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T K With

Publications and source records attributed to T K With.

At least 19 recordsLinked to original sources
PubMed1982

The first Swedish case of congenital erythropoietic porphyria, with a revised view of the porphyrin excretion pattern in this disease.

A case of congenital erythropoietic porphyria (CEP) in a Swedish boy is described. It is the second case of this extremely rare disease encountered in Scandinavia. Like the first Scandinavian case--a Norwegian boy--this patient showed a porphyrin excretion pattern like that of porphyria cutanea tarda (PCT) which was regarded as pointing to a hepatic type of porphyria. In the Norwegian boy this was regarded as atypical, but our study of the literature revealed two older cases with features pointing to a similar pattern and disclosed that analysis of the porphyrin pattern with modern methods, such as thin-layer chromatography, has not been performed in the great majority of the cases of CEP, because such methods were not available at the time these cases were published. Therefore, it is necessary to study more cases of CEP with modern analytical methods in order to get an adequate picture of the porphyrin pattern typical of this disease.

Child, Preschool↗
PubMed1979

Urinary total porphyrins by ion exchange analysis: reference values for the normal range and remarks on preformed prophyrins in acute porphyria urine.

Determination of total porphyrin with a rapid and east method (ion exchange; spectrophotometry) was performed on 57 morning urine samples from laboratory personnel, 59 arbitrary day urine samples from blood donors, and 90 24-hour urines from medical inpatients. The upper reference limit for morning urine was 0.32 mumol/l but even a value as high as 0.7 mumol/l was found. In the donor urines the upper limit was 0.155 mumol/l. The 24-hour urines showed an upper reference limit of 271 microgram/24 h. These values are in good agreement with values from the literature, mostly based on extraction analyses. Tracings of the absorption curves in the region of 380--430 nm were performed in all analyses and showed that the non-porphyrin absorption was close to linear in most cases. Studies of porphyric urines gave no support to the claim that preformed porphyrins not formed from porphobilinogen are excreted in this disease.

Acute Disease↗
PubMed1978

Ion exchange analysis of porphyrins in urine.

An easy and cheap analysis of total prophyrin in urine is described based on the ion exchange resin Dovex 2 X 8, 50--100 mesh. Recovery of added porphyrin varied between 65 and 90% for uro- and 70 and 95% for coproporphyrin. The use of the method with internal standard is described and exemplified. Ammoniacal solutions of crystalline free porphyrins were used as standards.

Humans↗