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PubMed1996

Idiopathic dysautonomia treated with intravenous gammaglobulin.

BACKGROUND: A previously healthy 23-year-old man presented with a short history of abdominal pain and diarrhoea followed by blurred vision, severe postural hypotension, reduced sweating and unremitting fever. METHODS: Examination revealed fixed dilated pupils, impaired sweating and postural hypotension. Clinical and neurophysiological examination showed no motor or sensory deficit. A diagnosis of idiopathic autonomic neuropathy was made. He became gravely ill with profound life-threatening hypotension and a prolonged ileus. FINDINGS: Within 36 h of receiving intravenous gammaglobulin (IVGG) his pupillary areflexia and severe hypotension resolved. 2 weeks later the autonomic failure recurred but again responded to treatment with IVGG. IVGG is a recognised treatment for Guillain-Barré syndrome. INTERPRETATION: This case report demonstrates that IVGG is also effective in the rare pure dysautonomic variant.

Adult↗

PubMed1998

"Rhabdoid" meningioma: an aggressive variant.

It is has been suggested that rhabdoid morphology is associated with a poor prognosis, regardless of tumor histogenesis. We report a series of 15 meningiomas with rhabdoid features. Nine patients had undergone multiple resections. In six, the rhabdoid component was histologically apparent only in recurrences. Rhabdoid morphology was defined as sheets of loosely cohesive cells with eccentric nuclei and hyaline, paranuclear inclusions. Ultrastructurally, the latter consisted of whorls of intermediate filaments often entrapping lysosomes or other organelles. Meningothelial features included whorl formation and nuclear pseudoinclusions, immunohistochemical coexpression of vimentin and epithelial membrane antigen, and the ultrastructural finding of interdigitating cell membranes and intercellular junctions. At the histologic level, a conventional meningioma component was noted in most tumors; only four lesions were entirely rhabdoid. Histologic malignancy (brain invasion or anaplasia) was observed in nine cases, another two tumors being considered malignant on the basis of extracranial metastasis. In the majority, increased cell proliferation was evidenced by a high mitotic rate or MIB-1 LI. At last follow-up, 13 patients (87%) had experienced at least one recurrence and 8 (53%) were dead of disease. Median time to death was 5.8 years after initial surgery and 3.1 years after the first appearance of rhabdoid morphology. Our findings corroborate those from a smaller series recently reported by Kepes et al. on the same entity (Kepes JJ, Moral LA, Wilkinson SB, Abdullah A, Llena JF. Rhabdoid transformation of tumor cells in meningiomas: A histologic indication of increased proliferative activity. Report of four cases. Am J Surg Pathol 1998;22:231-8). They further suggest that rhabdoid meningiomas are highly aggressive tumors and that the rhabdoid phenotype represents a marker of malignant transformation in meningiomas.

Adolescent↗

PubMed1995

Two novel antithrombin variants, Asn187Asp and Asn187Lys, indicate a functional role for asparagine 187.

Three unrelated families have been identified with mutations involving asparagine 187. Two of these families are asymptomatic and were identified during the screening of random blood donors, whilst the third has a history of recurrent thromboembolic disease. In two families the mutation (6460 AAC-->GAC) results in an asparagine to aspartate substitution and is associated with normal immunological levels of antithrombin but a reduction in functional activity. In the third family the mutation (6462 AAC-->AAA) results in an asparagine to lysine substitution at residue 187 and is associated with a parallel reduction in both immunological and functional antithrombin levels. Asparagine 187 is located in the middle of the F helix of antithrombin and forms the major link between the F helix and strand 3 of the A sheet. The F helix is seen to overlie the A sheet of the molecule and moves with strands 2 and 3 of this sheet as they open to allow entry of the reactive site loop to form strand 4. Substitutions of asparagine 187 are, therefore, likely to disrupt this sliding movement leading to a loss of inhibitory activity.

Adult↗

PubMed2006

[Effectiveness and safety of clopidogrel bisulfate in complex therapy of patients with acute coronary syndrome with ST segment elevation].

