Biomedical subjects
Publications and source records attributed to J Kunze.
We report on the sensitization of a baker against alpha-amylase contained in a dough additive. Positive test reactions to alpha-amylase were observed after 20 min., 6 h, and 72 h, even following heat inactivation. Specific IgE, IgG, and IgM antibodies could be proved in the serum. We assume mixed reactions of type I, III and IV.
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We report two families with seven siblings with de Barsy syndrome. Characteristic features include severe mental retardation, hypermobility with athetoid movements, grimacing, muscular hypotonia, laxity of small joints and brisk deep tendon reflexes, progeroid aspect with cutis laxa, atrophy of skin with hyperpigmentation, isolated depigmentations, reduction of subcutaneous fatty tissue, translucent vein pattern, short stature, frontal bossing in the young child, large prominent ears with dysplastic helices and corneal clouding or cataracts. The syndrome probably has autosomal recessive inheritance.
A female newborn of healthy parents demonstrates the combination of postaxial hexadactyly type B of the hands (X-ray investigations show in addition duplicated terminal phalanges of the thumbs), preaxial hexadactyly of the toes, partial and total syndactyly of the fingers and toes. These findings are compatible with the diagnosis of Greig cephalopolysyndactyly syndrome. Dyscrania can not be demonstrated. In addition, our patient exhibits genu recurvatum on the left side resulting from intrauterine deformation. Sonography of the head reveals normal formation of the corpus callosum. The neurological status of our baby is inconspicious, and the psychomotor development up to the age of 2 5/12 years normal. The differential diagnosis of this characteristic combination of pre- and postaxial polydactyly has to consider the acrocallosal syndrome. In sporadic cases of Greig cephalopolysyndactyly syndrome it is important to investigate and follow the neurological status of the patients and in particular to use sonography to document the intact corpus callosum. Patients with Greig cephalopolysyndactyly syndrome have a normal development, children with the acrocallosal syndrome are retarded.
A total of 315 psoriasis patients were treated on the basis of short-contact "minutes" therapy: 230 with 0.1-3% dithranol-2% salicylic acid-white soft vaseline (DSV) for 10-20 min daily; 85 patients in left-right comparison with modified therapeutic schemes. The object was to study the influence of concentration, contact time, psoriasis type, self-treatment at home, frequency of application, ointment base, and the admixture of corticosteroids on the efficacy of "minutes therapy." The clearing quotient for the individual psoriasis types was varied; it reached on an average 75% with a treatment period of 29.4 days. Even lower dithranol concentrations below 1% proved efficacious with part of the patients. Self-treatment at home and irregular applications diminished the efficacy. Neither prolongation of the contact time to 1 hr nor the addition of corticosteroids to dithranol did anything to improve the therapy results. The relapse-free period averaged 3.9 months. Undesirable irritation was avoided to a great extent by adjustment of the treatment intensity to individual tolerances. The simultaneous application of dithranol and corticosteroids did not hinder or diminish the dithranol erythema. For additional safety, a preliminary test treatment can be confined to a limited area for 1 week. Fortunately, the staining due to dithranol brown can be reliably removed from certain textiles and from the bath tub or shower cabin by the use of hypochlorite.
We studied 14 biopsies of congenital melanocytic nevi of children up to 1 year of age. In 11 biopsies we found two different types of melanocytic cells. In the reticular dermis, they were small cells. They had small nuclei, little cytoplasm, and no detectable pigment. They did not come in nests, but were scattered between the collagen bundles. The other type of cells was found within the epidermis or closely under it. These cells were large and round, and had abundant cytoplasms and evenly distributed melanin. Sometimes these cells were arranged in nests and in two moles they were also found in the epithelia of follicles. In all of these biopsies (with three exceptions) the superficial, large pigmented cells were separated from the deep, small, nonpigmented ones by a zone of tissue without any melanocytic cell. Early in life both types of cells are clearly separated, and the superficial ones are few. At later ages the superficial large cells are found in increased numbers, whereas the number of the deep ones does not change with age.
