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Biomedical subjects

K Stehr

Publications and source records attributed to K Stehr.

At least 19 recordsLinked to original sources

Pulmonary artery sling associated with tracheobronchial malformations.

We describe three cases of pulmonary artery slings associated with tracheal stenoses by complete cartilaginous rings and abnormalities in the tracheobronchial branching pattern. This association implicates special problems of management that are different from the simple pulmonary artery sling. Pathologic anatomy, symptoms, diagnostic procedures, and the problems of therapy are described. Considering similar cases in the literature, we conclude that thorough diagnostic evaluation of the tracheobronchial and the cardial system should be carried out in all cases of pulmonary artery sling. Simple correction of the aberrant vessel without correcting the tracheal stenosis is of no value in these cases. In some milder cases, a conservative approach is possible and probably less harmful than an operation.

Abnormalities, Multiple

Serious pertussis overlooked in infants.

Two infants with life-threatening pertussis are presented in whom the diagnosis was delayed. A review of pertinent literature suggests that the diagnosis of pertussis in infants is frequently missed and therefore the morbidity and mortality from this disease is underestimated.

Diagnostic Errors

[Pertussis--an illness with typical clinical symptoms?].

There has been a noticeable increase in the incidence of pertussis in West Germany over the last decade. Since the availability of adequate bacteriological diagnosis a much broader clinical spectrum can be attributed to infections with B. pertussis. Three patients with an unusual clinical presentation of pertussis are presented. A three month old infant presented with severe apneic spells without cough as the sole clinical symptoms of the infection. B. pertussis was isolated in the nasopharyngeal swab. A nine month old premature infant with bronchopulmonary dysplasia after long time intubation and artificial ventilation presented with apneic spells, pulmonary and cardiac decompensation and required ventilatory support. The diagnosis was suggested by a massive leucocytosis with lymphocytosis. The diagnosis on the patient was established by serologic methods. Adult contacts of this patient developed longstanding cough and clinical signs of pertussis. The diagnosis of pertussis in these persons was established by nasopharyngeal culture. The third patient with trisomy 21 and a corrected AV canal suffered from nonspecific cough and gradually developed signs of congestive heart failure with pneumonia. B. pertussis was isolated from the nasopharynx. This patient showed neither the typical paroxysmal coughing spells nor disclosed the typical lymphocytosis in his white blood count. Microbiological investigations of patients with symptoms of respiratory tract infections should include the isolation of B. pertussis. Thus, additional cases of pertussis not suspected on the basis of their initial clinical presentation will be detected.

Administration, Oral

[Single incisor syndrome and growth hormone deficiency].

The syndrome of single incisor belongs to the group of midline malformations and is characterized by a single incisor in the patient's maxilla. A growth hormone deficiency (GHD) can be associated. We report about two of our patients with this syndrome. A GHD was diagnosed at the age of 11.0 and 7.6 years, respectively, although a single incisor had already been present during their first dentition. The first patient was treated with HGH to a chronological age of 18.6 years (final height 168 cm), the second patient is still on therapy. The growth of children with craniofacial dysmorphic features must be controlled closely in order to diagnose a GHD in time.

Child

[Haemophilus influenzae type B. Disease and prevention].

Haemophilus influenzae is a gram-negative rod, causing severe infections in childhood, including meningitis, sepsis, epiglottits, pneumonia and otitis. Most of the invasive infections are due to serotype b. Since ampicillin-resistance is increasing, modern cephalosporines like cefotaxime and ceftriaxone are the antibiotics of choice in severe disease. Bacterial meningitis due to Haemophilus influenzae and epiglottitis are both still life-threatening diseases with a lethality of 5% to 25%, and there are severe sequelae in 35% of meningitis cases. Efforts have been made to develop efficacious vaccines. While immunogenicity of type b polysaccharide was low in the high-risk age (below 18 months), conjugated vaccines with either diphtheria-toxoid or Neisseria meningitis outer membrane protein and the Hib polysaccharide were found to be strongly immunogenic even in the first months of life. These vaccines show every few side-effects and can easily be combined with other immunizations such as DPT and DT. Thus, the incidence of invasive infections due to Haemophilus influenzae type b might decline in future.

Anti-Bacterial Agents

[Effect of obstetric-perinatal measures on mortality and early morbidity of premature infants weighing 500 to 1,500 grams].