UNLABELLED: Efficacy of clopidogrel in acute myocardial infarction (AMI) was studied only in two trials. However efficacy and safety of loading dose of this drug and its long-term effectiveness were not studied in these trials. AIM: To assess effects of clopidogrel loading dose and long term therapy in addition to standard treatment on death, re-infarction, recurrence of angina and bleedings rate in patients with ST segment elevation acute coronary syndrome. METHODS: Patients (n=107) with AMI who met the criteria for thrombolytic therapy (TLT) were assigned randomly into either clopidogrel group A (n=51) or conventional therapy group B (n=56). Group A received loading dose of clopidogrel (300 mg) in addition to conventional therapy (TLT, aspirin, statin, ACE inhibitor and beta-blocker). Group B received only conventional therapy. The follow-up was 6 months after inclusion during which patients in group A continued to receive clopidogrel (75 mg/day after Day 2 of the study). Primary endpoint included death, re-infarction, recurrence of angina and bleedings. In addition, changes of ST segment after TLT and local contractility were assessed. RESULTS: During 30 days of follow-up rates of primary endpoint were 2.0 and 41.1% in groups A and B, respectively (p=0.003). Subgroup analyses showed that this difference depended on the rate of angina recurrence (2.4 and 36.1% in groups A and B, respectively, p=0.002). These differences were maintained during all follow-up period. Odds ratios for clopidogrel were 0.235 for primary endpoint (95% CI 0.104-0.528, p=0.0003), 0.078 for angina recurrence (95% CI 0.022-0.279, p=0,0001). No significant differences were obtained for mortality, re-infarction and bleeding rate. TLT in group A was more effective. ST depression 90 min after TLT was 86.23+/-4.38 and 61.00+/-6.97% (p=0.010), reperfusion arrhythmia rate - 72.6+/-3.27 and 33.9+/-2.78% (p=0.005) in groups A and B, respectively. CONCLUSION: The use of clopidogrel in addition to standard therapy for AMI is safe and effective. Long-term clopidogrel treatment decreases angina recurrence rate.

Acute Disease↗

PubMed1999

Extraskeletal myxoid chondrosarcoma: a reappraisal of its morphologic spectrum and prognostic factors based on 117 cases.

Extraskeletal myxoid chondrosarcoma (EMC), a phenotypically and genotypically distinctive entity, has generally been viewed as a low-grade sarcoma. No studies regarding clinical and morphologic prognostic factors have been performed on a large series of cases with long-term follow-up because of the rarity and protracted clinical course of EMC. The clinical, morphologic, and immunohistochemical features of 117 previously unreported cases were studied and statistically analyzed. The male-to-female ratio was 2:1. The median patient age was 52 years (range, 6-89 years), and the median tumor size was 7 cm (range, 1.1-25 cm). All tumors occurred within the deep subcutis or deeper soft tissues, with 80% occurring in the proximal extremities or limb girdles and 20% in the trunk. Most initial tumor excisions were intralesional or marginal. Follow-up information was available in 99 cases (median, 9 years: range, 2 months-22 years). Forty-eight patients were disease-free, and 41 patients had evidence of disease (18 of these had died of disease). Ten additional patients survived, but their disease status was unknown. There were local recurrences in 40 (48%) of 83 patients, 23 (58%) of whom had multiple local recurrences. Metastases occurred in 35 (46%) of 76 patients. The estimated 5-, 10-, and 15-year survival rates were 90%, 70%, and 60%, respectively. All cases had histologic features characteristic of classical EMC, at least focally. Cellular foci devoid of myxoid matrix and reminiscent of chondroblastoma, Ewing's sarcoma, monophasic and poorly differentiated synovial sarcoma, fibrosarcoma, and rhabdoid tumor were identified in 29% cases. Older patient age, larger tumor size, and tumor location in the proximal extremity or limb girdle were adverse prognostic factors identified by multivariate analysis. Metastasis also adversely affected survival, although local recurrence did not. This study shows that EMC has a unique clinical course, including a high rate of local recurrence, prolonged survival after metastasis in some cases, and eventually a high rate of death due to tumor. These features distinguish EMC from low-grade sarcomas. This study shows that histologic grading is of no prognostic value in EMC because prognosis is dictated primarily by certain clinical features. Histologic recognition of classical EMC and cellular and solid, nonmyxoid variants is important, however, in view of EMC's distinctive biologic behavior.

Adolescent↗

PubMed2002

Macular serpiginous choroiditis.

