutaneous Nephrolithotomy  

PubMed Central

Purpose Percutaneous nephrolithotomy (PCNL) has been the preferred procedure for the removal of large renal stones in Iran since 1990. Recently, we encountered a series of devastating neurologic complications during PCNL, including paraplegia and hemiplegia. There are several reports of neurologic complications following PCNL owing to paradoxical air emboli, but there are no reports of paraplegia following PCNL. Materials and Methods We retrospectively reviewed the medical records of patients who had undergone PCNL in 13 different endourologic centers and retrieved data related to neurologic complications after PCNL, including coma, paraplegia, hemiplegia, and quadriplegia. Results The total number of PCNL procedures in these 13 centers was 30,666. Among these procedures, 11 cases were complicated by neurologic events, and four of these cases experienced paraplegia. All events happened with the patient in the prone position with the use of general anesthesia and in the presence of air injection. There were no reports of neurologic complications in PCNL procedures performed with the patient under general anesthesia and in the prone position and with contrast injection. Conclusions It can be assumed that using room air to opacify the collecting system played a major role in the occurrence of these complications. Likewise, the prone position and general anesthesia may predispose to these events in the presence of air injection. PMID:23526482

Basiri, Abbas; Soltani, Mohammad Hossein; Kamranmanesh, Mohammadreza; Tabibi, Ali; Mohsen Ziaee, Seyed Amir; Nouralizadeh, Akbar; Sharifiaghdas, Farzaneh; Poorzamani, Mahtab; Gharaei, Babak; Ozhand, Ardalan; Lashay, Alireza; Ahanian, Ali; Aminsharifi, Alireza; Sichani, Mehrdad Mohammadi; Asl-Zare, Mohammad; Ali Beigi, Faramarz Mohammad; Najjaran, Vahid; Abedinzadeh, Mehdi

2013-01-01

328

Serotonin Syndrome  

PubMed Central

Background Serotonin syndrome is a potentially life-threatening syndrome that is precipitated by the use of serotonergic drugs and overactivation of both the peripheral and central postsynaptic 5HT-1A and, most notably, 5HT-2A receptors. This syndrome consists of a combination of mental status changes, neuromuscular hyperactivity, and autonomic hyperactivity. Serotonin syndrome can occur via the therapeutic use of serotonergic drugs alone, an intentional overdose of serotonergic drugs, or classically, as a result of a complex drug interaction between two serotonergic drugs that work by different mechanisms. A multitude of drug combinations can result in serotonin syndrome. Methods This review describes the presentation and management of serotonin syndrome and discusses the drugs and interactions that can precipitate this syndrome with the goal of making physicians more alert and aware of this potentially fatal yet preventable syndrome. Conclusion Many commonly used medications have proven to be the culprits of serotonin syndrome. Proper education and awareness about serotonin syndrome will improve the accuracy of diagnosis and promote the institution of the appropriate treatment that may prevent significant morbidity and mortality. PMID:24358002

Volpi-Abadie, Jacqueline; Kaye, Adam M.; Kaye, Alan David

2013-01-01

329

Bullous Lesions in a Patient with Systemic Lupus Erythematosus  

PubMed Central

Bullous eruptions in patients with lupus erythematosus can be difficult to diagnose as bullous lesions can develop in lupus-specific lesions, and primary blistering disorders can also occur. Additionally, these patients tend to have multiple co-morbidities making them more likely to be on many medications that can lead to bullous drug reactions. A thorough history, the clinical presentation, and histopathological findings along with direct immunofluorescence can be helpful in diagnosing most cases. The authors report the case of a woman with a long history of systemic lupus erythematosus who initially presented in their clinic for diagnosis and management of erythema dyschromicum perstans and one year later developed bullae in atypical targetoid lesions on the extremities and trunk. They discuss several blistering disorders that have been reported in patients with lupus erythematosus with a focus on features that help distinguish erythema multiforme, fixed drug eruption, and lupus erythematosus from Stevens-Johnson syndrome/toxic epidermal necrolysis. In the patient described herein, the authors favor a diagnosis of Stevens-Johnson syndrome, but the classification between erythema multiforme major and Stevens-Johnson syndrome/toxic epidermal necrolysis cannot be made in some cases. PMID:24563697

Smith, Jennifer L.

2014-01-01

330

Down's syndrome.  

PubMed

The sequencing of chromosome 21 and the use of models of Down's syndrome in mice have allowed us to relate genes and sets of genes to the neuropathogenesis of this syndrome, and to better understand its phenotype. Research in prenatal screening and diagnosis aims to find methods to identify fetuses with Down's syndrome, and reduce or eliminate the need for amniocentesis. Other areas of active research and clinical interest include the association of Down's syndrome with coeliac disease and Alzheimer's disease, and improved median age of death. Medical management of the syndrome requires an organised approach of assessment, monitoring, prevention, and vigilance. Improvements in quality of life of individuals with Down's syndrome have resulted from improvements in medical care, identification and treatment of psychiatric disorders (such as depression, disruptive behaviour disorders, and autism), and early educational interventions with support in typical educational settings. Approaches and outcomes differ throughout the world. PMID:12699967

Roizen, Nancy J; Patterson, David

2003-04-12

331

"Sickle Cell Disease in the Emergency Department: Atypical Complications and Management"  

PubMed Central

Sickle cell disease is the most common inherited blood disorder in the United States. This disorder of hemoglobin structure leads to a chronic hemolytic anemia and complex chronic disease manifested by sudden, severe, and life-threatening complications. These acute complications can occur in any organ system beginning in early childhood and lasting throughout life. The intermittent nature and acuity of these complications lend the emergency department to be an important site of care. The hallmark of sickle cell disease is the vasoocclusive painful event. Other more “typical” complications include fever, acute chest syndrome, priapism, and ischemic stroke. Children with sickle cell disease can also present with other “atypical” complications that can have devastating consequences if they are unrecognized. Detailed discussion of these “atypical” sickle cell disease complications, organized by organ system involved, will be the focus of this article. PMID:21927581

Brandow, Amanda M.; Liem, Robert

2011-01-01

332

Aicardi syndrome.  

PubMed

Aicardi syndrome is a rare neurodevelopmental disease characterised by congenital chorioretinal lacunae, corpus callosum dysgenesis, seizures, polymicrogyria, cerebral callosum, chorioretinopathy and electroencephalogram abnormality. We present a case of Aicardi syndrome with callosal hypogenesis in a 4.5-month-old baby who presented with infantile spasms. Ophthalmoscopy revealed chorioretinal lacunae. The clinical and magnetic resonance imaging features were diagnostic of Aicardi syndrome. PMID:22815034

Singh, Paramdeep; Goraya, Jatinder Singh; Saggar, Kavita; Ahluwalia, Archana

2012-07-01

333

[Kounis syndrome].  

PubMed

Kounis syndrome was described in 1991 by Kounis and Zavras as the coincidental occurrence of acute coronary syndromes with allergic reactions (anaphylactic or anaphylactoid). Today, allergic angina and allergic myocardial infarction are referred to as Kounis syndrome, and the latter has been reported in association with a variety of drugs, insect stings, food, environmental exposures and medical conditions, among other factors. The incidence is not known, as most of the available information comes from case reports or small case series. In this article, the clinical aspects, diagnosis, pathogenesis, related conditions and therapeutic management of the syndrome are discussed. PMID:22154226

Rico Cepeda, P; Palencia Herrejón, E; Rodríguez Aguirregabiria, M M

2012-01-01

334

[Surgical management of visceral heterotaxy syndrome].  

PubMed

Surgical management of the heterotaxy syndrome including asplenia or polysplenia is still challenging, because they have not only congenital heart defects but also gastrointestinal abnormalities. In most cases, they are Fontan candidates, however, indications and procedures of surgical strategy toward Fontan operation are quite difficult because of cardiac complications or abdominal manifestations. We mentioned indications, surgical technique, peri- and post-operative managements of asplenia syndrome with our experiences and results. PMID:22868426

Fujimoto, Y; Sakamoto, K

2012-07-01

335

Haemolytic-Uraemic Syndrome in Typhoid Fever  

Microsoft Academic Search

Among 48 patients with a typhoid infection 6 (12·5%) developed the haemolytic-uraemic syndrome. Neither glucose-6-phosphate dehydrogenase deficiency nor therapy with chloramphenicol could be incriminated as the causal factor. Evidence presented here suggests that the mechanism is localized intravascular coagulation.The presence of leucocytosis in typhoid fever suggests a complication and should alert one to the possibility of the haemolytic-uraemic syndrome. Furthermore,

N. M. Baker; A. E. Mills; I. Rachman; J. E. P. Thomas

1974-01-01

336

Cerebrovascular involvement in Ehlers-Danlos syndrome  

Microsoft Academic Search

Opinion statement  Ehlers-Danlos syndrome type IV is one of the most prominent heritable disorders of connective tissues associated with neurovascular\\u000a disease. It is an uncommon disorder characterized by thin translucent skin, distinctive facial features, excessive bruising,\\u000a and rupture of blood vessels or viscera. The typical neurovascular complications of this syndrome are carotid cavernous fistulas,\\u000a intracranial aneurysms, and cervical artery dissections. Because

Wouter I. Schievink

2004-01-01

337

Neurologic Complications in Infective Endocarditis  

PubMed Central

Neurologic complications of infective endocarditis (IE) are common and frequently life threatening. Neurologic events are not always obvious. The prediction and management of neurologic complications of IE are not easily approached algorithmically, and the impact they have on timing and ability to surgically repair or replace the affected valve often requires a painstaking evaluation and joint effort across multiple medical disciplines in order to achieve the best possible outcome. Although specific recommendations are always tailored to the individual patient, there are some guiding principles that can be used to help direct the decision-making process. Herein, we review the pathophysiology, epidemiology, manifestations, and diagnosis of neurological complications of IE and further consider the impact they have on clinical decision making. PMID:25360207

Morris, Nicholas A.; Matiello, Marcelo; Samuels, Martin A.

2014-01-01

338

Biliary complications after liver transplantation  

Microsoft Academic Search

Opinion statement  \\u000a \\u000a \\u000a \\u000a \\u000a – \\u000a \\u000a The incidence of biliary complications after liver transplant is estimated to be 8% to 20%. Post-liver transplant biliary\\u000a complications may lead to acute and chronic liver injury. The early recognition and prompt treatment of such complications\\u000a improves the long-term survival of the patient and graft.\\u000a \\u000a \\u000a \\u000a \\u000a – \\u000a \\u000a An understanding of the type of biliary reconstruction, the rationale for

Sanjay Jagannath; Anthony N. Kalloo

2002-01-01

339

Complications of intraoperative radiation therapy  

SciTech Connect

The authors have studied the severe complications occurring after treatment with intraoperative radiation therapy (IORT) in patients with locally advanced carcinoma of the rectum. Four groups of patients were compared: Group 1 (80 patients) had treatment with surgery alone for mobile and resectable tumors; Group 2 (23 patients) had treatment with high dose preoperative irradiation followed by surgical resection for tumors which were fixed to adjacent structures and initially unresectable for cure; Group 3 (24 patients, primary disease) and Group 4 (17 patients, locally recurrent disease) had locally advance tumors as in Group 2 but were treated with IORT after preoperative irradiation and attempted surgical resection. All but 3 complications occurred within one year of therapy. Severe complications were seen in 16% of patients in Group 1, 35% in Group 2, 21% in Group 3 and 47% in Group 4 (32% in Groups 3 and 4 combined).

Tepper, J.E.; Gunderson, L.L.; Orlow, E.; Cohen, A.M.; Hedberg, S.E.; Shipley, W.U.; Blitzer, P.H.; Rich, T.

1984-10-01

340

Endoscopic treatment of Crohn's complications.  

PubMed

The progression to fistula and strictures is part of the natural history of Crohn's disease (CD) and these complications negatively affect the quality of life of CD patients. Surgery is the traditional treatment of CD strictures. However, due the chronicity of the inflammatory process and the associated fibrosis, postoperative recurrence occurs frequently. The lack of specific drug to treat fibrotic strictures and their irreversible nature has drawn the attention to less invasive and bowel-sparing therapeutic modalities. Endoluminal therapies may provide effective option in relieving symptoms associated with CD complications and reduce the need for repeated surgery with substantial clinical benefit. This review will discuss the current use and efficacy of the endoscopic treatment of CD complications. New endoscopic modalities and recent advances will be also evaluated. PMID:24849124

Buda, Andrea; Okolo, Patrick I

2014-11-01

341

Gastrointestinal complications after cardiac surgery.  

