Feocromocitoma asociado a leiomiomatosis cutánea y uterina y cáncer renal en un paciente con una mutación germinal en el gen de la fumarato hidratasa / Pheochromocytoma associated with cutaneous and uterine leiomyomatosis and renal cancer in a patient with a germline mutation in the fumarate hydratase gene

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Nódulos subcutáneos en un recién nacido / Subcutaneous nodules in a newborn

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Lupus eritematoso neonatal: 4 casos y revisión clínica / Neonatal lupus erythematosus: 4 cases and clinical review

El lupus eritematoso neonatal (LEN) es una enfermedad poco frecuente del recién nacido debido al paso transplacentario de anticuerpos maternos anti-Ro/SSA, anti-La/ SSB y/o anti-U1RNP en la que las afectaciones cutáneas y cardiacas son las más destacadas. Realizamos un estudio retrospectivo de casos diagnosticados de LEN en los últimos 10 años en el Hospital Universitario Insular de Gran Canaria en el que se obtuvieron datos completos de 4 enfermos. En 3 casos se presentaron anticuerpos circulantes anti-Ro en la madre y en los neonatos, mientras que en el otro era anti-RNP. Dos madres estaban diagnosticadas de lupus sistémico, una de enfermedad mixta del tejido conjuntivo y otra de vasculitis leucocitoclástica. Las lesiones cutáneas consistieron en lesiones urticariformes y descamativas. Un paciente presentó ulceración. El estudio histológico de las lesiones urticariformes mostró un infiltrado perivascular inespecífico; las lesiones descamativas fueron compatibles con lupus eritematoso subagudo

Neonatal lupus erythematosus (NLE) is an infrequent disease in newborns caused by the transplacental passage of maternal Anti-Ro/SSA, Anti-La/SSB and/or Anti-U1 RNP antibodies. The most common manifestations are cutaneous and cardiac. We carried out a retrospective study of cases of NLE diagnosed in the last 10 years at the Hospital Universitario Insular in Gran Canaria. Complete data was obtained for 4 patients. Three cases had circulating Anti-Ro antibodies in the mother and in the newborns, while in the fourth case they were Anti-RNP. Two mothers were diagnosed with systemic lupus, one with mixed connective tissue disease and the other with leucocytoclastic vasculitis. The skin lesions consisted of urticaria-like and desquamative lesions. One patient presented with ulceration. The histological study of the urticaria-like lesions showed a non-specific perivascular infiltrate; the desquamative lesions were consistent with subacute lupus erythematosus

[Neonatal lupus erythematosus: 4 cases and clinical review]. / Lupus eritematoso neonatal: 4 casos y revisión clínica.

Neonatal lupus erythematosus (NLE) is an infrequent disease in newborns caused by the transplacental passage of maternal Anti-Ro/SSA, Anti-La/SSB and/or Anti-U1 RNP antibodies. The most common manifestations are cutaneous and cardiac. We carried out a retrospective study of cases of NLE diagnosed in the last 10 years at the Hospital Universitario Insular in Gran Canaria. Complete data was obtained for 4 patients. Three cases had circulating Anti-Ro antibodies in the mother and in the newborns, while in the fourth case they were Anti-RNP. Two mothers were diagnosed with systemic lupus, one with mixed connective tissue disease and the other with leucocytoclastic vasculitis. The skin lesions consisted of urticaria-like and desquamative lesions. One patient presented with ulceration. The histological study of the urticaria-like lesions showed a non-specific perivascular infiltrate; the desquamative lesions were consistent with subacute lupus erythematosus.

Evaluation of acute hepatotoxic effects exerted by environmental estrogens nonylphenol and 4-octylphenol in immature male rats.

Nonylphenol (NP) and 4-Octylphenol (4OP) have shown estrogenic properties both in vivo and in vitro. Researchers have known for years that estrogens induce a wide number of hepatotoxic actions in rodents. In order to study the acute hepatic effects exerted by NP and 4OP on rat liver the following endpoints were evaluated: relative liver weight (RLW), morphology, cell cycle and ploidy status, monooxygenase enzymes content and levels of both, cytosolic estrogen receptor (cER) and microsomal binding sites for estrogens (mEBS). Immature male Sprague-Dawley rats were injected intraperitoneally (i.p.) with 60 mg/kg of NP or 4OP for 1, 5 or 10 days. Despite the fact that RLW of the animals was not modified but any treatment, the histopathological study revealed the presence of an increase in the percentage of both, mitotic activity and Ki-67-labeling index (LI) in the livers from animals treated with alkylphenols in absence of any degenerative lesion. Furthermore, all the livers from alkylphenols-treated groups showed the presence of abnormal mitosis and c-mitosis. Although the levels of both, cER and cytochrome P450 (Cyt. P450) were not affected by any treatment, concentration of the mEBS was decreased after 10 days of treatment with alkylphenols. These findings taken together suggest that the exposition to alkylphenols induce cell proliferation and spindle disturbances and that these compounds are capable of modulating the expression of putative membrane receptors for estrogens.

Steroid hormone progesterone induces cell proliferation and abnormal mitotic processes in rat liver.

The aim of this study was to evaluate the acute hepatic effects exerted by the steroid hormone progesterone (PR) in the rat. Although the liver is not a target tissue for this hormone, a number of hepatic actions of PR have been described, and, furthermore, a specific binding site for PR (PBS) exists in rat liver microsomes. Immature male rats were treated intraperitoneally with 60 mg/kg PR per day for 1, 5 or 10 days, and different parameters were evaluated in order to detect possible alterations in liver cells. Morphological study of the livers did not present images of cytotoxicity in any group of animals. The presence of a clear hyperplasia of smooth endoplasmic reticulum (SER) was noteworthy, mainly seen in perilobular hepatocytes. Despite this SER increase, the levels of cytochrome P450 (Cyt P450) significantly decreased after 10 days of PR administration. Similarly, the concentration of PBS was significantly decreased after 10 days of treatment with PR. On the other hand, these studies revealed a clear increase of mitotic activity and Ki-67 labelling index in the livers of animals treated with PR; furthermore, livers of PR-treated animals showed an increased percentage of binucleate hepatocytes. Flow cytometry analysis showed that although ploidy status of liver cells was not modified in any case the percentage of diploid nuclei in S-phase decreased during treatment with PR. The most relevant finding was the presence of abnormal mitosis and c-mitosis in livers from animals from all PR-treated groups. This study demonstrates that PR (a) does not induce cytotoxicity although it can induce cell proliferation and spindle disturbances in liver cells, (b) may also modulate the drug-metabolizing liver enzyme function, and (c) downregulates the expression of its own microsomal specific binding site.