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Biomedical subjects

M Ries

Publications and source records attributed to M Ries.

At least 19 recordsLinked to original sources
PubMed1995

Severe intracranial hemorrhage in a newborn infant with transplacental transfer of an acquired factor VII:C inhibitor.

We describe a woman in whom inhibitors to factor VIII:C developed in her first pregnancy. The neonate had a factor VIII:C level of 1% and an inhibitor titer of 5.2 Bethesda units. After an uneventful vaginal delivery, the boy had an intracranial hemorrhage at 5 days of age. To our knowledge, this is the first report of a bleeding complication in a newborn infant with transplacental transfer of an acquired factor VIII:C inhibitor.

Cerebral Hemorrhage↗
PubMed1995

Age-related differences in a clot lysis assay after adding different plasminogen activators in a plasma milieu in vitro.

PURPOSE: The fibrinolytic system is involved in a wide variety of biological phenomena and differs physiologically in newborns compared to older children or adults. Because the newborn has hypoplasminogenemia and a possible existence of a dysfunctional plasminogen with normal adult levels of plasminogen activator inhibitor type 1 and alpha 2-antiplasmin and elevated levels of plasminogen activator inhibitor type 2, it could be expected that the response to standard concentrations and doses of plasminogen activators would be reduced. PATIENTS AND METHODS: We have studied the kinetics of in vitro fibrinolysis after adding different concentrations of streptokinase(SK), urokinase(UK), and recombinant tissue plasminogen activator(rt-PA) by use of a microtiter clot lysis assay. RESULTS: Geometrical dilution rows showed characteristic dose response curves. After clot formation a rapid lysis was seen with all plasminogen activators. The 50% lysis time correlated to the plasminogen activator dose and showed no differences among normal adults, children aged 1-6 years, and children age 7-14 years. Newborns demonstrated a significantly prolonged 50% lysis time with all urokinase concentrations. The 50% lysis time with recombinant tissue plasminogen activator and streptokinase was significantly prolonged only at high concentrations, whereas we could not see any differences at lower concentrations. CONCLUSION: The experience with thrombolytic agents in newborns is limited, and no controlled investigations have been reported. Our results of the fibrinolytic potential in a plasma milieu in vitro after adding different plasminogen activators can be helpful to establish dosage guidelines for thrombolytic therapy in newborns and older children.

Adolescent↗
PubMed1995

[Thrombolysis of modified Blalock-Taussig shunts in childhood with recombinant tissue-type plasminogen activator].

Since 1983, when c-DNA was isolated, recombinant tissue plasminogen activator (rtPA), an endothelial-cell-produced activator of fibrinolysis is used, more increasingly often in therapy of thrombosis. Whereas some studies have been published regarding efficacy and safety rtPA in different thrombotic states of adults, only case reports exist in children. Doses vary widely (0.8-6 mg/kg/d), bleeding complications are reported in up to 50%. We report on four infants with complex cyanotic congenital heart disease who developed an early post-operative thrombosis of a modified Blalock-Taussig shunt. By local low dosage application of rtPA we could achieve a complete lysis of the thrombus in three of our four patients. In one patient we were unsuccessful due to a distal stenosis of the shunt. This infant required repeat surgery with creation of a central aortopulmonary shunt. We saw severe bleeding in one, requiring transfusion of packed cells, and formation of a perigraft reaction in another patient. In our experience local application of rtPA in low doses is a good therapeutical option in patients with thrombosis of aorto-pulmonary shunts, especially in the first postoperative days.

Dose-Response Relationship, Drug↗
PubMed1994

Sonographic demonstration of multiple intrahepatic meconium masses in a newborn with meconium peritonitis.

We report on a patient with atresia of the colon transversum and a large meconium pseudocyst adherent to the liver. Sonography additionally revealed multiple cystic structures due to intrahepatic meconium masses. Surgical evacuation of the meconium pseudocyst resulted in massive diffuse hemorrhage from the liver which could not be stopped. The child died intraoperatively due to hemorrhagic shock. Histologically the intrahepatic meconium masses were surrounded by various degrees of necrosis and hemorrhage without a well-defined capsule of fibrous granulation tissue.

