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Biomedical subjects

D Ullrich

Publications and source records attributed to D Ullrich.

At least 19 recordsLinked to original sources

Asymmetry of motion VEP in infantile strabismus and in central vestibular nystagmus.

Norcia et al. [1] found a nasal-temporal asymmetry of visually evoked potentials (VEP) elicited by motion stimuli in patients with infantile strabismus. Patients with infantile strabismus typically present with an asymmetry of the monocular optokinetic nystagmus (OKN). We here address the question whether the asymmetry of the motion VEP indicates a sensory defect in the afferent visual pathway that could explain the OKN asymmetry. We recorded the VEP to a horizontally oscillating vertical sinusoidal grating in 20 patients with infantile strabismus (esotropia, asymmetry of the monocular optokinetic nystagmus, latent nystagmus) and in 10 normal controls. No asymmetry occurred in the 10 controls. Eight of the 20 patients with infantile strabismus showed a clear difference between the VEPs evoked by back and forth movements with a mirror-like asymmetry between the two eyes (phase shift 180 +/- 20 degrees). However, there was no significant correlation between the degree of VEP and OKN asymmetries. Therefore, we assume that the VEP asymmetry does not reflect the primary cause of the OKN asymmetry. Rather, the OKN asymmetry may be due to a sensory-motor defect in the efferent subcortical pathway, and the VEP asymmetry could be an epiphenomenon. Some of the VEP asymmetry may be a consequence of the latent nystagmus typically released under monocular stimulation, leading to adaptation of the afferent retino-cortical pathway. This suggestion is supported by a marked VEP asymmetry that we found in two patients with an acquired central vestibular nystagmus, an abnormality most likely not combined with a primary defect of the retino-cortical pathway.

Adolescent

A prospective study of hyperlipidemia as a pathogenic factor in sudden hearing loss.

The pathogenic role of hyperlipidemia in sudden hearing loss (SHL) was examined in a prospective study. Twenty-five patients (14 males, 11 females; age range, 23-59 years) with a first event of SHL (group I) were compared with 9 patients (4 males, 5 females; age range, 28-86 years) with a repeated event of SHL (group II). Audiological examination revealed different types of SHL in group I vs group II: high-frequency loss, 76% vs 22%; low-frequency loss, 12% vs 22%; pancochlear hearing loss, 12% vs 56%. Serum lipid patterns and atherogenic risk factors in both groups were not different and corresponded to lipid patterns in the average population. These findings indicate that both hyperlipidemia and atherogenic risk factors are not of major pathological importance in SHL.

Adult

Urinary caffeine metabolites in man. Age-dependent changes and pattern in various clinical situations.

In an exploratory study the 24-h urinary excretion pattern of caffeine and 14 of its major metabolites was studied in 32 volunteers (adults, adolescents and children), 14 patients either with end stage renal disease or liver cirrhosis, 7 heavy smokers and 27 patients on therapy with cimetidine, allopurinol, theophylline or phenytoin. Caffeine and its metabolites were quantified by UV-absorption after liquid/liquid-extraction and HPLC-separation, which ensured proper analysis of 1-methyluric acid. In adults the renal excretion of caffeine derivatives corresponded to an intake of 509 mg caffeine/day, with 1-methyluric acid as the predominant metabolite. About 69% of caffeine was degraded by the paraxanthine pathway, and theobromine- (19%) and the theophylline pathway (14%) were less important. The ratio of paraxanthine formation to urinary caffeine concentration (= clearance equivalent) was about 2.2 ml.min-1.kg-1 in adults, and the corresponding ratios for theophylline and theobromine were 0.43 ml.min-1.kg-1 and 0.59 ml.min-1.kg-1, respectively. As expected, caffeine degradation was impaired in patients with cirrhosis and was increased in persons who smoked heavily or who were on phenytoin therapy. The results document the possibility of noninvasively investigating gross differences in caffeine disposition by analysis of the urinary pattern of its metabolites.

Adolescent

[Minimally invasive surgery of sinugenic orbital complications in childhood].

