Aortic thrombosis in a neonate with hereditary antithrombin III deficiency: successful outcome with thrombolytic and replacement treatment.

We report the case of a newborn male who presented an aortic thrombosis during the neonatal period and was subsequently diagnosed as having antithrombin III (AT III) hereditary deficiency type I. This hypercoagulable condition is known to predispose young adults to venous thrombosis, but in our patient the primary thrombotic incident affected the arterial vessels within the first few days of life. Combined treatment with thrombolytic agents and AT III concentrates recovered aortic permeability, suggesting that the use of AT III may be beneficial for the treatment of thrombotic complications during the first few days of life.

Cost containment in cardiac surgery: results with a critical pathway for coronary bypass surgery at the New York hospital-Cornell Medical Center.

Purpose: A multidisciplinary project was undertaken at The New York Hospital-Cornell Medical Center to develop critical pathways for open-heart surgery to help reduce cost, shorten hospital length of stay (LOS), and streamline patient care.

Methods: A critical pathway for elective coronary artery bypass grafting instituted on March 1, 1995, was developed through a cooperative effort involving surgeons, anesthesiologists, nurses, social workers, physical therapists, nutritionists, and patient case managers. Prospective data collected on consecutive patients forming a critical pathway group (n = 114) over a 6-month period were compared with retrospective data on consecutive patients forming a cohort group (n = 382) who underwent elective coronary artery bypass grafting in 1994.

Sleep-epilepsy interactions in patients with intractable generalized tonic seizures and depth electrodes in the centro median thalamic nucleus.

Sleep-epilepsy interactions were studied in ten patients with Lennox-Gastaut Syndrome (LGS) suffering intractable generalized tonic seizures associated with generalized ictal fast spikes and interictal slow spike-wave complexes, where electrodes were implanted in the centromedian thalamic nucleus (CM) as part of a neuroaugmentive procedure for seizure control. In these patients, continuous all night recordings according to International Guidelines were performed to quantitatively determine changes in sleep patterns produced by the epileptic condition and changes in ictal and interictal surface and CM EEG activities produced by sleep stages. LGS patients showed significantly longer wakefulness (W) and shorter slow wave sleep (SWS) II than normals.

Persistence of the typical spike-wave EEG pattern after surgical excision of a temporal lobe astrocytoma and apical lobectomy.

This is a report on a patient with intractable 'primary' generalized seizures and typical spike-wave EEG patterns, in whom an unexpected temporal lobe astrocytoma was detected by MRI studies. Clinical and electrophysiological studies were performed before and after surgical excision of the tumor and apical temporal lobectomy in an attempt to determine whether 'primary' generalized seizures and EEG patterns and the temporal lobe tumor were only coincident neurological disorders or were indeed related. Before resection, the patient consistently showed a typical spike-wave EEG pattern with no background and paroxysmal activities suggestive of 'secondary' bilateral synchrony in 10 consecutive conventional EEG recordings; neither spontaneous interictal nor ictal ECoG activities suggested focal temporal lobe epileptogenesis.

Electrical stimulation of the centromedian thalamic nucleus in control of seizures: long-term studies.

Five patients with chronic incapacitating seizures averaging 15-5,000/month were selected for study. All patients had more than one seizure type and had received maximal doses of antiepileptic drugs (AEDs). The centromedian thalamic nucleus (CM) was stimulated electrically through bilateral multicontact platinum electrodes stereotaxically placed in CM and connected to internalized pulse generators.

Neurotransmitter levels in cerebrospinal fluid in relation to severity of symptoms and response to medical therapy in Parkinson's disease.

Determinations of biopterin (BP), homovanilic acid (HVA), glutamic acid (GTA), and glutamine (GT) levels in cerebrospinal fluid (CSF) obtained through a lumbar tap were performed in 20 parkinsonian patients in different stages of evolution and without medication. In patients with motor symptoms not related to Parkinson's disease (dystonia, dyskinesia and essential tremor) (n = 4). In 7 other neurological patients subjected to spinal tap for diagnostic procedures neurotransmitters were also determined and taken as control groups.

Long-term effects of medical and surgical treatments on Parkinson's disease.

