Favourable Prognosis (favourable + prognosis)

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Distribution within Medical Sciences


Selected Abstracts


Specific immunoglobulin E and immunoglobulin G antibodies to toluene diisocyanate-human serum albumin conjugate: useful markers for predicting long-term prognosis in toluene diisocyanate-induced asthma

CLINICAL & EXPERIMENTAL ALLERGY, Issue 4 2002
H.-S. Park
Summary Background Our previous study reported that more than 50% of toluene diisocyanate (TDI)-induced asthma patients had persistent asthmatic symptoms even after complete avoidance. Although specific IgE (sIgE) has been detected in a portion of patients with TDI-asthma, a recent investigation suggests that the presence of serum specific IgG (sIgG), not sIgE, is more closely associated with positive bronchoprovocation test (BPT) results. Objective To evaluate the possible role of sIgE and sIgG in predicting long-term prognosis of TDI-asthma. Materials and methods Forty-one TDI-asthma patients whose diagnosis was confirmed by TDI-BPT, and 20 unexposed healthy controls were enrolled. Both sIgE and sIgG to TDI-human serum albumin (HSA) conjugate were detected by ELISA. All patients with persistent asthmatic symptoms took anti-asthmatic medications during the follow-up period (mean: 67.5 months) and were instructed to avoid exposure to TDI. Airway hyper-responsiveness to methacholine (AHM) was monitored every year during the study period. The patients were classified into three groups according to changing patterns of AHM and asthmatic symptoms as follows: group I, no improvement with persistent asthmatic symptoms (n = 12); group II, partial improvement with persistent asthmatic symptoms (n = 13); group III, in remission (n = 16). Results Favourable prognosis was associated with a mild degree of AHM at initial diagnosis (P < 0.05). Although there were no significant differences in the prevalence of sIgE antibody to TDI-HSA conjugate among the three groups (P > 0.05), prevalence of sIgG in group I tended to be higher than in group II (0.05 < P < 0.1). However, the levels of sIgG were significantly higher in group I than in group II (P = 0.05), whereas levels of sIgE were significantly higher in group II than in group I (P = 0.014). No significant differences were noted in exposure duration, sex, age, atopic status, and total IgE level among the three groups (P > 0.05). Conclusion This study confirmed that a favourable outcome is related to a mild degree of AHM and to low levels of sIgG to predict persistent asthmatic symptoms, it also suggested that the presence of high serum-specific IgE at initial diagnosis may represent a better prognosis. [source]


Two clinical manifestations of desmopathy of the accessory ligament of the deep digital flexor tendon in the hindlimb of 23 horses

EQUINE VETERINARY JOURNAL, Issue 6 2005
E. ELIASHAR
Summary Reasons for performing study: Desmopathy of the accessory ligament of the deep digital flexor tendon (ALDDFT) in the hindlimb is an unusual cause of lameness in horses, and reports of the condition are sparse. Objectives: To describe the clinical and ultrasonographic findings, therapy and outcome of 23 horses treated for desmopathy of the ALDDFT in the hindlimb. Methods: Records of 23 horses with ultrasonographic evidence of desmopathy of the ALDDFT in one or both hindlimbs from 3 referral centres were reviewed retrospectively. Age, breed, sex, duration and nature of clinical signs, results of clinical and lameness examinations, treatment and outcome were recorded. Results: In 13 horses (Group A), there was an acute onset of unilateral lameness. Ten horses (Group B) had an insidious or sudden onset of postural abnormality. There were 10 cobs, 5 British native-breed ponies and 8 horses of various larger breeds. Twenty horses were used for general purposes, and mean age was 12 years. Enlargement of the ALDDFT in the affected hindlimb(s) was identified in all horses. In 44% of horses, ultrasonographic abnormalities were localised to part of the ALDDFT. Treatment included box-rest and controlled exercise, and 10 horses were subjected to desmotomy or desmectomy of the ALDDFT. Seventy-three percent of horses in Group A returned to full function, while 90% of those in Group B remained lame. Conclusions: Two distinct clinical conditions are associated with the ALDDFT of the hindlimb. Traumatically induced injury resulting in acute onset lameness appears to have a favourable prognosis, with most horses returning to previous work. However, postural changes, once present, are irreversible and indicate a poor prognosis. Potential relevance: Desmopathy of the ALDDFT should be recognised as a potential cause of hindlimb lameness and this study provides clinical and prognostic information. Knuckling and/or semiflexion of the metatarsophalangeal joint may accompany the condition; therefore, if a horse is presented with a flexural deformity of this joint, desmopathy of the ALDDFT should be considered as a primary differential diagnosis. [source]


