Congenital fetal and neonatal visceral chylous effusions: neonatal chylothorax and chylous ascites revisited. A multicenter retrospective study.

This retrospective study was carried out at eight Neonatal Intensive Care Units (NICU) Centers worldwide on 33 newborns presenting at birth with pleural, pericardial, or abdominal chylous effusions. Diagnosis of chylous effusion is based on findings of fluid with a milk-like appearance, a concentration of triglycerides in pleural effusion >1.1 mmol/l, and a total cell count >1,000 cells/ml with a predominance of >80% lymphocytes. Thirty-three newborns met the inclusion criteria and were studied. Six subjects who presented at birth with fetal effusion were treated by in-utero pleuro-amniotic shunt. Five of these patients are alive at follow-up. At birth, pleural drainage was performed in 29/33 patients and abdominal drainage was carried out in 3/33. Total parenteral nutrition (TPN) was given to 32/33 patients; 19/23 patients were fed a medium-chain triglycerides (MCT). No adverse effects were observed. Eight patients were treated with Octreotide at dosages ranging from 1 to 7 mcg/kg/hour for 8 to 35 days. All patients showed decreased chylous production. Two patients were treated by pleurodesis. Twenty-two babies are alive after at least 6 months follow-up, 9/33 are deceased, and 2 were lost to follow-up. Clinical conditions of survivors are basically good except for lung involvement [chronic lung disease (CLD) or lung lymphangiectasia] and lymphedema. All patients were using a MCT diet at follow-up with good control of chylous effusion. Visceral chylous effusions of the fetus and neonate are rare disorders, and there currently is only partial agreement on decision-making strategies. We suggest the need for an international prospective trial in an effort to establish the efficacy and effectiveness of diagnostic and therapeutic options described in this article.

Life-threatening hemorrhage from a vulvar hemangioma.

Capillary hemangioma, the most common tumor of infancy, usually does not require any treatment, but in a minority of cases it may cause morphologic, functional, or life-threatening complications. The authors report a case of a vulvar emangioma complicated by life-threatening hemorrhage not responsive to corticosteroids therapy, which showed no signs of spontaneous involution. The therapeutic approach consisted of 3 steps of selective arterial embolizations followed 5 months later by surgical excision and reconstruction of the vulva. Selective embolization rarely is used in the treatment of hemangiomas but may be required in cases of intractable bleeding, severe heart failure, large and poorly involuting hemangiomas, and as preparation for surgery. The results of the combined approach in this case are reported, and the indications discussed.

TT virus-related acute recurrent hepatitis. Histological features of a case and review of the literature.

TT virus is a recently discovered virus, of which the pathogenetic potential is still uncertain. The present paper describes the histopathological features of a case of TT virus-related acute recurrent hepatitis. The patient is a 28-year-old woman with no history of drug or alcohol abuse, presenting with repeated episodes of hypertransaminasemia evidenced during the last 4 years. No other markers of viral or autoimmune disease were found. On histological analysis, the liver parenchyma showed a preserved architecture. The histological features were those of a mild acute hepatitis. The clinicopathological findings suggest th

[Closure of patent ductus arteriosus in thoracoscopy: analysis of the experience in the Gaslini Institute of Genoa]. / Chiusura del dotto arterioso persistente in toracoscopia: analisi dell'esperienza dell'istituto Gaslini di Genova.

In 1993, Laborde et al described a new surgical approach for closure of the patent ductus arteriosus (PDA), using a videoassisted technique. For the first time, videosurgery was utilized in pediatric cardiovascular surgery. The advantages of this new method are that it is minimally invasive (thoracotomy is replaced by three small holes), it reduces postoperative pain, recovery is more rapid with a shorter hospital stay and convalescence, and last but not least, it offers better aesthetic results. Between July 1994 and June 1996, we utilized the videoassisted thoracoscopic technique described by Laborde in 23 patients with PDA (age: 8 m-12 yrs, mean 45.2 m +/- 37.8; weight: 5.3-32 kg, mean 23 kg +/- 10.3). In two patients, we converted the procedure to standard thoracotomy due to anatomic problems. Echo-doppler evaluation, performed in the operating room shortly after the procedure, confirmed the complete closure of the PDA in all patients. There was neither morbidity nor mortality. The average hospital stay was four days. At a mean follow-up interval of 7.2 months (range 1-13 months), all patients showed excellent cosmetic results. We conclude that thoracoscopic closure of PDA is a valuable therapeutic option that can be used as an alternative to open-chest surgery and interventional cardiology.

