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Biomedical subjects

M Matrunola

Publications and source records attributed to M Matrunola.

14 recordsLinked to original sources
PubMed1999

Adult height in girls with central precocious puberty treated with gonadotropin-releasing hormone analogues and growth hormone.

GnRH analogues (GnRHa) represent the treatment of choice in central precocious puberty (CPP), because arresting pubertal development and reducing either growth velocity (GV) or bone maturation (BA) should improve adult height. However, in some patients, GV decrease is so remarkable that it impairs predicted adult height (PAH); and therefore, the addition of GH is suggested. Out of twenty subjects with idiopathic CPP (treated with GnRHa depot-triptorelin, at a dose of 100 microg/kg im every 21 days, for at least 2-3 yr), whose GV fall below the 25th percentile for chronological age, 10 received, in addition to GnRHa, GH at a dose of 0.3 mg/kg x week s.c., 6 days weekly, for 2-4 yr; and 10 matched for BA, chronological age, and duration of GnRHa treatment, who showed the same growth pattern but refused GH treatment, served to evaluate the efficacy of GH addition. No patient showed classical GH deficiency. Both groups discontinued treatment at a comparable BA (mean +/- SEM): 13.2 +/- 0.2 in GnRHa plus GH vs. 13.0 +/- 0.1 yr in the control group. At the conclusion of the study, all the patients had achieved adult height. Adult height was considered to be attained when the growth during the preceding year was less than 1 cm, with a BA of over 15 yr. Patients of the group treated with GH plus GnRHa showed an adult height significantly higher (P < 0.001) than pretreatment PAH (160.6 +/- 1.3 vs. 152.7 +/- 1.7 cm). Target height (TH) was significantly exceeded. The group treated with GnRH alone reached an adult height not significantly higher than pretreatment PAH (157.1 +/- 2.5 vs. 155.5 +/- 1.9 cm). TH was just reached but not significantly exceeded. The gain in centimeters obtained, calculated between pretreatment PAH and final height, was 7.9 +/- 1.1 cm in patients treated with GH combined with GnRHa; whereas in patients treated with GnRHa alone, the gain was just 1.6 +/- 1.2 cm (P = 0.001). Furthermore, no side effects have been observed either on bone age progression or ovarian cyst appearance and the gynecological follow-up in the GH-treated patients (in comparison with those treated with GnRHa alone). In conclusion, a gain of 7.9 cm in adult height represents a significant improvement, which justifies the addition of GH for 2-3 yr during the conventional treatment with GnRHa, especially in patients with CPP, and a decrease in GV so marked as to impair PAH, not allowing it to reach even the third centile.

Adult↗
PubMed1998

Crossed renal ectopia with fusion.

The case of three-year old girl with right to left crossed renal ectopia with fusion is described. This malformation presented only microhematuria and urinary tract infections. The diagnosis was established by means of biochemical and radiographic investigations.

Child, Preschool↗
PubMed1996

Primary pulmonary rhabdomyosarcoma in childhood: clinico-biologic features in two cases with review of the literature.

The cases of two children under three years of age with primary pulmonary rhabdomyosarcoma and no associated lung malformations are reported and a review of the literature is presented. In both, complete surgical removal of the tumor was performed and histologic examination revealed embryonal subtype. Flow cytometric assessment showed a tumor-cell diploid DNA content. Postoperative radio- and chemotherapy were carried out, but in spite of treatment both girls died because of disease progression, fourteen and nine months after diagnosis. The importance of associated cystic lung malformations and DNA content in predicting clinical outcome of primary pulmonary rhabdomyosarcoma is evaluated.

Child, Preschool↗
PubMed1996

Enucleative surgery for stage I nephroblastoma with a normal contralateral kidney.

PURPOSE: Tumor enucleation is not recommended for children with nephroblastoma and a normal contralateral kidney. However, in adults with unilateral low stage renal cell carcinoma tumor enucleation may offer an alternative to radical nephrectomy, since functioning renal tissue is preserved without a greater risk of residual microscopic disease. Enucleative surgery may be more reasonable in children with nephroblastoma, because the risk of relapse can be reduced with chemotherapy. Therefore, we prospectively evaluated the feasibility of enucleative surgery in children with stage I unilateral nephroblastoma. MATERIALS AND METHODS: Between 1992 and 1995, 13 children with nephroblastoma and a normal contralateral kidney were consecutively admitted to our surgical unit. Possible candidates for tumor enucleation were evaluated according to certain criteria, including stage I disease at diagnosis, well-defined margins on post-contrast computerized tomography and at least 50% of the functioning kidney could be preserved. Preoperative and postoperative chemotherapy was given in all cases. RESULTS: Of 4 children with preservation of more than 50% of the functioning kidney 3 were considered eligible for enucleation. The tumors, which were confined to the mid kidney in 2 children and upper renal pole in 1, were successfully enucleated without hypothermia or vascular occlusion. All 3 children are disease-free at 49, 48 and 26 months of followup, respectively. Renal function has been almost completely restored postoperatively. CONCLUSIONS: These preliminary data suggest that enucleative surgery may be a reasonable option in select children with stage I nephroblastoma and a normal contralateral kidney.

Child↗
PubMed1996

[Spondylitis in infancy. A case report].

The authors report on a case of a spondylodiskitis in three year old child. Diskitis is an inflammatory process of the intervertebral disk space, not often described in pediatric age. The etiology is infective and generally presents with lumbar spine localization. Clinically it is characterized by lumbar pain at times radiating to a lower extremity. Symptoms are aspecific and radiological signs do not appear for several weeks after the onset of symptoms. Interest in this pathology derives from the fact that it may be more common than previously recognized. This case offered us the opportunity to review principal symptoms of diskitis and to describe the main diagnostic technics used.