The so called "minutes" therapy is a fundamental new method of psoriasis treatment with dithranol (anthralin). Dithranol is applied to the lesions for only 10 to 20 minutes and thereafter washed off thoroughly under a shower. We use an ointment of 0.5 to 3% dithranol with 2% salicylic acid in yellow soft paraffin which is applied with a plastic glove to the lesions. This regimen makes it now possible to offer dithranol routinely for home treatment. The patient should be well informed and must consult the dermatologist weekly. The course of treatment usually starts with 1% dithranol for 10 minutes. Depending on the healing effect and the degree of irritation, the concentration and the contact time is increased step by step. Up to now, 200 patients have been treated, 64 of them as in-patients and 136 carrying out the treatment at home. The clearance rate was 77.5% (84% for the in-patients and 74% for the outpatients). The average clearing time for out-patients was 5.5 days longer than for in-patients (31.5 to 26 days). The rate of non-responders in both groups was 10%. The application of corticosteroids or oral retinoids in addition to the "minutes" therapy showed no better therapeutic results. The "minutes" therapy has medical, social and financial advantages. Dithranol is very effective and has no permanent side-effects. Therefore, corticosteroids, retinoids and PUVA can be restricted. Treatment at home enables the patients to enjoy normal family and professional life. Finally, the "minutes" therapy saves large amounts of money in comparison to a hospital stay for several weeks.
Patch testing with Iodine/potassium-iodide (3%) and commercialized povidone (PVP)-iodine preparations (Betaisodona-ointment and solution, Polyvidon-Jod-ointment "Braun", Braunol-solution "Braun", Traumasept-ointment, and Traumasept solution--not marketed--, were carried out in 104 patients of the Department of Dermatology of the Municipal Hospital Kassel. In addition, the according ointment and solution-bases were tested. 22 tests were patients with ulcera crurum, 82 patients with infectious skin diseases or skin tumors. 35 out of the 104 patients showed positive reactions to iodine/potassium-iodide. Hyperreactivity to one or more PVP-iodine-ointments was found in 12 patients, to one or more PVP-iodine solutions in 29 patients. If there was no positive reaction to iodine/potassium-iodide, PVP-iodine ointments were always tolerated, while PVP-iodine-solutions produced positive reactions in 9 cases even then. Our results show that allergic skin reactions must be expected in 2-9% of all patients using PVP-iodine ointments, and in 12-20% of all patients using PVP-iodine-solutions. If iodine-allergy is known the rate of positive reactions to PVP-iodine ointments is 6-25% to the solutions 34-60%.
We report on a female newborn baby with a "myopathic" stiff face, open mouth, high-arched palate, microgenia, generalized muscular hypotonia, limited extension of elbows, wrists and knees, flexed adducted thumbs, velopharyngeal insufficiency, and hypertrichosis. Death occurred at 3 months due to respiratory insufficiency. Muscular biopsy revealed myopathic abnormalities.
Four boys are described with partial gigantism of the hands and/or feet, pigmented nevi, hemihypertrophy, subcutaneous hamartomatous tumors and macrocephaly, and/or other skull anomalies. Three of these patients showed an accelerated growth in their first years of life. Two suffered from cystiform pulmonary abnormalities. The children showed normal mental development with the exception of one with traumatic brain damage. Parental consanguinity was not disclosed. As a result of a review of the literature, we can say that these cases do not conform to any well defined entity and would appear to represent a 'new' syndrome to be categorized under congenital hamartomatous disorders. The mode of inheritance of the undoubtedly genetically determined syndrome is yet not clearly understood. We propose the term Proteus syndrome for this 'new' syndrome.
This paper reports on the synthesis and pharmacological activity of 6-aryl-4,5-dihydro-3(2H)-pyridazinone derivatives. The compounds exhibit an aggregation inhibiting action on human platelets in vitro and on rat platelets under ex vivo conditions, as well as a hypotensive action on rats. The strongest pharmacological effects were found with dihydropyridazinones, which have a 6-[p-[(chloroalkanoyl)amino]phenyl] substituent, together with a methyl group in the 5-position. The antiaggregation activity of compounds of this type is in vitro up to 16000 times and ex vivo up to 370 times greater than that of acetylsalicylic acid; the hypotensive action is up to 40 times as great as that of the comparative substance dihydralazine.