Three groups of patients with Parkinson's disease (PD) were studied: (1) PD patients treated medically with L-Dopa and anticholinergics, (2) PD patients treated with thalamo-subthalamotomies plus medical treatment, and (3) PD patients treated with autologous adrenal medullary transplantation to right caudate nucleus plus medical treatment. Evaluations prior and 1, 6 and 12 months after treatment were accomplished with PD scales (NYPDS, UPDS, Schwab and England and Hoehn and Yahr scales). No attempt was made to compare one PD after treatment in each group.

Effect of chronic electrical stimulation of the centromedian thalamic nuclei on various intractable seizure patterns: II. Psychological performance and background EEG activity.

We studied the effect of electrical stimulation of centromedian thalamic nuclei (ESCM) on seizure control and paroxysmal EEG activity in 23 patients. We report the effect of chronic ESCM on psychological performance and background EEG activity of patients with various intractable seizure patterns. In each patient, a simple specifically designed neuropsychological scales (one for adults and one for children) was administered at the end of the baseline (BL), ESCM, and poststimulation (Post) periods; and 14 consecutive EEG recordings during these periods were performed to determine the degree of neuropsychological improvement and the temporal course of EEG changes.

Effect of chronic electrical stimulation of the centromedian thalamic nuclei on various intractable seizure patterns: I. Clinical seizures and paroxysmal EEG activity.

Twenty-three patients with various intractable seizure patterns were divided into four groups based on their most frequent seizure type and their clinical and EEG response to chronic electrical stimulation of the centromedian thalamic nuclei (ESCM): group A, generalized tonic-clonic (GTC, n = 9); group B, partial motor (Rasmussen type) (n = 3); group C, complex partial seizures (CPS, n = 5); and group D, generalized tonic seizures (Lennox-Gastaut type) (n = 6). CM were radiologically and electrophysiologically localized by means of stereotaxic landmarks and by thalamically induced scalp recruiting-like responses and desynchronization. ESCM consisted of daily 2-h stimulation sessions for 3 months.

Meningioma-like intracranial granulocytic sarcoma (chloroma). Radiologic and surgical findings.

We present the case of a 23 years old female with chronic granulocytic leukemia and two intracranial tumors with all the radiological characteristics of intracranial meningiomas. Successful removal of the largest tumor was accomplished using the technique described for meningiomas. The recovery was very good and the histopathologic diagnosis was of granulocytic sarcoma.

Endotoxin-induced pulmonary dysfunction is prevented by C1-esterase inhibitor.

In septic shock, hypotension, disseminated intravascular coagulation, and neutrophil activation are related to the activation of the blood coagulation contact system. This study evaluates in dogs the effect of the C1-esterase inhibitor (C1-INH), a main inhibitor of the blood coagulation contact system, on the cardiovascular and respiratory dysfunction associated with endotoxic shock. Two groups were included: controls, which received Escherichia coli endotoxin, and a C1-INH group in which C1-INH was infused before E.

Fibrinolytic activity during hemodialysis: a biocompatibility-related phenomenon.

According to recent reports, fibrinolytic activity may be enhanced during hemodialysis. The aim of this work was to study the fibrinolytic activity in uremic patients, and to evaluate the effect of membrane biocompatibility on the fibrinolytic system during hemodialysis. Tissue plasminogen activator (t-PA), t-PA antigen, plasminogen activator-inhibitor (PAI), plasminogen (PL) and alpha-2-antiplasmin (AP) were measured in 10 uremic patients on maintenance hemodialysis treated sequentially with Cuprophane and polyacrylonitrile (AN69) membranes.

Coagulation, fibrinolytic and kallikrein systems in neonates with uncomplicated sepsis and septic shock.

This study evaluates the contact system, coagulation inhibitors and fibrinolysis in 23 full-term newborns with sepsis (8 with septic shock). The results were compared with a group of 20 healthy newborns. Blood samples were obtained at the time of clinical diagnosis and 3 days after the antibiotic therapy was started.

Serum TNF levels in neonatal sepsis and septic shock.

Tumor necrosis factor (TNF-alpha) has been implicated as a principal mediator in the pathogenesis of septic shock. TNF-alpha was measured by immunoradiometric assay in serum samples from 23 full-term infants with sepsis (15 with severe infection and 8 with septic shock) and in 20 healthy full-term newborns. Serum TNF-alpha levels were significantly higher in the group with sepsis, at the time of admission to the neonatal intensive care unit, than in the healthy neonates.

Electroencephalographic and magnetic resonance correlations in children with intractable seizures of Lennox-Gastaut syndrome and epilepsia partialis continua.