[Differential types of pulmonary flow in bidirectional cavo-pulmonary anastomosis with an additional flow source]. / Differenti tipi di flusso polmonare nell'anastomosi cavo-polmonare bidirezionale con sorgente addizionale di flusso.

While Fontan operation is considered a palliative procedure for the treatment of complex congenital heart disease, its morbidity and mortality are certainly not negligible, especially in high-risk patients. In our opinion, a bidirectional cavo-pulmonary anastomosis with an additional source of blood flow to the lungs (Blalock-Taussig shunt, pulmonary artery banding, native pulmonary stenosis) represents a valuable surgical option in this subset of patients. From November 1992 to September 1994, sixteen patients with complex congenital heart disease underwent bidirectional cavo-pulmonary anastomosis, leaving an additional source of blood flow in place (modified Blalock-Taussig shunt in 9 cases, pulmonary artery banding in 6 cases, native pulmonary stenosis in 1 case). Eight of these patients (50%) were reinvestigated after a mean follow-up of 15.7 months (range 1-26 m). It has been possible to differentiate two types of pulmonary blood flow, according to the nature of the additional flow source; in the case of cavo-pulmonary anastomosis and pulmonary artery banding or pulmonary stenosis, pulmonary blood flow was pulsatile and evenly distributed to both lungs, while in the case of bidirectional cavo-pulmonary anastomosis and Blalock-Taussig shunt, pulmonary flow was non pulsatile and asymmetric (angiographic criteria). There were no hospital deaths. Late mortality was 12.5% (2 pts) due to worsening of atrioventricular valve regurgitation. In one of these two patients, prolonged pleural and pericardial effusion occurred. In conclusion, bidirectional cavo-pulmonary anastomosis with an additional flow source is a good alternative to classic or fenestrated Fontan operation in the treatment of complex congenital heart disease. The best results are obtained with an additional flow source represented by pulmonary artery banding or pulmonary stenosis, due to pulsatility and uniform distribution of blood flow in the pulmonary district. Morbility and mortality compare favorably with the rates observed in classic or fenestrated Fontan operation.

Bidirectional cavo-pulmonary anastomosis with an additional flow source to the lungs: clinical experience in 21 cases.

The Fontan operation is considered a palliative procedure for the treatment of complex congenital heart diseases with a morbidity and mortality that are not negligible, especially in high-risk patients. In these cases, a bidirectional cavopulmonary anastomosis with additional source of blood flow to the lungs can represent a valuable surgical option. Between November 1992 and December 1995, 21 patients with univentricular heart physiology were submitted to bidirectional cavopulmonary anastomosis with additional blood flow the additional flow consisted of an aortopulmonary shunt in 13 cases, pulmonary artery banding in seven cases, and pulmonary artery stenosis in one case. Mean age at operation was 35.5 months (range 4 months to 12 years) and mean body weight 10.9 (range 4-24.4) kg. Hospital mortality was zero. Morbidity (pleuropericardial effusions) was significant in one case. There were three late deaths (14%) caused by worsening atrioventricular valve regurgitation: two of these occurred in patients with right isomerism. Late haemodynamic evaluation documented a significant increase in cardiac output (P < 0.01) and oxygen saturation (P < 0.05) during pharmacological stress testing. Angiographic characterization of blood flow distribution to the lungs showed better results when the additional blood flow was represented by an anterograde source (pulmonary artery banding or native pulmonary artery stenosis). In conclusion, bidrectional cavopulmonary anastomosis with additional blood flow is a good alternative to classic or fenestrated Fontan technique in high-risk patients. Secondly, the diagnosis of isomerism could be a contraindication to this type of surgery, because the persistent volume overload could worsen atrioventricular valve regurgitation. Thirdly, the pharmacological stress testing shows a favourable behaviour of cardiac output and arterial oxygen saturation. Fourth, the type of additional blood flow seems to condition both pulsatility and distribution of pulmonary blood flow.