Orbital complications of acute sinusitis are classified into inflammatory edema, orbital cellulitis, subperiostal abscess and orbital abscess. The diagnosis is based on endoscopy of the nose, computed tomography of sinuses and orbit and an ophthalmological examination. Endonasal sinus surgery improves drainage and ventilation of sinuses and is free of long-term complications as observed with previous surgical techniques. Thus, the early surgical treatment of orbital complications is indicated even in children. Inflammatory edema and orbital cellulitis will still be treated conservatively. Subperiostal abscess and orbital abscess are treated surgically.

Child

[Pseudotumor of the hypopharynx and other primary diseases in the head and neck region in HIV infections. A case report and review of the literature].

The present article describes a pseudotumor of the hypopharynx, and deteriorating breathing and swallowing in a 72-year-old man. The pseudotumor was due to advanced HIV-infection and is the first such case to be described. Additionally, the article reviews the literature concerning otorhinolaryngeal diseases as primary manifestations of HIV infection.

Acquired Immunodeficiency Syndrome

The influence of gestational age on bilirubin conjugation in newborns.

Unconjugated, mono- and diconjugated bilirubin levels were determined in serum soon after birth, and followed up for several days. Fourteen preterm neonates were studied with a gestational age below 33 weeks (n = 7) or between 34 and 37 weeks (n = 7), respectively, as well as 19 full-term newborns either untreated (n = 9) or treated by phototherapy (n = 10). Bilirubin and its derivatives were analysed by alkaline methanolysis and spectrometry after separation by thin-layer chromatography. In normal full-term neonates total and unconjugated bilirubin reached peak levels at days 2-4. Thereafter, a decline of 11% per day was detectable. Monoconjugates in serum amounted to 3.1 +/- 1.1% of total pigment and remained at that level. The relative amount of diconjugates increased from 0.55 +/- 0.25% (2-4th postnatal day) to 1.62 +/- 0.99% (9-13th day of life). The rapid decline of unconjugated bilirubin paralleled by an increase of diconjugates are an expression of the maturation process for bilirubin conjugation. The premature neonates with less than 33 weeks gestation exhibited an increase of unconjugated serum bilirubin up to the 4-5th postnatal day, the decline thereafter amounted 2% per day. The fraction of 2.3 +/- 1.1% monoconjugates was small and exhibited only a moderate increase in the follow up. In contrast diconjugates were undetectable or very low and remained at this level. These results suggest the presence of a more severe immaturity as well as a slower maturation process of bilirubin conjugation in preterm newborns.(ABSTRACT TRUNCATED AT 250 WORDS)

Bilirubin

Quantum yield of CHAPSO-solubilized rhodopsin and 3-hydroxy retinal containing bovine opsin.

The quantum yields of bleaching for two artificial pigments, bovine opsin combined with (3R)-3-hydroxy retinal or (3R,S)-3-methoxy retinal, were determined in comparison to the value for regenerated bovine rhodopsin. Regeneration of the visual pigments was performed by incubation of 3-[(3-Cholamidopropyl)-dimethylammonio]-2-hydroxy-1- propanesulfonate (CHAPSO)-solubilized opsin with the 11-cis isomers of retinal and the respective retinal derivatives. The extinction coefficients of the pigments in CHAPSO were determined to 35,000 M-1 cm-1 (native rhodopsin), 35,300 M-1 cm-1 (regenerated rhodopsin) and 34,500 M-1 cm-1 (3-OH retinal opsin). With respect to rhodopsin (lambda max: 500 nm), the pigments carrying the substituted chromophores exhibit blue shifted absorbance maxima (3-hydroxy and 3-methoxy retinal opsin: 488 nm). In parallel experiments under absolutely identical conditions we find related to the value of CHAPSO solubilized rhodopsin (identical to 1) a quantum efficiency of bleaching for the 3-hydroxy pigment of 1.2.

Animals

[Surgical anatomy of the optic nerve and the internal carotid artery in the lateral wall of the sphenoid sinus. An anatomic study of the cranial base].

