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PubMed2006

Activation of the NF-kappaB pathway by the leukemogenic TEL-Jak2 and TEL-Abl fusion proteins leads to the accumulation of antiapoptotic IAP proteins and involves IKKalpha.

Abnormal activation of tyrosine kinases and of signaling pathways they control plays a critical role in the neoplastic process of human hematopoietic malignancy. The nuclear factor-kappaB (NF-kappaB) pathway is one of the signalings activated by the TEL-Jak2 and TEL-Abl oncoproteins and required for their antiapoptotic activity. To define the signal relay responsible for this activation, we used mouse embryonic fibroblast (MEF) cells and observed that TEL-Jak2- and TEL-Abl-mediated NF-kappaB induction was abolished in cells lacking the IkappaB kinase (IKK)alpha but not in IKKbeta(-/-) cells. Similar observations were performed with oncogenic forms of the FMS-like tyrosine kinase 3 (Flt-3) involved in the pathogenesis of one-third of acute myeloid leukemias. Rescue of TEL-Jak2-mediated NF-kappaB activation was obtained with a kinase-proficient form of IKKalpha in IKKalpha(-/-) MEF. Hematopoietic cells transformed by TEL-Jak2 and TEL-Abl showed sustained IKKalpha activity without promotion of NF-kappaB2/p100 processing, generally associated to IKKalpha functions. Furthermore, IAP1, IAP2 and XIAP, which are central regulators of the NF-kappaB-mediated survival pathway, were highly expressed in cells transformed by these oncoproteins. Our results indicate that these oncogenic tyrosine kinases preferentially use an IKKalpha-dependent mechanism to induce a persistent NF-kappaB activity and allow the production of antiapoptotic effectors that participate to their leukemogenic properties.

Animals↗
PubMed2002

Conversion motor paralysis disorder: overview and rehabilitation model.

It is important to consider a differential diagnosis between paralysis on an organic basis and paralysis and disability due to psychological mechanisms in people with physical impairment secondary to trauma, without evidence of organic etiology. We review the most dramatic type of conversion disorder (CD)-'Conversion Motor Paralysis'. Recent important medical literature concerning the accepted treatment and rehabilitation management will be reviewed and discussed. The inter-disciplinary in-patient team management approach in a rehabilitation setting offers the benefits of a comprehensive assessment and treatment. The diagnosis is temporary and conditional, since there may be a long delay until the appearance of organic findings. A complete medical assessment is essential in order to rule out any possibility of an organic etiology. In as many as 25% to 50% of patients diagnosed as conversion, an organic medical diagnosis was found.

Behavior Therapy↗
PubMed1976

The LOT test and school-age children.

The LOT test was administered to 100 normal-hearing subjects ranging in age from seven years, six months to 10 years, six months. The test was conducted at 500, 1000, 2000, and 4000 Hz with 99 subjects who were able to perform the required task. The LOT test misclassified (false-positive result) only 4% of the normal-hearing children at 1000 Hz. The test misclassified 3% at 2000 Hz, 3% at 4000 Hz, and 14% at 500 Hz. A new criterion for the use of the LOT test with children is proposed which reduced the percentage of misclassification to 2% at 1000, 2000, and 4000 Hz. The frequency of 500 Hz is not recommended for the preadolescent population.

Age Factors↗
PubMed1977

Effects of sophistication on four tests for nonorganic hearing loss.

Three groups of 15 normal-hearing subjects were asked to simulate a hearing loss in one ear. Each subject was then tested on four measurements often used in determining the presence of pseudohypacusis (a comparison of the SRT to the pure-tone average, ascending vs descending pure-tone tests, the Stenger test, and pure-tone delayed auditory feedback). Results showed that as practice and sophistication on each of the procedures increased, there tended to be more improvement in the subjects' ability to "beat" some of the tests than others.

Adult↗
PubMed1999

Trauma-induced dissociative amnesia in World War I combat soldiers.

OBJECTIVE: This study relates trauma-induced dissociative amnesia reported in World War I (WW I) studies of war trauma to contemporary findings of dissociative amnesia in victims of childhood sexual abuse. METHOD: Key diagnostic studies of post-traumatic amnesia in WW I combatants are surveyed. These cover phenomenology and the psychological dynamics of dissociation vis-à-vis repression. RESULTS: Descriptive evidence is cited for war trauma-induced dissociative amnesia. CONCLUSION: Posttraumatic amnesia extends beyond the experience of sexual and combat trauma and is a protean symptom, which reflects responses to the gamut of traumatic events.

Adult↗
PubMed2003

Local excision of rectal carcinoma not-exceeding the muscularis layer.