Surgical management of complete atrioventricular canal associated with tetralogy of Fallot.

Between 1984 and 1993, 12 children with an atrioventricular canal and tetralogy of Fallot underwent surgical repair. The mean(s.d.) age at operation was 58(18) months, and the mean(s.d.) body weight 15(4) kg. Nine patients underwent 11 palliative procedures. The ventricular septal defect was closed through a combined (right atrial and right ventricular) approach in nine cases, and through a right atrial approach in three, using a prosthetic patch with a wide anterior extension, secured with a running suture. The 'ostium primum' defect was closed with a separate prosthetic patch in 11 cases (double-patch technique). Right ventricular outflow obstruction was relieved by a composite infundibular patch (seven cases) or a transanular patch (five). There were four hospital deaths (33%). These were caused by low cardiac output in three cases and infection in one (three deaths occurred in patients with a transanular patch). One patient has so far died during follow-up. Assessment at 50(36) months by echo-Doppler showed moderate-to-severe 'mitral' regurgitation in three cases, and moderate 'tricuspid' regurgitation with right ventricular dysfunction in one case. Two patients have required further surgery.

Effects of SKF 108922, an HIV-1 protease inhibitor, on retrovirus replication in mice.

Rationally designed synthetic inhibitors of retroviral proteases inhibit the processing of viral polypeptides in cultures of human T lymphocytes infected with human immunodeficiency virus type 1 (HIV-1) and therefore suppress the infectivity of HIV-1 in vitro. We have previously reported the antiviral activity in vitro of HIV-1 protease inhibitors against the C-type retrovirus Rauscher murine leukemia virus (RMuLV) and the lentivirus simian immunodeficiency virus (SIV). The same compounds which blocked the infectivity of HIV-1 also inhibited the infectivity of RMuLV and SIV in vitro. This report extends these findings by testing the antiviral activity of HIV-1 protease inhibitors in vivo in the RMuLV model. RMuLV-infected mice were treated twice a day (bid) with either an active (SKF 108922) or inactive (SKF 109273) compound for fourteen days by the intraperitoneal (i.p.) route. Compared with excipient control, SKF 108922, formulated with hydroxypropyl-beta-cyclodextrin (HPB), reduced virus-induced splenomegaly, viremia, and serum reverse transcriptase (RT) levels, while SKF 109273 was inactive. The HPB vehicle by itself enhanced replication of RMuLV. The effects of changing the formulation and the route of administration were examined. SKF 108922, formulated in HPB, had similar antiviral activity when administered by the i.p. or subcutaneous (SC) routes. However, SKF 108922 administered as a colloidal suspension in cholesterol sulfate (CS) had no detectable antiviral effect. Measurements of the circulating levels of the protease inhibitor in plasma explained this result. Plasma concentrations of SKF 108922 exceeded 1000 nM within 10 min after SC administration of the compound solubilized in HPB, but SKF 108922 was not detected in plasma after SC administration of the same dose formulated with CS. Information on optimal conditions for administering these agents should prove useful in guiding their clinical application Therefore, RMuLV should provide a good model for the preclinical evaluation and development of this class of agents for the treatment of HIV.

Aortopulmonary septal defects. A review of the literature and report of ten cases.

From 1981 to 1992, 10 infants with aortopulmonary septal defect (APSD) underwent surgical repair. The mean age at operation was 5.6 +/- 5.5 months, and the mean weight 4.6 +/- 2 kg. Intracardiac associated anomalies were as follows: ventricular septal defects (7 cases), tetralogy of Fallot (2 cases), aortic valve stenosis (2 cases), atrial septal defect (3 cases), patent ductus arteriosus (3 cases), pulmonary valve stenosis (1 case). Cardiac catheterization was performed in 8 out of 10 patients. Eight patients had type I (proximal) defect, 1 had type II (distal) defect and 1 had type III (absent aortopulmonary septation) defect. A variety of surgical procedure was employed. APSD closure with hemoclip was feasible in 3 cases with small window. In 6 patients, during a period of cardiopulmonary by-pass, a side biting clamp was positioned on the ascending aorta close the defect; the border of the window was divided leaving a flap of pulmonary wall on the left side to close the aortic defect; the pulmonary artery was repaired by an autologous pericardial patch. In the patient with type III APSD, aortopulmonary septation was carried out through a transwindow approach. Associated anomalies were repaired in all infants except one. Hospital mortality was 10% (1 case). No late deaths occurred. At a mean follow-up of 47 +/- 35 months 8 patients are asymptomatic and 1 is awaiting for repair of associated anomalies. Conclusions. APSD is a rare but nonetheless well identifiable anomaly. Surgery is indicated as soon as the diagnosis is established, regardless of the patient's age.(ABSTRACT TRUNCATED AT 250 WORDS)