PubMed Central

Gastrointestinal complications after cardiac surgery are uncommon, but are associated with a high morbidity and mortality. Over 11 years 8559 procedures requiring cardiopulmonary bypass were performed in this unit and 35 patients were identified who developed gastrointestinal complications after surgery, an incidence of 0.41%. There were nine deaths in this group, a mortality of 25.7% compared with an overall mortality after cardiac surgery in Ireland ranging from 3.24% to 4.81%. These complications required surgery in 21 patients. The most common indication for surgical intervention was upper gastrointestinal bleeding in 10 patients, three patients had acute pancreatitis, two patients had perforated peptic ulcer; two patients had intestinal ischaemia, with five cases of colon pathology. The difficulties of making an early diagnosis are outlined and a low threshold to exploratory laparotomy is recommended. PMID:8422146

Egleston, C. V.; Wood, A. E.; Gorey, T. F.; McGovern, E. M.

1993-01-01

342

Osler-Weber-Rendu syndrome during pregnancy.  

PubMed

Osler-Weber-Rendu syndrome is a very rare systemic fibrovascular dysplasia. Rupture of angiomas can cause haemorrhages, which sometimes can be severe with difficult bleeding control. The main manifestation is recurrent epistaxis. Treatment of this disorder is symptomatic. During pregnancy, there may be an increased risk of complications. We describe a case of a pregnant woman with Osler-Weber-Rendu syndrome. Besides frequent epistaxis and microcytic hypochromic anaemia that resolved with oral iron treatment, she had a normal pregnancy, vaginal delivery and puerperium without complications. PMID:23814092

Inocêncio, G; Braga, A; Lima, T; Buchner, G

2013-01-01

343

Nicolau Syndrome after Intramuscular Benzathine Penicillin Injection  

PubMed Central

A 3-year-old boy was admitted to the emergency department with right lower limb pain, edema, and livedoid discoloration that occurred immediately after intramuscular injection of benzathine penicillin. The patient was diagnosed with Nicolau syndrome, a rare complication of intramuscular injection presumed to be related to the inadvertent intravascular injection. It was first reported following intramuscular injection of bismuth salt, but it can occur as a complication of various other drugs. Fasciotomy was carried out due to the resultant compartment syndrome and medical therapy with heparin, corticosteroid, and pentoxifyllin was initiated.

Noaparast, Morteza; Mirsharifi, Rasoul; Elyasinia, Fezzeh; Parsaei, Reza; Kondori, Hessam; Farifteh, Sara

2014-01-01

344

Caregiving, bereavement and complicated grief  

PubMed Central

Most deaths are preceded by chronic illness and disability and the provision of support by family caregivers. The purpose of this article is to describe how the caregiving experience affects bereavement, with an emphasis on the relationship between challenging caregiving situations and difficult grieving processes – often referred to as `complicated grief'. The article starts with a brief summary of the general literature on caregiving and bereavement. It then defines complicated grief and discusses why some caregivers may struggle with the death of their loved one. Finally, it offers practical suggestions for what professionals can do to help caregivers both before and after the death has occurred. PMID:20463850

Boerner, Kathrin; Schulz, Richard

2010-01-01

345

Complications from international surgery tourism.  

PubMed

Medical tourism is an increasing trend, particularly in cosmetic surgery. Complications resulting from these procedures can be quite disruptive to the healthcare industry in the United States since patients often seek treatment and have no compensation recourse from insurance. Despite the increasing number of plastic surgery patients seeking procedures abroad, there have been little reported data concerning outcomes, follow-up, or complication rates. Through a survey of American Society of Plastic Surgeons (ASPS) members, the authors provide data on trends to help define the scope of the problem. PMID:21813883

Melendez, Mark M; Alizadeh, Kaveh

2011-08-01

346

Surgical Complications of Gynecologic Surgery  

PubMed Central

Complications of gynecological surgery are considerable and when reviewed in detail are almost frightening. There is no substitute for experience and intimate knowledge of the intricate pelvic structures in health and disease. Anyone who is active in the field is sooner or later going to experience some difficulty whether it be due to his miscalculation or to innate conditions in the patient which are beyond his/her control. It is the responsibility of the pelvic surgeon to recognize the complication and apply proper corrective measures. The patient should not be given false hopes of sure success nor should she be deprived of whatever hope for success does exist. PMID:572875

Weekes, Leroy R.; Gandhi, Shobhana Anil; Gandhi, Anil Krishnakumar

1977-01-01

347

What is new in the Marfan syndrome?  

PubMed

The Marfan syndrome is an autosomal dominant disorder of connective tissue, caused by mutations in the FBN1 gene on chromosome 15. More than 500 mutations have been identified and almost all are unique to an affected individual or family. Genotype--phenotype correlations in the Marfan syndrome have been complicated by the large number of unique mutations reported, as well as by clinical heterogeneity among individuals with the same mutation. A relatively unknown cardiovascular manifestation of Marfan syndrome is dilatation of the main pulmonary artery. Of 50 patients with Marfan syndrome, MR imaging showed in 74% patients an enlarged pulmonary artery root above the upper limit of normal. Aortic elasticity determined by measurement of local distensibility and flow wave velocity with MR imaging is decreased in non-operated patients with Marfan syndrome. Aortic distensibility of the thoracic descending aorta appeared to be the strongest predictor for descending aortic complications. Over the past 30 years improvement of diagnostic modalities and aggressive medical and surgical therapy, have resulted in considerable improvement of life expectancy of patients with Marfan syndrome. Further studies are needed to investigate the role of modulating genes and genotype--phenotype correlations. Long-term follow-up studies may reveal the prognostic significance of aortic elasticity and may identify patients at risk of aortic complications. PMID:15590086

Nollen, G J; Mulder, B J M

2004-12-01

348

Outcomes with the Boston Type 1 Keratoprosthesis at Instituto de Microcirug?a Ocular IMO  

PubMed Central

Purpose To report the outcomes on the Boston Type 1 Keratoprosthesis at our institution. Design Retrospective analysis case series. Participants We analyzed 54 eyes of 53 patients who previously underwent Boston Type 1 Keratoprosthesis surgery at our institution from July 2006 to March 2011. Methods Preoperative and postoperative parameters were collected and analyzed. Main outcome measures Visual acuity and keratoprosthesis stability. Results Common preoperative diagnoses were penetrating keratoplasty failure in 49 eyes (90.7%), chronic keratitis in 2 eyes (3.7%), ocular cicatricial pemphigoid in 1 eye (1.85%), Stevens Johnson syndrome in 1 eye (1.85%) and corneal vascularization in 1 eye (1.85%). Additionally, 40 eyes (74%) had preoperative glaucoma, and an Ahmed valve was implanted in 55% of them. Preoperative BCVA ranged from 20/200 to light perception. At an average follow-up of 20.15 months ± 12.7 (range, 1–56), postoperative vision improved to ?20/200 in 18 eyes (33.3%) and ?20/50 in 4 eyes (7.4%). The graft retention was 96%. Conclusions The Boston Type 1 keratoprosthesis is a valid option for high-risk patients. The design improvements in the Boston keratoprosthesis, as well as the daily implementation of the therapeutic methods, have notably diminished occurrence of the most serious complications, such as corneal necrosis and endophthalmitis. As such, glaucoma and its subsequent complications now stand as the most prevalent prognostic factor in the long term. PMID:23960937

Guell, Jose L.; Arcos, Edilio; Gris, Oscar; Aristizabal, Diego; Pacheco, Miguel; Sanchez, Claudia L.; Manero, Felicidad

2011-01-01

349

Wolf syndrome  

Microsoft Academic Search

Since the initial description in 1965 of Wolf syndrome, or deletion of the short arm of chromosome number four, over one hundred cases have been reported. Much less, however, has been published on the radiologic findings in this disorder. We report a case with both typical and unusual features of the 4p- syndrome, including “bottle opener” deformity of the clavicles,

D. S. Katz; T. H. Smith

1991-01-01

350

Joubert Syndrome  

MedlinePLUS

... our understanding of Joubert syndrome, and for developing methods of treatment and prevention. NINDS, in conjunction with the NIH Office of Rare Disorders, sponsored a symposium on Joubert syndrome in 2002. Research priorities for the disorder were outlined at this meeting. ...

351

Barth syndrome  

Microsoft Academic Search

Key words Disease name and synonyms Definition\\/diagnostic criteria Epidemiology Abstract Barth syndrome is a metabolic disorder characterized by a cardiomyopathy of the dilated type, more rarely of the hypertrophic type, neutropenia, skeletal myopathy, diminished statural growth and 3-methylglutaconicaciduria. However the clinical presentation can be of variable expression. The disease can be slowly progressive or sudden. In most cases, Barth syndrome

Pascale de Lonlay; Dimitri Schlemmer; Paola Melacini

352

Stroke Syndromes  

Microsoft Academic Search

We gave a short overview of the most important stroke syndromes in the clinical setting. Knowledge of these syndromes helps\\u000a to understand the complex pathophysiology of cerebral ischemia. Combination of clinical findings with the data from the new\\u000a and evolving imaging techniques certainly facilitates and improves care for stroke patients.

Georg Gahn

353

Pfeiffer syndrome  

Microsoft Academic Search

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

Annick Vogels; Jean-Pierre Fryns

2006-01-01

354

Bazex Syndrome*  

PubMed Central

Acrokeratosis Paraneoplastica or Bazex syndrome is a dermatologic manifestation usually associated with the diagnosis of squamous cell carcinoma of the upper aerodigestive tract. We report a case with exuberant clinical manifestations, exemplifying the typical cutaneous lesions in this rare syndrome, in a patient with squamous cell carcinoma of the esophagus. PMID:24346922

Rodrigues Junior, Ismael Alves; Gresta, Leticia Trivellato; Cruz, Rafaela Carolina; Carvalho, Giselly Gomes; Moreira, Melissa Heringer Chamon Barros Quintao

2013-01-01

355

Hepatorenal Syndrome  

Microsoft Academic Search

Hepatorenal syndrome (HRS) is a serious event during the course of decompensated cirrhosis. Although the most characteristic feature of the syndrome is a functional renal failure due to intense renal vasoconstriction, it is a more generalized process affecting the heart, brain and splanchnic organs. There are two types of HRS. Type 1 HRS is characterized by a rapidly progressive impairment

Mónica Guevara; Pere Ginès

2005-01-01

356

KBG syndrome  

Microsoft Academic Search

KBG syndrome is a rare condition characterised by a typical facial dysmorphism, macrodontia of the upper central incisors, skeletal (mainly costovertebral) anomalies and developmental delay. To date, KBG syndrome has been reported in 45 patients. Clinical features observed in more than half of patients that may support the diagnosis are short stature, electroencephalogram (EEG) anomalies (with or without seizures) and

Francesco Brancati; Anna Sarkozy; Bruno Dallapiccola

2006-01-01

357

Marfan Syndrome  

MedlinePLUS

... Connective tissue helps support all parts of your body. It also helps control how your body grows and develops. Marfan syndrome most often affects ... A mutation, or change, in the gene that controls how the body makes fibrillin causes Marfan syndrome. Fibrillin is a ...

358

Poland syndrome  

PubMed Central

Poland's syndrome is a rare congenital condition, characterized by the absence of the sternal or breastbone portion of the pectoralis major muscle, which may be associated with the absence of nearby musculoskeletal structures. We hereby report an 8-year-old boy with typical features of Poland syndrome, the first documented case from Uttar Pradesh, India. PMID:24959021

Sharma, Chandra Madhur; Kumar, Shrawan; Meghwani, Manoj K.; Agrawal, Ravi P.

2014-01-01

359

Anesthesia management in a patient of Apert syndrome  

PubMed Central

Apert syndrome is autosomal dominant disease associated with multiple craniofacial and limb deformities. These children usually face many orthopedic, orthodental operative procedures. As anesthetist, we face difficulties in airway management due to mid-facial hypoplasia, craniosynostosis. We report a case of Apert syndrome which was referred to us for syndactyly release, focusing on the difficulties and complications related to it.

Patel, Kiran; Chavan, Dilip; Sawant, Pradnya

2013-01-01

360

Innominate artery involvement in type iv Ehlers-Danlos syndrome  

Microsoft Academic Search

We report two cases of innominate artery involvement in patients with Ehlers-Danlos syndrome. In the first patient, spontaneous dissection of the innominate artery was treated successfully. In the other, the patient died of spontaneous rupture of the innominate artery in the early postoperative course after operation for aneurysm of the celiac artery. Arterial complications occurring in Ehlers-Danlos syndrome are rare

Alain Valverde; Jean-François Tricot; Bruno de Crepy; Hayssam Bakdach; Koskrow Djabbari

1991-01-01

361

Propofol infusion syndrome and Brugada syndrome electrocardiographic phenocopy.  

