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Biomedical subjects

G Jacobi

Publications and source records attributed to G Jacobi.

At least 19 recordsLinked to original sources

Desmoplastic supratentorial neuroepithelial tumours of infancy.

The clinicopathological features of two infants with desmoplastic supratentorial neuroepithelial tumours are described. The cases were similar to the 26 cases reported previously as desmoplastic infantile ganglioglioma and superficial cerebral astrocytoma attached to dura. Neuronal differentiation was absent on routine stains, but was immunohistochemically established. We review the literature and suggest classifying this clinicopathological entity by using a general designation such as desmoplastic supratentorial neuroepithelial tumours of infancy, a term which indicates the variability in the amount and lines of differentiation.

Astrocytoma

EEG and evoked potentials in HIV-infected children.

Forty-seven HIV-seropositive children were investigated by EEG and evoked potentials (BAEP, SEP). Twenty-three children were symptomatic (P2), 8 seropositive without symptoms (P1), and 16 children were less than 15 months of age (P0). Some of them were investigated at different stages of HIV infection. During the neonatal period, 7 newborns of drug-addicted mothers had seizures and frequent spikes and sharp waves in their EEGs. Among (P2) children 6/23 showed background slowing and 1 had rhythmic theta activity (6 with and 1 without neurological symptoms). In BAEP, bilateral prolonged interpeak latencies (IPL) were found in 1 child with severe AIDS encephalopathy. Side differences greater than or equal to 0.4 ms in IPL were seen in 2 (P2), 1 without and 1 with neurological symptoms. A late onset was seen in 2 (P1) and 4 (P2) children. Median SEPs were normal in 24/26 patients; N20/N13 amplitude ratio was reduced in 2 (P1) patients. EEG and BAEP revealed nonspecific abnormal features in HIV encephalopathy. The the progression of the disease. However, also in the symptomatic group, normal results of EEG and BAEP dominated. SEP in the symptomatic group revealed only normal values. For monitoring the effectiveness of AZT treatment in HIV encephalopathy, EEG seems to be a relevant investigation; for evoked potentials more data and experience are needed.

AIDS Dementia Complex

Immunocytochemical characterization of cytomegalovirus (CMV) infected giant cells in perinatal acquired human immunodeficiency virus (HIV) infection.

In a pediatric case of necrotizing CMV myelitis after perinatal HIV infection characteristic cytomegalic cells, which could not be attached to a particular cell line by cell morphology, were studied after immunostaining with monoclonal and polyclonal antibodies raised against GFAP, S100 protein, NSE, synaptophysin, factor VIII, vimentin, macrophages, leukocytes, CMV, HSV I + II, toxoplasma, and HIV 1 gp41. Astrocytes, oligodendrocytes, neurons, ependymal and endothelial cells, macrophages, and Schwann cells stained positively with CMV antiserum. With regard to their immunological features the majority of cytomegalic cells ("owl eye cells") was identified as astrocytes, and in decreasing frequency, the remainder was characterized as macrophages, mesenchymal, and endothelial cells. It is concluded that CMV giant cells represent one phase of virus induced cell transformation, not only one single, but numerous cell types are exposed to after CMV infection.

Acquired Immunodeficiency Syndrome

Rapid development of giant aneurysm at the base of the brain in an 8-year-old boy with perinatal HIV infection.

An 8-year-old boy with perinatal HIV infection developed a large fusiform aneurysm in the circle of Willis two years prior to death which was confirmed by radiological studies. The postmortem examinations revealed a predominantly intimal, proliferative lesion, and partial destruction of the internal elastic lamina in the involved arteries. Within the intima hyperplasia of fibroblasts and smooth muscle cells was observed. No inflammatory alterations, no granulomas and no multinucleated giant cells could be noted in the vascular walls and in the cerebral parenchyma. A small ischemic infarct was present in the left thalamus. Cerebellum, brainstem and medulla showed multiple areas of progressive multifocal leukoencephalopathy (PML). Immunohistochemistry with anti-gp41, a monoclonal antibody against HIV envelope did not exhibit any positive results. These findings implicate that the vascular lesion might be attributed to primary infection of the brain by HIV which led to a defect of elastic lamina and consecutive intimal hyperplasia. A second hypothesis could be based on the effect of extremely high dose AZT therapy avoiding inflammatory reaction after HIV infection.

Acquired Immunodeficiency Syndrome

[Post-traumatic epilepsy].