Nucleophosmin (NPM1) mutations in adult and childhood acute myeloid leukaemia: towards definition of a new leukaemia entity,

HEMATOLOGICAL ONCOLOGY, Issue 4 2009
Rachel Rau
Abstract Nucleophosmin (NPM) is a ubiquitously expressed chaperone protein that shuttles rapidly between the nucleus and cytoplasm, but predominantly resides in the nucleolus. It plays key roles in ribosome biogenesis, centrosome duplication, genomic stability, cell cycle progression and apoptosis. Somatic mutations in exon 12 of the NPM gene (NPM1) are the most frequent genetic abnormality in adult acute myeloid leukaemia (AML), found in approximately 35% of all cases and up to 60% of patients with normal karyotype (NK) AML. In children, NPM1 mutations are far less frequent, occurring in 8,10% of all AML cases, and in approximately 25% of those with a NK. NPM1 mutations lead to aberrant localization of the NPM protein into the cytoplasm, thus the designation, NPMc+ AML. NPMc+ AML is seen predominantly in patients with a NK and is essentially mutually exclusive of recurrent chromosomal translocations. Patients with NPM1 mutations are twice as likely as those who lack an NPM1 mutation to also have a FMS-like tyrosine kinase (FLT3) internal tandem duplication (ITD) mutation. NPMc+ AML is also characterized by a unique gene expression signature and microRNA signature. NPMc+ AML has important prognostic significance, as NPMc+ AML, in the absence of a coexisting FLT3-ITD mutation, is associated with a favourable outcome. NPM1 mutations have also shown great stability during disease evolution, and therefore represent a possible marker for minimal residual disease detection. Given its distinctive biologic and clinical features and its clear clinical relevance, NPMc+ AML is included as a provisional entity in the 2008 WHO classifications. There is still much to be learned about this genetic alteration, including its exact role in leukaemogenesis, how it interacts with other mutations and why it confers a more favourable prognosis. Further, it represents a potential therapeutic target warranting research aimed at identifying novel small molecules with activity in NPMc+ AML. Copyright 2009 John Wiley & Sons, Ltd. [source]


Distinguishing medullary carcinoma of the breast from high-grade hormone receptor-negative invasive ductal carcinoma: an immunohistochemical approach

HISTOPATHOLOGY, Issue 7 2010
Uta Flucke
Flucke U, Flucke M T, Hoy L, Breuer E, Goebbels R, Rhiem K, Schmutzler R, Winzenried H, Braun M, Steiner S, Buettner R & Gevensleben H (2010) Histopathology,56, 852,859 Distinguishing medullary carcinoma of the breast from high-grade hormone receptor-negative invasive ductal carcinoma: an immunohistochemical approach Aims:, Medullary carcinomas (MCs) represent a rare breast cancer subtype associated with a rather favourable prognosis compared with invasive ductal carcinomas (IDCs). Due to histopathological overlap, MCs are frequently misclassified as high-grade IDCs, potentially leading to overtreatment of MCs. Our aim was to establish novel diagnostic markers distinguishing MCs from hormone receptor-negative high-grade IDCs. Methods and results:, Sixty-one MCs and 133 hormone receptor-negative IDCs were analysed in a comparative immunohistochemical study. Applied markers included a comprehensive panel of cytokeratins (CKs), vimentin, smooth muscle actin (SMA), p63, p53, cell adhesion molecules [N-CAM (CD56), syndecan-1 (CD138), E-cadherin and P-cadherin] and development associated transcription factors (AP-2,, AP-2,). A significantly higher proportion of IDCs displayed increased expression of CK7, AP-2, and HER2 in contrast to MCs (CK7: 91% of IDCs versus 77% of MCs; AP-2,: 77% versus 57%; and HER2: 26% versus 7%, each P < 0.01). Vice versa, MCs were slightly more frequently positive for SMA and vimentin (P > 0.05). Conclusions:, Hormone receptor-negative high-grade IDCs are significantly associated with luminal differentiation, Her2 and AP-2, overexpression, whereas MCs tend to display myoepithelial features. Markers analysed in this study are of diagnostic value regarding the differential diagnosis of MCs. [source]