A study was performed of EEG-magnetic resonance imaging (MRI) abnormalities in 7 Lennox-Gastaut syndrome (LGS) children and 3 epilepsia partialis continua (EPC) children with intractable generalized and partial motor seizures, respectively. In 4 children with LGS and 3 children with EPC, depth electrodes were implanted in the centromedian thalamic nuclei (CM) for seizure control. In all children with LGS, hyperdense, T2-weighted MRI signals were observed at the mesencephalic level of the brainstem, whereas none of the 3 children with EPC had this finding.

ACTH secreting pituitary microadenomas disclosed only by gadolinium DTPA enhanced MRI.

Purpose: To assess the usefulness of Gd-DTPA enhancement in disclosing microadenomas of the pituitary gland that had not been demonstrated by non enhanced MRI in patients with Cushing's disease.

Methods: Six patients with Cushing's disease underwent pre and post enhancement MRI scanning and the findings were correlated at surgery and confirmed by histological and immunohistochemistry studies of the surgical specimen.

Results: In all cases post enhancement MR scans demonstrated circumscribed hypointense foci that corresponded well with the location and extension of the adenomatous tissue found at surgery.

Role of the centromedian thalamic nucleus in the genesis, propagation and arrest of epileptic activity. An electrophysiological study in man.

Twenty-eight patients underwent electrical stimulation of the centromedian (CM) thalamic nucleus as a neuro-augmentative procedure to control intractable seizures of various types. To assess the correct placement of electrodes, electrical stimulation at 3 and 6 Hz, 1.0 msec and 600-2000 uA pulses in trains of 30-60 sec were used while the EEG was recorded by scalp electrodes.

Retroperitoneal Castleman's disease.

A case of Castleman's disease localized in the retroperitoneal space is reported. A 29-year-old patient had a mass 15 cm in diameter with radial calcification. After surgical resection, both the patient's anemia and hypergammaglobulinemia disappeared.

[A genetic and hormonal study of 5 patients with the nonclassical form of congenital adrenal hyperplasia due to 21-hydroxylase deficiency].

Background: To study the association between the 21-hydroxylase deficiency with the HLA histocompatibility complex in a mediterranean ethnic group.

Methods: 5 patients with late-onset 21-hydroxylase deficiency, diagnosed on the basis of a high plasma level of 17-hydroxyprogesterone, along with 23 family members were typed. 17-hydroxyprogesterone response to iv ACTH stimulus was measured too in the family members.

Diminished anticoagulant and fibrinolytic activity following liver transplantation.

This study analyzed the coagulation changes in twenty patients after orthotopic liver transplantation. The procoagulant, anticoagulant, and fibrinolytic systems were studied during the first two postoperative weeks. Within the first postoperative day all extrinsic and intrinsic pathway factors became normal except factors IX, VII, and X, which recovered within the next 24 hr.

[Inflammatory pseudotumor of the liver].

Inflammatory pseudotumor is a pathological process whose cause is unknown, and whose macroscopic appearance is that of a malignant tumor, but it is in fact of inflammatory nature. Inflammatory pseudotumor of the liver is infrequent, but must be taken into account in the differential diagnosis of liver masses. A case report is presented, with review of the literature.

Increase in the D-dimer levels during treatment in patients with acute myelogenous leukemia.

Plasma concentration of thrombin-antithrombin III complex (TAT), tissue-type plasminogen activator (t-PA), plasminogen activator inhibitor 1 (PAI-1), PAI-2, D-dimer complex and urokinase-plasminogen activator (u-PA) activity were studied in 30 patients with acute nonlymphoblastic leukemia (ANLL), before and during antileukemic therapy. Fifteen patients showed signs of disseminated intravascular coagulation (DIC), 10 of them classified as M3, 2 as M2 and 3 as M5 subtypes. The initial levels of TAT complex were elevated in all ANLL patients.

Protein C, protein S and C4b-binding protein in neonatal severe infection and septic shock.

We have studied the behaviour of total protein S, free protein S, protein C and C4b-binding protein fifteen neonates with severe infections, eight with septic shock and in a group of ten healthy newborns. Protein C was decreased in shock and septic patients, but only the shock group showed significant differences compared to normal neonates. Total protein S was normal in both groups of patients, although free protein S had significantly lower values in shock and nonshock infants.