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Biomedical subjects

F Walther

Publications and source records attributed to F Walther.

At least 19 recordsLinked to original sources

High-risk mitral valve replacement in severe pulmonary hypertension--30 years experience.

OBJECTIVE: In the past 30 years, 2316 patients underwent mitral valve replacement (MVR) at our institution; 382 of them had severe pulmonary hypertension (pulmonary artery pressure (PAP) > 50 mmHg; pulmonary vascular resistance (PVR), 690 +/- 46 dyn/s per m2). We reviewed our early and late results in this high-risk subgroup. METHODS: We used 336 mechanical and 46 biological devices for MVR. The follow-up was 95%, with an observation period of 3208 patient-years and a mean of 8.4 +/- 0.2 years per patient. The overall early mortality rate was 10.5% (n = 40) and stayed at about the same level over the years, although patients characteristics have changed to much older patients and more reoperations. To clarify this fact we divided our data in results according to the decades in which the operations were carried out. The clinical preoperative status and results were as follows (*P < 0.05; **P < 0.01 compared with previous decade). In the decades between 1963 and 1973 (I), 1974 and 1983 (11) and 1984 and 1993 (III) we operated on n = 95 (I), n = 185 (II), and n = 102 (III) patients with a mean age of 43 +/- 1 (I), 50 +/- 1** (II), and 58 +/- 1** (III) years. The incidence of reoperations among these patients was 3.2 (I), 4.9 (II), and 22.6%** (III). The early mortalities were 13.7 (I), 8.6* (II) and 10.8% (III); late mortalities lowered from 5.77 (I), over 4.95 (II), and up to 3.39%** (III) patients/year. The mean functional status according to New York Heart Association (NYHA) class improved from preoperatively 3.0 +/- 0.1 (I), 3.2 +/- 0.1 (II) and 3.3 +/- 0.1 (III) to 2.4 +/- 0.2 (I), 2.4 +/- 0.1 (II) and 2.3 +/- 0.1 (III) postoperatively. RESULTS: Compared with routine elective MVR with a mortality rate of 3.6% (P < 0.01), early mortality is high. But once the patient survives the perioperative course, late results show no difference compared with patients without pulmonary hypertension. The functional results as well are not significantly different. In spite of on average 15 years older multimorbid patients with therefore higher complication rates, early results improved slightly, which could be explained by better operative techniques, perioperative treatment and nursing (online monitoring with immediate therapeutic substitution). Surprisingly the increased number of reoperations had no negative impact on patients' outcomes. CONCLUSION: According to our results, we recommend MVR in severe pulmonary hypertension even in the elderly, with a high but acceptable risk and good long-term results.

Adult↗

[Metastatic malignant melanoma in treated non-Hodgkin's lymphoma].

Secondary cutaneous neoplasia, e.g. basal cell malignant carcinomas or squamous cell carcinomas, is frequently observed in patients with malignant lymphomas. The association between malignant lymphoma and secondary malignant melanoma has only been rarely reported. We describe a patient with a low-grade nodal B-cell lymphoma who, while on treatment with steroids and an alkylating agent, developed a malignant melanoma level IV which rapidly metastasized to lymph nodes involved by the malignant lymphoma.

Adult↗

Tracer kinetic studies on a methionine-supplemented soy-based infant formula using 1-13C- and 15N-methionine as tracers.

A tracer-kinetic study using 1-13C- and 15N-labeled L-methionine was conducted in order to measure the retention rate of free methionine added to commercially-produced soy-based infant formulas. Twelve male infants, fed on a soy formula, received a single-pulse labeling by oral administration of L-1-13C-methionine (5 mg/kg) and L-15N-methionine (10 mg/kg). The abundance of expired 13C-labeled CO2 was measured up to 7 h after administration at 15-, 30-, and 60-min intervals. Additionally, enrichment of total 15N and 15N in urinary ammonia were determined up to 48 h after administration. Retention rates of the labeled carboxyl group amounted to an average of 91.2% (SD 4.1) of the intake. A similar retention rate was measured for the 15N-label of methionine (90.0%, SD 4.3). The data point at the efficacy of methionine supplementation of soy-based infant formulas.