The optic nerve and the internal carotid artery lying in the cavernous sinus contact the bony wall of the sphenoid sinus, and can easily be injured during surgery. The maxillary sinus, the sphenoid sinus and the ethmoid cells were opened on both sides during ten resections of the skull base. After removing the bony part of the lateral wall of the sphenoid sinus the following measurements were performed: the distance between the optic nerve and the frontal dura; the distance between the optic nerve and the internal carotid artery; the length and width of the optic nerve and the internal carotid artery in the area contacting the bony wall of the sphenoid sinus. This study illustrates the regularity of the structures of the posterior nasal wall. Landmarks are offered for finding the orbital aperture of the optic canal. The necessity of orientation by landmarks is emphasized.

Carotid Artery, Internal

Subfractionation of serum bilirubins by alkaline methanolysis and thin-layer chromatography. An aid in the differential diagnosis of icteric diseases.

The determination of direct and indirect-reacting bilirubin fractions by diazo procedures does not allow a definite diagnosis of icteric diseases. Therefore, the clinical relevance of serum bilirubin subfractionation by alkaline methanolysis and subsequent thin-layer chromatography (AM-TLC) was evaluated. Esterified bilirubins could be detected and quantitated in all serum samples investigated. The ratio of serum esterified to total bilirubin was 10-28% in 60 healthy adults (mean 17 +/- 5% S.D.), 1-11% in 77 patients with Gilbert's syndrome (mean 6 +/- 2%), and 2 and 3%, respectively, in two patients with Crigler-Najjar disease type II. The difference was highly significant (p less than 0.001) and the overlap was restricted to three of 139 individuals. The ratio of esterified to total bilirubin was similar to that obtained with HPLC when corrected for with a blank run. The absolute concentration of bilirubin esters in serum from Gilbert's syndrome patients was similar to that from healthy controls, but the unconjugated pigment was increased. In patients with chronic haemolysis (n = 9) and chronic persistent hepatitis (n = 12), the hyperbilirubinaemia consisted of a proportional increase of both unconjugated and esterified bilirubin. As such, the ratio of conjugated to total bilirubin was not significantly different from control values. Patients with acute hepatitis during the first (n = 18) and third ('remission') week of the disease (n = 15), liver cirrhosis (n = 34), and extrahepatic cholestasis (n = 20) predominantly showed an increase in bilirubin conjugates.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent

["Sudden deafness" in childhood and adolescence. Symptoms, therapy and prognosis--a retrospective study].

Fourteen children and adolescents aged between four and 17 years with genuine sudden hearing loss were analyzed in a retrospective study. The symptoms of hearing loss, tinnitus and vertigo were similar to those found in adults. The study's findings suggest that younger children particularly below the age of 14, will not suffer from these symptoms. Consequently, the disease was frequently diagnosed in younger children after the parents had observed defective hearing. In an 13-year-old child it was not possible to provoke a caloric vestibular response. As in the case of the other children (below 14 years of age) this patient did not complain of vertigo. -The prognosis of sudden hearing loss in children is poor. Thus 22% (two out of nine) of all patients treated with drugs became deaf. Moreover, 29% (four out of 14) of all patients examined (with and without therapy) remained deaf after sudden hearing loss. It is concluded that, due to this poor prognosis, a therapeutic attempt should be performed analogous to the treatment given to adults.

Adolescent

Low fractions of mono- and diconjugated bilirubin in patients with beta-thalassemia: an approach to characterize hepatic conjugation capacity.

