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Biomedical subjects

M Guglielmi

Publications and source records attributed to M Guglielmi.

At least 37 records · Page 2Linked to original sources

Nodular regenerative hyperplasia of the liver: description of two cases.

Nodular regenerative hyperplasia (NRH) of the liver is a condition of unknown origin, rarely occurring in children, usually accidentally discovered, described in association with a variety of clinical conditions and drugs. Confusion with other types of hepatic masses may pose a problem and for this reason NRH is considered a "tumor-like lesion." Histologically it consists of single or multiple regenerative foci. Hepatic failure and rupture of the liver have been rarely described as complications in adults, and not in children, and malignant transformation has not been demonstrated. Neither surgical removal nor other treatment is needed. These features are described as they were found in two patients we encountered.

Biopsy↗

Necrotizing enterocolitis: late surgical results after enterostomy without resection.

Between January 1985 and December 1992, 36 neonates were treated for necrotizing enterocolitis (NEC). 13/36 were treated conservatively and 2 (18%) died. 23/36 required surgical treatment at birth and 6/23 (26%) died; 14, out of 17 surviving initial surgery, underwent reintervention to restore intestinal continuity, 3 to 26 months later. In these 14 infants the acute necrotizing process involved the whole colon and ileum in 3 cases, the whole colon in 5, part of the colon in 3, part of the colon and ileum in 2 and was confined to the ileum in 1. 11 cases were perforated. During their first laparotomy all 14 cases had a decompressive enterostomy without resection. 21 reoperations were later needed in these 14 patients: simple enterostomy closure was performed in only 1 case, while resection of delayed strictures was required in 13. 12 cases underwent partial colectomy, 3 of these had multiple segmental resections for skip lesions and only 1 child had a total colectomy. The only postoperative complication was one anastomotic leak. Two children had short-gut syndrome but later outgrew their need for parenteral nutrition. Our experience does not support the need of resecting the gangrenous bowel in the acute phase. A simple diverting enterostomy seems to be effective treatment. Delaying the intestinal resection up to the time of recanalisation allows spontaneous healing and results in the sparing of a significant length of bowel.

Colectomy↗

Resection of primary tumor at diagnosis in stage IV-S neuroblastoma: does it affect the clinical course?

PURPOSE: To determine whether resection of primary tumor has a favorable influence on outcome of infants (age 0 to 11 months) with stage IV-S neuroblastoma. PATIENTS AND METHODS: Between March 1976 and December 1993, 97 infants with previously untreated neuroblastoma diagnosed in 21 Italian institutions were classified as having stage IV-S disease. Seventy percent were younger than 4 months. Adrenal was the primary tumor site in 64 of 85 patients with a recognizable primary tumor. Liver was the organ most often infiltrated by the tumor (82 patients), followed by bone marrow and skin. RESULTS: The overall survival (OS) rate at 5 years in 80% and event-free survival (EFS) rate 68%. In 24 infants, the effect of resection of primary tumor could not be evaluated because of rapidly fatal disease progression (n = 8), absence of a primary tumor (n = 12), or partial resection (n = 4). Of 73 assessable patients, 26 underwent primary tumor resection at diagnosis: one died of surgical complications, one relapsed locally and died, and two others relapsed (one of these two locally) and survived, for a 5-year OS rate of 92% and EFS rate of 84%. Of the remaining 47 patients who did not undergo primary tumor resection at diagnosis 11 suffered unfavorable events, of whom five died, for an OS rate of 89% and EFS rate of 75% (no significant difference from previous group). Disease recurred at the primary tumor site in only one five who died, and in only one of six survivors of progression or relapse; in these patients, the primary tumor, located in the mediastinum, was successfully resected. CONCLUSION: Infants who underwent resection of the primary tumor at diagnosis had no better outcome than those in whom the decision was made not to operate.

Adrenal Gland Neoplasms↗

A simple exam to screen superficial masses: fine-needle aspiration cytology.

Fine-needle aspiration cytology (FNAC) is now considered a useful tool in the evaluation of adult patients with mass lesions. We reviewed the experience with FNAC in our Paediatric Surgical Department. One hundred and eleven FNACs were performed in children with a superficial mass and no definite diagnosis. All the exams were done by the same physician using a fine needle and no anesthesia. We routinely used May-Grüwald-Giemsa and Papanicolaou staining. Patients age ranged from 20 days to 17 years, with a mean age of 6.5 years. A clinically benign pathology was cytologically confirmed in 90 cases (81%). All children did well at follow-up. Malignancy was diagnosed in eight cases (7.2%) and in all was confirmed with a surgical biopsy. In nine children (8.1%) the specimen was considered insufficient for definite diagnosis. In four cases (3.6%) the pathologist diagnosed a possible malignancy that was excluded at surgical biopsy. The sensitivity was 100% and the specificity was 96%. Our experience confirms that FNAC is a fast, cheap, simple, and accurate diagnostic method and should be used for screening in all children with doubtful superficial masses.