Expression of c-MET, low-molecular-weight cytokeratin, matrix metalloproteinases-1 and -2 in spinal chordoma

HISTOPATHOLOGY, Issue 5 2009
Takahiko Naka
Aims:, In skull base chordoma, c-MET expression has been reported to correlate with younger patient age and favourable prognosis; however, it also contributes to tumour invasiveness, especially in recurrent lesions, suggesting variable roles for c-MET according to clinical status. The aim of this study was to investigate the significance of c-MET expression in spinal chordoma, which affects patients who are 10,20 years older than those with skull base chordoma. Methods and results:, Using immunohistochemical techniques, the expression of c-MET and its ligand, hepatocyte growth factor (HGF) was investigated in 34 primary spinal chordomas and compared with other clinicopathological parameters. Expression of c-MET and HGF was observed in 85.3 and 21.7% of lesions, respectively. c-MET expression correlated with the expression of an epithelial marker, low-molecular-weight cytokeratin (CAM5.2). Lesions with higher c-MET expression showed significantly stronger expression of proteinases, including matrix metalloproteinase (MMP)-1 and MMP-2. However, c-MET expression was not associated with patient age, proliferative ability estimated by MIB-1 labelling index, or prognosis. Conclusions:, c-MET expression was observed in most spinal chordomas and correlated with the expression of CAM5.2, suggesting a relationship to an epithelial phenotype. [source]


Total pancreatectomy in six patients with intraductal papillary mucinous tumour of the pancreas: the treatment of choice

HPB, Issue 4 2001
J Bendix Holme
Background Intraductal papillary mucinous tumours (IPMT) were described as a distinct entity in 1982. The extent of surgical resection remains controversial. Methods Six patients with a diffuse dilatation of the main pancreatic duct were treated with total pancreatectomy for cure of IPMT. Results Histological examination showed one IPM adenoma, four IPM non-invasive carcinomas and one IPM invasive carcinoma. In all but one case multifocal extensive intraductal changes were found, affecting either most of the pancreas or the whole organ. All patients survived the operation and remain alive 5,56 months later. Post-pancreatectomy diabetes has been moderately well controlled. Discussion IPMTs represent a subgroup of pancreatic neoplasms with a favourable prognosis, and the resection should aim at removing all dysplastic foci. In cases with diffuse dilatation of the main pancreatic duct, widespread tumour involvement of the duct system can be expected, so total pancreatectomy should be the operation of choice. [source]


Enlargement and the European employment strategy: turbulent times ahead?

