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Cystic fibrosis is a multisystem disease with extremely variable onset, symptoms and course. One of the onset modality but also a complication of the disease is the pseudo-Bartter syndrome, characterized by hyponatremia, hypochloremic dehydration and metabolic alkalosis in absence of any renal disease. This syndrome occurs more frequently in the first year of life and has a peak in the summer. In this article, we describe two cases of cystic fibrosis associated with pseudo-Bartter syndrome in childhood. Excluding every possible cause of metabolic alkalosis associated with hyponatremia was crucial for our diagnostic pathway, and the experience gained with the first case helped a lot with the second one., (Copyright by Società Italiana di Nefrologia SIN, Rome,Italy.)

Hepatocellular adenoma is a benign neoplasm that accounts for 2-4% of pediatric liver tumors. The inflammatory variant of the adenoma can be expressed at the clinical level only with the evidence of persistent elevation of the inflammatory indices, since the hepatic mass is itself silent unless there are complications. The main complications relate to the bleeding risk, the chronic inflammatory state and the low risk of malignant evolution. The predictive marker of hemorrhagic complication is the size and in particular if the diameter is greater than 5 cm surgical treatment is recommended. We describe the case of a 9-year-old girl with a hepatocellular adenoma in the telangiectatic inflammatory variant, with a clinical presentation of loss of appetite and high inflammatory indices.

Objective: Administration of cytomegalovirus (CMV)-specific immunoglobulins to pregnant women with primary CMV infection in order to inhibit viral activity., Materials and Methods: We considered 2 groups of patients including 24 pregnant women., Group a: 12 women with primary maternal-fetal CMV infection, shown by CMV culture and CMV DNA detection in 9 (75%) and by only CMV DNA detection in 3 (25%) of the amniotic fluid (AF) samples. These pregnant women were treated with infusions of CMV-specific immunoglobulins (200 U/Kg of maternal weight and 400 U/Kg of fetal weight to prevent CMV pneumonia and gastroenteritis). As control group we considered 15 pregnant women, 5 of whom had CMV-positive AF samples demonstrated by polymerase chain reaction (PCR) and 10 also by CMV culture., Group B: 12 women with primary CMV infection, who were treated with monthly infusions of specific immunoglobulins to prevent the transmission of CMV to the fetus. In the control group we followed up 53 patients who were not treated, including 15 subjects with CMV-positive AF samples, 20 with CMV-negative AF samples and 18 women who did not accept amniocentesis., Group a: All 9 neonates born to mothers with culture and DNA positive AF samples were CMV infected. On the contrary, the babies born to 3 women with only PCR-positive amniotic fluid were CMV-negative by culture and DNA detection. Of 9 neonates infected, 8 were asymptomatic and 3 became culture-negative before they were one year old. The only symptomatic baby (IUGR and ventriculomegaly diagnosed by ultrasound during the pregnancy) was treated with ganciclovir and foscarnet. Of 15 non-treated patients, 11 had ultrasound signs of placental and/or fetal CMV involvement. Seven of these (46%) aborted and 4 (27%) delivered neonates with severe symptomatic infection. The other 4 patients delivered oligo-symptomatic CMV infected neonates positive by culture or PCR only. Overall, the prevalence of symptomatically infected neonates or fetuses was significantly higher (p = 0.02) among non-treated than treated women. Moreover, the babies of treated women showed less prolonged (p = 0.01) viruria than those of non-treated patients., Group B: all 11 pregnant women, treated with immunoglobulins delivered CMV-negative neonates. One patient, who had interrupted infusions at the 24th gestation week, delivered an asymptomatic CMV infected baby. In the control group, 4 women with CMV-negative AF samples delivered neonates with asymptomatic infection. Of 18 patients who did not undergo amniocentesis, 9 (50%) aborted; of remaining 9 women, 3 (33%) delivered CMV infected neonates., Conclusions: Our data suggest that CMV immunoglobulins may be effective for treatment or prevention of fetal CMV infection.

A new orthodontic device is proposed by the author: an alloy layer welded to first upper bicuspids bands. It's a matter of an an activator-like device being no obstructing for tongue, cheeks and lips. To alter vertical dimension in oral cavity, it is not difficult to use the last generation of bonding agents.

The Author reports a case of chronic closed lock of TMJ treated by inferior repositioning appliance. In management of internal derangement is useful to perform a diagnostic follow up using tomographic images of TMJs before and during treatment. In this way you can drive the splint therapy and set a new position of the mandible up in the better way.