In a retrospective analysis of perinatal influencing factors in 186 premature newborns of the Department of Gynaecology of the University of Erlangen covering the period from 1982-1987 with birth weights between 500 and 1500 grams, the mortality and early morbidity were analysed, as characterised by cerebral haemorrhages, respiratory distress syndrome and infections insofar, as they had been connected with the obstetrical approach and paediatric intensive-care treatment, 45 infants born in 1982/83 were compared with 141 infants, who had been subjected to a different treatment approach during 1984 to 1987. During the second period, there was a marked drop both in mortality and in the incidence of asphyxia-induced severe cerebral haemorrhage and of the respiratory distress syndrome. A shortened latency period after premature rupture of the amnion, and a more pronounced presence of a neonatologically experienced team of paediatricians were found to be significant obstetric liberal influencing factors in determining the need to perform Caesarean section. The triplication of the frequency of Caesarean section observed resulted in a 50% reduction in perinatal mortality and morbidity. Infants with pelvic presentation benefited most from the more liberal performance of Caesarean section, as did infants with vertex presentation. Shortening of the latency phase in premature rupture resulted in a marked reduction in infection morbidity and mortality. Therefore we conclude, that the frequently practised procrastination with the aim to await an improvement in lung maturity should be replaced by a more active obstetric management, avoiding both infection and birth trauma. Obstetric decisions should be based rather on prenatal estimation of weight than on the calculated gestational age. At present, the lowest birth weight associated with the expectation of a healthy life is considered to be 750 grams.

Asphyxia Neonatorum

High-molecular-mass ("biliary") isoenzyme of alkaline phosphatase and the diagnosis of liver dysfunction in cystic fibrosis.

The high-Mr isoenzyme of alkaline phosphatase (AP, EC 3.1.3.1), a highly sensitive index to cholestasis, was measured by liquid chromatography in 45 patients with cystic fibrosis. Results of serum tests for liver dysfunction--including gamma-glutamyltransferase, aspartate aminotransferase, alanine aminotransferase, total AP, bilirubin, and bile acids--were compared with those for high-Mr AP. Values for high-Mr AP were increased in 44.4% of our patient population, with activities ranging from 0.4 to 17.3 U/L. The upper limit in the control group was 2.5 U/L. We find increased high-Mr AP to be a more sensitive indicator of liver dysfunction in patients with cystic fibrosis than are other tests.

Adolescent

High-molecular-mass alkaline phosphatase: simplified and highly sensitive determination by liquid chromatography.

This simplified HPLC method for measurement of high-molecular-mass alkaline phosphatase (high-Mr AP; EC 3.1.3.1) in serum and bile is rapid (time for column preparation and separation 30 min), reproducible (CV 4.2%), and highly sensitive (detects high-Mr AP in healthy controls at 1-3% of total AP activity in serum), and is suitable for processing small batches of sample. We characterized high-Mr AP in serum and bile by incubating samples with L-phenylalanine, neuraminidase, 1-butanol, or wheat-germ lectin, and by determining stability to heat. High-Mr AP activity was determined in sera of patients with various liver diseases (4-32% of total AP serum activity) and results were compared with those by electrophoresis on agarose.

Alkaline Phosphatase

[Chronic hemorrhagic pancreatitis in gallbladder polyposis as an initial symptom of metachromatic leukodystrophy].

A 4 1/2 year old boy without previous neurologic disorders developed chronic hemorrhagic pancreatitis and was shown to have polyposis of the gallbladder. Neurologic symptoms emerged at the age of 5 years. The sonographic pattern of an echogenic gallbladder was suspect of metachromatic leukodystrophy. The definitive diagnosis was made by the findings of very low arylsulfatase A activity in the white blood cells and deposits of sulfatides in the stroma of the polyps of the gallbladder.

Cerebroside-Sulfatase

[An unusual case of invagination].

Intussusception is an usual pediatric problem in the first two years of life. Nevertheless, it may occur in older children as well, but then often with an uncharacteristic history and atypical x-ray findings. Even symptoms lasting over weeks or months do not exclude intussusception. Ultrasonography is a useful diagnostic approach. In difficult cases endoscopy is indicated.

Child

Extreme variant of septo-optic dysplasia.

A newborn female is demonstrated, the first child of healthy unrelated parents who was born after an uneventful pregnancy. Computerized tomography of the brain revealed gross malformations of the parietal and occipital lobes, of the cerebellum and of midline structures. The pictures are reminiscent of hydranencephaly. The association with bilateral coloboma of the papilla and diabetes insipidus is considered an extreme variant of septo-optico dysplasia.

Brain

Deficiencies of essential fatty acids and vitamin E in cystic fibrosis.

In 25 children (13 male; 12 female) with cystic fibrosis aged 6 months to 16 years and 24 matched controls total serum vitamin E levels and fatty acid patterns of serum cholesterol esters, phospholipids and triglycerides are demonstrated. Compared to controls (1.02 +/- 0.24 mg/dl) the total serum vitamin E levels are significantly decreased in patients with cystic fibrosis (0.30 +/- 0.26 mg/dl) (p less than 0.01). There is no significant difference comparing the fatty acid patterns of the serum ester fractions of both groups. Differences can be seen best in the cholesterol ester fraction. In this fraction linoleic acid shows a trend to be decreased in the cystic fibrosis patients compared to the control group. A possible influence of height velocity on the levels of essential fatty acids is discussed.

Adolescent