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Biomedical subjects

M Sauer

Publications and source records attributed to M Sauer.

At least 73 records · Page 4Linked to original sources

A double-blind placebo controlled trial of diltiazem in Duchenne dystrophy.

The role of calcium accumulation in the pathogenesis of Duchenne muscular dystrophy (DMD) has already been discussed. Several trials with different calcium-blocking drugs have revealed no clinical benefit. In addition, the present study includes histological investigations and computer tomography to verify therapeutic effects. In a randomized placebo-controlled double-blind study, 13 DMD patients aged from 3-10 years (mean, 7 years) were treated with 5 mg/kg diltiazem daily for 1 year. Compared with before therapy, the number of calcium-positive muscular fibres was remarkably reduced in the treated DMD patients, but not in the placebo group. The evaluation of all other biochemical and clinical parameters revealed no significant effects of the diltiazem therapy. The muscular X-ray density measured by computer tomography decreased under treatment. After the evaluation of the double-blind study, the code was broken. Therapy, however, was continued in the treated group and started in the placebo group. After 3 years of diltiazem therapy the clinical status of all 26 patients of the study and 20 additional DMD patients who were treated with diltiazem was compared with 46 untreated DMD patients of the same age and stage in our department. No obvious clinical benefit of diltiazem therapy could be observed.

Calcium↗

Diagnosis of brain death by transcranial Doppler sonography.

The blood flow velocities in the basal cerebral arteries can be recorded at any age by transcranial Doppler sonography. We examined nine children with either initial or developing clinical signs of brain death. Soon after successful resuscitation increased diastolic flow velocities indicated a probable decrease in cerebrovascular resistance; this was of no particular prognostic importance. As soon as there was a clinical deterioration, there was a reduction in flow velocities with retrograde flow during early diastole, probably due to an increase in cerebrovascular resistance; this indicated a doubtful prognosis. In eight of the nine children with clinical signs of brain death a typical reverberating flow pattern was found, which was characterised by a counterbalancing short forward flow in systole and a short retrograde flow in early diastole. This indicated arrest of cerebral blood flow. One newborn showed normal systolic and end diastolic flow velocities in the basal cerebral arteries for two days despite clinical and electroencephalographic signs of brain death. Shunting of blood through the circle of Willis without effective cerebral perfusion may explain this phenomenon. No patient had the typical reverberating flow pattern without being clinically brain dead. Transcranial Doppler sonography is a reliable technique, which can be used at the bedside for the confirmation or the exclusion of brain death in children in addition to the clinical examination.

Basilar Artery↗

Ferric-coprogen receptor FhuE of Escherichia coli: processing and sequence common to all TonB-dependent outer membrane receptor proteins.

Iron transport via siderophores requires outer membrane receptor proteins and the TonB protein. The FhuE protein of Escherichia coli functions as the receptor for ferric coprogen and ferric-rhodotorulic acid. A chromosomal DNA fragment bearing the fhuE gene was cloned into pACYC184. The gene was localized by insertion mutagenesis by using the transposon Tn1000. Expression in minicells revealed a FhuE precursor with an apparent molecular weight of 82,000 and a FhuE protein with a molecular weight of 76,000. The transcription polarity of the fhuE gene was deduced from the size of truncated polypeptides derived from Tn1000 insertions, which were mapped by restriction analysis. The processing of truncated precursors that were synthesized by insertion mutants was strongly reduced even when the insertion site was close to the carboxy terminus of the FhuE protein. It is concluded that either the efficient insertion of proFhuE into the cytoplasmic membrane or the rate of cleavage of the signal peptide requires a particular conformation of the proFhuE protein, which is only formed by the complete primary structure. The amino-terminal amino acid sequence deduced from the nucleotide sequence was confirmed by gas-phase sequencing of the precursor and the mature form, which were separated by electrophoresis on polyacrylamide gels. The precursor contained an unusually long signal peptide of 36 amino acids. The amino-terminal end of the mature form contained the sequence Glu-Thr-Val Ile-Val. A pentapeptide starting with either Glu or Asp, followed by Thr, and two uncharged residues ending with Val were found in all outer membrane receptor proteins that were constituents of TonB-dependent transport systems.