Ammonia↗

[An increased incidence of megacolon in psychiatric and neurologic patients].

The incidence of megacolon is elevated in neuropsychiatric patients. Siegmund was the first, in 1935, to report on the development of megacolon and megasigmoid as the result of chronic atropine therapy of patients with postencephalitic parkinsonism and the associated risk of stercoraceous ulcers and ileus or even sudden death. The etiology of increased frequency of megacolon among neuropsychiatric patients is assumed to be nonuniform, and to include organic defects of centers of the autonomous nervous system in the diencephalon and/or hypothalamous, pharmacodynamic, psychogenic and neurogenic influences on the autonomic nervous system, akinesia and increased obstipation among psychiatric patients, insufficient pressure in the abdominal wall especially in the mentally retarded, with frequently associated weakness of the connective tissue. In cases of long-term therapy with psychotropic drugs the anticholinergic side effects with the risk of megacolon and resulting ileus, sometimes with fatal outcome should be borne into mind.

Cholinergic Antagonists↗

Response to sodium benzoate treatment in non-ketotic hyperglycinaemia.

Therapy with benzoic acid in a case of classic neonatal non-ketotic hyperglycinaemia (NKH) was successful in stopping seizures but not in promoting mental development. Serum glycine levels were normalizable even by administering low doses of 53 mg sodium benzoate/kg body mass (BM) per day. Despite giving a higher dosage (240 mg/kg BM per day) normalization of glycine concentration in cerebrospinal fluid (CSF) was not achieved. However, seizures ceased. Restriction of protein intake (< or = 2 g/kg BM per day) seemed to be profitable. CSF glycine concentrations below 100 mumol/L may be sufficient to prevent seizures in older infants who have adapted to neuronal glycine exposure. No toxicity of sodium benzoate treatment was detected when administering doses of up to 470 mg/kg BM per day but side effects such as itching and hyperactivity were obvious.

Benzoates↗

[Aarskog syndrome in association with mental and psychological retardation, grand mal epilepsy and tardive dyskinesia and apparent radicular paralysis of the fibular nerve in torsion scoliosis].

The case of an about 22-year-old patient with AARSKOG-syndrome is reported whose scoliosis caused a radicular impairment with resulting severe paresis of the right fibular nerve. The syndrome was also associated with moderate mental retardation, epilepsy, premature craniosynostosis and tardive dyskinesias after neuroleptic therapy because of disorders of behaviour. The risks of idiopathic scoliosis in patients with dysmorphia-retardation syndromes should be taken into consideration and such children should be supervised by an orthopaedist.

Abnormalities, Multiple↗

[Early infantile autism and excessive aerophagy with symptomatic megacolon and ileus in a case of Ehlers-Danlos syndrome].

A case of Ehlers-Danlos syndrome is reported, which was associated with mental retardation and early infantile autism. The patient died at the age of 19 by ectasy of the stomach as the result of extreme aerophagy causing a megacolon and by compression so finally resulting in a mechanical ileus. Investigation of the cerebellum revealed a significant rarefication and diminuation of the Purkinjè cells as well as the cells of the stratum granulare in the lobuli VI and VII, obviously due to a genetically determined malformation.

Adolescent↗

Controlled trial of a single dose of synthetic surfactant at birth in premature infants weighing 500 to 699 grams. The American Exosurf Neonatal Study Group I.