PURPOSE: To report a variant form of serpiginous choroiditis, that initially or predominantly involved the macular area. METHODS: Nine eyes of 6 patients with the macular form of serpiginous choroiditis were evaluated clinically and angiographically in a longitudinal fashion for a period of 12-36 months. The active stage and the recurrences were treated by oral and periocular cortico steroids; and two patients were supplemented with oral azathioprine. Most of these patients were referred to our center with varied diagnoses. RESULTS: In this group, 4 were male and 2 were female with an average age of 30.5 years. Three patients had bilateral macular lesions, two had typical serpiginous choroiditis in the fellow eye and the remaining one had unilateral macular involvement alone. The initial visual acuity was 6/60 or less in 60% eyes whereas the final visual acuity was 6/18 or better in 66% eyes. Angiographic findings were typical of serpiginous choroiditis characterised by early hypofluorescence followed by leakage and staining of the borders and the lesion itself without any evidence of choroidal ischaemia or retinal vascular abnormalities. CONCLUSION: The macular variant of serpiginous choroiditis can mimic many other macular pathologic lesions, thus posing a diagnostic dilemma. Because of its relentless destructive course, early diagnosis and prompt treatment is required to prevent sight-threatening complications.

Adolescent↗

PubMed2004

Ectopic hamartomatous thymoma: a clinicopathologic and immunohistochemical analysis of 21 cases with data supporting reclassification as a branchial anlage mixed tumor.

This report describes the clinicopathologic and immunohistochemical findings in 21 cases of a highly distinctive tumor with a strong predilection for the lower neck region of adult males. Our study group consisted of 20 males and one female. The patients were 28 to 79 years old (mean age, 47 years; median age, 40 years), and they presented with solitary, lobular or multilobular masses ranging in size from 2.0 to 19.0 cm in greatest dimension (mean size, 5.1 cm; median, 4 cm). The tumors principally involved the lower neck region, usually in close proximity to the sternoclavicular joint. The preoperative duration of the lesions ranged from 2 months to 30 years. Histologically, the tumors were typically well marginated and composed of plump spindled cells, delicate spindled cells, mature adipose tissue, and epithelial cells, including both squamous and glandular elements. Epithelial-lined cysts were a focal finding in most cases and measured up to 2 cm in greatest dimension. Mitotic counts for the tumors ranged from 0 to 7 mitotic figures per 50 high power fields (mean mitotic count, 1.1 mitotic figures per 50 HPFs). Our immunohistochemical analysis revealed a complex immunophenotype with a diverse keratin profile. The plump spindled cells had a myoepithelial phenotype, as evidenced by the coexpression of keratins (5, 5/6, and 14), alpha-smooth muscle actin, CD10, and to a lesser extent, calponin. No compelling evidence for thymic differentiation was observed. The patients were initially managed by biopsy or partial resection (n = 4), simple local excision (n = 16), or an unspecified procedure (n = 1). Clinical follow-up of > or =3 years was available for 10 patients (48%). Two patients had recurrent disease, but there were no metastases or tumor-related deaths. A derivation from sequestered branchial epithelium is likely, but evidence for a thymic component is tenuous, at best. Our data support reclassification of this distinctive process as a branchial anlage mixed tumor. The differential diagnosis includes conventional mixed tumors of skin adnexal or salivary gland origin, synovial sarcoma, a peripheral nerve sheath tumor variant, and cystic teratoma.

Adult↗

PubMed1985

Desmoplastic melanoma of the anus.

To date a desmoplastic melanoma, an unusual variant of malignant melanoma, has been described only in the skin. The present report describes a case of desmoplastic melanoma that arose in the anal canal. The patient was without evidence of recurrence or metastases 18 months after surgical removal of the lesion.

Anal Canal↗

PubMed1997

[The placement of a Wiktor stent for the treatment of vasospastic angina: a case report].

A 46-year-old male with a fixed stenosis in the mid-segment of the left anterior descending artery underwent balloon angioplasty. The procedure included the placement of two Wiktor stents because of severe dissection. Five months later he complained of Prinzmetal angina with ST elevation in the anterior wall. A metilergobasine test during the coronary arteriogram showed a discrete, severe spasm on the proximal segment of the left anterior descending artery. Because of a lack of symptomatic improvement with high-dose nitrates and calcium blockers, a Wiktor coronary stent was successfully implanted in the proximal left anterior descending artery, resulting in complete relief of the angina.

Angina Pectoris, Variant↗

PubMed1998

Ovarian cancer gene therapy.