PubMed

This anesthetic drug may cause a rare condition named propofol infusion syndrome, characterized by unexplained lactic acidosis, lipemia, rhabdomyolysis, cardiovascular collapse and Brugada-like electrocardiographic pattern or Brugada electrocardiographic phenocopy changes following high-dose propofol infusion over prolonged periods of time. Several articles have contributed to our understanding of the cause of the syndrome, and the growing number of case reports has made it possible to identify several risk factors. Uncertainty remains as to whether a genetic susceptibility exists. The favorable recovery profile associated with propofol offers advantages over traditional anesthetics in clinical situations in which rapid recovery is important. Propofol is a safe anesthetic agent, but propofol infusion syndrome is a rare lethal complication. PMID:20544610

Riera, Andrés Ricardo Pérez; Uchida, Augusto Hiroshi; Schapachnik, Edgardo; Dubner, Sergio; Filho, Celso Ferreira; Ferreira, Celso

2010-01-01

362

Frey syndrome in neurofibromatosis 1  

PubMed Central

A healthy 18-month-old girl presented with a history of intermittent hemifacial flushing when eating. Her symptom seemed to be exclusively triggered by chewing. Examination revealed cutaneous features of neurofibromatosis type 1 (NF1) and mild facial asymmetry. Imaging confirmed cerebral vacuolisation changes seen in NF1 and a left facial plexiform neurofibroma involving the parotid gland. This is the first reported case of Frey syndrome complicating NF1. PMID:22698902

Ibrahim, Laila Farah; Brenner, Clare; McMenamin, Joe; Webb, David

2011-01-01

363

Major Depression and Complicated Grief  

MedlinePLUS

... and bereavement Next Topic Coping with loss Major depression and complicated grief Depression It’s common for people to have sadness, pain, ... might be getting worse—going into a major depression. About 1 in 5 bereaved people will develop ...

364

The complication of pneumatic retinopexy.  

PubMed Central

There have been 26 published series with a total of 1274 detachments operated with pneumatic retinopexy. Eighty percent were reattached with a single procedure and 98% with reoperations. New breaks occurred in 13% and PVR in 4%. The complications published in 101 papers on pneumatic retinopexy in the last 5 years are analyzed as to frequency, prevention, management, and results. PMID:2095021

Hilton, G F; Tornambe, P E; Brinton, D A; Flood, T P; Green, S; Grizzard, W S; Hammer, M E; Leff, S R; Mascuilli, L; Morgan, C M

1990-01-01

365

Constraints complicate centrifugal compressor depressurization  

SciTech Connect

Blowdown of a centrifugal compressor is complicated by process constraints that might require slowing the depressurization rate and by mechanical constraints for which a faster rate might be preferred. The paper describes design constraints such as gas leaks; thrust-bearing overload; system constraints; flare extinguishing; heat levels; and pressure drop.

Key, B. (Hoover and Keith Inc., Houston, TX (United States)); Colbert, F.L. (Paragon Engineering Services Inc., Houston, TX (United States))

1993-05-10

366

Complicating the Concept of Culture  

ERIC Educational Resources Information Center

This essay argues against a simple, reified view of culture as a set of ideas and norms belonging to a group or nation, and considers the implications of a more complicated concept for discussion of world culture and the global/local nexus. Most anthropologists define culture as the making of meaning, with an emphasis on the process itself as…

Anderson-Levitt, Kathryn M.

2012-01-01

367

Permanent makeup: indications and complications  

Microsoft Academic Search

Cosmetic tattoos, simulating makeup, have become very popular in the last decades; the technique of micropigmentation consists of implantation of pigment into the skin using a tattoo pen. The procedure can also be used to camouflage vitiligo, to mask scars, and as an adjunct to reconstructive surgery. Risks and complications include infections, allergic reactions, scarring, fanning, fading, and dissatisfaction about

Christa De Cuyper

2008-01-01

368

Behavioral Complications of Early Pallidotomy  

Microsoft Academic Search

A review of stereotactic medial pallidotomy of the 1950s in five neurosurgical centers is presented. The surgical technique varied from one center to the other. The results of surgery, however, seemed to be quite equal, being positive in 70–90% of the patients. The surgical mortality ranged from 0 to 13%. Behavioral complications were adequately analyzed and reported from one center

Lauri V. Laitinen

2000-01-01

369

Imaging the Complications of Osteochondromas  

Microsoft Academic Search

Most osteochondromas are asymptomatic and incidental with typical radiographic characteristics. When symptomatic, complications due to space-occupying effects, mechanical deformity, or malignant transformation are usually the underlying causes. This article addresses the imaging strategy for their identification and evaluation. Lee, J. M.et al. (2002). Clinical Radiology57, 18–28.

K. C. Y. Lee; A. M. Davies; V. N. Cassar-Pullicino

2002-01-01

370

Complications of mediastinal neural tumours  

Microsoft Academic Search

Thirty-two mediastinal neural tumours were seen in the East Anglian Regional Thoracic Surgical Unit at Cambridge between October 1952 and July 1970. The descending order of frequency was neurofibroma, ganglioneuroma, neurilemmoma, neurofibrosarcoma, and neuroblastoma. The literature relating to these tumours is reviewed and the pathological and clinical complications encountered in this series and in the literature are described.

Christopher Parish

1971-01-01

371

Aortic complications after bariatric surgery  

Microsoft Academic Search

Obesity and its correlated comorbities are nowadays considered a public health concern. In the last few years a myriad of strategies searched an effective procedure to reduce weight and decreases related comorbities. Among these strategies, the bariatric procedures have been achieved a significantly increase, being able to promote an expressive and lasting weight reduction. Diverse early and late complications are

Diego Felipe GAIA; José Honório PALMA; João Nelson; Rodrigues BRANCO; Enio BUFFOLO

372

Anaesthetic complications in plastic surgery.  

PubMed

Anaesthesia related complications in plastic surgeries are fortunately rare, but potentially catastrophic. Maintaining patient safety in the operating room is a major concern of anaesthesiologists, surgeons, hospitals and surgical facilities. Circumventing preventable complications is essential and pressure to avoid these complications in cosmetic surgery is increasing. Key aspects of patient safety in the operating room are outlined, including patient positioning, airway management and issues related to some specific conditions, essential for minimizing post-operative morbidity. Risks associated with extremes of age in the plastic surgery population, may be minimised by a better understanding of the physiologic changes as well as the pre-operative and post-operative considerations in caring for this special group of patients. An understanding of the anaesthesiologist's concerns during paediatric plastic surgical procedures can facilitate the coordination of efforts between the multiple services involved in the care of these children. Finally, the reader will have a better understanding of the perioperative care of unique populations including the morbidly obese and the elderly. Attention to detail in these aspects of patient safety can help avoid unnecessary complication and significantly improve the patients' experience and surgical outcome. PMID:24501480

Nath, Soumya Sankar; Roy, Debashis; Ansari, Farrukh; Pawar, Sundeep T

2013-05-01

373

Manufacturing Complicated Shells And Liners  

NASA Technical Reports Server (NTRS)

Explosive forming, wax filling, and any one of welding, diffusion bonding, or brazing used in method of manufacturing large, complicated shell-and-liner vessels or structures. Method conceived for manufacture of film-cooled rocket nozzles but applicable to joining large coaxial shells and liners in general.

Sobol, Paul J.; Faucher, Joseph E.

1993-01-01

374

Gastrointestinal complications in renal transplantation  

Microsoft Academic Search

One wonders whether the use of cyclosporin, histamine receptor antagonists, low doses of steroids, and early diagnosis and treatment actually modify the incidence, morbidity, and mortality of gastrointestinal (GI) and pancreatic complications in renal transplantation. To find out, we reviewed 614 kidney transplant recipients between January 1984 and December 1988. One hundred patients (16.2%) were found to have GI and\\/or

G. Benoit; M. Moukarzel; G. Verdelli; C. Hiesse; C. Buffet; H. Bensadoun; B. Charpentier; A. Jardin; D. Fries

1993-01-01

375

The use of botulinum toxin type A in the treatment of Frey and crocodile tears syndromes  

Microsoft Academic Search

PurposeWe sought to investigate the efficacy of botulinum toxin type A in the treatment of Frey and crocodile tears syndromes. Frey syndrome is a common complication after surgical intervention or injury in the region of the parotid gland. Crocodile tears syndrome is unusual and manifests after facial nerve paralysis and other causes such as head trauma.

Dionysios E Kyrmizakis; Aris Pangalos; Chariton E Papadakis; John Logothetis; Nicolas J Maroudias; Emmanuel S Helidonis

2004-01-01

376

Colonic diverticulitis in adolescents: an index case and associated syndromes  

Microsoft Academic Search

Diverticular disease of the colon, a common problem among adults, is diagnosed rarely in children. We report an adolescent\\u000a patient with sigmoid diverticulitis who required operative treatment. Pediatric patients with the complications of diverticula\\u000a typically have conditions that result in genetic alterations affecting the components of the colonic wall. Our patient had\\u000a Williams–Beuren syndrome, although Ehlers–Danlos syndrome, Marfan syndrome, and

Brian J. Santin; Vinay Prasad; Donna A. Caniano

2009-01-01

377

Prader-Willi syndrome in a child with XYY  

Microsoft Academic Search

We report a 26-month-old boy with XYY syndrome, with the complication of Prader-Willi syndrome (PWS) due to uniparental maternal\\u000a disomy of chromosome 15. To our knowledge, this is the first case of XYY syndrome and PWS. Clinical findings were fully compatible\\u000a with the diagnostic criteria for PWS. Molecular analysis revealed a maternal heterodisomy of chromosome 15, indicating that\\u000a non-disjunction of

Akira Honma; Reiko Ishii; Aiko Ito; Mitsuhiro Kato; Shinji Saitoh; Kiyoshi Hayasaka

1999-01-01

378

Nevoid basal cell carcinoma syndrome (Gorlin syndrome)  

PubMed Central

Nevoid basal cell carcinoma syndrome (NBCCS), also known as Gorlin syndrome, is a hereditary condition characterized by a wide range of developmental abnormalities and a predisposition to neoplasms. The estimated prevalence varies from 1/57,000 to 1/256,000, with a male-to-female ratio of 1:1. Main clinical manifestations include multiple basal cell carcinomas (BCCs), odontogenic keratocysts of the jaws, hyperkeratosis of palms and soles, skeletal abnormalities, intracranial ectopic calcifications, and facial dysmorphism (macrocephaly, cleft lip/palate and severe eye anomalies). Intellectual deficit is present in up to 5% of cases. BCCs (varying clinically from flesh-colored papules to ulcerating plaques and in diameter from 1 to 10 mm) are most commonly located on the face, back and chest. The number of BBCs varies from a few to several thousand. Recurrent jaw cysts occur in 90% of patients. Skeletal abnormalities (affecting the shape of the ribs, vertebral column bones, and the skull) are frequent. Ocular, genitourinary and cardiovascular disorders may occur. About 5–10% of NBCCS patients develop the brain malignancy medulloblastoma, which may be a potential cause of early death. NBCCS is caused by mutations in the PTCH1 gene and is transmitted as an autosomal dominant trait with complete penetrance and variable expressivity. Clinical diagnosis relies on specific criteria. Gene mutation analysis confirms the diagnosis. Genetic counseling is mandatory. Antenatal diagnosis is feasible by means of ultrasound scans and analysis of DNA extracted from fetal cells (obtained by amniocentesis or chorionic villus sampling). Main differential diagnoses include Bazex syndrome, trichoepithelioma papulosum multiplex and Torre's syndrome (Muir-Torre's syndrome). Management requires a multidisciplinary approach. Keratocysts are treated by surgical removal. Surgery for BBCs is indicated when the number of lesions is limited; other treatments include laser ablation, photodynamic therapy and topical chemotherapy. Radiotherapy should be avoided. Vitamin A analogs may play a preventive role against development of new BCCs. Life expectancy in NBCCS is not significantly altered but morbidity from complications can be substantial. Regular follow-up by a multi-specialist team (dermatologist, neurologist and odontologist) should be offered. Patients with NBCCS should strictly avoid an excessive sun exposure. PMID:19032739

Lo Muzio, Lorenzo

2008-01-01

379

Lymphatic complications after vascular interventions  

PubMed Central

Introduction Lymphorrhea due to classical and mini-invasive surgical interventions on femoral and popliteal arteries is a serious hindrance to patient treatment. Depending on the experience of a particular center, the incidence and frequency of this type of complication may constitute a serious clinical problem. While the level of lymphorrhea intensity and its duration result in certain foreseeable consequences, their treatment can be a time-consuming and multistep procedure. Aim To compare different types of vascular interventions with lymphorrhea occurrence. Material and methods The authors conducted a retrospective analysis of lymphatic complications based on the material collected between 2005 and 2012 at the Department of Vascular and Endovascular Surgery of the Military Institute of Medicine in Warsaw and in the Department of Interventional Cardiology and Angiology of the Institute of Cardiology in Anin, Warsaw, in 2009–2012. Results Maintaining due thoroughness when dissecting tissues and treating the cutting line in this area with ligatures and tissue puncture are the most reliable methods of minimizing the risk of lymphatic leakage after surgical procedures performed in a classical way. The lymphatic complication under analysis is far less likely to occur when procedures are performed as planned and an endovascular technique is used – statistical significance p < 0.05. Minimally invasive and fully percutaneous procedures performed via needle puncture, including the use of the fascial closure technique to close the femoral artery, eliminate the likelihood of the occurrence of this vascular complication – statistical significance was found with p value less than 0.05. Conclusions We concluded that in every case by minimizing the vascular approach we protected the patient against lymphatic complications. PMID:25337168

Obara, Andrzej; Maruszynski, Marek; Witkowski, Adam; Dabrowski, Maciej; Chmielak, Zbigniew

2014-01-01

380

Case series on tropical diabetic hand syndrome.  