Colonic Diseases↗
PubMed1994

[Total cavopulmonary anastomosis: risk factors and results in patients under 4 years of age].

In recent years, an increasing number of modified Fontan-operations has been performed in children younger than 4 years of age. The purpose of this study was to identify preoperative risk factors in this age group. From February 1990 until February 1993, we performed in our center a modified Fontan-operation using the technique of total cavopulmonary anastomosis (TCPA) in 37 consecutive patients (17 pts. < 4 years = group I, 20 pts. > 4 years = group II). Early postoperative mortality occurred in patients of group I only (n = 3 pts.). All of these patients had additional preoperative risk factors. Pulmonary vascular resistance (PVRI) > 2 U x m2 was a significant risk factor for the younger patients while pulmonary artery size alone (expressed as the McGoon-ratio or Nakata-index) could not be identified as a separate risk factor. Using two additional indices (McGoon-ratio/PVRI and Nakata-index/PVRI), we were able to identify patients with unfavorable postoperative hemodynamics as high-risk patients. In our experience, TCPA can be performed in patients younger than 4 years of age with a low mortality, if there are no additional preoperative risk factors. For high-risk patients we recommend either a bidirectional Glenn-anastomosis as a first step procedure or a TCPA with fenestration of the intraatrial tunnel-patch.

Adolescent↗
PubMed1992

Colour Doppler imaging of intracranial vasculopathy in severe infantile sialidosis.

Neonatal ascites is usually attributed to prenatal infections, lysosomal storage disease and anomalies of the genitourinary tract, gastrointestinal tract or cardiovascular system. We report one case of neonatal ascites associated with infantile sialidosis. Cerebral sonography showed stripe-like intracerebral echogenicities in the region of the basal ganglia. Colour Doppler imaging demonstrated blood flow within the echogenicities confirming the suspected diagnosis of intracranial vasculopathy.

Adult↗
PubMed1992

Amyotrophic lateral sclerosis.

Amyotrophic lateral sclerosis (ALS) is a clinical entity differentiated during the last few years into definite, probable, possible and suspected ALS. There are many hypotheses trying to explain its genesis: slow virus hypothesis, trace elements, immunologic and trophic factors, excitotoxins, metabolic influences, DNA anomalies, and so on. It is necessary to differentiate ALS like syndromes and ALS variants. Some of the ALS like syndromes can be treated. Only the disease elaborated like that may be submitted to clinical therapeutical trials or molecular-genetic research. Palliative therapy is still necessary. It does not prolong life, it makes its quality better.

Amyotrophic Lateral Sclerosis↗
PubMed1992

New chromogen for assay of glucose in serum.

We describe a colorimetric assay for glucose determination in human serum, with use of the chromogen 2-amino-4-hydroxybenzenesulfonic acid (AHBS), glucose oxidase, and peroxidase. With this assay, glucose concentrations less than or equal to 27.8 mmol/L can be measured in serum, with a sample/reagent volume ratio as low as 0.0025. The chromogen itself is easily soluble in water and does not require other components for the color change, making the reagent composition less complex. A single working reagent is used, and the reaction is completed within 10 min at 37 degrees C. The absorbance of the yellow reaction product is measured at 415 nm, and a blank sample measurement is not needed. The average analytical recovery of glucose in different human sera was 97.6%, with no significant interference of reducing compounds in serum. The results of the recommended procedure correlated well with those of the phenol/4-aminoantipyrine method of Trinder.

Blood Glucose↗
PubMed1991

Microheterogeneity of urinary albumin and tubular proteinuria in juvenile diabetes mellitus.

We studied differential urinary albumin excretion by a double one-dimensional gel electrophoresis with decyl sodium sulphate-polyacrylamide gel electrophoresis in the first, and isoelectric focusing in the second dimension in 37 diabetic children and 20 healthy subjects. In addition, total proteins, albumin, beta 2-microglobulin and molecular size distribution of urinary proteins were measured, the latter using sodium dodecyl sulphate-polyacrylamide gel electrophoresis. Whilst albuminuria was not significantly different from controls we found an increased microheterogeneity of urinary albumin in 38% of patients. In addition, low molecular weight protein (P less than 0.05) and beta 2-microglobulin excretion (P less than 0.01) were elevated. It is suggested that the appearance of highly heterogenous albumin in the pI range of 5.3-5.9 is the result of a decreased tubular reabsorption.