There might be 3 different forms of epilepsy following head injury: earliest seizures with occasional characteristics, early seizures prompting considerations of differential diagnosis, and, late seizures which might have a chronic course = posttraumatic epilepsy proper. The risks to have posttraumatic epilepsy for a child suffering from head injury are: penetrating cranial trauma, early seizures, intracranial hemorrhage, unconsciousness > 24 hours, depressed fracture with dural laceration and unconsciousness > 24 hours, fractures on the base of the skull, focal synchronous activity in the EEG which is related to the site of brain injury. If a or > or = 2 of b-g are given we recommend prophylactic antiepileptic therapy: in the younger (< 5 years) with a low dose of phenobarbital and in the elder (> 5 years) carbamazepine for at least 2 years of treatment.

Brain Injuries

[Physiologic blood coagulation studies in idiopathic arterial thrombosis].

QUESTIONING: The prevalence of inherited thrombotic syndromes in the general population appears to be higher than that of inherited bleeding disorders. However, the most important candidates for screening are patients with unexplained thromboembolism at ages of less than 40 years: In 19 children suffering from "idiopathic" arterial thrombosis laboratory screening has been performed. METHODS: PT, PTT, TT, platelet count, spontaneous platelet aggregation, von Willebrand-factor, fibrinogen, plasminogen, antithrombin III, protein C, C1-inactivator, alpha-1-antitrypsin, alpha-1-antichymotrypsin, alpha-2-antiplasmin and alpha-2-macroglobulin have been investigated. RESULTS: Compared to an age matched healthy control group we could demonstrate in children with arterial thrombosis in vitro platelet activation with significant enhanced platelet aggregation, elevated levels of von Willebrand-factor and alpha-1-antichymotrypsin at the onset of disease. Protein C and alpha-2-antiplasmin were significantly decreased. These changes turned back to normal in the following 6 to 9 months. PT, PTT, TT, platelet count, plasminogen, alpha-1-antitrypsin, c1-inactivator and alpha-2-macroglobulin showed no alterations compared to controls. CONCLUSIONS: Platelet activation and alteration of platelet function have been shown in vivo and in vitro to initiate thrombosis. The von Willebrand's VIII molecule is involved in this step. The lowering of protein C levels at the onset of thrombotic diseases is discussed to be due to an increased turnover, whereas the decreased levels of alpha-2-antiplasmin might be a counter-regulation to the thrombotic event, showing an "activated" fibrinolytic system.

Adolescent

Central nervous system involvement of children with HIV infection.

The neurological findings in 41 HIV-seropositive children are described. 23 children were symptomatic, eight seropositive but without symptoms and 10 seropositive children less than 15 months of age had no other evidence of immunodeficiency. Acquired microcephaly, developmental regression and progressive motor deterioration indicated HIV encephalopathy, as did developmental delay, mental retardation, cerebellar symptoms and behavioural changes. Three children with progressive encephalopathy improved after treatment with azidothymidine (AZT). In eight children treated with prophylactic intravenous immunoglobulin therapy (IVIG) and seven treated with both IVIG and AZT, no mental deterioration has been observed since the beginning of therapy. One child with advanced encephalopathy and severe pyramidal tract involvement did not improve.

Adolescent

Expression of the HSV-2 ribonucleotide reductase subunits in adenovirus vectors or stably transformed cells: restoration of enzymatic activity by reassociation of enzyme subunits in the absence of other HSV proteins.

We have cloned the large subunit (RR1) of the HSV-2 ribonucleotide reductase into a helper-independent adenovirus 5 vector under control of the viral major late promoter. Infection of 293 cells with the AdRed-1 recombinant virus resulted in the expression of the HSV-2 RR1 protein. We have also produced cells which constitutively express the small (RR2) subunit of the HSV-2 enzyme by transfecting 293 cells with a plasmid encoding this protein and the neo resistance marker (pSV2neo-RR2). Infection of the A439-14 producer cells with AdRed-1 resulted in the expression of enzymatically active HSV-2 ribonucleotide reductase. HSV-2 reductase activity could also be detected upon mixing of extracts from cells expressing either subunit. Our results indicate that the HSV-2 holoenzyme can be reconstituted in vivo and in vitro and that no HSV-2 proteins, beyond the enzyme subunits, are required for the formation and activity of the viral reductase.