Amino Acid Sequence↗

Functional characterization of prethymic T cells committed to alloreactivity.

The results of previous experiments on MHC fully allogenic bone marrow transplantation (BMT) in nonthymectomizd recipients indicated that anti-MHC alloreactivity starts to become irreversibly committed at the prethymic level. This is a matter of some controversy. Since it is possible that conflicting results depend on the methods chosen, we reexamined our previous results by applying two new approaches. Adult thymectomized (ATX) Balb/c mice received a syngeneic fetal thymus either 3 weeks before or 3 weeks after lethal irradiation and reconstitution with C57BL/6 BM incubated in antiserum. Since monoclonal antibodies such as anti-Thy 1 are of limited value for investigations of the above type (Thy 1 antigen crosses the prethymic/thymic border), we used two highly selective, excessively cytotoxic xenoantisera for incubation of the donor BM--either a specific anti-T cell serum (SAT) that eliminated only mature T cells, or a specific antilymphocyte serum (SAL) that reacted additionally with a subset of prethymic T cells (PTC). In both experimental approaches the results were similar: Recipients of SAT-BM developed antihost reactivity, in contrast to recipients of SAL-BM. SAT-BM recipients became immunodeficient, whereas SAL-BM chimeras were immunocompetent. Late mortality was observed only following SAT treatment. Preliminary morphological findings in the lymphoid tissue of BM recipients agree fully with the functional observations. We conclude that the data confirm our previous results in nonthymectomized BM recipients--i.e., PTCs initiate antihost reactivity in MHC fully allogeneic BMT--and PTC commitment is thymus/thymus factor independent. These conclusions are discussed with regard to the problems of MHC allogeneic clinical BMT.

Animals↗

A new X-linked syndrome with muscle atrophy, congenital contractures, and oculomotor apraxia.

Six men from three generations of one family had manifestations of a possible new syndrome. All had congenital contractures of the feet at birth, a slowly progressive predominantly distal muscle atrophy, dyspraxia of the eye, face, and tongue muscles, and mild mental retardation. The pedigree is compatible with X-linked recessive inheritance with no detectable manifestations in the obligate carriers. Linkage analysis excludes close linkage with the Xg locus and a polymorphic DNA sequence from the long arm of the X chromosome (DXS17).

Adolescent↗

Unilateral vulvar edema after intraperitoneal Hyskon administration.

Two patients are presented who developed vulvar edema after intraperitoneal instillation of Hyskon. Regardless of the route of extravasation, Hyskon's high molecular weight and osmotic effects may lead to further soft tissue swelling. Correct diagnosis warrants the exclusion of wound infection, hematoma, or seroma. Special attention to ensuring peritoneal closure may lessen the risk for the development of this complication.

Adult↗

Placenta previa: an analysis of three years experience.

A retrospective study of 45 cases of complete placenta previa is presented. Expectant management of the premature infant using tocolytics, blood transfusion, and cesarean section was practiced. All patients were suspected of having placenta previa based upon ultrasound examination; 90% reported third-trimester bleeding; 48% required prepartum blood transfusion; and 80% experienced one or more complications. Premature delivery occurred in 67% of patients; tocolysis was initiated in 53%. Patients with previa were older, of higher parity, and more likely to have had a previous cesarean section or abortion than matched controls.

Adult↗

[Disobliteration of coronary arteries by the laser. Peroperative experience].

The authors report their experience of coronary artery disobliteration by laser in 10 patients. This was a preliminary study to assess the effects of an Argon laser on atheromatous coronary stenosis in vivo. This technique was used during coronary bypass surgery. Two series of patients were treated: an initial group of 5 patients who underwent laser therapy associated with coronary bypass surgery; a second group undergoing laser therapy alone without distal bypass grafting. The results were assessed by immediate angiography in the first series and by the passage of calibrated probes in both series. Secondary control angiography after 3 weeks was carried out in all patients. The immediate results showed a constant improvement (less than 25%) in the degree of stenosis. However, secondary angiography showed secondary occlusion in 88% of cases. These preliminary results show: the immediate efficacy of Argon laser in reducing the size of atheromatous plaques, the innocuity of the method as there were no postoperative deaths, a high incidence of secondary failure which could be related to the type of indication (competitive flow in the first group and poor distal run off in the second group of patients) or to the type of laser used. The authors consider this to be a promising technique but a lot of clinical and experimental work remains to be done before it can be adopted for routine use.