Retroviral-mediated delivery of BRCA1 gene therapy (LXN-BRCA1sv, a normal splice variant form of BRCA1) was tested extensively in mouse models. It was found to be effective in reducing tumor burden and to be minimally toxic. Twelve phase I clinical trial patients with recurrent or persistent epithelial ovarian cancer were treated with one to three cycles of intraperitoneal vector. There was minimal toxicity, four patients developed fevers (< 102.5 degrees F) and three had sterile peritonitis, which resolved within 48 hours. The vector was found to be fairly stable in some patients at 24 hours as well as transferred into and expressed in patient tissues. Stable disease was noticed in 8 of the 12 patients, suggesting that the peritoneal cavity may be an appropriate site for gene therapy.

Animals↗

PubMed2001

MDR1 gene-related clonal selection and P-glycoprotein function and expression in relapsed or refractory acute myeloid leukemia.

The expression of P-glycoprotein (P-gp), encoded by the MDR1 gene, is an independent adverse prognostic factor for response and survival in de novo acute myeloid leukemia (AML). Little is known about MDR1 expression during the development of disease. The present study investigated whether MDR1 gene- related clonal selection occurs in the development from diagnosis to relapsed AML, using a genetic polymorphism of the MDR1 gene at position 2677. Expression and function of P-gp were studied using monoclonal antibodies MRK16 and UIC2 and the Rhodamine 123 retention assay with or without PSC 833. No difference was found in the levels of P-gp function and expression between diagnosis and relapse in purified paired blast samples from 30 patients with AML. Thirteen patients were homozygous for the genetic polymorphism of MDR1 (n = 7 for guanine, n = 6 for thymidine), whereas 17 patients were heterozygous (GT). In the heterozygous patients, no selective loss of one allele was observed at relapse. Homozygosity for the MDR1 gene (GG or TT) was associated with shorter relapse-free intervals (P =.002) and poor survival rates (P =.02), compared with heterozygous patients. No difference was found in P-gp expression or function in patients with AML with either of the allelic variants of the MDR1 gene. It was concluded that P-gp function or expression is not upregulated at relapse/refractory disease and expression of one of the allelic variants is not associated with altered P-gp expression or function in AML, consistent with the fact that MDR1 gene-related clonal selection does not occur when AML evolves to recurrent disease. (Blood. 2001;97:3605-3611)

ATP Binding Cassette Transporter, Subfamily B, Mem↗

PubMed1990

Meckel-Gruber syndrome. Importance of prenatal diagnosis.

Prenatal sonographic findings are reported in six fetuses with the Meckel-Gruber syndrome to illustrate the variety of sonographic findings associated with this disorder and to emphasize the importance of prenatal sonography in helping to establish the correct diagnosis. All six fetuses demonstrated evidence of renal cystic dysplasia. In five cases the kidneys were large and echogenic, demonstrating small discrete cysts in the range of 2 to 5 mm. The remaining fetus demonstrated unilateral renal cystic dysplasia and contralateral renal agenesis. Oligohydramnios was noted in all cases and was evident as early as 14 weeks. An occipital cephalocele was demonstrated on sonography in each case although the size and contents of the cephalocele varied significantly. Two fetuses, both in the same family, also demonstrated a cystic mass in the posterior fossa and partial absence of the cerebellum consistent with a Dandy-Walker variant or cerebellar hypoplasia. The concurrence of marked oligohydramnios and bilateral severe renal anomalies should initiate a search for anomalies of the central nervous system indicative of the Meckel-Gruber syndrome. Recurrence of Meckel-Gruber syndrome may be evaluated as soon as 14 weeks, but it may not be reliably excluded until 20 weeks.

Abnormalities, Multiple↗

PubMed2002

[Cyclothymic obsessive-compulsive disorder. Clinical characteristics of a neglected and under-recognized entity].

OBJECTIVE: Clinical research is largely focused on depressive comorbidity in obsessional compulsive disorder (OCD). However some recent publications have suggested that bipolar comorbidity occurs in authentic OCD and its presence has a differential impact on the clinical picture and course of OCD. METHOD: Recent data from the collaborative survey conducted with AFTOC (French Association of patients suffering from OCD) have revealed a high rate of bipolar comorbidity in OCD: 30% for hypomania and 50% for cyclothymia. RESULTS: The present paper presents further comparative analyses between OCD with (n = 302) versus without cyclothymia (n = 272). The sub-group "Cyclothymic OCD" is characterized by a different clinical picture (higher frequency of aggressive, impulsive, religious and sexual obsessions, and compulsions of control, hoarding, repetition), episodic course, higher rate of major depressive episodes (with more intensity and recurrence) associated with higher rates of suicide attempts and psychiatric admissions, and less favorable response to anti-OCD treatments. CONCLUSION: These data suggested that cyclothymic OCD could represent a specific distinct variant form of OCD. More vigilance is needed toward this entity which is largely under-recognized in clinical practice.