AIM: The aim of the study was to compare the results of treatment by local excision of two different clinical stages of the rectal cancer. MATERIAL AND METHODS: Fifty-eight patients with early rectal carcinoma were operated on during the last 26 years using different methods of local excision. The carcinomas were initially assessed as not-exceeding the muscularis layer of the rectal wall. The tumours, localized up to 12 cm from the anal margin, were removed by means of "parachute" excision (47 patients). In 6 patients, carcinoma localized in the central part of rectum, was excised by means of the Localio method. Transanal endoscopic microsurgery was applied in 5 cases of carcinoma localized on the depth of 5-20 cm from the anal margin. RESULTS: After local excision the patients were divided into two groups: I, tumours of low degree of malignancy, not exceeding submucosal layer (26 patients); II, tumours of low or median degree of malignancy with infiltration of muscularis layer (32 patients). There was a significant difference in cancer relapses between groups I and II. One patient in group I and 9 in group II developed local recurrences (P < 0.05) and 5 patients in group II had neoplastic dissemination (15.6%). CONCLUSIONS: Best results were obtained in patients with carcinoma not exceeding submucosal membrane. In cases of rectal muscular layer infiltrations, the risk of carcinoma relapses was markedly higher. The use of transanal endoscopic microsurgery has permitted removal of tumours from the upper rectum.

Adult↗
PubMed1996

Prenatal ultrasonographic diagnosis of fetal hepatic hyperechogenicities: clinical significance and implications for management.

The clinical significance of sonographically detected fetal hepatic hyperechogenicities has not been fully established. The aim of this study was to assess various aspects of fetal hepatic hyperechogenicities detected in utero, including natural history, prenatal investigation and pregnancy outcome. In a retrospective survey of pregnant women presenting to the ultrasonographic unit, five fetuses with a mean gestational age of 20 weeks (range 14-24 weeks) were antenatally diagnosed as having hepatic hyperechogenicities. All cases underwent extensive investigation to establish the etiology and to determine the spectrum of ultrasonographic presentation. In three fetuses, only parenchymal lesions were found, while two had combined lesions: parenchymal and peritoneal. The earliest prenatal ultrasonographic diagnosis was made at 14 weeks' gestation in a fetus with combined hyperechogenic lesions, which, at 12 weeks' gestation, demonstrated unexplained fetal ascites. Associated abnormalities were found in only one fetus in which a solitary parenchymal lesion was associated with direct communication of the intrahepatic portion of the umbilical vein with the right atrium, and high output cardiac failure. In this case, termination of pregnancy confirmed ischemic hepatic necrosis. In the other four cases, no direct etiology could be determined, and all four pregnancies continued to term with normal neonatal follow up until 12 months of age. The present data suggest that in cases of fetal hepatic hyperechogenicities, where apparent intrauterine infection or neoplasia can be ruled out, a vascular disruption phenomenon is a putative etiology. If there are no associated morphological abnormalities or abnormal karyotypes, the prognosis may be favorable.

Adult↗
PubMed1998

Outcome of fetuses with isolated borderline unilateral ventriculomegaly diagnosed at mid-gestation.

OBJECTIVE: To evaluate the outcome of fetuses with isolated borderline, unilateral ventriculomegaly. DESIGN: A retrospective survey was conducted at four perinatal centers in Israel. SUBJECTS AND METHODS: Only fetuses with one ventricular width of > or = 11 mm and the other < 10 mm were included in the study. In all cases, the difference of the ventricular width between the two ventricles was > 2.4 mm (two standard deviations). Fetuses with other malformations, chromosomal abnormalities, or those with evidence of in utero infection, were not included in the study. RESULTS: Unilateral ventriculomegaly was found in 27 subjects (after excluding one case with unilateral ventriculomegaly and Down's syndrome). The mean width of the enlarged ventricle was 11.7 +/- 0.9 mm, while the other normal ventricle was 7.2 +/- 0.9 mm. The mean gestational age at diagnosis of the unilateral ventriculomegaly was 23.6 +/- 2.7 weeks. In one case, pregnancy was terminated, and pathological examination of the fetal brain failed to detect any structural abnormality. Twenty-five patients delivered at term and only one at 34 weeks' gestation. The neurological development in all 25 fetuses was normal, and one fetus had petit mal seizures. CONCLUSION: Fetuses with isolated, borderline unilateral ventriculomegaly, but without other abnormalities, have a good neurological outcome.

Cerebral Ventricles↗
PubMed1999

Screening efficacy of the subcutaneous tissue width/femur length ratio for fetal macrosomia in the non-diabetic pregnancy.