PubMed

Tropical diabetic hand syndrome is a term used to describe diabetes complication of the hand affecting people in the tropics. It consists of localized cellulitis with variable swelling and ulceration of the hands, progressive, fulminant hand sepsis and gangrene in extreme cases. This syndrome is not well-recognized and is therefore less frequently reported. The authors describe three different female patients who were known diabetics of varying duration presenting with this syndrome at our tertiary health center and who were successfully managed by both the surgical and medical units. The need for early diagnosis and aggressive management is emphasized. PMID:24909485

Ezeani, I U; Edo, A E

2014-01-01

381

Papillon-Lefevre syndrome: A case report  

PubMed Central

Papillon–Lefevre syndrome (PLS) is a rare autosomal recessive disorder of keratinization, characterized by palmoplantar hyperkeratosis, periodontal involvement and precocious loss of dentition. The purpose of this report was to describe the case of an 18-year-old girl who presented to the out patient department of Navodaya Dental College and Hospital, Raichur, Karnataka, India, with the chief complaint of multiple loss of teeth. Her gingiva appeared erythematous, edematous and bled readily on probing, and the teeth were mobile. Hyperkeratosis of palms and soles were found. These findings are consistent with Papillon–Lefèvre syndrome. The clinical presentation, differential diagnosis, complications and management of this syndrome are discussed. PMID:24151407

Pavankumar, Kalwa

2010-01-01

382

Carpal tunnel syndrome — a comprehensive review  

Microsoft Academic Search

\\u000a Purpose. To provide a comprehensive review of the management of carpal tunnel syndrome.\\u000a \\u000a \\u000a Methods and results. A systematic literature review is provided of the history, anatomy, pathophysiology, epidemiology, diagnostic criteria,\\u000a investigative surgical techniques, results and complications for carpal tunnel syndrome.\\u000a \\u000a \\u000a \\u000a \\u000a Conclusion. Surgery for carpal tunnel syndrome requires meticulous attention to history-taking, investigation, counseling, training\\u000a and surgical technique if unsatisfactory results

J. Haase

383

[Leukostasis and tumor lysis: important complications of hyperleukocytosis].  

PubMed

The occurrence of hyperleukocytosis (leukocytes >?100.000/?l) is associated with complications such as leukostasis, tumor lysis and consumption coagulopathy in patients with acute leukemia much more often than in patients with chronic malignant hematological diseases. To manage these situations may be complex as organ failure is often imminent or manifest, infectious complications arise and indications for induction chemotherapy are usually urgent. Prophylaxis and therapy of the tumor lysis syndrome consists of hydration, lowering of uric acid and the management of electrolyte disturbances. Leukostasis requires immediate reduction of the leukocyte count by leukapheresis, administration of hydroxycarbamide and, ultimately, by causative and specific treatment of the underlying disease itself. In patients with curable diseases or favorable long-term prognosis, transfer to the intensive care unit must be evaluated early in the course of impending organ dysfunction, especially in cases of acute respiratory failure. PMID:23943008

Schellongowski, P; Staudinger, T

2013-09-01

384

[Neurologic complications of surgery of the spine in children].  

PubMed

Neurological deficiency can occur during or after spinal surgery. The most severe complications are seen after instrumental correction for scoliosis or kyphosis. Regression of paraplegia, paraparesia and Brown-Sequard syndrome is never a certainty and usually incomplete. Preoperative manoeuvres and evoked potentials do not provide absolute security and metal instrumentation should always be used prudently. The main risk factors are vertebral malformation, major kyphosis, preoperative signs of neurological deficit, excessive correction and double anterior and posterior access. Finally, the canal is poorly vascularized from T4 to T8 or T9 which can raise further problems. Cordal deficiency during or following almost always requires removal of the metal implant, and exploration of the canal possibly with MRI. Injury include direct contusion of the spinal cord, devascularization and compressive haematomas. The frequency of neurological complications is currently about 1% and only extreme prudence and knowledge of causes can reduce this rate. PMID:8665810

Carlioz, H; Ouaknine, M

385

Emerging risk factors and prevention of perioperative pulmonary complications.  

PubMed

Modern surgery is faced with the emergence of newer "risk factors" and the challenges associated with identifying and managing these risks in the perioperative period. Obstructive sleep apnea and obesity hypoventilation syndrome pose unique challenges in the perioperative setting. Recent studies have identified some of the specific risks arising from caring for such patients in the surgical setting. While all possible postoperative complications are not yet fully established or understood, the prevention and management of these complications pose even greater challenges. Pulmonary hypertension with its changing epidemiology and novel management strategies is another new disease for the surgeon and the anesthesiologist in the noncardiac surgical setting. Traditionally most such patients were not considered surgical candidates for any required elective surgery. Our review discusses these disease entities which are often undiagnosed before elective noncardiac surgery. PMID:24578647

Bhateja, Priyanka; Kaw, Roop

2014-01-01

386

Brown's syndrome: diagnosis and management.  

PubMed Central

PURPOSE: To better understand the various etiologies of Brown's syndrome, define specific clinical characteristics of Brown's syndrome, describe the natural history of Brown's syndrome, and evaluate the longterm outcome of a novel surgical procedure: the silicone tendon expander. Also, to utilize a computer model to simulate the pattern of strabismus seen clinically with Brown's syndrome and manipulate the model to show potential surgical outcomes of the silicone tendon expander. METHODS: Charts were reviewed on patients with the diagnosis of Brown's syndrome seen at a children's hospital ophthalmology clinic from 1982 to 1997, or seen in the author's private practice. Objective fundus torsion was assessed in up gaze, down gaze, and primary position in 7 Brown's syndrome patients and in 4 patients with primary superior oblique overaction. A fax survey was taken of members of the American Association of Ophthalmology and Strabismus (AAPOS) listed in the 1997-1998 directory regarding their results using the silicone tendon expander procedure for the treatment of Brown's syndrome. A computer model of Brown's syndrome was created using the Orbit 1.8 program by simulating a shortened superior oblique tendon or by changing stretch sensitivity to create an inelastic muscle. RESULTS: A total of 96 patients were studied: 85 with Brown's syndrome (38 with congenital and 47 with acquired disease), 6 with masquerade syndromes, 1 with Brown's syndrome operated on elsewhere, and 4 with primary superior oblique overaction in the torsion study. Three original clinical observations were made: 1. Significant limitation of elevation in abduction occurs in 70% of Brown's syndrome cases surgically verified as caused by a tight superior oblique tendon. Contralateral pseudo-inferior oblique overaction is associated with limited elevation in abduction. 2. Traumatic Brown's syndrome cases have larger hypotropias than nontraumatic cases (P < .001). There was no significant hypotropia in primary position in 56 (76%) of 74 congenital and nontraumatic acquired cases despite severe limitation of elevation. 3. Of 7 patients with Brown's syndrome, 6 had no significant fundus torsion in primary position, but had significant (+2 to +3) intorsion in up gaze. Spontaneous resolution occurred in approximately 16% of acquired nontraumatic Brown's syndrome patients. The silicone tendon expander was used on 15 patients, 13 (87%) were corrected with 1 surgery and 14 (93%) with 2 surgeries. The only failure was a Brown's syndrome not caused by superior oblique pathology. Five of the silicone tendon expander patients had at least 5 years follow-up (range, 5 to 11 years). Four (80%) of the 5 patients had an excellent outcome with 1 surgery, final results graded between 9 and 10 (on a scale of 1-10, 10 is best). The fifth patient had a consecutive superior oblique paresis and a good outcome after a recession of the ipsilateral inferior oblique muscle. The AAPOS survey had a mean outcome score of 7.3, with 65% between 8 and 10. There were 9 (6%) complications reported: 4 related to scarring and 5 extrusions of the implant. Three of the 5 extrusions were reported from the same surgeon. The computer model of an inelastic superior oblique muscle-tendon complex best simulated the motility pattern of Brown's syndrome with severe limitation of elevation in adduction, mild limitation of elevation in abduction, minimal hypotropia in primary position, no superior oblique overaction, and intorsion in up gaze. CONCLUSIONS: The presence of mild to moderate limitation of elevation in abduction is common, and its presence does not eliminate the diagnosis of Brown's syndrome. The majority of Brown's syndrome patients have a pattern of strabismus consistent with an inelastic superior oblique muscle-tendon complex that does not extend, but can contract normally; not the presence of a short tendon. The presence of inelastic or tethered superior oblique muscle-tendon can be diagnosed without forced duction testing by observing the pattern of strabismus including torsion. Because of

Wright, K W

1999-01-01

387

Hyperinsulinemic hypoglycemia of infancy in Sotos syndrome.  

PubMed

Sotos syndrome (OMIM #117550) is a congenital syndrome characterized by overgrowth with advanced bone age, macrocephaly, and learning difficulties. Endocrine complications of this syndrome have not yet been fully described in previous reports. We here investigated the clinical manifestations of Sotos syndrome in Japanese patients who presented with hyperinsulinemic hypoglycemia of infancy. We recruited patients diagnosed as having Sotos syndrome who presented with the complication of hyperinsulinemia during the neonatal period using a survey of the abstracts of Pediatric Meetings in domestic areas of Japan from 2007 to 2011. As a result, five patients (four females and one male) were recruited to evaluate the clinical presentation of Sotos syndrome by reference to the clinical record of each patient. A 5q35 deletion including the NSD1 gene was detected in all patients. Major anomalies in the central nervous, cardiovascular, and genito-urinary systems were frequently found. Hypoglycemia occurred between 0.5 and 3?hr after birth and high levels of insulin were initially found within 3 days of birth. The patients were treated with intravenous glucose infusion at a maximum rate of 4.6-11.0?mg/kg/min for 12-49 days. Three of the five patients required nasal tube feeding. One patient received medical treatment with diazoxide. This study shows that patients with Sotos syndrome may present with transient hyperinsulinemic hypoglycemia in the neonatal period. PMID:23239432

Matsuo, Terumichi; Ihara, Kenji; Ochiai, Masayuki; Kinjo, Tadamune; Yoshikawa, Yoko; Kojima-Ishii, Kanako; Noda, Marie; Mizumoto, Hiroshi; Misaki, Maiko; Minagawa, Kyoko; Tominaga, Koji; Hara, Toshiro

2013-01-01

388

Mortality in Prader-Willi Syndrome  

PubMed Central

Persons with Prader-Willi syndrome have been known to have a high mortality rate. However, intellectual disability, which usually accompanies Prader-Willi syndrome, is also associated with a higher mortality rate than in the general population. In this study, the death rates in a longitudinal cohort of people with Prader-Willi syndrome are compared with those for an epidemiologically derived control sample of people with intellectual disability from other causes. We found that those with Prader-Willi syndrome had a higher mortality rate than did controls. After the protective effect of mild intellectual disability or average intellectual function was accounted for, the hazard ratio for Prader-Willi syndrome versus controls was 6.07. Obesity and its complications were factors contributing to the mortality identified in this study. PMID:16597186

Einfeld, Stewart L.; Kavanagh, Sophie J.; Smith, Arabella; Evans, Elizabeth J.; Tonge, Bruce J.; Taffe, John

