Biomedical subjects
Publications and source records attributed to R Boldrini.
A case of brown tumor of hyperparathyroidism in a young girl with a long history of chronic renal failure and hemodialysis therapy is reported. The diagnosis can be reached with the aid of the classic skeletal radiological sign which address the evaluation of both CT and MR imaging.
The clinical, morphological and biochemical findings of a sporadic case, showing accumulation of desmin-type intermediate filaments in skeletal muscle and myocardium are described. Desmin storage was demonstrated by immunofluorescence, sodium dodecyl sulfate gel electrophoresis and two-dimensional gel electrophoresis. These findings are in agreement with those of Rappaport et al. (FEBS lett. 231:421-425, 1989). A sensory-motor polyneuropathy was established by electrophysiological studies and, ultrastructurally, intramuscular nerves showed accumulation of neurofilaments and neurotubules with formation of axonal spheroids. These findings are discussed considering all previous reports with related conditions.
Testicular dermoid cysts are rare in the pediatric age group: their discovery in an undescended testis is exceptional. The origin of this lesion is still unclear, but it should be considered as a monolayer expression of a mature teratoma. Though surgeons when confronted with these intratesticular cysts have traditionally opted for castration, in presence of small, compact masses, simple enucleation may be recommended, provided that the integrity of the neighboring parenchyma is not affected.
Pulmonary interstitial emphysema (PIE) may occur spontaneously, as a complication of endotracheal tube displacement, intrauterine viral pneumonia or massive aspiration of formula. Nevertheless PIE occurs more frequently in neonates requiring mechanical ventilation for RDS. Untoward effects of large air collections in the extra-alveolar spaces are based on decreased perfusion and ventilation of the affected lung tissues, compression of adjacent pulmonary parenchyma and mediastinum, possible air embolism. Sometimes PIE spontaneously regress, but in some instances the process is self perpetuating, leading to an "air block syndrome". Fourteen (19.4%) of 72 infants ventilated for RDS in a three-year period had radiological evidence of PIE during the first few days of life. All 5 bilateral, 7 diffuse and 2 localized cases were treated with "vigorous pulmonary therapy". In addition to these procedures, 4 neonates were submitted to a selective bronchial intubation (SBI) and 5 different infants improved after HFV. No one of our patients underwent a surgical procedure. Infants with fine linear hyperlucencies improved sooner. All pneumothoraces (7 of 14) were preceded by X-ray appearances of PIE. Three neonates died. Mortality was observed in newborns with bilateral PIE, because of an intraventricular hemorrhage in two and an intractable under tension pneumothorax in one patient. Plain chest roentgenograms, histological pictures and treatment modalities of PIE remain separated from these considered for congenital lobar emphysema, congenital cystic adenomatoid malformation, bronchogenic cyst or lung sequestration. Surgical treatment of PIE is not as universally accepted as in congenital cystic lesions of the lung, in which it's mandatory.
One of the aims of necropsy at neonatal and infantile ages is to identify the causes of death in this population, which is decreasing continuously because of the decay in natality seen in Italy as well as in other western countries over the last few decades. Analysis of 2410 autopsies performed on 92% of deaths in infants under one year of age occurring at the Bambino Gesù Hospital in Rome in 1974-89 shows a striking change in the principal causes of death in recent decades: Malformations, neonatal anoxia and immaturity have now become the main causes of death in the first year of life, while there is a very low rate of nutritional and infectious diseases, which predominated in the past. The autopsy results obtained indicate, however, that the policy of paediatric care in public health systems must be changed.
A muscle biopsy and autopsy study of a child who died at 14 months of respiratory failure is described. A diagnosis of infantile cytoplasmic body myopathy was made due to the high percentage of cytoplasmic bodies (CBs), particularly in respiratory muscles. No pathological abnormalities were found in the central nervous system, peripheral nerves or visceral organs. Immunohistochemical studies suggested that the central core of CBs was stained for fibrillary actin, being surrounded by a positive signal for desmin. A differential diagnosis as to other conditions involving proliferation of CBs is discussed.
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The authors report on a case of disseminated Langerhans' cell histiocytosis with a clinical presentation and a bone marrow simulating, at onset, an acute leukemia non lymphoid. A hepatic needle biopsy performed for the progressive enlargement of the liver oriented the diagnosis towards a Langerhans' cell histiocytosis. The morphological, immunohistochemical and ultrastructural study of these cells showed them to be undetermined, i.e. Langerhans' cell precursors.
Since there is little information regarding the possible prognostic significance of tumor rupture in localized neuroblastoma, we have analyzed the clinical courses of 163 children registered from 1979-1990 in 12 italian pediatric oncology Centers participating in the Neuroblastoma Cooperative Group of the A.I.E.O.P. (Italian Association for Paediatric Haematology-Oncology). Ten instances (6%) of tumor rupture were described. Ruptures occurred preoperatively in one child, during the operation in 9; among these 9, two were provoked by the surgeon to allow radical tumor excision, 7 were accidental. Of these 10 children, 7 relapsed at 3-25 months (median, 8 months) from diagnosis. Relapses were local in 5 children (2 of the 5 died), disseminated in one (who died), local + disseminated in one (presently alive with disease). Two local relapses were followed by bony or haematologic spread at 4 and 8 months, respectively. Of the 7 children who relapsed, 2 are alive in complete remission at 29, 100 months, respectively; two are alive with disease at 3 and 65 months, 3 died at 8, 15 and 24 months, respectively. We conclude that rupture of a localized neuroblastoma is a factor predisposing to relapse and may compromise the chance of cure. The surgeon should be aware of the risks connected with this complication and make any effort to avoid it.