Whereas the omphalocele develops from a malformation of the mesoderm during the embryonal period, the gastroschisis appears to be the result of an intrauterine vascular occlusion; defined as disruption. Definite signs of malformations are seen in omphalocele. In our patient population observed during a three year period, eight cases of abdominal wall defects were seen, four of which with Wiedemann-Beckwith syndrome and one with trisomy 18, two with an isolated omphalocele and one with a gastroschisis. The concomitance of genetic malformation syndromes implies the necessity of intrauterine diagnosis evaluation. In proven cases of chromosomopathies and signs of omphalocele in ultrasound an interruption of pregnancy may be indicated. Similarly with very unfavourable prognosis such as Edwards syndrome, a post partum operative intervention is not necessary. This decision however can only be reached through interdisciplinary consultation between the pediatrician, pediatric surgeon, midwife and parents.
A total of 11 epileptic mothers treated with carbamazepine (CBZ) as well as their 12 newborns were included in this study. Maternal CBZ concentrations remained rather constant during pregnancy and slightly increased after parturition. Carbamazepine-10, 11-epoxide ( CBZE ) levels were less predictable and either increased or decreased during pregnancy. Fetal/maternal serum concentration ratios at birth were 0.78 +/- 0.14 (n = 5) for CBZ and 0.75 +/- 0.09 (n = 5) for CBZE . Neonatal half-lives were 28 +/- 11 hours (n = 4) for CBZ and 20 and 24 hours (n = 2) for CBZE . maternal milk/serum concentration ratios of CBZ and CBZE were 0.39 +/- 0.22 (N = 11) and 0.49 +/- 0.28 (n = 6), respectively. The steady-state CBZ serum levels of nursed infants were about 1.0 micrograms/ml in all cases but one, where a maximum concentration of 4.7 micrograms/ml was reached. One of the infants had major malformations. Minor anomalies were less frequent in the CBZ group, compared to the whole group of infants exposed to anticonvulsive drugs other than CBZ and as frequent as in a matched pair control group of unexposed neonates. Neonatal somatic data were found to be below the corresponding values of neonates exposed to antiepileptic drugs other than CBZ.
Clioquinol- and tribromphenolwismut zinc oxide preparations were tested in vitro in order to investigate their bactericidal and bacteriostatic efficacy against strains of staphylococcus aureus, E. coli, pseudomonas as well as against the fungi candida albicans and trichophyton rubrum. The results show that there was both a bacteriostatic and a bacteriocidal effect of clioquinol except against pseudomonas. Tribromphenolwismut has less efficacy. In spite of some suggestions in the literature our results confirm that the efficacy of clioquinol is not impaired if combined with zinc oxide. The use of this well tolerated preparation is recommended in the treatment of infectious dermatoses even today.
630 outpatients and inpatients under rest as well as under conditions of physical loads and psychic stress were examined with the help of a radiobiotelemetry device of own construction, which corresponds to a combination of traditional radiotelemetry and tape storage. In 81.4% of the patients with hitherto unclarified disturbances of the cardiac rhythm telemetry obtained their qualitative clarification. In 50% of all cases with classical angina pectoris symptomatology the ischaemic heart disease was verified by determination of the usual criterias of ECG at the telemetry stripe. Of these 50% only 7 patients had no arrhythmia. In 57.5% of all examined patients with pacemakers of permanent frequency we could prove a considerable parasystoly in load by telemetry. 9 patients with R-demand-pacemakers became evident by triggering defects in load. During the psychoanalytic examination we registered extrasystoles in 25%, apart from this once developed a pancardiac asystolism. The radiobiotelemetry is a valuable completion of the well-known methods of load.
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