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Biomedical subjects

J Ferrando

Publications and source records attributed to J Ferrando.

At least 127 records · Page 7Linked to original sources
PubMed1975

[Hepato-erythrocytic porphyria. A new type of porphyria].

A case of porphyria with symptomatology beginning in early infancy with peculiar biochemical features is described. There were crops of blisters, an extraordinary hypertrichosis and scarring of the exposed skin. The porphyrin examination showed an abnormal excretion pattern of urinary porphyrins with large amounts of 5-COOH carboxyle porphyrins and the protoporphyrin level of the red cells was raised. There was fluorescence of the erythrocytes and erythroblasts. The porphyrin excretion pattern of the stools was similar to those of the PCT. This case is closely similar to another published by the authors in 1969 and to 2 published by Schneider and Simon. The authors consider that could represent a new type of porphyria.

Child↗
PubMed1975

[Ultrastructural and cytological study of atypical fibroxanthoma].

A case of atypical fibroxanthoma of the skin is related. Ulstrastructural findings include: fibroblast-like cells, histiocytic-like cells, xanthomatous cells, and multinucleated cells. Strikingly similar findings were found by Fu and cols. (1975) in four cases of malignant fibrous histiocytoma. The authors postulated that both tumours could represent different aspects of a single proliferating process.

Aged↗
PubMedDate not supplied

Maximal biliary transport of sulfobromophthalein in patients with a T-tube placed in the common bile duct.

In 19 adult patients with choledocholithiasis who were operated on, excretion of free and conjugated sulfobromophthalein (BSP) in the bile collected through a T-tube inserted in the common bile duct was determined. The transport maximum (Tm) for BSP was calculated by the constant-infusion technique after an intravenous infusion of the dye at a rate of 0.3 and 0.09 mg/kg/min for the first and second hour, respectively. Free and conjugated BSP were measured in blood samples obtained at 30, 40, and 50 min of each hourly-infusion period, and in bile collected during the first 30 min (sample A) and between 30-50 min (sample B) after starting the first BSP infusion, and during the first 30 min (sample C) and between 30-50 min (sample D) after starting the second infusion. No correlations between Tm of BSP and glutathione transferase activity and between Tm and bilirubin and alkaline phosphatase in serum were found. Although there was an overall correlation between Tm of BSP and biliary excretion of BSP after 30 min of starting the BSP infusion (samples B, C and D) (r = 0.4716; P = 0.41), Tm values were always lower than recoveries of free BSP in bile. It seems that Tm of BSP (measured with the Wheeler's method) overestimates the actual values of biliary excretion of free BSP, and that the percentage of conjugated BSP in serum is related to the degree of impairment of biliary transport of BSP.

Aged↗
PubMedDate not supplied

[Social support and perceived health status in non-institutionalized population over 60 years of age].

OBJECTIVES: The purpose of this study is to describe the degree of social support in a sample of non-institutionalized population aged 60 and over and to explore possible relations between social support and perceived health. METHODS: We analyse data coming from Barcelona 1992 Health Survey. Sociodemographic and perceived health variables are analysed together with several social support variables based on the presence and number of available people for instrumental and emotional support necessity and perceived aid need for personal care and daily needs. RESULTS: In the study period, 1156 subjects aged 60 and over were interviewed at home. 20% of the sample (28.9% were women) lived alone and 28.2% (41.7% were women) were widowed. 43% had an extensive social support and 51% had a good emotional support. The men with less emotional support had a worse perceived health (OR = 2.7, IC 95% = 1.3-5.5). Only aid need for daily works in women was associated with worse perceived health (OR = 6.3, IC 95% = 3.2-12.4). CONCLUSIONS: Instrumental and emotional support availability weren't associated with perceived health in women in spite of they lived alone in a greatest proportion. In men, only emotional support absence was associated with worse perceived health.

Aged↗
PubMedDate not supplied

Loose anagen hair in hypohidrotic ectodermal dysplasia.

We report a 4 year-old boy affected with hypohidrotic ectodermal dysplasia in whom loose anagen hair syndrome (LAHS) was suspected clinically. The diagnosis was confirmed by examination of hair by optic and scanning electron microscopy. Loose anagen hairs have not been previously described in the ectodermal dysplasias. It is possible that our patient had real LAHS or an ectodermal dysplasia with loose anagen hair as an epiphenomenon.

Child, Preschool↗
PubMedDate not supplied

Trichothiodystrophy associated with urologic malformation and primary hypercalciuria.

Trichothiodystrophy (TTD) is a hair abnormality that may be associated with a large number of alterations affecting the skin phenotype and skin appendages, nervous system, eyes, bones, and immune, gonadal, and endocrine systems. We report the first case of TTD associated with a urologic malformation and primary hypercalciuria. Our patient had congenital ichthyosis, physical and mental retardation, and a dysmorphic facies associated with a complex urologic malformation and primary hypercalciuria. Characteristic features of his hair under microscopic examination (trichoschisis, alternating light and dark bands under polarizing microscopy, and a severely defective cuticle) and low sulfur content by radiographic microanalysis confirmed the diagnosis. We discuss the meaning of this new association in the spectrum of abnormalities related to TTD.

Abnormalities, Multiple↗
PubMedDate not supplied

Acquired partial curly hair.

We report 6 adolescent females apparently affected by diffuse partial woolly hair. The patients complained of thinning and curling of some hair of the scalp. Examination revealed two distinct hair populations. The first hair population was straight, normally pigmented and of normal length, thus considered "normal" hair. The second hair population was wavy, curly, thinner and apparently slightly shorter, thus considered abnormal. Direct examination of the abnormal hair population under a light microscope revealed a curled pattern of the hair shaft, and single torsions. With light microscopy the clinically normal hair had no abnormalities. Under scanning electron microscopy (SEM) the abnormal hair had curly and flattened hair shafts of 30-60 mum in diameter, oval shaped sections, canalicular formations, single torsions and cuticular weathering. With SEM, clinically "normal" hair had a diameter between 60-80 mum, with no major relevant abnormalities except for weathering. These results are suggestive of a new variant of acquired kinking (curling) of hair simulating diffuse partial woolly hair. The absence of clinical alterations in the proximal area of the affected hair and the spontaneous improvement with time, suggests that this variant may result from environmental exposure (weathering, cosmetic procedures) in predisposed individuals.

Adolescent↗