Adolescent↗

The safety of home tracheostomy care for children.

Home tracheostomy care avoids prolonged hospitalization for tracheostomized children who must remain cannulated for a long time; however the safety aspects of this form of management are still under discussion. Since 1987 the authors have tutored parents in tracheostomy care. Once parental training was complete and the necessary equipment (aspirator, humidifier, etc) provided, children in stable condition were discharged and had period follow-up as outpatients. Thirty-four children had a total of 710 months in home care; 11 of them had decannulation. The only reported complications were two partial obstructions of the cannula and two accidental decannulations. The low number of documented accidents and the parents' profound appreciation in having their children at home indicate that patients with a tracheostomy in need of long-term care can be safely managed at home.

Accidents↗

Double fetus in fetu: diagnostic imaging.

Fetus in fetu is a rare pathological condition, presenting as a congenital tumor. It consists of a malformed parasitic twin that is found within the body of its sibling. Less than 70 cases have been reported and in most of them a definite diagnosis was only made during surgery. We present a case with two fetuses in the retroperitoneum and describe the criteria to be kept in mind for a correct preoperative diagnosis using the current imaging modalities.

Abdominal Neoplasms↗

Paratesticular rhabdomyosarcoma (RMS) and paraaortic lymphadenectomy.

This study is based on the experience of the Italian Cooperative Studies "AIEOP-RMS 79 and RMS 88". Between October 1979 and December 1989 299 children under 15 years of age with a histologically proven rhabdomyosarcoma (RMS) were examined. We analyzed the problem of the paratesticular localization paying particular attention to the role of surgery in dealing with paraaortic nodes. In the first study (RMS 79) the surgical exploration of retroperitoneal nodes was required: this procedure performed in 11 out of 14 patients affected by paratesticular RMS confirmed the negative data observed with diagnostic investigations. In RMS 88 study the sampling of retroperitoneal nodes was not required in cases with negative radiological findings but suggested in those cases with uncertain signs; two out of 8 patients underwent a laparotomy. In these two cases the results were negative. These 22 children were treated with an adjuvant chemotherapy after surgery and at present they show no evidence of disease.

Adolescent↗

Primary occlusion of the iliac and femoral artery in two newborn infants: efficacy of medical treatment.

Arterial occlusion not associated with umbilical artery catheterization is a rare condition in the newborn period. Two neonates with femoral and iliac artery occlusion in the first days of life are presented. While almost all the cases described in the literature underwent surgical treatment, these two infants received only pharmacological treatment with good results. Although angiography remains the examination of choice for the diagnosis, noninvasive angiologic evaluations such as oscillometry and Doppler flow examination have been used for both diagnosis and follow-up. The etiology in these two cases remains unknown. The results obtained with these two neonates suggest that with prompt recognition of this disorder and an aggressive medical treatment, a complete recovery could be achieved.

Arterial Occlusive Diseases↗

Effectiveness and toxicity of cisplatin and doxorubicin (PLADO) in childhood hepatoblastoma and hepatocellular carcinoma: a SIOP pilot study.

Sixteen children, aged 16 days to 13 years with hepatoblastoma (HB) (13 patients) or hepatocellular carcinoma (HCC) (3 patients), were given a total of 89 courses of cisplatin and doxorubicin (PLADO) as IV continuous infusion. All tumors were confined to the liver except for 1 hepatoblastoma patient with pulmonary metastases at presentation. Tumor response to PLADO was evaluable in 10 children (8 HB, 2 HCC) treated with preoperative chemotherapy and in another 2 HB patients treated when they developed pulmonary metastases after initial treatment with surgery alone. There were 2 complete responses (2 HB with pulmonary recurrences), 7 very good partial responses (6 HB and 1 HCC), 2 partial responses (1 HB, 1 HCC), and 1 stable disease (HB). The last patient underwent orthotopic liver transplantation whereas all the other patients had their tumor completely excised at delayed surgery. Documented toxicity was BM depression (16 patients), infection (11), vomiting (11), mucositis (3), hearing loss (1), and cardiotoxicity (1). These data indicate that PLADO in continuous infusion is effective in the treatment of malignant epithelial liver tumors with acceptable toxicity.

Adolescent↗

Arteriovenous fistula between descending aorta and hemiazygos vein.

The article describes a rare case of congenital A-V fistula between descending aorta and hemiazygos vein in a 9-year old girl. Differential diagnosis in these cases is difficult and only sonography and angiography can lead to a correct diagnosis. The recent literature of this subject is reviewed.

Angiography, Digital Subtraction↗

Neonatal malignant solid tumors.