Unconjugated and mono- and diconjugated bilirubin was determined by alkaline methanolysis and thin-layer chromatography in serum from 16 pediatric patients with homozygous beta-thalassemia (aged 1-23 years), in 14 age-matched controls, and in 26 healthy adults. Total bilirubin amounted to 21 +/- 20 mumol/L in patients with thalassemia compared with 7 +/- 4 mumol/L in healthy children and 11 +/- 3 mumol/L in healthy adults. The fractions of bilirubin conjugates in the various groups were 8 +/- 4% (patients with thalassemia), 18 +/- 5% (age-matched controls; p less than 0.001), and 16 +/- 5% (healthy adults; p less than 0.001). The low fraction of bilirubin conjugates in patients with thalassemia showed no correlation to any other physical or laboratory finding. It is concluded that subnormal values of bilirubin conjugates in thalassemia indicate defective bilirubin conjugation.

Adolescent

Glucuronyl transferase deficiency and mild hereditary spherocytosis: effect of splenectomy.

In a 6-year-old girl an association of hereditary spherocytosis and a defect in hepatic bilirubin metabolism has been found. The patient suffered from mild compensated haemolytic anaemia and excessive hyperbilirubinaemia (maximum concentration 581 mumol/l), the serum activity of liver enzymes was slightly increased. Examination of the erythrocyte membrane proteins revealed a deficiency of the major membrane skeletal protein, spectrin (about 75% of normal) which is probably the basic genetic defect of hereditary spherocytosis. Examination of the patient's family revealed a recessive mode of inheritance. The concentration of bilirubin conjugates in the patient's serum was decreased due to a reduced UDP-glucuronyl transferase activity found in homogenates of liver tissue. Histological liver examination showed an intrahepatic cholestasis, which is a secondary and reversible alteration resulting from severe hyperbilirubinaemia. After splenectomy, normalization of the increased haemolysis and hepatic dysfunction was observed. The excessive hyperbilirubinaemia can be explained by the association of an increased bilirubin load due to haemolytic anaemia and the diminished hepatic conjugation of bilirubin.

Anemia, Hemolytic

Paracetamol as a test drug to determine glucuronide formation in man. Effects of inducers and of smoking.

A simple, noninvasive procedure was developed to monitor glucuronidation and sulphation in patients using paracetamol as the test drug. Urinary paracetamol and its metabolites were determined by UV absorption and electrochemical detection after separation by HPLC. The metabolite to paracetamol ratio (M/P) was used as an approximation of the partial clearance due to metabolite formation. In 14 healthy volunteers, all nonsmokers without medication, M/P was 18 +/- 5 for glucuronides and 12 +/- 4 for sulphate esters. The test was validated in patients treated with enzyme inducers. In 10 patients with epilepsy given phenytoin 0.3 g/day, and in 10 patients with tuberculosis treated with rifampicin 0.6 g/day, the M/P value for glucuronidation was significantly increased to 41 +/- 11 and 35 +/- 7, respectively. In contrast, M/P values for sulphation were not significantly different from untreated controls. In 9 heavy smokers (about 40 cigarettes/day) M/P values for glucuronidation were also significantly increased to 33 +/- 11. However, in 4 moderate smokers (about 10 cigarettes/day) no significant increase was found. The results suggest that in man glucuronidation of paracetamol is inducible both by phenobarbital- and 3-methylcholanthrene-type inducers. Monitoring the ratios of various urinary paracetamol conjugates/paracetamol may be useful as a new tool for the evaluation of factors determining glucuronide and sulphate ester formation in man.

Acetaminophen

Normal pathways for glucuronidation, sulphation and oxidation of paracetamol in Gilbert's syndrome.

A group of eleven subjects with Gilbert's syndrome was characterized by conventional tests and determination of bilirubin and its conjugates in plasma by alkaline methanolysis and thin layer chromatography. After a 1 g dose of paracetamol h.s. the drug and its metabolites were measured by high performance liquid chromatography (HPLC) in the overnight 8-h urine sample. The amounts of paracetamol and of its metabolites recovered in urine were almost identical with those found in the control group (n = 10). The glucuronide:paracetamol ratio, which is considered to be an index of glucuronidation, was not correlated with the fraction of bilirubin present in plasma as glucuronides. These data do not suggest that in subjects with Gilbert's syndrome therapeutic doses of paracetamol are associated with an increased risk for hepatic or systemic toxicity.

Acetaminophen