[Splenic embolization and hepatic chemoembolization: combined transcatheter treatment of hepatocellular carcinoma in cirrhosis with hypersplenism]. / Embolizzazione splenica e chemioembolizzazione epatica: trattamento combinato transcatetere del carcinoma epatocellulare su cirrosi con ipersplenismo.

Liver cirrhosis with hypersplenism is often associated with HCC. In many such cases, chemoembolization (TACE) may be very difficult because of the high incidence of hemorrhagic complications due to treatment and/or following portal hypertension, as well as for poor hematologic tolerance to antiblastic drugs in cirrhotic patients. Six patients with nodular HCC and cirrhosis (Child B) with hypersplenism were treated by combined TACE and partial splenic embolization (PSE) to reduce splenic size and to improve hematologic and hepatic function rates. Actual and long-lasting (up to 6 months since TACE/PSE) positive results were observed in splenic size and in hepatic function synthesis, as well as on hematologic and hemocoagulation factors. The clinical-laboratory improvement following TACE/PSE allowed TACE to be completed in all cases, following the usual protocol based on 3 procedures. Therefore, in the patients with advanced/decompensated cirrhosis and hypersplenism associated with HCC, the combined one-step TACE/PSE treatment can be said to improve patients' tolerance to antiblastic drugs and to reduce the risk of hemorrhagic complications due to invasive radiologic procedures and/or to portal hypertension.

Goretex patch aortoplasty for coarctation in children: nuclear magnetic resonance assessment at 7 years.

From 1979 to 1990, 56 children ranging between 4 days and 16 years of age (mean 73 +/- 51 months) underwent Goretex patch aortoplasty for coarctation of the aorta. The mean weight at operation was 20.2 +/- 3.5 kg (range 3.3-42 kg). Forty-two patients had primary repair, and the remaining 14 had reoperation for recoarctation. The aorta was opened through a standard left thoracotomy, the posterior fibrous ridge was partially excised when it was prominent, and a large patch from a Goretex tube was sutured into place. The postoperative complications were as follows: paradoxical hypertension in 14 cases, massive haemorrhage due to aortic wall rupture in a diabetic child, and intestinal bleeding in 1 case. There were no early deaths and only 1 case of late death, which was not related to coarctation repair (mortality rate 1.8%). The average follow-up was 48 +/- 26 months. Continuous wave-Doppler examination at rest showed no arm-leg systolic gradient in 52 cases and a gradient of about 15 mmHg in 4 cases. Graded exercise testing showed only 1 case with an arm-leg gradient higher than 35 mmHg. Nuclear magnetic resonance (NMR) imaging, performed on 26 patients at a mean of 7 years from operation, showed excellent morphology and size of the aortoplasty. No cases of recoarctation or late aneurysm formation were found. We conclude that Goretex patch aortoplasty can be performed effectively and safely in children. Nuclear magnetic resonance provides high resolution imaging of the coarctation repair site.

Investigations on intravascular coagulation in liver disease: souble fibrin monomer complexes in liver cirrhosis.

Souble fibrin monomer complexes (SFMC) have been quantitated in 18 patients with liver cirrhosis by means of plasma gel filtration on an agarose column. The concentration of SFMC (as related to total fibrinogen) was increased in cirrhotic patients. The difference between controls and patients, as well as the difference between compensated and decompensated cirrhotic subjects, was statisically significant. Although these data, together with the results of heparin administration in one reported case, suggest intravascular coagulation, some alternative explanations should also be considered.