Adolescent↗
PubMed1990

Simultaneous palsy of facial and vestibular nerve in a child with Lyme borreliosis.

We describe a boy with borreliosis characterized by lymphocytic meningitis and simultaneous palsy of facial and vestibular nerves on the left side. A mild sensoneural affection was also confirmed by brainstem evoked response audiometry. While symptoms of vestibular dysfunction quickly decreased during antibiotic treatment, facial palsy improved only slowly.

Child↗
PubMed1990

Demonstration of perivascular echogenicities in congenital cytomegalovirus infection by colour Doppler imaging.

Two children with congenital cytomegalovirus infection and intracerebral echogenicities were investigated by computer sonography and colour Doppler imaging (CDI). By simultaneous imaging of brain tissue and CDI, blood flow within the stripe-like echogenicities of the basal ganglia was demonstrated. Using CDI the echogenicities were identified as the walls of thalamostriate vessels.

Basal Ganglia↗
PubMed1990

[Evaluation of diagnostic efficacy in radiology. Literature review].

The recent development of new imaging procedures has lead the radiologists to assessing the diagnostic efficacy of the available examination techniques in order to reduce medical care costs. In cooperation with epidemiologists and statisticians, several methods of statistic analysis of the diagnostic efficacy of the procedures such as sensitivity and specificity evaluation, ROC analysis, disease prevalence, predictive value of tests, etc... have been developed. Benefit-cost and cost-efficiency analyses have also been performed, with a view to improving the quality of long-term medical care for the patient at a lower cost both for himself and the society.

Costs and Cost Analysis↗
PubMed1989

Cortical pelvic strains with varying size hemiarthroplasty in vitro.

Four human pelves were dissected of soft tissues and instrumented with rosette strain gages. The pelves were left intact (not separated at the pubic symphysis or sacroiliac joints) although each of the eight hemipelves was tested separately. The pelves were loaded to simulate single leg stance with use of a wire mesh cemented to the wing of the ilium, representing the abductor muscles. Loading was carried out with the intact hip joint and 1 mm undersized, correct sized, and 1 mm oversized hemiarthroplasty. The correct sized component produced a strain pattern closet to normal. The oversized component was associated with an increase in strain at the medial acetabular dome.

Acetabulum↗
PubMed1989

Normal pelvic strain pattern in vitro.

Four cadaver pelves were dissected of soft tissue and each of the eight hemipelves instrumented with ten rosette strain gauges. Static loading was conducted to simulate single leg stance, and applied through the intact hip joint. The medial portion of the pelvis was under tension directed vertically and the lateral ilium was in compression. This strain pattern is consistent with bending applied to the ilium from the action of the abductor and joint reaction forces.

Biomechanical Phenomena↗
PubMed1989

[An unusual late manifestation of hemorrhage caused by vitamin K deficiency].

After an uneventful pregnancy and birth at home without vitamin K-prophylaxis, a 13-day-old breast-fed newborn showed an unexplained swelling of his right elbow and avoided movement of his right arm. One week later a swelling of his left thigh and hip with extensive hematoma led to the diagnosis of vitamin K-deficiency. The initial symptoms can be interpreted as an unusual primary manifestation of late hemorrhagic disease caused by vitamin K-deficiency.

Blood Coagulation Tests↗
PubMed1988

Total dislocation of the talus. Case report with a 13-year follow up and review of the literature.

Simultaneous dislocation of the talus from the tibiotalar, talocalcaneal, and talonavicular joints is uncommon and usually occurs from considerable violence. Total dislocation of the talus is frequently an open injury, or the skin may be tented over the dislocated talus leading to skin slough. Infection and avascular necrosis may follow. Treatment options include closed or open reduction, talectomy, and arthrodesis. The following case and literature review illustrate some of the problems encountered and factors that influence the outcome of this injury.

Adult↗