INDUSTRIAL RELATIONS JOURNAL, Issue 5 2003
Mike Ingham
The European Employment Strategy (EES) is set to remain centre stage as the EU embraces ten new member states. The evidence regarding the prospects of the accession countries meeting the increasingly explicit targets that the EES has set for the years up to 2010 does not yield a favourable prognosis. [source]


Zygomycosis , a case report and overview of the disease in India

MYCOSES, Issue 4 2007
Amit Diwakar
Summary A case of zygomycosis caused by Rhizopus oryzae in a diabetic patient previously misdiagnosed as invasive pulmonary aspergillosis and an overview of the disease in India are presented. The case was diagnosed by direct microscopy, histopathologic examination and culture. Following surgical resection of pulmonary cavity under cover of amphotericin B administration, the patient recovered completely. Of 461 cases reported to-date, approximately 70% had been diagnosed at the Postgraduate Institute of Medical Education and Research, Chandigarh, in north India. This may be attributed to better awareness, expertise and infrastructural facilities for mycological diagnosis than to any particular regional preponderance of the disease. Rhino-orbito-cerebral manifestations were the most common feature of zygomycosis (269 cases), followed by cutaneous disease (66 cases), which is in conformity with the pattern prevalent worldwide. The etiologic agents encountered were Rhizopus oryzae, Apophysomyces elegans, Saksenaea vasiformis, Cunninghamella bertholletiae, Absidia corymbifera, Basidiobolus ranarum and Conidiobolus coronatus. In contrast to cases from the developed world where transplant recipients and patients with haematological malignancies seem to be most vulnerable to zygomycosis, the most common risk factor in India was uncontrolled diabetes mellitus. Amphotericin B was the mainstay of various treatment modalities employed. The relevance of a strong clinical suspicion and early diagnosis of zygomycosis for favourable prognosis can hardly be over-emphasised. [source]


The effect of warfarin use on clinical stage and histological grade of prostate cancer

PHARMACOEPIDEMIOLOGY AND DRUG SAFETY, Issue 5 2010
V. Tagalakis MD
Abstract Purpose Prolonged warfarin use may decrease the risk of prostate cancer. We aimed to assess the effect of warfarin on histological grade and clinical stage of prostate cancer at diagnosis. Methods We carried out a retrospective population-based cohort study of men older than 50 years of age diagnosed with prostate cancer between 1985 and 2002 and registered with the Saskatchewan Cancer Registry. We compared a composite score of histological grade and clinical stage of prostate cancer at diagnosis according to warfarin use in the 5 years preceding the diagnosis of prostate cancer. Results Compared with non-users, men with at least 2 years of cumulative warfarin use in the 5 year period preceding the diagnosis of prostate cancer were at a lower risk of a poor prognosis composite score at the time of their prostate cancer diagnosis (OR 0.40, 95%CI (0.19,0.83)), and when intermediate and poor prognosis scores were combined, a similar estimate of association was observed (OR 0.55, 95%CI (0.33,0.91)), adjusted for age at diagnosis and year of diagnosis. However, an increased risk of poor prognosis disease was observed with 4 years of cumulative warfarin use compared to never use (OR 2.2, 95%CI (1.03,4.81)). Conclusions There is a suggestion that at least 2 years of warfarin use is associated with a more favourable prognosis but that extended duration of use beyond 2 years may be associated with poor prognosis disease. Further investigation with a more complete assessment of confounders and that addresses potential detection biases is warranted. Copyright 2010 John Wiley & Sons, Ltd. [source]


Methylthioadenosine phosphorylase and activated insulin-like growth factor-1 receptor/insulin receptor: potential therapeutic targets in chordoma,