Child, Preschool↗
PubMed1996

Delayed gastric emptying in an infant with Sandifer syndrome.

The case of an infant with Sandifer syndrome is reported. Real-time ultrasonography showed delayed gastric emptying time, which returned to normal when the patient was asymptomatic. The importance of gastric motility investigations in Sandifer syndrome is stressed since delayed gastric emptying could play a role in the pathogenesis of this disease.

Female↗
PubMed1995

[Budd-Chiari syndrome in children: case report].

This work aims at describing a particular case of Budd-Chiari syndrome in a ten-year-old girl. In our patient, the subjective and objective features of this disease, which is already rare during childhood, are associated with a well-assessed polyposis in the blind intestine (intestinum caecum). In our opinion, a possible connection between these two pathological events, a connection unheard of in the literature so far, is an interesting issue to be dealt with in this case report. The follow-up of our patient took place at the Pediatric Hospital of the University of Rome "La Sapienza" and lasted an overall period of 6 months. The girl was hospitalized twice and she underwent a large number of radiological and laboratory tests. The therapy consisted only of medical treatment and support measures. Liver transplant is regarded as the only chance to obtain a full recovery, but it has not yet been performed on our patient. It is by comparing those researches carried out on similar cases with the outcome of our personal study, that we come to the conclusion that, as for our patient, the Budd-Chiari syndrome can be classified as an idiopathic syndrome. Moreover, the fact that this syndrome and the polyposis of caecum occur at the same time in a patient can probably be considered as a coincidence.

Budd-Chiari Syndrome↗
PubMed1995

[Crossed renal ectopy with fusion. Report of a case].

Crossed renal ectopia is a rare urinary tract anomaly, often associated with malformations involving various organs and systems. We report a case of crossed renal ectopy with fusion, without associated abnormalities. Recurrent abdominal pain, sometimes with microhematuria, were the clinical features, never accompanied by alteration of the renal function.

Abdominal Pain↗
PubMed1995

[High resolution computerized tomography in cystic fibrosis. Clinico-radiologic correlations in 25 patients].

Twenty five patients with a clinical and radiological diagnosis of cystic fibrosis underwent high resolution Computed Tomography (HRCT) with the aim to study pulmonary parenchymal localization of disease. Both patients with initial pulmonary alterations and patients in a more advanced phase of the disease were studied. HRCT proved an excellent method for detecting very early lesions such as bronchiolar ectasia and bronchiolar obstruction by mucous accumulation. Moreover, HRCT proved to be very useful in detecting centrolobular and panlobular parenchymal lesions, with diagnostic information on axial and peripheral interlobular connective tissue. HRCT provided a better spatial definition of bronchiectasia and subpleural air if compared to conventional radiology. It also allowed for correct diagnosis of pneumothorax and detection of pleural fibrosis as a result of iatrogenic complications.

Adolescent↗
PubMedDate not supplied

Ultrasound in the management of hepatic veno-occlusive disease in three children treated with dactinomycin and vincristine.

Three children, treated with dactinomycin and vincristine without radiotherapy showed hepatic toxicity consistent with diagnostic criteria for hepatic veno-occlusive disease (VOD). Two patients were affected with Wilms' tumor and the third with malignant fibromatosis. The clinical manifestations of VOD were mild and regressed in all patients after supportive therapy. Serial ultrasonography (US) was performed in all cases and was useful in confirming the diagnosis and in evaluating the severity of the disease. US features of VOD were hepatomegaly, gallbladder wall thickening, ill-defined borders of the hepatic vessels, and ascites. Parenchymal heterogeneity was still present after several months as a result of the hepatic injury. The hazy appearance of the portal vessels and the gallbladder wall thickening seemed directly correlated with the degree of hepatic involvement in the early phase of VOD.

Child↗
PubMedDate not supplied

[Bilateral intravesical ureteroceles associated with hydroureteronephrosis and hyperechogenic spots in kidneys. Report of a neonatal case].

Ureterocele is a cystic dilatation of the terminal ureter. In children it is most commonly ectopic and associated with the duplex collecting system, less frequently it is intravesical and associated with the single collecting system. We present the radiological and ultrasound findings in a male newborn with bilateral intravesical ureterocele associated with hydroureteronephrosis and hyperechogenic spots in both kidneys. In the literature this association has never been reported in neonatal age.

Humans↗
PubMedDate not supplied

[The interpretation of a negative intradermal reaction to tuberculin in a case of tuberculous lymphadenitis].

This work aims at describing a particular case of tuberculous lymphadenitis with negative tuberculin skin test in a five-year child who has afterwards developed Hodgkin disease. BK presence is initially demonstrated through the examination of a first bioptic sample of a lymph-node, then confirmed by the presence in the serum of mycobacterial antigens on the peripheral lymphocytes and monocytes. Nine months later the second bioptic sample puts in evidence the presence of Reed-Sternberg cells. The follow-up of our patient took place at the Pediatric Department of the University of Rome "La Sapienza" and lasted an overall period of twenty days. The child was hospitalized twice and underwent a large number of radiological and laboratory tests. The therapy consisted only of medical specific treatment. Our study results lead us to evaluate the presence of immunological suppressive mechanisms in some particular tuberculosis cases, justifying the tuberculin skin-test negativity. When Hodgkin's disease has turned up, it has been too difficult to outline a precise temporal sequence of events, we could only advance hypothesis.

Biopsy↗