Adenoviridae

[Van der Woude syndrome in combination with ring chromosome 18].

7-month-old girl with a combination of van der Woude syndrome and ring chromosome 18 showed the following malformations: medial palate cleft, fistulas of the lower lips, hypertelorism, epicanthus, antimongoloid position of the eyelids, a small hymenal appendix, plantar placing of the third toe, atrial septum defect, stenosis of the auditory canal. The combination of fistulas of the lower lip and cleft lip or palate already established the diagnosis of Van der Woude Syndrome. Because of the additional malformations observed a chromosome analysis was performed which demonstrated a ring chromosome 18. The phenotypic features of ring-chromosome 18, a 18p-/18q- syndrome, vary according to the extend of deletion of the short or long arm. However, all patients show craniofacial dysmorphism, intellectual and developmental retardation, defective hearing, speech disorder, and more than 50% have stenosis or atresia of the auditory canal.

Abnormalities, Multiple

[Damage patterns in severe child abuse with and without fatal sequelae].

In 62 battered children with involvement of the central nervous system clinically 3 patterns of impact to the skull, brain and its coverings could be distinguished: In 22 babies (mean age 6 months) the brain was damaged mainly by violent shaking. Many of those infants were in shock and epileptic status on admission. Retinal, subarachnoidal, and later subdural bleedings were recognizable. At first, all patients survived, but later 3 of them died in a vegetative state. Retinal bleedings are prompted by subarachnoidal hemorrhage due to shearing of bridging veins and by compression of the chest which immediately is followed by raise of venous pressure in the upper half of the body and by arteriospasms, leading to endothelial damage and increase of vascular permeability (Purtscher's disease). Both mechanisms result in severe retinal hemorrhage which might intrude into the vitreous body (Terson-syndrome). The consequences of violent shaking for the child might be disastrous mental retardation, microcephaly, spasticity, and epilepsies. The child might turn blind on one or both eyes; the visual failure is due to retinal scar formation, retinal detachment and fibrous organisation within the vitreous body on the one hand, due to raised intracranial pressure on the other hand, adding further damage to the visual pathway. In 19 children whose mean age was 13 months massive impact on the skull resulted in major brain damage: acute subdural hematoma, contusional bleedings, compound, diastatic or impression fractures. Ten of them died immediately or were picked up dead from home by different emergency services.(ABSTRACT TRUNCATED AT 250 WORDS)

Battered Child Syndrome

Cranial nerve damage after paediatric head trauma: a long-term follow-up study of 741 cases.

A report is given on transient and permanent (6 months) impairment of cranial nerves after paediatric head injuries (N: 741). There is a link between severity of the injury, fractures on the base of the skull, its foramina and channels, and the frequency of cranial nerve involvement. One should try to establish whether a posttraumatic dysfunction of the cranial nerves is primary or secondary in nature, i.e. due to raised intracranial pressure or haemorrhage. In children after head injuries often the cranial nerves of the oculomotor system are affected (20.2%/7.0%)--transient (permanent), followed by optic atrophy (4.88%), lesion of the trigeminal nerve (4.2%/2.2%), and the facial nerve lower motor type (4.1%/1.7%). Loss of hearing (3.3%/1.2%) and of smell (3.2%/1.2%) are less frequent in children than in adults.

Child

[Double urologic tumors: reduced immunocompetence?].

55 patients with primary multiple neoplasias underwent immunostaging with determination of the immunoglobulins, B- and T-lymphocyte counting, H3-lymphocyte stimulation (ConA, PWM, PHA) and DNCB-epidermal test. There were no significant differences between the PMN-group and a group of patients with solitary tumors, whereas both cancer patient groups revealed a diminished immunocompetence compared with a non-malignant control group of patients.

Adult

[Microrheological studies in healthy subjects. Effect of cigarette smoke and oral contraceptives on erythrocyte flexibility].

Erythrocyte flexibility was tested in 29 healthy subjects, using whole-blood filtration through membrane filters with 5 micron pores, special attention being given to cigarette smoking and oral contraceptives as risk factors. Red cell flexibility was significantly reduced in male smokers compared with non-smokers (P is less than 0.0001), and in all male and female smokers (P is less than 0.05) An influence of oral contraceptives to erythrocyte flexibility could not be found. Reduced erythrocyte flexibility in smokers as a factor contributing to an abnormal microcirculation and to the development of arterial occlusive disease is discussed.

Adolescent