THE JOURNAL OF PATHOLOGY, Issue 5 2010
Josh Sommer
Abstract Currently there is no effective chemotherapy for chordoma. Recent studies report co-expression of insulin-like growth factor-1 receptor (IGF1R) and its cognate ligand in chordoma, but it is unknown whether this receptor tyrosine kinase is activated in these tumours. Additionally, genetic studies have confirmed frequent deletions of chromosome 9p in chordomas, which encompasses the cyclin-dependent kinase inhibitor 2A (CDKN2A) locus. Another gene in this region, methylthioadenosine phosphorylase (MTAP), is an essential enzyme of the purine salvage pathway and has therapeutic relevance because MTAP-deficient cells are particularly sensitive to inhibitors of de novo purine synthesis. We investigated whether these pathways might be potential therapeutic targets for chordoma. Paraffin-embedded tissue samples from 30 chordomas were analysed by immunohistochemistry for expression of the phosphorylated isoforms of IGF1R or the insulin receptor (pIGF1R/pIR) and selected downstream signalling molecules, including BCL2-associated agonist of cell death protein (BAD). Expression of CDKN2A and MTAP proteins was also assessed. Skeletal chondrosarcomas, benign notochordal cell tumours, and fetal notochord were studied for comparison. Phosphorylated IGF1R/IR was detected in 41% of chordomas, together with activated downstream signalling molecules, and pIGF1R/pIR was absent in benign notochordal cell tumours and fetal notochord. Thirty-nine per cent of chordomas were negative for MTAP immunoreactivity. Patients with pIGF1R/pIR-positive tumours showed significantly decreased median disease-free survival in multivariate survival analysis (p = 0.036), whereas phosphorylation of BAD at serine-99 was found to be associated with a favourable prognosis (p = 0.002). Approximately 40% of chordomas demonstrate evidence of activation of the IGF1R/IR signalling pathway or loss of a key enzyme in the purine salvage pathway. Aberrant signalling cascades and disrupted metabolic pathways such as these may represent opportunities for novel targeted therapeutic approaches for the treatment of chordoma. Copyright 2009 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd. [source]


Fas ligand and tumour counter-attack in colorectal cancer stratified according to microsatellite instability status

THE JOURNAL OF PATHOLOGY, Issue 1 2003
Julie M Michael-Robinson
Abstract Expression of membrane-bound Fas ligand (FasL) by colorectal cancer cells may allow the development of an immune-privileged site by eliminating incoming tumour-infiltrating lymphocytes (TILs) in a Fas-mediated counter-attack. Sporadic colorectal cancer can be subdivided into three groups based on the level of DNA microsatellite instability (MSI). High-level MSI (MSI-High) is characterized by the presence of TILs and a favourable prognosis, while microsatellite-stable (MSS) cancers are TIL-deficient and low-level MSI (MSI-Low) is associated with an intermediate TIL density. The purpose of this study was to establish the relationship between MSI status and FasL expression in primary colorectal adenocarcinoma. Using immunohistochemistry and a selected series of 101 cancers previously classified as 31 MSI-High, 30 MSI-Low, and 40 MSS, the present study sought to confirm the hypothesis that increased TIL density in MSI-High cancers is associated with low or absent membrane-bound FasL expression, while increased FasL in MSS cancers allows the killing of host TILs. TUNEL/CD3 double staining was also used to determine whether MSS cancers contain higher numbers of apoptotic TILs in vivo than MSI-High or MSI-Low cancers. Contrary to the initial hypothesis, it was found that MSI-High cancers were associated with higher FasL expression (p = 0.04) and a stronger intensity of FasL staining (p = 0.007). In addition, mucinous carcinomas were independently characterized by increased FasL expression (p = 0.03) and staining intensity (p = 0.0005). Higher FasL expression and staining intensity did not correlate with reduced TIL density or increased numbers of apoptotic TILs. However, consistent with the hypothesis that curtailment of the host anti-tumour immune response contributes to the poor prognosis in MSS cancers, it was found that apoptotic TILs were most abundant in MSS carcinomas and metastatic Dukes' stage C or D tumours (p = 0.004; p = 0.046 respectively). This study therefore suggests that MSS colorectal cancers are killing incoming TILs in an effective tumour counter-attack, but apparently not via membrane-bound FasL. Copyright 2003 John Wiley & Sons, Ltd. [source]


Tubular carcinoma of the breast: Prognosis and response to adjuvant systemic therapy