2008-01-01

389

Ehlers-Danlos syndrome type IV  

PubMed Central

Ehlers-Danlos syndrome type IV, the vascular type of Ehlers-Danlos syndromes (EDS), is an inherited connective tissue disorder defined by characteristic facial features (acrogeria) in most patients, translucent skin with highly visible subcutaneous vessels on the trunk and lower back, easy bruising, and severe arterial, digestive and uterine complications, which are rarely, if at all, observed in the other forms of EDS. The estimated prevalence for all EDS varies between 1/10,000 and 1/25,000, EDS type IV representing approximately 5 to 10% of cases. The vascular complications may affect all anatomical areas, with a tendency toward arteries of large and medium diameter. Dissections of the vertebral arteries and the carotids in their extra- and intra-cranial segments (carotid-cavernous fistulae) are typical. There is a high risk of recurrent colonic perforations. Pregnancy increases the likelihood of a uterine or vascular rupture. EDS type IV is inherited as an autosomal dominant trait that is caused by mutations in the COL3A1 gene coding for type III procollagen. Diagnosis is based on clinical signs, non-invasive imaging, and the identification of a mutation of the COL3A1 gene. In childhood, coagulation disorders and Silverman's syndrome are the main differential diagnoses; in adulthood, the differential diagnosis includes other Ehlers-Danlos syndromes, Marfan syndrome and Loeys-Dietz syndrome. Prenatal diagnosis can be considered in families where the mutation is known. Choriocentesis or amniocentesis, however, may entail risk for the pregnant woman. In the absence of specific treatment for EDS type IV, medical intervention should be focused on symptomatic treatment and prophylactic measures. Arterial, digestive or uterine complications require immediate hospitalisation, observation in an intensive care unit. Invasive imaging techniques are contraindicated. Conservative approach is usually recommended when caring for a vascular complication in a patient suffering from EDS type IV. Surgery may, however, be required urgently to treat potentially fatal complications. PMID:17640391

Germain, Dominique P

2007-01-01

390

Maternal serum soluble HLA-G in complicated pregnancies.  

PubMed

Preeclampsia, intrauterine growth retardation (IUGR), oligohydramnios, abortus, preterm birth and premature rupture of the membranes (PROM) are significant complications of pregnancy. Insufficient trophoblastic invasion plays an important role in the pathophysiology of pregnancy complications. Soluble human leukocyte antigen-gestation (HLA-G)1/G5 is a molecule associated with trophoblast invasion. When pregnancy complications are predicted early, strategies to prevent these complications can be implemented. The aim of this study was to investigate the relationship between first trimester maternal serum soluble HLA-G1/G5 levels and high-risk pregnancies. A total of 232 pregnant women were followed prospectively. Maternal blood samples were collected for determination of soluble HLA-G1/G5 levels at 11-14 weeks, during which routine serum free beta human chorionic gonadotropin (?hCG) and pregnancy associated plasma protein-A (PAPP-A) level determinations in addition to nuchal translucency (NT) measurements for Down's syndrome screening were done during 20-22 weeks gestation. The subjects were classified into normal pregnancy, preeclampsia, oligohydramnios, IUGR, preterm birth and PROM groups. First trimester maternal serum soluble HLA-G1/G5 levels were not significantly different between the groups. First trimester soluble HLA-G1/G5 did not predict high-risk pregnancies. Studies with larger number of cases are need to confirm our findings. PMID:23795671

B?y?k, Ismail

2014-03-01

391

Hemihyperplasia syndromes  

Microsoft Academic Search

Obective  Hemihyperplasia is a heterogenous group of disorders characterized by asymmetric limb growth. There is considerable confusion\\u000a regarding their classification and ascertainment into various syndromes. We tried to look into the various aspects of hemihyperplasia\\u000a syndromes.\\u000a \\u000a \\u000a \\u000a Methods  Records of 17 consecutive cases of hemihyperplasia were reviewed and were ascertained into various syndromes based on available\\u000a literature and diagnostic criteria.\\u000a \\u000a \\u000a \\u000a Results  Of the 17

Ashwin B. Dalal; Shubha R. Phadke; Mandakini Pradhan; Sheetal Sharda

2006-01-01

392

CLOVES syndrome.  

PubMed

A cohort of patients with overgrowth syndromes has been identified with congenital lipomatous overgrowth, dysregulated fat deposits, and mixed vascular malformations. The acronym CLOVES was given on a heuristic basis to stand for congenital lipomatous overgrowth (CLO), vascular malformation (V), epidermal nevi (E), and scoliosis and spinal deformities (S). These patients have upper limb anomalies with variable phenotypes. Although hand anomalies alone cannot make the diagnosis, the foot, truncal, cutaneous and spinal anomalies are particularly diagnostic. CLOVES syndrome has emerged as a distinct clinical entity diagnosed by clinical and radiographic examinations. The overgrowth pattern is now easily distinguished from other overgrowth syndromes. PMID:24161472

Bloom, Jacob; Upton, Joseph

2013-12-01

393

Cushing Syndrome  

PubMed Central

A variety of diagnostic advances including radioimmunoassay of adrenocorticotropic hormone (ACTH) have increased the number of methods for laboratory investigation of Cushing syndrome.* However, experience with these procedures has led to a recognition of their limitations. We have developed an algorithm which incorporates these newer techniques and minimizes the number of procedures required to diagnose the various causes of Cushing syndrome. At present, we recommend pituitary surgical operations for pituitary-dependent Cushing syndrome because we believe this disease is caused by the development of a pituitary ACTH-secreting tumor. PMID:6992458

Cook, David M.; Kendall, John W.; Jordan, Richard

1980-01-01

394

Oral Complications in Hematopoietic Stem Cell Recipients: The Role of Inflammation  

PubMed Central

Hematopoietic stem cell transplantation (HSCT) is widely used as a potentially curative treatment for patients with various hematological malignancies, bone marrow failure syndromes, and congenital immune deficiencies. The prevalence of oral complications in both autologous and allogeneic HSCT recipients remains high, despite advances in transplant medicine and in supportive care. Frequently encountered oral complications include mucositis, infections, oral dryness, taste changes, and graft versus host disease in allogeneic HSCT. Oral complications are associated with substantial morbidity and in some cases with increased mortality and may significantly affect quality of life, even many years after HSCT. Inflammatory processes are key in the pathobiology of most oral complications in HSCT recipients. This review article will discuss frequently encountered oral complications associated with HSCT focusing on the inflammatory pathways and inflammatory mediators involved in their pathogenesis. PMID:24817792

Haverman, T. M.; Raber-Durlacher, J. E.; Rademacher, W. M. H.; Vokurka, S.; Epstein, J. B.; Huisman, C.; Hazenberg, M. D.; de Soet, J. J.; de Lange, J.; Rozema, F. R.

2014-01-01

395

Retinal complications after bungee jumping.  

PubMed

Bungee jumping is becoming a popular sport in the Western world with some cases of ophthalmic complications being reported in recent literature. The authors reported a case of a 23-year-old healthy female who presented retinal complications following a bungee jumping. Her fundi showed superficial retinal hemorrhages in the right eye and a sub-internal limiting membrane hemorrhage affecting the left eye. A general examination, including a full neurological examination, was normal and laboratorial investigations were all within normal values. More studies are necessary to identify risk factors and the true incidence of related ocular lesions, but until then, we think this sport activity should be desencouraged, especially to those that are not psychological and physically fit. PMID:7642337

Filipe, J A; Pinto, A M; Rosas, V; Castro-Correia, J

396

Suicide bereavement and complicated grief  

PubMed Central

Losing a loved to suicide is one is one of life's most painful experiences. The feelings of loss, sadness, and loneliness experienced after any death of a loved one are often magnified in suicide survivors by feelings of quilt, confusion, rejection, shame, anger, and the effects of stigma and trauma. Furthermore, survivors of suicide loss are at higher risk of developing major depression, post-traumatic stress disorder, and suicidal behaviors, as well as a prolonged form of grief called complicated grief. Added to the burden is the substantial stigma, which can keep survivors away from much needed support and healing resources. Thus, survivors may require unique supportive measures and targeted treatment to cope with their loss. After a brief description of the epidemiology and circumstances of suicide, we review the current state of research on suicide bereavement, complicated grief in suicide survivors, and grief treatment for survivors of suicide. PMID:22754290

Tal Young, Ilanit; Iglewicz, Alana; Glorioso, Danielle; Lanouette, Nicole; Seay, Kathryn; Ilapakurti, Manjusha; Zisook, Sidney

2012-01-01

397

Surgical management of complicated diverticulitis  

Microsoft Academic Search

One hundred forty patients who had complicated diverticular disease were identified in a retrospective review at the Lahey\\u000a Clinic between 1967 and 1982. Of these patients, 86 underwent resection with primary anastomosis with a 1 percent mortality\\u000a rate and an 18 percent morbidity rate; 13 had resection with anastomosis and creation of a proximal colostomy with no death\\u000a and a

Alan W. Hackford; David J. Schoetz; John A. Coller; Malcolm C. Veidenheimer

1985-01-01

398

Neurologic Complications of Lung Cancer  

Microsoft Academic Search

Lung cancer frequently causes neurological complications from direct and indirect effects. Brain metastases occur in 41% of\\u000a patients with non-small cell lung cancer and 35% with small cell lung cancer at autopsy. Presenting symptoms can be quite\\u000a protean. MRI continues to be the gold standard of detecting metastases. Biopsy or resection should be considered for patients\\u000a with a single lesion,

Suriya A. Jeyapalan; Anand Mahadevan

399

Complications following Acute Ischemic Stroke  

Microsoft Academic Search

The objective of this study was to assess typical early-onset complications following ischemic stroke in a large, hospital-based cohort to provide clinical data for future randomized trials and quality standards in clinical routine. 3,866 patients with acute ischemic stroke were prospectively documented in 14 Neurology Departments with an acute stroke unit. Within the first week after admission, increased intracranial pressure

Christian Weimar; Michael P. Roth; Gesine Zillessen; Jörg Glahn; Martin L. J. Wimmer; Otto Busse; Roman L. Haberl; Hans-Christoph Diener

2002-01-01

400

[Respiratory complications in compressive goiters].  

PubMed

Authors' experience in respiratory complications of compressive goiter is reviewed. Three cases with typical symptoms, relative therapy and outcome are reported. Appearance of respiratory distress should urge to avoid delay in elective surgery performing. In case of acute respiratory failure, immediate emergency tracheostomy is mandatory, subsequently followed by elective intervention. In case of malignancy, if radical surgery is not possible, tracheostomy is the short and medium-term palliative surgical treatment of choice. PMID:1751337

De Toma, G; Sgarzini, G; Gabriele, R; Campli, M; Adami, E A; De Cesare, E

1991-01-01

401

Genetic hypofibrinolysis in complicated pregnancies  

Microsoft Academic Search

Objective: To assess the hypofibrinolytic 4G\\/4G mutation of the plasminogen activator inhibitor (PAI-1) gene as a possible factor contributing to severe preeclampsia, abruptio placentae, fetal growth restriction, and stillbirth.Methods: We compared 94 women from a previous report who had obstetric complications to 95 controls with normal pregnancies matched for ethnic background and age. We collected blood and extracted DNA after

Charles J Glueck; Michael J Kupferminc; Robert N Fontaine; Ping Wang; Babette B Weksler; Amiram Eldor

2001-01-01

402

Management of complicated duodenal diverticula.  

PubMed

The duodenum is the second most common location of intestinal diverticula after the colon. Duodenal diverticulum (DD) is usually located in the second portion of the duodenum (D2), close to the papilla. Most duodenal diverticula are extraluminal and acquired rather than congenital; more rare is the congenital, intraluminal diverticulum. DD is usually asymptomatic and discovered incidentally, but can become symptomatic in 1% to 5% of cases when complicated by gastroduodenal, biliary and/or pancreatic obstruction, by perforation or by hemorrhage. Endoscopic treatment is the most common first-line treatment for biliopancreatic complications caused by juxtapapillary diverticula and also for bleeding. Conservative treatment of perforated DD based on fasting and broad-spectrum antibiotics may be offered in some selected cases when diagnosis is made early in stable patients, or in elderly patients with comorbidities who are poor operative candidates. Surgical treatment is currently reserved for failure of endoscopic or conservative treatment. The main postoperative complication of diverticulectomy is duodenal leak or fistula, which carries up to a 30% mortality rate. PMID:23810155

Oukachbi, N; Brouzes, S

2013-06-01

403

Complications of acute otitis media in children  

Microsoft Academic Search

The clinical picture and the treatment of acute otitis media (AOM) and its complications have changed during the past decades.\\u000a The availability of antibiotics has decreased the incidence of complications of AOM significantly. The treatment of complications\\u000a of AOM is conservative in most cases. Mastoidectomy is needed when abscess-forming mastoiditis or intracranial complications\\u000a develop. Although intratemporal and intracranial complications of

Kimmo Leskinen

2005-01-01

404

[Autoinflammatory syndromes/fever syndromes].  