In a multicenter, double-blind, placebo-controlled trial conducted at 23 hospitals in the United States, a single prophylactic 5 ml/kg dose of a synthetic surfactant (Exosurf Neonatal) or air placebo was administered shortly after birth to 215 infants with birth weights of 500 to 699 gm. Despite stratification at entry by birth weight and gender, by chance female infants predominated in the air placebo group and male infants predominated in the surfactant group. Among infants receiving synthetic surfactant, improvements in oxygen requirements were significant at 2 hours after birth (p = 0.014) and persisted for 3 days (p = 0.001); improvements in the alveolar-arterial partial pressure of oxygen gradient were significant at 6 hours after birth (p = 0.01) and persisted for 3 days (p = 0.008). Improvements in mean airway pressure were not significant at 2 or at 6 hours after birth (p = 0.622 and 0.083, respectively), but became significant thereafter and persisted for 3 days (p = 0.002). Pneumothorax was reduced by slightly more than half (25 vs 11; p = 0.014); death from respiratory distress syndrome (RDS) was also reduced (26 vs 15; p = 0.046). Overall neonatal mortality, however, was not significantly reduced (58 vs 46; p = 0.102). Other complications of RDS and prematurity were not altered, except that pulmonary hemorrhage occurred significantly more frequently in infants receiving synthetic surfactant (2 vs 12; p = 0.006). These findings indicate that a single prophylactic dose of synthetic surfactant in infants weighing 500 to 699 gm at birth improves lung function, incidence of air leak, and death from RDS but not overall mortality. The only safety problem identified was an increase in pulmonary hemorrhage.

Bronchopulmonary Dysplasia↗

[Neuropsychiatric manifestations of Saethre-Chotzen syndrome].

Four psychiatric patients with SCS are described. All exhibited essential tremor, minimal cerebral dysfunctions, speech disorders, bradyphrenia, disorders of personality with psychasthenie and suspicious traits, restrictions, increased irritability and depressivity, one case of mutism due to abnormal reaction and one of alcohol hallucinosis. The possibility of cosmetic handicap caused by craniofacial dysplasia should be taken into mind and perhaps surgically corrected. Because the craniofacial dysplasia is mostly moderate and the syndactylies are slight the SCS remains often unrecognized, but apart from psychic maldevelopment it is significant as the result of frequent association with other malformations.

Acrocephalosyndactylia↗

[Primary hypogonadism associated with neuropsychiatric disorders].

Among 43 female patients aged 17-46 years, most with severe oligophrenia, there were 4 with primary hypogonadism (olfactory-genital dysplasia, Smith-Lemli-Opitz syndrome and lastly a Kanner syndrome). The incidence of genital underdevelopment is assumed to be higher among mentally retarded female patients. In cases of hypogonadism and hypogenitalism a search should always be made for possible mental and neurological disorders.

Abnormalities, Multiple↗

[Hirschsprung-Galant infantilism].

Report about a 17 1/2 year-old girl with severe mental retardation, dwarfism, hypogenitalism and short segment type of HIRSCHSPRUNG's disease, abortive SMITH-LEMLI-OPITZ syndrome is supposed. Literature about etiology and genetics of HIRSCHSPRUNG's disease is reviewed.

Abnormalities, Multiple↗

[Oculo-auriculo-vertebral dysplasia with abnormal social development].

Report of a 17-year-old boy with oculo-auricolo-vertebral dysplasia. He exhibited microphthalmos on the left and auricular deformity at hemifacial microsomia, scoliosis, dysraphia and other malformations of vertebras and ribs further other minor anomalies of face and hands. The disfiguring microphthalmia with ptosis was an essential cause of a social maldevelopment with temporary important aggressivity. Patients with oculoauriculovertebral dysplasia should be examined for associating cardial and renal dysplasias and cases of psychosocial disorders treated psychotherapeutically in time.

Adolescent↗

[Chotzen-Saethre syndrome with oligophrenia and psychological abnormal development].

A weak minded man aged 31 years exhibited with moderate acrobrachycephalia, basal cutaneous syndactylies of the 2nd and 3rd fingers, marking symptoms of the Saethre-Chotzen syndrome (SCS), with dominant inheritance in three generations. Additionally there were symptoms of Ascher's syndrome, blepharochalasis, goitre and broad lower lip. The psychic maldevelopment is considered in association with a syndrome of the frontal lobe. Severe craniostenosis is rare at SCS; cosmetic handicap and obstruction of the nasal airway can be corrected by craniofacial surgeon.

Acrocephalosyndactylia↗