ANZ JOURNAL OF SURGERY, Issue 1 2001
P. R. B. Kitchen
Background: Tubular carcinoma of the breast is an uncommon and usually small tumour, and is thought to have a favourable prognosis. The present study examined the long-term prognosis of patients with tubular breast carcinoma and the roles of axillary dissection and adjuvant therapy. Methods: Eighty-six tubular cases were identified from a large worldwide database of 9520 breast carcinoma patients entered into randomized adjuvant therapy trials run by the International Breast Cancer Study Group from 1978 to 1999. These patients were followed for a median of 12 years. Results: Forty-two (49%) cases were node-positive, of which 33 (79%) had 1,3 nodes involved. Ten (32%) of the 31 smaller tumours (, 1 cm in size) were node-positive. Patients with node-positive tubular carcinoma had a significantly better 10-year relapse-free survival (P = 0.006) and survival (P < 0.0001) compared with non-tubular node-positive cases. Overall survival was similar for node-positive and node-negative tubular carcinoma. Overall, 71 patients (83%) received some form of adjuvant systemic therapy. Of the 86 cases, 43 (50%) received more than one course of chemotherapy. There was an 85% decrease in the risk of death for patients who received more than one course of chemotherapy compared to those who did not (hazard ratio 0.15, 95% confidence interval (CI): 0.03,0.82; P = 0.03). Conclusions: Compared to other histological types of breast cancer, tubular carcinoma has a better long-term prognosis. Adjuvant chemotherapy may further improve prognosis and involvement of axillary nodes may not be an indicator for early death due to breast carcinoma. [source]


Maternal and neonatal outcomes in 54 triplet pregnancies managed in an Australian tertiary centre

AUSTRALIAN AND NEW ZEALAND JOURNAL OF OBSTETRICS AND GYNAECOLOGY, Issue 3 2004
Andrea BARKEHALL-THOMAS
Abstract Background:, To provide current data on maternal and neonatal outcomes in triplet pregnancies in an Australian population. Methods:, Retrospective case note review of all triplet pregnancies managed within a single Australian tertiary centre. Results:, Fifty-four sets of triplets were managed from January 1996 to October 2002. A total of 59% resulted from the use of assisted reproductive technologies. The median gestation at delivery was 32.5 weeks (range: 21,36 weeks); 14% delivered prior to 28 weeks and 43% delivered before 32 weeks. Preterm labour and preterm rupture of membranes were the most common antenatal complications occurring in 57 and 22% of pregnancies, respectively. A total of 93% of pregnancies were delivered by Caesarean section and 37% of mothers experienced at least one post-partum complication. A total of 96% of neonates were liveborn, with a median birthweight of 1644 g (range: 165,2888 g). The two most common neonatal complications were jaundice and hypoglycaemia in 52 and 43% of liveborn neonates, respectively. A total of 28% of neonates were below the 10th centile for gestational age and sex. A total of 8% of neonates demonstrated congenital anomalies. The perinatal mortality at a gestational age of 20,24 weeks was 100%, 22% at 25,28 weeks and zero for those babies born at 29 weeks or beyond. Conclusion:, Assisted reproductive technologies contribute significantly to the incidence of triplet pregnancies. Gestational age at delivery and perinatal mortality is comparable to published international data. Triplets born in a tertiary centre beyond 28 weeks gestation have a very favourable prognosis in the newborn period. [source]


Prospective monitoring of BCR-ABL1 transcript levels in patients with Philadelphia chromosome-positive acute lymphoblastic leukaemia undergoing imatinib-combined chemotherapy

BRITISH JOURNAL OF HAEMATOLOGY, Issue 4 2008
Masamitsu Yanada
Summary The clinical significance of minimal residual disease (MRD) is uncertain in patients with Philadelphia chromosome-positive acute lymphoblastic leukaemia (Ph+ ALL) treated with imatinib-combined chemotherapy. Here we report the results of prospective MRD monitoring in 100 adult patients. Three hundred and sixty-seven follow-up bone marrow samples, collected at predefined time points during a uniform treatment protocol, were analysed for BCR-ABL1 transcripts by quantitative reverse transcription polymerase chain reaction. Ninety-seven patients (97%) achieved complete remission (CR), and the relapse-free survival (RFS) rate was 46% at 3 years. Negative MRD at the end of induction therapy was not associated with longer RFS or a lower relapse rate (P = 0800 and P = 0964 respectively). Twenty-nine patients showed MRD elevation during haematological CR. Of these, 10 of the 16 who had undergone allogeneic haematopoietic stem cell transplantation (HSCT) in first CR were alive without relapse at a median of 29 years after transplantation, whereas 12 of the 13 who had not undergone allogeneic HSCT experienced a relapse. These results demonstrate that, in Ph+ ALL patients treated with imatinib-combined chemotherapy, rapid molecular response is not associated with a favourable prognosis, and that a single observation of elevated MRD is predictive of subsequent relapse, but allogeneic HSCT can override its adverse effect. [source]