BACKGROUND: Antenatal weight estimations have limited sensitivity and specificity for the detection of macrosomia. The objective of our study was to examine the screening efficacy of the subcutaneous tissue width/femur length ratio for the intrapartum detection of fetal macrosomia in a non-diabetic population at term. STUDY DESIGN: Intrapartum sonographic measurements were performed in 178 well-dated gravidas at 37-41 weeks' gestation with negative glucose tolerance screens. The biparietal diameter, femur length (FL), abdominal circumference and subcutaneous tissue width of the thigh (SCT) were determined. Subsequently, predictions for macrosomia (actual birth weights above the 90th centile) were made using varying cut-off points of the examined parameters or estimated fetal weights. RESULTS: Macrosomia occurred in 27 newborns (15.1%). The SCT/FL ratio was independent of gestational age (r = -0.017). Maternal age, gravidity, parity, gestational age and the ratio of male-to-female infants were similar in pregnancies resulting in appropriate-for-gestational-age and macrosomic infants (NS). There was no difference in the SCT/FL ratio between these groups (p = 0.067; 99% power to detect 2 standard deviation differences). Comparison of screening efficacy by the univariate z score for the area under receiver operating characteristic (ROC) curves (theta) revealed that the abdominal circumference had the best sensitivity-specificity trade-off (theta = 0.8843; p < 0.0001 for comparison with SCT/FL ROC curve), followed by weight estimations based on the Hadlock formula (theta = 0.8773; p < 0.0005), the Shepard formula (theta = 0.8606; p < 0.0001), subcutaneous tissue thickness alone (theta = 0.6872; p < 0.01) and the SCT/FL ratio (theta = 0.6303). CONCLUSIONS: We conclude that the SCT/FL ratio is a poor sonographic predictor of fetal macrosomia in the non-diabetic pregnancy and does not improve fetal weight estimations by conventional sonographic parameters.

Adolescent↗
PubMed1999

Prenatal diagnosis of interrupted inferior vena cava as an isolated finding: a benign vascular malformation.

We report two cases of interrupted inferior vena cava with azygous continuation diagnosed as an isolated finding during routine prenatal scans. Visualization of the venous vasculature of the abdomen and thorax in the mid-sagittal plane failed to visualize the segment of the inferior vena cava between the kidneys and the liver. A vessel with venous flow was observed parallel, adjacent and posterior to the aorta between the kidney and the right atrium. This blood vessel connected with the superior vena cava. Axial planes of the thorax confirmed the presence of two vessels running paravertebrally. A detailed ultrasound examination of the fetal anatomy failed to demonstrate other anomalies. The neonatal course of both fetuses was uneventful. Isolated interruption of the inferior uena cava can be a vascular malformation without known pathological consequences.

Adult↗
PubMed2002

A normal second-trimester ultrasound does not exclude intracranial structural pathology.

OBJECTIVE: To report the prenatal diagnosis and management of 34 fetuses with various intracranial structural pathologies diagnosed following a normal second-trimester ultrasound examination. METHODS: We retrospectively reviewed the images of 203 abnormal central nervous system ultrasound examinations performed between 13 and 37 weeks of gestation at our prenatal diagnosis unit. In 34 (16.7%) of them at least one previous second-trimester ultrasound examination had been performed and considered normal. These 34 fetuses represent the study group. RESULTS: The following intracranial pathologies were diagnosed: dysgenesis of the corpus callosum, ventriculomegaly, cerebral cysts or hemorrhage, migrational disorders, vermian dysgenesis, arachnoid cysts, macrocephaly, enlarged subarachnoid space, brain calcifications and microcephaly. CONCLUSION: A normal second-trimester ultrasound scan does not rule out significant intracranial anomalies. Parents and physicians should be informed about the limitations of second-trimester sonography as far as brain diagnosis is concerned. A repeat third-trimester scan may enable more accurate diagnosis and counseling.

Brain↗
PubMed2002

Congenital periventricular pseudocysts: prenatal sonographic appearance and clinical implications.

