27 research outputs found

    Intrapartum stillbirth for sepsis complicating Arabin cervical pessary placement in a twin pregnancy

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    There is little evidence regarding the best treatment in case of dilated cervix and exposed membranes in twins. Current options for its management include vaginal progesterone, cervical cerclage and cervical pessary, but none of them had shown effectiveness compared to expectant management. We presented a case of twin pregnancy at 22 6/7 weeks gestation admitted to the hospital because of cervical insufficiency with bulging of membranes. An Arabin pessary was positioned after a failed attempt of cervical cerclage and no antibiotic was given in absence of signs of infection. Cesarean delivery was performed at 24 weeks gestation because of spontaneous preterm labor and spontaneous rupture of membranes with the first baby in a transverse lie position. At the uterus section, the first baby was stillborn, with Arabin pessary strictly adherent to his ecchymotic head, while the second baby was born alive. After diagnostic histopathological and microbiological investigations, we hypothesized that the first twin died for funisitis/sepsis and mechanical insult due to the strict adhesion of the pessary to the fetal head. We concluded that in case of bulging membranes and dilated cervix, antibiotic treatment should be evaluated, also in absence of signs/symptoms of infection or suspicion of rupture of the membranes, and pessary insertion should be avoided, namely at second trimester, because of the risk of its dislocation inside the uterus when contractions start and potential hurt to extremely preterm fetus

    Growth in children after bone marrow transplantation.

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    Growth velocity pattern and growth hormone (GH) secretion were evaluated in 18 prepubertal patients (13 males, 5 females), receiving an allogeneic (7 patients) or autologous (11 patients) bone marrow transplantation (BMT). Children were affected by oncological or hematological malignancies and the age range was between 2 and 11 years. Nine patients received a conditioning regimen consisting of chemotherapy and fractionated total body irradiation (TBI) (12 Gy in 6 fractions over 3 days), whereas 9 children also received previous prophylactic cranial irradiation during first-line chemotherapy. GH secretion in response to pharmacological stimuli (insulin, arginine and/or L-Dopa) was evaluated when growth failure occurred. The 9 prepubertal patients who had received previous prophylactic cranial irradiation during first-line chemotherapy, showed a significant decrease in growth rate already 1 year after BMT and this reduced growth rate presented a progressive further decrease in the 2nd and 3rd year after BMT. On the contrary, in the 9 prepubertal children treated with TBI and chemotherapy alone, growth rate presented an impressive decrease only during the 3rd year. In the two groups of patients, pretransplantation growth rates were comparable, while, due to the earlier growth failure in children receiving TBI and previous prophylactic cranial irradiation, mean standard deviation score (SDS) significantly differed at 1 and 2 years following BMT. Such a difference disappeared at 3 years after BMT, because of the late decrease in growth rate in patients given TBI and chemotherapy alone.(ABSTRACT TRUNCATED AT 250 WORDS

    Muscle phosphofructokinase deficiency in a myopathic child with severe mental retardation and aplasia of cerebellar vermis

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    Muscle phosphofructokinase (PFK) deficiency in man is responsible for at least two forms of myopathy; one is characterized by painful contractures of muscles and typically occurs in adults, whereas the other is often disabling and typically occurs in childhood, with psychomotor and growth retardation. In this investigation, a young myopathic patient with severe mental retardation and aplasia of the cerebellar vermis presented with muscular hypotrophy of the limbs, generalized hypotonia, convergent strabismus and marked pain during passive movement. Biopsy of quadriceps femoris muscle showed variation in the fiber size with sarcoplasmic areas positive for periodic acid-Schiff stain. Histochemical qualitative reaction for PFK showed no staining of muscle fibers; ultrastructural studies showed abnormal accumulation of glycogen granules in both intermyofibrillar and subsarcolemmal areas. While some enzyme activities in the muscular crude extract were significantly lower than in controls, direct assay of PFK revealed no activity, thus demonstrating that the child's myopathy was due to the lack of PFK activit
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