PubMed

Hereditary periodic (fever) syndromes, also called autoinflammatory syndromes, are characterized by relapsing fever and additional manifestations such as skin rashes, mucosal manifestations, or joint symptoms. Some of these disorders present with organ involvement and serological signs of inflammation without fever. There is a strong serological inflammatory response with an elevation of serum amyloid A (SAA), resulting in an increased risk of secondary amyloidosis. There are monogenic disorders (familial mediterranean fever (FMF), hyper-IgD-syndrome (HIDS), cryopyrin-associated periodic syndromes (CAPS), "pyogenic arthritis, acne, pyoderma gangrenosum" (PAPA), and "pediatric granulomatous arthritis (PGA) where mutations in genes have been described, which in part by influencing the function of the inflammasome, in part by other means, lead to the induction of the production of IL-1?. In "early-onset of enterocolitis (IBD)", a functional IL-10 receptor is lacking. Therapeutically, above all, the IL-1 receptor antagonist anakinra is used. In case of TRAPS and PGA, TNF-antagonists (etanercept) may also be used; in FMF colchicine is first choice. As additional possible autoinflammatory syndromes, PFAPA syndrome (periodic fever with aphthous stomatitis, pharyngitis and adenitis), Schnitzler syndrome, Still's disease of adult and pediatric onset, Behçet disease, gout, chronic recurrent multifocal osteomyelitis (CRMO) and Crohn's disease also are mentioned. PMID:21541834

Schedel, J; Bach, B; Kümmerle-Deschner, J B; Kötter, I

2011-05-01

405

Serotonin syndrome  

MedlinePLUS

... medicine). Drugs of abuse, such as ecstasy and LSD have also been associated with serotonin syndrome. ... Always tell all of your healthcare providers what medicines you take. ... especially right after starting a medicine or increasing ...

406

Caplan syndrome  

MedlinePLUS

... who have been exposed to mining dust containing coal. The lung disease is called coal worker's pneumoconiosis . ... Caplan syndrome is caused by breathing in coal mining dust. This ... small lung lumps (nodules) and mild asthma-like airway disease. ...

407

Bloom's Syndrome  

MedlinePLUS

... a high incidence of breaks in an individual’s chromosomes. The most serious manifestations of this condition are ... the symptoms of this disorder may recommend specialized chromosome testing or testing of the Bloom’s syndrome gene. ...

408

Down Syndrome  

MedlinePLUS

... the key to healthier, happier, more independent lives. Chromosomes Are the Cause To understand why Down syndrome happens, you need to understand a little about chromosomes. What are chromosomes? They're thread-like structures ...

409

Hunter syndrome  

MedlinePLUS

Genetic counseling is recommended for couples who want to have children and who have a family history of Hunter syndrome. Prenatal testing is available. Carrier testing for female relatives of affected males is available at a few centers.

410

Aicardi Syndrome  

MedlinePLUS

... encephalopathy that affects newborn infants. ) Is there any treatment? There is no cure for Aicardi syndrome nor is there a standard course of treatment. Treatment generally involves medical management of seizures and ...

411

Apert syndrome  

MedlinePLUS

... by ridging along sutures (craniosynostosis) Frequent ear infections Fusion or severe webbing of the 2nd, 3rd, and ... face Skeletal (limb) abnormalities Short height Webbing or fusion of the toes Several other syndromes can lead ...

412

Levator Syndrome  

MedlinePLUS

... Abscess Anorectal Fistula Foreign Objects in the Rectum Hemorrhoids Levator Syndrome Pilonidal Disease Proctitis Rectal Prolapse Merck ... exclude other painful rectal conditions (such as thrombosed hemorrhoids, fissures, or abscesses). The physical examination is often ...

413

Ohtahara Syndrome  

MedlinePLUS

... Ohtahara syndrome is a neurological disorder characterized by seizures. The disorder affects newborns, usually within the first ... first 10 days) in the form of epileptic seizures. Infants have primarily tonic seizures, but may also ...

414

HELLP syndrome  

MedlinePLUS

... in 10-20% of pregnant women with severe preeclampsia or eclampsia . Most often HELLP develops before the ... have high blood pressure and are diagnosed with preeclampsia before they develop HELLP syndrome. In some cases, ...

415

Williams Syndrome  

MedlinePLUS

... cognitive, personality, and neurological characteristics of WS. NIH Patient Recruitment for Williams Syndrome Clinical Trials At NIH ... on the treatment or care of an individual patient should be obtained through consultation with a physician ...

416

Marfan syndrome.  

PubMed

Marfan syndrome is a rare autosomal dominant disorder of the connective tissue, with skeletal, ligamentous, orooculofacial, pulmonary, abdominal, neurological and the most fatal, cardiovascular manifestations. It has no cure but early diagnosis, regular monitoring and preventive lifestyle regimen ensure a good prognosis. However, the diagnosis can be difficult as it is essentially a clinical one, relying on family history, meticulous physical examination and investigation of involved organ systems. Patients of Marfan syndrome portray very typical physical and orofacial characteristics, suggesting obvious recognition, but due to variable phenotypic expression, cases often go unnoticed unless a full range of attributing features is apparent. Dental practitioners are very likely to encounter patients of Marfan syndrome at an early age as they frequently present for dental treatment. The present case report illustrates the preliminary screening of Marfan syndrome in a dental office followed by timely diagnosis and appropriate referrals. PMID:24336584

Jain, Eesha; Pandey, Ramesh Kumar

2013-01-01

417

Isaac's Syndrome  

MedlinePLUS

... typically caused by antibodies that bind to potassium channels on the motor nerve. Issacs' syndrome is only ... neurological conditions that can be caused by potassium channel antibodies. Is there any treatment? Anticonvulsants, including phenytoin ...

418

Paraneoplastic Syndromes  

PubMed Central

Neoplasms can produce a variety of remote effects on the host; these are referred to as paraneoplastic syndromes. The syndromes may affect any of the systems of the body, may precede or follow the diagnosis of the underlying neoplasm, and may or may not parallel the course of the neoplasm in severity. The diagnosis of and therapy for these syndromes can be challenging to a physician, but successful therapy may bring about worthwhile relief for the patient. In addition, the syndromes and the substances that cause them are sometimes useful in diagnosing and in following the course of certain neoplasms. Perhaps of greater importance, study of these remote effects of neoplasia may shed light on the nature of the neoplastic process itself. PMID:6990627

Stolinsky, David C.

1980-01-01

419

Asperger Syndrome  

MedlinePLUS

... AS) is a developmental disorder. It is an autism spectrum disorder (ASD), one of a distinct group ... of thought and behavior. Other ASDs include: classic autism, Rett syndrome, childhood disintegrative disorder, and pervasive developmental ...

420

Sheehan syndrome  

MedlinePLUS

... occur in a woman who bleeds severely during childbirth. Sheehan syndrome is a type of hypopituitarism . ... Severe bleeding during childbirth can result in tissue death in the pituitary gland. This causes the gland to lose its ability to work ...

421

Down Syndrome  

MedlinePLUS

... help improve skills. They may include speech, physical, occupational, and/or educational therapy. With support and treatment, many people with Down syndrome live happy, productive lives. NIH: National Institute of Child Health and Human Development

422

Syndromic diarrhea/Tricho-hepato-enteric syndrome  

PubMed Central

Abstract Syndromic diarrhea/Tricho-hepato-enteric syndrome (SD/THE) is a rare and severe bowel disorder caused by mutation in SKIV2L or in TTC37, 2 genes encoding subunits of the putative human SKI complex. The estimated prevalence is 1/1,000,000 births and the transmission is autosomal recessive. The classical form is characterized by 5 clinical signs: intractable diarrhea of infancy beginning in the first month of life, usually leading to failure to thrive and requiring parenteral nutrition; facial dysmorphism characterised by prominent forehead and cheeks, broad nasal root and hypertelorism; hair abnormalities described as woolly and easily removable; immune disorders resulting from defective antibody production; intrauterine growth restriction. The aetiology is a defect in TTC37, a TPR containing protein, or in the RNA helicase SKIV2L, both constituting the putative human ski complex. The ski complex is a heterotetrameric cofactor of the cytoplasmic RNA exosome which ensures aberrants mRNAs decay. The diagnosis SD/THE is initially based on clinical findings and confirmed by direct sequencing of TTC37 and SKIV2L. Differential diagnosis with the other causes of intractable diarrhea is easily performed by pathologic investigations. During their clinical course, most of the children require parenteral nutrition and often immunoglobulin supplementation. With time, some of them can be weaned off parenteral nutrition and immunoglobulin supplementation. The prognosis depends on the management and is largely related to the occurrence of parenteral nutrition complications or infections. Even with optimal management, most of the children seem to experience failure to thrive and final short stature. Mild mental retardation is observed in half of the cases. Abstract in French Les diarrhées syndromiques ou syndrome tricho-hepato-enterique (SD/THE) sont un syndrome rare et sévère dont l’incidence est estimée à 1 cas pour 1 million de naissances et la transmission autosomique récessive. La forme typique associe 5 signes cliniques: une diarrhée grave rebelle nécessitant dans la majorité des cas une nutrition parentérale du fait de la malnutrition, une dysmorphie avec un front large et bombé, une racine du nez large et un hypertélorisme, des anomalies des cheveux qui sont fragiles, cassants, incoiffables et qualifiés de « laineux », un retard de croissance intra utérine et des anomalies de l’immunité à type de déficit en immunoglobuline ou d’absence de réponse aux antigènes vaccinaux. Des anomalies de deux protéines peuvent être à l’origine du syndrome SD/THE: TTC37, une protéine à motif TPR et SKIV2L, une hélicase à ARN, toutes 2 étant des constituants du complexe SKI humain. Le complexe SKI est un co-facteur de l’exosome cytoplasmique qui assure la dégradation des ARN aberrants ou exogènes. Le diagnostic est d’abord clinique puis confirmé par le séquençage des gènes TTC37 et SKIV2L. Le diagnostic différentiel avec les autres formes de diarrhées intraitables est fait grâce aux analyses anatomopathologiques qui montrent dans les autres formes, des lésions spécifiques. La prise en charge clinique repose sur la nutrition parentérale et la supplémentation en immunoglobuline si nécessaire. Un certain nombre d’enfants peuvent être sevrés de la nutrition parentérale et des supplémentations en immunoglobulines. En cas d’atteinte hépatique, celle-ci peut être sévère et conduire au décès. Même avec une prise en charge optimale, les enfants présentent une petite taille et, dans la moitié des cas, un retard mental modéré. Disease name/synonyms – Syndromic diarrhea – Phenotypic diarrhea – Tricho-hepato-enteric syndrome – Intractable diarrhea of infancy with facial dysmorphism &#x

2013-01-01

423

CHARGE syndrome  

Microsoft Academic Search

CHARGE syndrome was initially defined as a non-random association of anomalies (Coloboma, Heart defect, Atresia choanae, Retarded growth and development, Genital hypoplasia, Ear anomalies\\/deafness). In 1998, an expert group defined the major (the classical 4C's: Choanal atresia, Coloboma, Characteristic ears and Cranial nerve anomalies) and minor criteria of CHARGE syndrome. Individuals with all four major characteristics or three major and

Kim D Blake; Chitra Prasad

2006-01-01

424

[Kabuki syndrome].  