Aged↗

Ultrasonographic diagnosis of brain tumors in infancy.

Fourteen intracranial tumors were diagnosed by means of mechanical sector scanning through the infant's open fontanelle in a series consisting of 1150 examinations. In addition, integrated pulsed Doppler-sonography (duplex-scan technique) was used to diagnose malformations of intracranial vessels. We established five diagnostic criteria: high echogenic structures are mostly seen in solid tumors of the periventricular region; low echogenic structures are seen in infiltrating tumors of the cerebral tissue and they are difficult to recognize; echo-free structures correspond with a cystic process, a necrosis or malformation of vessels; indirect sonographic signs of a cerebral tumor are all changes in shape and size of the normal anatomic structures; and abnormal blood-perfusion of a marked region, e.g. caused by dilatations of vessels, can be proved by integrated pulsed Doppler-sonography.

Brain Neoplasms↗

Small bowel obstruction following diagnostic laparoscopy.

The occurrence of an incarcerated small bowel hernia through a subumbilical laparoscopic incision site is a rare event. The obstruction occurred 7 weeks after laparoscopy and 1 week after bilateral tuboplasty through a Pfannenstiel incision. Following laparoscopy, the possibility of a bowel obstruction should be entertained in any patient presenting with signs consistent with this diagnosis regardless of the intervening period of time.

Adult↗

[The cavum septi pellucidi and cavum Vergae in infants -- an investigation with 2-dimensional sector-echo encephalography].

Using a two-dimensional sector-scanner the brains of 642 preterm and term infants between 28 weeks of gestation and 12 months of age were examined for the incidence of a cavum septi pellucidi (CSP) and a cavum Vergae (CV). In 100 out of 642 infants we saw a CSP. The highest incidence (52%) was found among the infants of very low gestational age (-33 weeks) within the first seven days of life. After the second month the incidence decreases rapidly (only 1% in infants - months). We saw a CV only in combination with a CSP, less frequent, however, than a CSP alone (26 out of 642 infants): the incidence was 33% in infants of very low gestational age (-33 weeks) within the first seven days of live, only 4% in term neonates and 0% after the second month of life. Finally, the rupture of a septum with CSP and CV by stretching forces is demonstrated in an infant with progressive hydrocephalus. The data of this paper contribute to the knowledge of the morphological development of the normal fetal and newborn brain.

Age Factors↗

[Doppler-sonography of cerebral blood-flow in infants].

Doppler-sonography of the intracranial arteries in the sagittal section through the open anterior fontanelle may yield valuable information on the cerebral blood-flow in infants. Applying the directional method, the anterior-cerebral artery could be recorded in all probands; by using the pulsed technique at least one artery was identified in 95% of the cases. There was a significant difference in pulsation-index (P.I.) between the pulsed and the directional method. Both methods revealed a reduction of the P.I. in infants with brain edema, and an increase of the P.I. after intracranial haemorrhage had occurred. The P.I. was significantly increased in infants younger than 36 gestational weeks, compared with healthy term newborns older than 40 weeks. The clinical relevance for diagnostic purposes as well as for therapeutic monitoring is discussed.

Brain Edema↗

Adrenal insufficiency, myopathic hypotonia, severe psychomotor retardation, failure to thrive, constipation and bladder ectasia in 2 brothers: adrenomyodystrophy.

Two brothers are described who manifested primary adrenal insufficiency, dystrophic myopathy, severe psychomotor retardation, failure to thrive, fatty degeneration of the liver, megalocornea, chronic constipation, and terminal bladder ectasia until their death at 3 8/12 and 1 7/12 years, respectively. The differential diagnosis to similar syndromes is discussed. This combination of problems apparently has not yet been described. The term adrenomyodystrophy is suggested.

Adrenal Insufficiency↗