Focal dermal hypoplasia with uterus bicornis and renal ectopia: case report and review of the literature.

Focal dermal hypoplasia (FDH) is a rare inherited genodermatosis with an X-linked dominant trait. FDH is associated with skin defects and other abnormalities of bone, nails, hair, limbs, teeth and eyes. We present the case of a 26-year-old female in the 27th pregnancy week and a previous history of miscarriage.

[Clinical and pathological correlation in fatal dengue cases found in Maracaibo, Venezuela].

Introduction: Given the importance of dengue in America, it is indispensable to know the cause of death of the infected people and to evaluate the few necropsy studies available.

Objective: To describe and to analyze the relevant clinical and pathological findings in fatal dengue cases of dengue in a Venezuelan endemic area.

Methods: Eight fatal cases, according to clinical and epidemiological dengue criteria and/or confirmed by complete serological tests were studied after complete clinical necropsy.

Ultrastructural cutaneous microvascular pathology of young adults aged up to 50 years with chronic kidney disease and vascular cognitive impairment.

Background: Studies suggest a link between chronic kidney disease and cognitive impairment. Skin biopsy is hire in the study of leucoaraiosis since permit establish the responsible vascular pathology of subcortical white matter changes because the pathological hallmark is systemic. The aim was to study the ultrastructural changes of cutaneous small vessel of patients aged up to 50 years with chronic kidney disease (CKD) and vascular cognitive impairment (VCI).

Electron microscopy morphology of the mitochondrial network in human cancer.

Mitochondria have been implicated in the process of carcinogenesis, which includes alterations of cellular metabolism and cell death pathways. The aim of this review is to describe and analyze the electron microscopy morphology of the mitochondrial network in human cancer. The structural mitochondrial alterations in human tumors are heterogeneous and not specific for any neoplasm.

Importance and repercussions of renal and cardiovascular pathology on stroke in young adults: an anatomopathologic study of 52 clinical necropsies.

Introduction: Stroke in young adults has seldom been studied in a necropsy series. The objective of the present clinical necropsy-based investigation was to analyze stroke and its relationship with cardiovascular and renal pathology in young adults.

Materials And Methods: The protocols of 52 clinical necropsies with diagnoses of stroke in patients aged 18 - 49 years, performed between the years 1990-2006, were reviewed.

Primary cerebral fibrosarcoma in a child.

Objective: To describe the case of a primary cerebral fibrosarcoma in a child.

Clinical Case: A 6-year-old boy presented with a history of headache, drowsiness, vomiting and seizures. He was referred to our institution and died shortly upon arrival to the ER.

[Frequency and clinical implications of lupus anticoagulant in patients with terminal chronic renal failure in hemodialysis].

The objective of the present study was to determine the frequency of lupus anticoagulant (LA), in patients with terminal chronic renal failure (TCRF), and its association with thrombotic events. Sixty three patients were separated into two groups: Group A, consisted of 32 patients under treatment with hemodialysis, and Group B was formed of 31 patients who were treated in a conservative manner. Presence of LA was found in 4 patients from Group A and none from Group B.

[Primary amebic meningoencephalitis: two new cases report from Venezuela].

Primary amebic menigoencephalitis (PAM) is rare. Two cases of PAM in Venezuelan patients are described. Case 1, a 10 year-old male with headache, fever, vomiting.

[Cerebrovascular disease: its relationship with cardiovascular and renal pathology in 134 clinical autopsies].

Objective: To establish the relationship between cardiopathy and chronic nephropathy, and cerebral vascular pathology in clinical necropsies performed in adult patients.

Material And Methods: The protocols of 861 clinical autopsies done during the lapse 1990-2000 were reviewed, of these, 134 cases with diagnosis of cerebrovascular disease (CVD) were selected. Analyzed features included: neuropathological study of CVD, renal and cardiovascular pathology, and cause of death.

[Acute non-specific splenitis as indicator of systemic infection. Assessment of 71 autopsy cases].

Classic pathology textbooks claim that acute splenitis reflects septic states, nevertheless, the evidence upon which that association is based remains unclear. We assessed the occurrence of acute splenitis, as an indicator of systemic infection, in 34 autopsies performed in patients in whom the cause of death was due to multiple organ dysfunction syndrome, secondary to sepsis (group A); and in 37 cases of death by non-infectious causes (group B). These necropsies were done during the period January 31, 1999-December 31, 2000, at the Pathology Department of the Hospital General del Sur "Dr.

[Non neoplastic space occupying lesions mimicking central nervous system tumors].

Objective: To establish the frequency and nature of space occupying lesions of the central nervous system (CNS) that mimic neoplastic growths.