PubMed

Kabuki syndrome is characterised by a peculiar face resembling the make-up of actors in Kabuki, the traditional Japanese theatre, postnatal growth deficiency, mild to moderate mental retardation, unusual dermatoglyphic patterns, and various skeletal and visceral anomalies. The author would like to draw attention to this less known condition in Hungary by a case-report of a 23 months old female patient with Kabuki syndrome. PMID:7651720

Stankovics, J

1995-08-20

425

Metabolic Syndrome  

Microsoft Academic Search

“Metabolic syndrome” refers to the phenomenon of risk factor clustering and is presumed to reflect a unifying underlying pathophysiology.\\u000a Clustering commonly occurs in the setting of obesity, insulin resistance and a sedentary lifestyle. Currently there are five\\u000a different criteria for metabolic syndrome, all of which are associated with increased risk of diabetes or cardiovascular disease.\\u000a Therapeutic lifestyle change that focuses

James B. Meigs

426

Jacobsen syndrome  

Microsoft Academic Search

Jacobsen syndrome is a MCA\\/MR contiguous gene syndrome caused by partial deletion of the long arm of chromosome 11. To date, over 200 cases have been reported. The prevalence has been estimated at 1\\/100,000 births, with a female\\/male ratio 2:1. The most common clinical features include pre- and postnatal physical growth retardation, psychomotor retardation, and characteristic facial dysmorphism (skull deformities,

Teresa Mattina; Concetta Simona Perrotta; Paul Grossfeld

2009-01-01

427

Ischaemic Stroke Following Percutaneous Transluminal Coronary Angioplasty (PTCA): A Rare Complication  

PubMed Central

Stroke following coronary interventions is a devastating and most dreaded complication with significant morbidity and mortality. Various factors have been ascribed for this complication including the technical errors. We hereby describe such a patient who presented to us with the diagnosis of acute coronary syndrome and underwent percutaneous coronary intervention (PCI) but unfortunately developed left sided hemiparesis due to ischaemic stroke (right middle cerebral artery). She was managed as per the standard treatment protocols for acute coronary syndrome and later on for ischaemic stroke which she nicely responded to and was discharged in a haemodynamically stable condition. On follow-up after 15 days, she was totally symptom-free. We will discuss all the possible preventive and treatment measures for this rare complication of (PCI). PMID:25121015

Sanghi, Anand B

2014-01-01

428

Popliteal entrapment syndrome caused by fibular osteochondroma.  

PubMed

Osteochondromas are the most common tumors of bone. In adolescence, they usually present as a painless growing mass. Femoropopliteal vascular complications associated with an osteochondroma are rare, with most cases involving pseudoaneurysms. This report describes an exceptional case of a popliteal entrapment syndrome caused by a proximal fibular osteochondroma. PMID:21665427

Holzapfel, Boris M; Seppel, Gernot; Wagner, Richard; Kenn, Werner; Meffert, Rainer

2011-10-01

429

Cardiovascular Magnetic Resonance in Marfan syndrome  

PubMed Central

This review provides an overview of Marfan syndrome with an emphasis on cardiovascular complications and cardiovascular imaging. Both pre- and post-operative imaging is addressed with an explanation of surgical management. All relevant imaging modalities are discussed with a particular focus on cardiovascular MR. PMID:23587220

2013-01-01

430

Neuroepidemiology of Acquired Immune Deficiency Syndrome  

Microsoft Academic Search

Neuroepidemiology is that branch of chronic disease epidemiology dealing with disorders that affect the nervous system. The potential for neurological involvement in the diagnosis and pathogenesis of Acquired Immune Deficiency Syndrome (AIDS) has recently been noted. The following review outlines the spectrum of neurological conditions seen in AIDS patients to date. These conditions include: (1) infectious central nervous system complications

Chester Nakamura; Craig A. Molgaard

1986-01-01

431

Central Pain Syndrome  

MedlinePLUS

NINDS Central Pain Syndrome Information Page Table of Contents (click to jump to sections) What is Central Pain Syndrome? Is there ... being done? Clinical Trials Organizations What is Central Pain Syndrome? Central pain syndrome is a neurological condition ...

432

Down Syndrome (For Parents)  

MedlinePLUS

About Down Syndrome Down syndrome (DS), also called Trisomy 21, is a condition in which extra genetic material causes delays ... rises to about 1 in 100. Continue How Down Syndrome Affects Kids Kids with Down syndrome tend to ...

433

Angelman Syndrome Foundation  

MedlinePLUS

... Syndrome Clinics Newsletter Privacy Policy Transparency Honoring Your Investment 50% of individuals with Angelman syndrome are originally ... Syndrome Clinics Newsletter Privacy Policy Transparency Honoring Your Investment Angelman Syndrome Foundation, Inc. (800-432-6435) 75 ...

434

Ocular Complications of Chloroquine Therapy  

PubMed Central

Ocular complications of long-term chloroquine therapy were observed in 18 of 45 patients so treated. This therapy was used in patients with rheumatoid arthritis, lupus erythematosus, sarcoidosis, discoid lupus and other chronic “collagen disease”. Thirteen patients had reversible corneal opacifications, and seven had irreversible retinal changes, with visual loss and visual field defects. Pathological evidence of chloroquine retinopathy was obtained in one patient. Physicians are therefore warned to use this drug only after careful consideration. If it is used, repeated ocular examinations should include assessment of visual acuity, visual fields on a tangent screen and fundus examination through a dilated pupil. ImagesFig. 4Fig. 7Fig. 8 PMID:14275038

Lloyd, Lois A.; Hiltz, John W.

1965-01-01

435

Postoperative Complications After Gynecologic Surgery  

PubMed Central

Objective To estimate the association of age, medical comorbidities, functional status, and unintentional weight loss (as a marker of frailty) with postoperative complications in women undergoing major gynecologic surgery. Methods We conducted a cross-sectional analysis of the American College of Surgeons National Surgical Quality Improvement Program 2005-2009 participant use data files were used to analyze gynecologic procedures. The primary outcome was composite 30-day major postoperative complications. Results A total of 22,214 women were included in our final analysis. The overall prevalence of composite 30 day major postoperative complications was 3.7% (n=817). Age 80 years or older (adjusted odds ratio (OR)=1.8(95% Confidence Interval (CI) 1.25,2.58)), dependent functional status (adjusted OR= 2.37(95% CI 1.53,3.68)), and unintentional weight loss (adjusted OR = 2.49(95% CI 1.48,4.17)) were significantly associated with postoperative morbidity after adjusting for diabetes mellitus (adjusted OR= 1.44(95% CI 1.15,1.79)), known bleeding disorder (adjusted OR= 2.29 (95% 1.49,3.52)), morbid obesity (adjusted OR= 1.77(95% CI 1.45,2.17)), ascites (adjusted OR= 3.27(95% CI 2.18,4.90)), preoperative systemic infection (adjusted OR= 3.02(95% CI 2.03,4.48)), procedures for gynecologic cancer (adjusted OR= 1.60(95% CI 1.27,2.0)), disseminated cancer (adjusted OR= 2.57(95% CI 1.64,4.03)), emergency procedures (adjusted OR = 1.82(95% CI 1.18,2.79)), operative time greater than 4 hours vs. less than 1 hour (adjusted OR = 2.91(95%CI 2.18,3.89)) and wound class 4 vs. 1(adjusted OR= 4.28(95%CI 1.82,10.1). Conclusion Age 80 years or older, medical comorbidities, dependent functional status, and unintentional weight loss are associated with increased major postoperative complications after gynecologic procedures. PMID:21934441

Erekson, Elisabeth A.; Yip, Sallis O.; Ciarleglio, Maria M.; Fried, Terri R.

2011-01-01

436

Neurological complications of coeliac disease  

PubMed Central

A variety of neurological disorders have been reported in association with coeliac disease including epilepsy, ataxia, neuropathy, and myelopathy. The nature of this association is unclear and whether a specific neurological complication occurs in coeliac disease remains unproved. Malabsorption may lead to vitamin and trace element deficiencies. Therefore, patients who develop neurological dysfunction should be carefully screened for these. However, malabsorption does not satisfactorily explain the pathophysiology and clinical course of many of the associated neurological disorders. Other mechanisms proposed include altered autoimmunity, heredity, and gluten toxicity. This review attempts to summarise the literature and suggests directions for future research. PMID:12151653

Pengiran, T; Wills, A; Holmes, G

2002-01-01

437

Ocular complications of bungee jumping  

PubMed Central

Aim In this paper, we will try to highlight the importance of various investigations and their crucial role in identifying whether the defect is structural or functional. Case history A 24-year-old woman presented with ocular complications after bungee jumping. Subsequently, although all ophthalmic signs resolved, she complained of decreased vision in her left eye. Conclusion Initial ophthalmic injury was detected by optical coherence tomography scan showing a neurosensory detachment of the fovea. This was not initially detected on slit-lamp examination or fluorescein angiography. On later examination, although the optical coherence tomography scan showed no structural damage, electrodiagnostic tests showed a functional defect at the fovea. PMID:23055687

Hassan, H Mohammed J; Mariatos, Georgios; Papanikolaou, Theocharis; Ranganath, Akshatha; Hassan, Hala

2012-01-01

438

[Neurological complications in cancer patients].  

PubMed

Neurological symptoms in cancer patients have a great impact on quality of life and need an interdisciplinary approach. They lead to significant impairment in activities of daily living (gait disorders, dizziness), a loss of patients independency (vegetative disturbances, wheel-chair dependency) and interfere with social activities (ban of driving in case of epilepsy). In this article we describe three main and serious neurological problems in the context of oncological patients. These are chemotherapy-induced polyneuropathy, malignant spinal cord compression and epileptic seizures. Our aim is to increase the awareness of neurological complications in cancer patients to improve patients care. PMID:25146945

Hundsberger, Thomas; Roth, Patrick; Roelcke, Ulrich

2014-08-20

439

Automatic Monitoring Of Complicated Systems  

NASA Technical Reports Server (NTRS)

Collection of computer programs developed for expert computer system performing complicated, tedious, and repetitive portions of analysis of telemetry data from spacecraft. Provides nonstop, accurate surveillance of incoming data, also frees operators to concentrate their expertise on unexpected abnormal operating conditions. When unable to explain discrepancies with certainty resulting from data out of synchronization or other falso-alarm conditions, triggers alarm devices to request assistance from designated individuals. Concept useful in such terrestrial systems as production lines, power-distribution networks, chemical processes, large airplanes, and other assemblies of interdependent equipment.

Schwuttke, Ursula M.

1990-01-01

440

Neonatal meningitis complicating with pneumocephalus  

PubMed Central

Pneumocephalus is a rare condition characterized by the presence of gas within the cranial cavity. This gas may arise either from a trauma, a tumor, a surgical, or a diagnostic procedure or occasionally from an infection. Pneumocephalus as a complication of bacterial meningitis, in absence of trauma or a procedure, is extremely rare, particularly in a newborn. A case of pneumocephalus occurring in a baby, suffering from neonatal meningitis, acquired probably through unsafe cutting and tying of the cord, is reported here. Cutting, tying, and care of the umbilical cord is of utmost importance to prevent neonatal infection as the same is a potential cause of serious anaerobic infections, besides tetanus. PMID:24741257

Kumari, Anita; Agrawal, Satish C.

2014-01-01

441

Radiation-induced moyamoya syndrome  

SciTech Connect

Purpose: The moyamoya syndrome is an uncommon late complication after radiotherapy (RT). Methods and Materials: A PubMed search of English-language articles, with radiation, radiotherapy, and moyamoya syndrome used as search key words, yielded 33 articles from 1967 to 2002. Results: The series included 54 patients with a median age at initial RT of 3.8 years (range, 0.4 to 47). Age at RT was less than 5 years in 56.3%, 5 to 10 years in 22.9%, 11 to 20 years in 8.3%, 21 to 30 years in 6.3%, 31 to 40 years in 2.1%, and 41 to 50 years in 4.2%. Fourteen of 54 patients (25.9%) were diagnosed with neurofibromatosis type 1 (NF-1). The most common tumor treated with RT was low-grade glioma in 37 tumors (68.5%) of which 29 were optic-pathway glioma. The average RT dose was 46.5 Gy (range, 22-120 Gy). For NF-1-positive patients, the average RT dose was 46.5 Gy, and for NF-1-negative patients, it was 58.1 Gy. The median latent period for development of moyamoya syndrome was 40 months after RT (range, 4-240). Radiation-induced moyamoya syndrome occurred in 27.7% of patients by 2 years, 53.2% of patients by 4 years, 74.5% of patients by 6 years, and 95.7% of patients by 12 years after RT. Conclusions: Patients who received RT to the parasellar region at a young age (<5 years) are the most susceptible to moyamoya syndrome. The incidence for moyamoya syndrome continues to increase with time, with half of cases occurring within 4 years of RT and 95% of cases occurring within 12 years. Patients with NF-1 have a lower radiation-dose threshold for development of moyamoya syndrome.

Desai, Snehal S. [Department of Radiology, Division of Radiation Oncology, Baylor College of Medicine, Methodist Hospital, Houston, TX (United States); Paulino, Arnold C. [Department of Radiology, Division of Radiation Oncology, Baylor College of Medicine, Methodist Hospital, Houston, TX (United States)]. E-mail: apaulino@tmh.tmc.edu; Mai, Wei Y. [Department of Radiology, Division of Radiation Oncology, Baylor College of Medicine, Methodist Hospital, Houston, TX (United States); Teh, Bin S. [Department of Radiology, Division of Radiation Oncology, Baylor College of Medicine, Methodist Hospital, Houston, TX (United States)

2006-07-15

442

Why Metabolic Syndrome Matters  

MedlinePLUS

Why Metabolic Syndrome Matters Updated:Jul 24,2014 Metabolic syndrome may be diagnosed when a patient has a cluster of risk factors for ... Syndrome • Home • About Metabolic Syndrome • Why Metabolic Syndrome Matters • Your Risk for Metabolic Syndrome • Symptoms & Diagnosis • Prevention & ...