Adolescent↗

PubMed1991

[Treatment of essential trigeminal neuralgia by Gasserian compression using balloon (50 cases)].

Between 1985 and 1989, 50 patients with trigeminal neuralgia were submitted for percutaneous gasserian ganglion compression, the procedure is the technic described by Mullan with some variants. The Fogarty catheter is introduced through a teflon catheter. The balloon is inflated to 0.5 cc during 8 minutes. So 46 patients were relieved of their pain; there were 7 (14%) recurrence for an interval of between 6 and 54 months (average 30 months). Dysesthesias were noted in two patients when the duration of compression was more prolonged. Two patients had transient oculomotor nerve palsy. The success rate after gasserian compression is similar to this obtained in the other percutaneous procedures. Despite the general anesthesia with intubation which is useful, the procedure is technically very easy and can be performed by a neurosurgeon who has not a long experience.

Adult↗

PubMed1990

[Gallopamil infusion for treatment of Prinzmetal angina].

The efficacy of a new calcium channel blocker, gallopamil, has been tested via a single blind, self-controlled versus placebo protocol in 9 consecutive patients admitted to our Coronary Care Unit because of repeated daily attacks of Prinzmetal variant angina. Exclusion criteria were age (greater than 65 years) bradycardia (less than 50 beats/min), recent myocardial infarction, heart failure, sinoatrial or atrioventricular block. After a 24 hours run-in period on saline drip, gallopamil was administered as 0.03 mg/kg bolus followed by continuous infusion at 0.02 mg/kg/h for the first 24 hours and 0.03 mg/kg/h for the last 48 hours. Treatment was then stopped and the patients were again kept on saline infusion for the next 30 hours. Holter monitoring was recorded during run-in, on third day of treatment and 6 hours after gallopamil withdrawal. Anginal attacks were significantly reduced in number by therapy (-63%, -91%, -84% in the 3 days of treatment). Holter monitoring during gallopamil infusion showed a statistically significant reduction in silent (-98%) and symptomatic (-93%) ischemic episodes (IE). During the last 24 hours of the washout period we observed a statistically significant increase in silent ischemic episodes. While no transient ST segment elevation was recorded in 3 patients, in 1 patient symptomatic IE were increased of 150% with respect to the run-in period. On the whole we observed complete suppression of IE in 7 patients (78%) at the third day of treatment with gallopamil. In 2 patients (22%) a greater than 75% reduction was observed.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

PubMed1989

[Dynamics and outcome of neuroses].

Based on the data obtained during examination of 320 neurotic patients the grouping of the varieties of the time-course of changes in neurotic disorders has been suggested. Variants of the lingering neuroses have been distinguished with regard to the presence or lack of the tendency towards progression and exacerbation of the symptomatology. Single attacks of neurotic disorders, repeated and recurrent neuroses were delineated within the framework of attack-like dynamics. Outcomes of neurotic disorders have been systematized.

Chronic Disease↗

PubMed1985

Impetigo herpetiformis and hypoparathyroidism.

A 64-year-old woman had recurrent bouts of impetigo herpetiformis triggered by episodes of hypocalcemia secondary to postsurgical hypoparathyroidism. Correction of the hypocalcemia resulted in rapid clearing of her skin disease. Impetigo herpetiformis should be considered a variant of generalized pustular psoriasis. Although best known for its association with pregnancy, this disease should also be considered an important cutaneous manifestation of the postthyroidectomy syndrome.

Calcium↗

PubMed1984

Verrucous carcinoma--changing concepts in management.

Verrucous carcinoma is an unusual, non-metastasizing, distinct variant of a well differentiated squamous cell carcinoma. Multiple biopsies are frequently necessary to establish its diagnosis. Seven of 10 patients treated with irradiation showed no evidence of anaplastic transformation. A high recurrence rate in tumors of the oral cavity treated with either irradiation or surgery alone is noted. Extensive lesions in this area may benefit from combined therapy. A good response to radiotherapy with a low recurrence rate in small laryngeal lesions is recognized. Irradiation should be considered in the management of this tumor without undue concern for change in its biologic behavior.

Aged↗