Patients And Methods: We reviewed the clinical records, imaging and neuropathological studies of patients operated in different hospitals of Maracaibo, Venezuela during the period January 1 1996 July 31 2002. These patients had a pre operative diagnosis of CNS tumor, and their definitive diagnosis was non neoplastic disease.

[Epidemiologic studies of cerebellopontine angle tumors surgically treated in Maracaibo, Venezuela, in 1985-1999].

Objective: To analyze the epidemiological, clinical and neuropathological data of cases of cerebellopontine angle (CPA) tumors.

Material And Methods: The clinical records, neuroimaging and neuropathological studies of 50 patients with diagnosis of CPA tumor operated in different hospitals of Maracaibo, Venezuela, during the lapse January 1st, 1985-December 31, 1999 were reviewed. The variables age, gender, side of the lesion and neuropathological diagnosis were analyzed.

[Clinical and tomographic aspects of hemorrhagic cerebrovascular disease associated with hypertensive crisis in adults under 50 years of age].

The purpose of this study was to analyze both the clinical and tomographic aspects of the hemorrhagic cerebrovascular disease (HCd), associated with hypertensive crisis in adults under 50 years of age. Forty six patients, who were not under anticoagulant therapy, were not using illegal drugs, who had not a cerebral tumor disease, and who had neither arteriovenous malformations nor past traumatic episodes, were studied. Seventy eight percent of the patients had preexisted arterial hypertension, 30% of them had at least a previous emergency for a hypertensive crisis.

[Lupus anticoagulant in preeclampsia and non-proteinuria gestational hypertension in primiparous women].

The purpose of this study was to determine the relationship between the lupus anticoagulant and the proteinuric and non-proteinuric Gestational Hypertension in primigravids without subjacent pathology. Sixty- five patients with a single gestation of twenty or more weeks long were studied. Thirty four patients (Group A) were pregnant women with a normal blood pressure.

[The classical clinical and neuroimaging features of meningiomas are mimicked by other intracranial, supratentorial expansive lesions].

Objective: To analyze the surgical neurology cases in which both the clinical and neuroimaging features suggested supratentorial meningioma, and resulted in different entities from the neuropathological point of view.

Patients And Methods: The clinical histories of patients harboring intracranial, supratentorial mass lesions diagnosed as meningioma operated in different hospitals of Maracaibo, Venezuela, during the period 1993-1997 were reviewed.

Results: Our analysis revealed 15 cases with different neuropathological diagnosis.

Solitary fibrous tumor of the meninges.

Intracranial fibrous tumors are unusual growths that occur almost exclusively in young individuals. Most of these lesions are intra-axial, however, on extremely rare occasions they arise from the leptomeninges and compress the adjacent brain. The authors report the case of a benign solitary fibrous tumor of the meninges in a 14-year-old girl who presented with a 5-month history of generalized headaches.

[Malondialdehyde and nitric oxide behaviour in patients with myocardial infarction].

Introduction And Objectives: The aim of this study was to determine the potential usefulness of malondialdehyde and nitric oxide as sensors of metabolic damage produced during acute coronary ischaemics events.

Methods: Serum malondialdehyde and nitric oxide levels were determined as thiobarbituric acid derivative and nitrites respectively in 15 male patients who were admitted to the emergency ward of the Hospital General del Sur de Maracaibo, because of acute stage of myocardial infarction.

Results: Our results show, upon follow-up and afterwards 30 days a highly significant increase in the malondialdehyde level during the acute phase of myocardial infarction (1.

Cholecystectomy via Kocher incision without peritoneal closure.

This investigation was carried out to evaluate the surgical wound outcome of patients who underwent cholecystectomy via Kocher incision, without peritoneal closure. Consecutive patients (n = 129) were randomized either to have the peritoneal closure (n = 66) or to have this step omitted (n = 63). Demographic data of the patients were similar in both groups.

[Surgical treatment of congenital cysts of the liver].

We report 9 patients who were surgically treated for symptomatic congenital liver cysts. Median age at presentation was 51 years and all the cases were females. Symptoms and clinical findings included a painful mass in the upper right quadrant of the abdomen.

[Intraventricular oligodendroglioma. Description of 2 cases].

Oligodendrogliomas usually arise in the cerebral hemispheres, less frequently they are found in the cerebellar hemispheres and very rarely they adopt an intraventricular location. The authors describe two cases of intraventricular oligodendroglioma. Case No.

[Gliomatosis cerebri. Ante-mortem diagnosis].

Gliomatosis Cerebri is an unusual neoplastic entity that affects the central nervous system (C.N.S.