OBJECTIVE: Periventricular pseudocysts (PVPC) are diagnosed in approximately 1% of premature newborns that undergo brain sonography during the first 24 h of life. These pseudocysts are thought to develop antenatally due to germinal matrix hemorrhage, but have not been described until now in prenatal ultrasound studies. The aim of this study was to report the identification, differential diagnosis, and prognosis of PVPC detected by prenatal ultrasound examination. DESIGN: Between 1997 and 2001 we made an ultrasound diagnosis of PVPC in 11 fetuses. In nine fetuses the findings were characteristic of PVPC and these patients represent our study group. Fetal magnetic resonance imaging was performed in five patients. Termination of pregnancy was carried out in three patients, in one case intrauterine fetal death occurred at 31 weeks, and one infant died in the neonatal period. The surviving four newborns are being followed in the pediatric neurology clinic. RESULTS: PVPC were diagnosed by ultrasound scan in fetuses between 16 and 37 weeks of gestation (mean, 29.7 weeks). Magnetic resonance imaging confirmed the presence of PVPC in two cases. In eight cases the pseudocysts were unilateral and in one case bilateral. They were an isolated finding in five patients. Four of these fetuses were delivered at term and have normal neurological development at ages ranging from 6 to 25 months. All fetuses with additional pathologies (coarctation of the aorta, hemimegalencephaly, cytomegalovirus infection, hypoplasia of the vermis with dysmorphism) did not survive. CONCLUSIONS: The prenatal diagnosis of PVPC warrants an extensive search for possible associated pathological findings. As an isolated finding, antenatal PVPC seem to carry a good prognosis.

Abortion, Induced↗
PubMed2003

A structured evidence-based review on the meaning of nonorganic physical signs: Waddell signs.

STUDY DESIGN: This is a structured, evidence-based review of all available studies addressing the concept of nonorganic findings: Waddell signs (WSs). OBJECTIVES: To determine what evidence, if any, exists for the various interpretations for the presence of WSs on physical examination. SUMMARY OF BACKGROUND DATA: WSs are a group of eight physical findings divided into five categories, the presence of which has been alleged at times to have the following interpretations: Malingering/secondary gain, hysteria, psychological distress, magnified presentation, abnormal illness behavior, abnormal pain behavior, and somatic amplification. At the present time, there is, therefore, significant confusion as to what these findings mean. METHODS: A computer and manual literature search produced 61 studies and case series reports relating to WSs. These references were reviewed in detail, sorted, and placed into tabular form according to the following subject areas: 1) Reliability (test-retest); 2) Reliability (inter-rater); 3) Reliability (factor analysis); 4) Validity, psychological distress; 5) Validity, correlation Minnesota Multiphasic Pain Inventory (MMPI); 6) Validity, correlation abnormal illness behavior; 7) Validity, other behaviors; 8) Validity, as a nonorganic phenomenon; 9) Validity, correlation pain drawing; 10) Validity, functional performance; 11) Validity, treatment outcome; 12) Validity, predicting surgical treatment outcome; 13) Validity, return to work outcome; 14) Validity, secondary gain correlation; and 15) Validity, pain correlation. Each study in each topic area was classified according to the type of study it represented according to the type of evidence guidelines developed by the Agency for Health Care Policy and Research (AHCPR). In addition, a list of 14 study quality criteria was used to measure the quality of each study. Each study was categorized for each criterion as positive, (criterion filled), negative (criterion not filled), or not applicable independently by two of the authors. A percent quality score was obtained for each study by counting the total number of positives obtained, dividing by 14 minus the total number of not applicables, and multiplying by 100. Only studies having a quality score of 75% or greater were used to formulate the conclusions of this review. The strength and consistency of the evidence represented by the remaining studies in each topic area (above) was then categorized according to the strength and consistency AHCPR guidelines. Conclusions of this review for each topic area are based on these results. RESULTS OF DATA SYNTHESIS: Of the 61 studies, four had quality scores below 75% and were not used to generate the results of this review. According to the AHCPR guidelines for strength and consistency of the reviewed data, the following results were obtained: 1) There was consistent evidence for WSs being associated with decreased functional performance, poor nonsurgical treatment outcome, and greater levels of pain; 2) There was generally consistent evidence for WSs not being associated with psychological distress, abnormal illness behavior, or secondary gain; 3) There was also generally consistent evidence that WSs are an organic phenomenon and that they cannot be used to discriminate organic from nonorganic problems; 4) There was inconsistent evidence that WSs do demonstrate inter-rater reliability, do not correlate with the neurotic triad of the MMPI, are associated with poorer surgical treatment outcome, and are associated with nonreturn to work; 5) There was little or no evidence that WSs demonstrate test-retest reliability, or reliable factors, and are associated with self-esteem problems, catastrophizing, or the nonorganic pain drawing. CONCLUSIONS: Based on the above results, the following conclusions were made: 1) WSs do not correlate with psychological distress; 2) WSs do not discriminate organic from nonorganic problems; 3) WSs may represent an organic phenomenon; 4) WSs are associated with poorer treatment outcome; 5) WSs are associated with greater pain levels; 6) WSs are not associated with secondary gain; and 7) As a group, WS studies demonstrate some methodological problems.

Acute Disease↗