Renal involvement in Hansen's disease was evaluated in 94 Portuguese patients, average age and duration of disease of 47.6 and 6.8 years respectively. Sixty-seven were studied retrospectively and 27 prospectively; renal biopsy was obtained in 4, fat-tissue needle aspiration for amyloidosis in 20, and tubular function was tested in ten. Mild proteinuria and/or haematuria was found in 33 patients, the severity increasing during erythema nodosum leprosum reactions, but without overt nephritic or nephrotic syndrome. Two patients had renal amyloidosis on biopsy and two more were confirmed by fat biopsy, a 10.5% incidence in those studied prospectively; all but one were of the lepromatous type, with frequent bouts of erythema nodosum leprosum. The two other renal biopsies showed mesangial glomerulonephritis, and one unexplained acute tubular necrosis; none had immune deposits by immunofluorescence. Proximal acidification was always normal, distal acidification tested by bicarbonate infusion was abnormal in one of nine patients, and six of nine patients had concentration defects. Leprosy causes frequent urinary sediment changes and concentration defects, usually without clinical expression; proteinuria and/or glomerular involvement is mainly due to amyloidosis.

Adolescent↗

PubMed1989

[Lichen amyloidosis with extensive skin lesions].

Lichen amyloidosus is a chronic pruritic skin disorder, unusual among Europeans, and classically seen over the shins. The exact nature of the amyloid fibril protein, in lichen amyloidosus, is a matter of controversy and no specific treatment is available. Diffuse cutaneous involvement in a 60-year-old portuguese man is described. New pathogenic aspects and recent therapeutic approaches are discussed.

Amyloidosis↗

PubMed1981

[Simple epidermolysis bullosa. Anatomo-clinical considerations with respect to 7 cases].

Seven patients, belonging to seven different families, are reported. The clinical picture is dominated by the bullous lesions which appeared during the delivery or soon after birth; in one patient (case 4) the lesions first appeared after the first year of life. Besides the bullae there were erosions, crusts, residual macules but no millia cysts or scars. The nails were affected in several patients; the mucosae were affected in 2 patients. The histological aspects of the lesions are described; in all the cases it was possible to establish the position of the cleavage in regard to the functional membrane. The patients were treated with many different kinds of treatment which included topical and systemic corticotherapy; recently alpha-tocopherol (Roche) was assayed; none of the medicaments showed efficacy. The management of the patients relies on protective measures in order to avoid the formation of new lesions; gentle and time consuming nursing care is necessary to treat the patients. This must be explained to the parents of the patients as the care will rely on them. It is important to explain also the basic cutaneous defect that provokes the skin trouble and the necessity to avoid trauma and the possibility of the appearance of a new child with the defect in any subsequent pregnancy. The situation improves with age and this form of E.B. is compatible with a normal adult life.

Adolescent↗

PubMed1981

[Incontinentia pigmenti (Bloch-Sulzberger. Report of a patient with cerebral changes].

The clinical case refers to a three months-old female patient with incontinentia pigmenti. The cutaneous lesions, were the predominant features of the dermatosis; it consisted in vesicles, bullae, hyperkeratosis and pigmentation forming bizarre patterns. Associated with the dermatosis there were neurological abnormalities, which consisted in cerebral ulegiria. The child died during an episode of bronchiolitis. The necropsy showed; ulegiria cerebral, severe bronchopneumonia lobar and the cutaneous lesions.

Brain↗

PubMed1976

[Treatment of female gonorrhea. Therapeutic results with pivampicillin-probenecid and penicillin procaine-probenecid combinations].

A controlled trial of the relative efficacies of procaine penicillin G and pivampicillin for single dose treatment of uncomplicated gonorrhea in female was performed. Confirmation of the disease was obtained by bacteriological isolation of Neisseria gonorrhea from uretral, cervical or rectal exudates inoculated in suitable media. Based upon their previous experience, the authors used probenecid, along with both antibiotics, as a way of increasing the effectiveness of these drugs. Twentynine patients received probenecid 1 g. followed by oral pivampicillin, in a single dose of 1,4 g. Therapeutical results were evaluated in twenty-four, with clinical and bacteriological cure of eighteen. The other group included fifty-two patients treated with probenecid 1 g. followed by a single intramuscular dose of 3.000.000 U. of procaine penicillin G. The therapeutical results in the last group were evaluated in forty patients, with clinical and bacteriological cure of thirty-three. According to the above mentioned results procaine penicillin G seems to offer a slightly superior probability of cure.

Adolescent↗