443

[Keratoprostheses. Clinical aspects].  

PubMed

In patients with dry eye, like pemphigoid, Fuchs-Stevens-Johnson syndrome and xerophthalmia, in eyes with severe vascularisation of the cornea due to severe burns, in a leucoma adherens or following recurrent transplant rejections as well as in cloudy corneas of silicone-filled eyes, restoration of visual acuity can often only be achieved by a keratoprosthesis. In the case of an intact posterior segment and retinal function a normal visual acuity can be anticipated but mostly with a restricted visual field. Complications vary from glaucoma, retroprosthetic membranes, necrosis of the recipient tissue at the rim of the prosthesis, endophthalmitis, loosening and loss of the prosthesis even to the loss of visual acuity. With some types of prostheses the visual acuity will be reduced again within a short time, but in osteo-odonto-keratoprostheses, using the root of a patients tooth as haptic material for the optical cylinder, good long-term results even for several decades are possible. In patients without suitable teeth, prostheses with biointegrable materials such as Pintucci keratoprostheses can be used. A basic rule is that a secondary glaucoma, which in this patient group frequently develops even preoperatively, must be treated adequately. PMID:12148298

Hille, K

2002-07-01

444

Limbal stem cell transplantation: new progresses and challenges.  

PubMed

Patients with limbal stem cell deficiency (LSCD) suffer from photophobia and a severe loss of vision uncorrectable by conventional PKP. This literature review shows that new strategies can be formulated for treating LSCD. Early cryopreserved amniotic membrane transplantation (AMT) as a temporary biological bandage with sutures or with sutureless ProKera in the acute stage of chemical burn and Stevens-Johnson syndrome prevents the occurrence of LSCD by preserving and expanding the remaining limbal epithelial stem cells. Similarly, remaining limbal stem cells can also be expanded in corneal surfaces with partial or nearly total LSCD if corneal pannus is removed and AMT is performed as a graft with or without sutures by the use of fibrin glue. Moreover, AMT as a temporary bandage and a graft using fibrin glue can also facilitate corneal surface reconstruction by reducing the size of a conjunctival limbal autograft (CLAU) to one 60 degrees graft for unilateral total LSCD as well as promote the success of a keratolimbal allograft (KLAL) for bilateral total LSCD. The latter success is further dictated by effective systemic immunosuppression and by measures to restore the ocular surface defenses, suppress conjunctival inflammation, and correct cicatricial complications so that a stable tear film can be maintained before surgery. This review also summarizes recent findings and outlines future challenges that we need to overcome in squamous metaplasia, that is, another major type of ocular surface failure. PMID:19098704

Liang, L; Sheha, H; Li, J; Tseng, S C G

2009-10-01

445

Marfan's syndrome with aortic valve endocarditis.  

PubMed

Marfans syndrome is an Autosomal dominant disorder of the connective tissues resulting in abnormalities of the musculoskeletal system, cardiovascular system and eyes. It has a prevalence of 1 in 100,000 population1 and occurs in all ethnic groups. It may be familial or due to new mutation (30%), in the fibrillin gene on arm of chromosome 15. It is estimated that one person in every 3000-5000 has Marfans syndrome may have cardiovascular abnormalities and may be complicated by infective endocartditis. About 90% of Marfan patients will develop cardiac complications2. The patient under discussion has musculoskeletal (Tall stature, reduced upper-lower segment ratio, arm-span to height ratio > 1.05, high arched palate) and Cardiovascular features (Severe aortic regurgitation complicated with infective endocarditis). PMID:16400220

Jaiswal, S; Magar, B-S; Poudel, M; Joshi, L-N; Neopane, A; Karki, D-B

2004-01-01

446

Metabolic complications of schizophrenia and antipsychotic medications--an updated review.  

PubMed

Metabolic syndrome is a cluster of risk factors comprising obesity, dyslipidaemias, glucose intolerance, insulin resistance (or hyperinsulinaemia), and hypertension, and is highly predictive of type 2 diabetes mellitus and cardiovascular disease. The life expectancy of people with schizophrenia is reduced by 20%, with 60% of the excess mortality due to physical illness. Schizophrenia itself may be a risk factor for metabolic syndrome and there is also increasing concern that antipsychotic drugs, particularly second-generation antipsychotics, have metabolic consequences that contribute to the risk. Various diagnostic guidelines, updated facts with regard to epidemiology, pathophysiology, risk factors, and complications of metabolic syndrome are discussed in this review. Moreover, the impact of various antipsychotics on metabolic syndrome and their possible mechanisms are comprehensively reviewed. The authors emphasise that, while many adults with schizophrenia receive little or no medical care, such care is important given the risk of metabolic abnormalities associated not only with antipsychotic medications, but also with schizophrenia in general. PMID:23535629

Yogaratnam, J; Biswas, N; Vadivel, R; Jacob, R

2013-03-01

447

Entry Complications in Laparoscopic Surgery  

PubMed Central

To review the complications associated with laparoscopic surgery and provide clinical direction regarding the best practice based on the best available evidence. The laparoscopic entry techniques and technologies reviewed include the classic pneumoperitoneum (Veress/trocar), the open (Hasson), the direct trocar insertion, the use of disposable shielded trocars, radially expanding trocars and visual entry systems. Medline, Pubmed and Cochrane Databases were searched for English language articles published before December 2008. It is an evidence based fact that minimal access surgery is superior to conventional open surgery since this is beneficial to the women, community and the healthcare system.Over the past 50 years, many techniques, technologies and guidelines have been introduced to eliminate the risks associated with laparoscopic entry. No single technique or instrument has been proved to eliminate laparoscopic entry associated injury. Proper evaluation of the women, supported by surgical skills and good knowledge of the technology and instrumentation is the keystone to safe access and prevention of complications during laparoscopic surgery. PMID:22442503

Krishnakumar, S; Tambe, P

2009-01-01

448

Bacterial infections complicating tongue piercing  

PubMed Central

Tongue piercing has become an increasingly popular form of body art. However, this procedure can occasionally be complicated by serious bacterial infections. The present article reports a case of prosthetic valve endocarditis caused by a Gemella species in a patient with a pierced tongue, and reviews 18 additional cases of local and systemic bacterial infections associated with tongue piercing. Infections localized to the oral cavity and head and neck region included molar abscess, glossal abscess, glossitis, submandibular lymphadenitis, submandibular sialadenitis, Ludwig’s angina and cephalic tetanus. Infections distal to the piercing site included eight cases of infective endocarditis, one case of chorioamnionitis and one case of cerebellar abscess. Oropharyngeal flora were isolated from all cases. While bacterial infections following tongue piercing are rare, there are reports of potentially life-threatening infections associated with the procedure. Both piercers and their clients should be aware of these potential complications, and standardized infection prevention and control practices should be adopted by piercers to reduce the risk. PMID:21358880

Yu, Catherine HY; Minnema, Brian J; Gold, Wayne L

2010-01-01

449

[Infectious complications after esophageal surgery].  

PubMed

The incidence of wound infection, which is an intrasurgical field infection, is lower than the incidence of pneumonia, which is an extrasurgical field infection, after esophageal cancer surgery. Several trials predicting postoperative infectious complications have been reported. One measured the phytohemagglutinin- and concanavalin A-induced proliferation of peripheral blood mononuclear cells in patients; one measured the white blood cell (WBC) count 2 h after surgery and the decrease in WBC count on first postoperative day; and another showed that the decrease in serum IgG2 level can predict the occurrence of methicillin-resistant Staphylococcus aureus (MRSA) infections. Useful strategies for managing infectious complications have also been reported. Applying mupirocin calcium hydrate ointment to the nasal cavity decreases the incidence of MRSA infections. Autologous blood collection reduces the need for allogeneic transfusion in patients undergoing resection of esophageal cancer, and avoidance of allogeneic transfusion may reduce the risk of postoperative infection. The total exposure to preoperative chemoradiotherapy should be limited to 40 Gy or less to prevent postoperative pneumonia. PMID:12599924

Ozawa, Soji; Kitagawa, Yuko; Okamoto, Nobuhiko; Shimizu, Yoshimasa; Kitajima, Masaki

2002-12-01

450

Hemophagocytic syndrome in severe dengue Fever: a rare presentation.  

PubMed

We describe a 2-year old boy developing virus-associated hemophagocytic syndrome in severe dengue fever. The condition was diagnosed according to the established criteria of the International Histiocyte Society. There was uneventful recovery with corticosteroid therapy. Secondary hemophagocytosis in children can mimic severe sepsis, systemic inflammatory response syndrome, or multi organ dysfunction syndrome and lead to diagnostic difficulties. This report adds to the limited pediatric cases of dengue related hemophagocytic syndrome reported in literature; and underlines the importance of prompt diagnosis and appropriate treatment of this rare but serious complication. PMID:25332549

Mitra, Subhashis; Bhattacharyya, Rajat

2014-09-01

451

[Skin complications of diabetes mellitus therapy].  

PubMed

Author summarizes potencial skin complications related to the therapy with insulin or peroral antidiabetics. These are mainly allergic reactions and lipodystrophic changes complicating or even precluding the therapy. PMID:16771093

Olsovský, J

2006-05-01

452

Managing Complications of Diabetes in Later Life  

MedlinePLUS

... previous page Related Topics Diabetes Related Documents PDF Living with Diabetes Download PDF Managing Complications of Diabetes in Later Life Download Join our e-newsletter! Resources Managing Complications ...

453

Neuropathy in Wolfram syndrome.  

PubMed

Wolfram syndrome (WFS) is a degenerative disease with neurological and endocrine disorders, characterized by the association of juvenile diabetes mellitus and bilateral optic atrophy. A polyneuropathy was exceptionally described but its characteristics are not well-established. In addition to our observation, we searched all case reports of patients with WFS in the medical literature (more than 600), and selected patients who underwent an EMG: twenty-one patients underwent an EMG, which was considered as abnormal in only 8 cases. The common profile was axonal sensory-motor polyneuropathy, sometimes with marked decrease of motor conduction velocities. This neuropathy could be due to diabetes mellitus, even though microangiopathic and macroangiopathic complications are rare in WFS. Another origin for this neuropathy could be a degenerative process in relationship with WFS. PMID:20888932

Mathis, Stéphane; Maisonobe, Thierry; Neau, Jean-Philippe

2011-01-01

454

Endometriosis, a modern syndrome  

PubMed Central

The identification of endometriosis has been a subject of intense debate over the last decade. There is, however, no doubt that Thomas Cullen was the first to describe endometriosis and adenomyosis as one disease characterized by the presence of endometrium-like tissue outside the uterine cavity. With the introduction of laparoscopy in the early 1960s three different clinical presentations of endometriosis were distinguished: peritoneal, deep adenomyotic and cystic ovarian. As soon as synthetic steroids became available, pioneer clinicians started utilizing these in an attempt to replace radical surgery by a medical treatment. While medical therapy may resort in relief, in most cases the current approach consists of a combination of medical and surgical therapy. While the pathogenesis of endometriosis is still enigmatic and complex, there is increasing evidence that endometriosis is part of a uterine reproductive dysfunction syndrome. For prevention of complications, it is very important that diagnosis is made as early as possible in a woman's life. PMID:21727656

Brosens, Ivo; Benagiano, Giuseppe

2011-01-01

455

Cowden syndrome.  

PubMed

Cowden syndrome (CS) is a rare inherited condition characterised by multiple hamartomas in a variety of tissues from all three embryonic layers. It is a cancer predisposition syndrome with an increased risk of developing malignancy in many tissues but especially breast, thyroid and endometrium. It is inherited in an autosomal dominant manner with ?80% of patients having a germ-line mutation of the PTEN tumour suppressor gene. Presenting signs and symptoms are highly non-specific. Nevertheless clinicians should be able to recognise this syndrome so that patients may be screened for cancerous growths and afforded the opportunity to have genetic testing to assist them and their family members in making medical management decisions. We present a review of this unusual but important condition with particular emphasis on the diagnostic criteria, clinical features, genetics, management and surveillance. PMID:20580873

Farooq, A; Walker, L J; Bowling, J; Audisio, R A

2010-12-01

456