A sensitive model for prediction of relapse in adult acute myeloid leukaemia with t(8;21) using white blood cell count, CD56 and MDR1 gene expression at diagnosis

BRITISH JOURNAL OF HAEMATOLOGY, Issue 4 2004
Markus Schaich
Summary Acute myeloid leukaemia (AML) carrying t(8;21) has an overall favourable prognosis. However, relapse occurs and the impact of multidrug resistance gene (MDR1) expression on recurring disease in this group of patients is not known. We determined quantifiable MDR1 expression in the bone marrow of 28 AML patients with t(8;21) by a validated real-time polymerase chain reaction assay. Using MDR1 expression, white blood cell count and CD56 expression at diagnosis we observed complete concordance of predicted and observed relapses. A calculated logit out of these three variables was a strong independent prognostic factor for overall (P = 0007) and disease-free survival (P = 0002). [source]


Long-term prognosis of children with papillary thyroid cancer presenting with pulmonary metastases

BRITISH JOURNAL OF SURGERY (NOW INCLUDES EUROPEAN JOURNAL OF SURGERY), Issue 9 2000
J. Brink
Background Papillary thyroid cancer (PTC) has a generally favourable prognosis, but elderly patients with distant metastases or extracapsular invasion fair poorly. In small studies with short follow-up, young patients presenting with such extracapsular invasion and pulmonary metastases have faired well. This retrospective study was undertaken to clarify the long-term prognosis of such patients with advanced PTC. Methods Twenty-one children and young adults (median age 14 (range 6,20) years) presenting with PTC and pulmonary metastases were treated at a single institution between 1947 and 1998. Mean maximal tumour diameter was 465 cm. Initial surgical treatment consisted of total thyroidectomy (n = 16), subtotal thyroidectomy (n = 4) and isthmectomy (n = 1), coupled with a variety of lymph node dissections (n = 20). After operation, 19 patients were treated with ablative and incremental doses of iodine-131 until disease free. All patients were placed on suppressive thyroid hormone after operation. Mean length of follow-up was 21 years (range 3 months to 47 years). Follow-up was less than 3 years in four patients. All patients have undergone post-treatment radionucleotide and radiological evaluation. Results Nine of the 21 patients developed recurrent disease. The risk of recurrence at 5 years was 39 (95 per cent confidence interval 14,57) per cent. Eight had cervical lymph node recurrence and no patient developed recurrent pulmonary disease. All patients with identifiable recurrent disease underwent selective lymph node resection, which involved multiple resections in four. At follow-up, 18 patients remain completely free of disease, one patient has recurrent cervical node disease and two patients have died. The disease-free survival at 5 years was 95 (95 per cent confidence interval 86,100) per cent. Cause-specific death occurred in a single patient who died from extensive local disease at age 29 years after 12 years of multiple cervical lymph node recurrences. Conclusion A stepwise treatment approach including total thyroidectomy, high-dose iodine-131 treatment and early surgical reintervention for suspected local recurrent disease allows long-term survival and frequent ,cure' for young patients with PTC and concomitant pulmonary metastases. 2000 British Journal of Surgery Society Ltd [source]


Autosomal dominant pericentral retinal dystrophy caused by a novel missense mutation in the TOPORS gene