We reviewed the 8 malignant solid tumors in children less than 1 month of age observed at the Pediatric Surgical Department of Padua University from 1968 to 1985. These were 6 neuroblastomas, 1 Wilms' tumor and 1 angiosarcoma; 5/8 are alive without evidence of disease with a median follow-up of 8 years. The importance of combined therapeutic approach and conservative surgery are stressed. The sensitiveness of infants to both chemotherapy and radiotherapy are considered. Surgery remains the treatment of choice when a radical resection is possible.

Combined Modality Therapy↗

Obstructive jaundice in Kawasaki's disease.

We describe a case of Kawasaki's disease in a 10-year-old child who had obstructive jaundice, intrahepatic biliary duct dilatation and gallbladder hydrops. The diagnosis was defined by echography, CT-scan and cholangiography. Immediate relief from symptoms and progressive resolution of jaundice was achieved by percutaneous transhepatic biliary drainage (PTBD). Laparotomy was avoided.

Catheters, Indwelling↗

Predictive value of visible lesions (cheeks, lips, oropharynx) in suspected caustic ingestion: may endoscopy reasonably be omitted in completely negative pediatric patients?

The relationship between absence or presence of grossly visible lesions in the cheeks, lips, and oropharynx (C.L.O. burns) and the incidence, site, and degree of visceral burns was evaluated in all children referred to our hospital for a suspected caustic ingestion during a 10-year period. All children underwent eso-gastro-duodenoscopy within 24 hours. Of the 156 children, 96 (61.6%) showed no visible signs of contact with the caustic substance; however, in 36/96 (37.5%), endoscopy revealed burns in one or more visceral sites. Eight of 36 children (22.2%) sustained potentially dangerous lesions (second to third degree). Sixty of 156 children (38.4%) showed visible lesions; in 30/60 (50%), endoscopy revealed other burns in one or more visceral sites. Fourteen of 30 patients (46.6%) sustained potentially dangerous lesions (second to third degree). A total of 50 esophageal burns have been recorded: first degree (E1), 32; second degree (E2), 12; third degree (E3), 6. Two of 12 patients with E2 lesions and 6/6 with E3 lesions developed esophageal stenosis. One patient in this latter group died because of complications related to a tracheostomy. A total of 31 gastric burns have been recorded: G1 (22), G2 (6), G3 (3). One gastric perforation was observed in the G3 group, whereas the remaining two lesions healed with residual asymptomatic scarring. Minimal scarring was observed in two of six patients with G2 burns. A total of eight lesions have been recorded in the larynx [L1 (3), L3 (1)] and in the duodenum [D1 (2), D2 (2)].(ABSTRACT TRUNCATED AT 250 WORDS)

Burns, Chemical↗

[The value of prenatal sonography in the diagnosis of congenital malformations].

Three hundred and forty-one prenatal ultrasound tests performed in 79 cases who presented congenital malformations at birth were studied retrospectively. A correct prenatal diagnosis was only made in 24 cases (30.3%), whereas there were 50 false negative (63.3%) and 5 dubious cases (6.3%). The percentage of false negative was greater in the group of cases examined in peripheral centres (56%) in comparison to those examined in our institute (35%).

Congenital Abnormalities↗

Long-term follow-up results after surgical repair of esophageal atresia.

Fiftytwo of 55 children, surviving surgical repair of tracheo-esophageal fistula between 1975 and 1985, were reviewed in relation to birth weight and Waterston's risk group after a mean follow-up of 7.5 years. The long-term results of esophageal atresia patients seem good in 30% of the follow-ups. Esophageal dysfunctions and respiratory difficulties were found respectively in 23 (44.2%) and 26 (50%) of the children; all the patients were improving after the first years of life. Scoliosis affected 27 children (51.9%) and its incidence increased with growth. Waterston's classification was a poor prognostic guide, but children weighing less than 2500 g at birth remained of relatively lower weight. The frequent development of scoliosis with age leads to continue evaluations after that respiratory and alimentary problems which might occur in the early years have been overcome.

Adolescent↗

[High-dose gynecologic endocavitary brachytherapy. Technical and dosimetric aspects].

The technical and dosimetric aspects are presented of high-dose intracavitary brachytherapy in gynecology. Fifty-five patients (203 insertions) were examined over two years with a remote loading Selectron HDR 60Co unit installed in a dedicated bunker. The dose to the rectal and bladder markers on AP and LL films was calculated before every irradiation, in order to allow the necessary corrections to be made. Uniform irradiation conditions were obtained at each treatment set-up for both tumoral target and bladder and rectal doses. High-dose intracavitary brachytherapy proved to be a safe, reliable and versatile method from the technical and dosimetric point of view both in the treatment of unoperated gynecological malignancies and in postoperative therapy.

Brachytherapy↗