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Biomedical subjects

A Ruggiero

Publications and source records attributed to A Ruggiero.

67 records · Page 4Linked to original sources
PubMedDate not supplied

Second case of hepatoblastoma in a young patient with Simpson-Golabi-Behmel syndrome.

Simpson-Golabi-Behmel syndrome (SGBS) is an X-linked overgrowth syndrome associated with multiple congenital anomalies caused by a mutant X-linked recessive trait. The authors report on a 14-month-old male patient affected by hepatoblastoma. As far as is known, this is the second patient reported with SGBS and hepatoblastoma. The observations emphasize that an increased risk of neoplasia in SGBS must be kept in mind, especially in young patients.

Genetic Diseases, X-Linked↗
PubMedDate not supplied

Early reactive neutrophil changes in an infant with Kawasaki syndrome.

An infant with Kawasaki syndrome presenting with fever, rash, and a high percentage of neutrophils with toxic granulation is reported. The peripheral blood smears of the patient showed a high proportion of neutrophils with marked vacuoles and toxic granulations. The reactive changes in neutrophils could be helpful in the early detection of the disease, especially in infants younger than 6 months of age who are at high risk for development of coronary artery disease.

Diagnosis, Differential↗
PubMedDate not supplied

Pulmonary hemorrhage complicating a typical hemolytic-uremic syndrome.

We describe a case of pulmonary bleeding and subsequent acute respiratory distress syndrome (ARDS) in a 20-month-old female suffering from a typical postdiarrheal hemolytic-uremic syndrome (HUS). Acute renal failure was treated early by peritoneal dialysis. It is of interest to underline that thrombocytopenia or any coagulative impairment was absent when this complication occurred, and spontaneous diuresis recovery was ongoing. All examinations failed to identify a unique site of bleeding in the main stem bronchi or trachea. Complete renal and clinical recovery was obtained in spite of this very uncommon complication. Intensivists should be aware of this rare and potentially fatal complication of typical (D+)HUS.

Acute Kidney Injury↗
PubMedDate not supplied

Alloimmune neonatal neutropenia treated with rHuG-CSF.

Infants with alloimmune neonatal neutropenia (ANN) may be at risk of life-threatening infection. Various modalities of treatment have been attempted but with differing results. We describe a case treated with rHuG-CSF. The use of rHuG-CSF should be considered in children with ANN especially when associated with life-threatening infection.

Female↗
PubMedDate not supplied

[Neutropenia in childhood].

The discovery of a neutropenia is a common problem in pediatric population. Some children with neutropenia have an uncomplicated clinical course and require no treatment. However, some patients are more severely affected and present a management challenge for the pediatrician and for the hematologist. The clinical approach to these children is directed primarily to understand the etiology of neutropenia in order to define a correct therapeutic behaviour, such as glucocorticoids, intravenous immunoglobulins or, more recently, rhG-CSF. In our article we have reviewed the etiology, the clinical course and the therapeutic options of constitutional and acquired neutropenias.

Child↗
PubMedDate not supplied

[Rhabdoid tumor of the kidney: a rare malignant neoplasm of infancy. A case report and review of the literature].

Rhabdoid tumor of the kidney (RTK) is a quite rare malignant neoplasm of early childhood. It has a very unfavourable prognosis, since it tends to give early metastases and shows a poor response to chemotherapy regimens. We report a case of an infant with RTK, who had a rapidly progressive course. Based upon our case and the review of the literature, we would like to stress the importance of a differential diagnosis with another kidney cancer, namely Wilms tumor, which is more frequent and has by far a better prognosis.

Diagnosis, Differential↗