ACTA OPHTHALMOLOGICA, Issue 3 2010
Kaja Kristine Selmer
Abstract. Purpose:, This study aimed to identify the genetic cause of autosomal dominant pericentral retinal dystrophy (adPRD) in a large Norwegian family with 35 affected members. Methods:, The family was characterized by clinical ophthalmological examination along with fundus photography, dark adaptometry and electroretinography. We performed a genome-wide linkage analysis followed by sequencing of a candidate gene to identify the mutation causing the disease. Results:, The ophthalmological examinations revealed an atypical form of retinitis pigmentosa (RP), which we prefer to call adPRD. Compared with classical RP, this phenotype has a favourable prognosis. Linkage analysis showed a linkage peak covering the most recently reported adRP gene TOPORS. This gene was sequenced in 19 family members and a novel missense mutation, c.1205a>c, resulting in an amino acid substitution p.Q402P, was detected in all affected members. The mutation showed complete co-segregation with the disease in this family, with a LOD score of 7.3. It is located in a highly conserved region and alignment with the appropriate DNA sequence from other species shows complete conservation of this amino acid. The mutation was not detected in 207 healthy, unrelated controls of Norwegian origin. Conclusions:, We present a novel mutation in the TOPORS gene co-segregating with a distinct phenotype of adPRD in a large Norwegian family. [source]


Isolated invasive Aspergillus tracheobronchitis: a clinical study of 19 cases

CLINICAL MICROBIOLOGY AND INFECTION, Issue 6 2010
N. Wu
Clin Microbiol Infect 2010; 16: 689,695 Abstract Isolated invasive Aspergillus tracheobronchitis (iIATB) is an uncommon clinical form of invasive Aspergillosis in which Aspergillus infection is limited entirely or predominantly to the tracheobronchial tree. In the present study, we retrospectively analyzed the medical records of 19 patients who had histological documented iIATB in the Department of Respiratory Medicine of Changhai Hospital between October 2000 and February 2008. Malignancy was the most common underlying disease, which existed in 14 patients (73.7%) in our series. Most patients had impaired airway structures or defence functions, whereas the systemic immune status was relatively normal. Only three patients (15.8%) had neutropenia. The clinical manifestations and chest radiograph were nonspecific. We classified iIATB into four different forms according to the bronchoscopic features of intraluminal lesions: superficial infiltration type (Type I, n = 4), full-layer involvement type (Type II, n = 2), occlusion type (Type III, n = 6) and mixed type (Type IV, n = 7). Type IV was the largest group in our study, followed by Type III. All patients with iIATB of Type IV had definite airway occlusion. Fourteen patients (73.7%) had a good response to antifungal treatments and five (26.3%) died as a result of the progression of Aspergillosis, all of whom had full-layer invasion of the involved bronchi. In conclusion, we found that iIATB could occur in moderately or non-immunocompromised patients with impaired airway structures or defence functions and may be an early period of invasive pulmonary Aspergillosis. Most of the iIATB patients had a favourable prognosis with early diagnosis and effective antifungal treatment. The morphological features of intraluminal lesions might be of prognostic value. [source]


Reversible brain lesions in childhood hypertension

ACTA PAEDIATRICA, Issue 9 2002
P Singhi
Posterior leukoencephalopathy syndrome is characterized by an acute, usually reversible, encephalopathy with transient occipital lobe abnormalities detected on MRI that occur mostly in association with acute hypertension. The clinical presentation includes seizures, headache, altered mental status and blindness. Disturbed autoregulation of cerebral blood flow and endothelial injury are central to the pathogenesis of this disorder. Prompt control of hypertension results in rapid and complete neurological recovery. In this report we discuss the cases of two children with acute onset hypertension of different aetiologies that presented with the characteristic features of posterior leukoencephalopathy syndrome. Conclusion: Early recognition of this readily treatable condition may obviate the need for extensive and invasive investigations. Despite the alarming lesions on the MRI, prompt control of hypertension carries a uniformly favourable prognosis. [source]