Respiratory Profile in Patients After Liver Transplantation.

BACKGROUND: Patients in the immediate post-operative liver transplantation (LxT) period can develop respiratory and functional complications. In the postoperative months, these functions tend to improve. Nevertheless, there are few studies that evaluate precisely and specifically respiratory function in post-LxT long-term after surgery. The objective of the study was to describe the respiratory profile of patients 1 to 6 months after LxT, accompanied by LxT outpatients. METHODS: We included patients between 25 and 60 years old. We excluded patients with chronic renal or cerebrovascular impairment, severe heart disease, and history of lung surgery or liver re-transplantation. Evaluations were carried out on 3 occasions: 1 month, 3 months, and 6 months after LxT. The following evaluations were submitted: respiratory muscle strength (manuvacuometer), value flows and lung volumes (spirometer), and surface electromyography analyzing root mean square in the right (RMS-R) and left (RMS-L) diaphragm. We analyzed MELD (Model for End-Stage Liver Disease). After normality tests, we used the Friedman test (non-parametric values) and ANOVA (parametric values), P ≥ .5 with the use of SPSS 21.0. RESULTS: Patients (n = 15) had a mean age of 53.0 ± 7.5 years and 25.9 ± 4.6 MELD score. The statistically significant value obtained at the 3 occasions of evaluation was RMS-R, with a decline during periods of evaluation. This can be caused by removal of the liver, resulting in a denervation and reduction in compliance of this portion of the muscle. CONCLUSIONS: Patients between 1 and 6 months after transplantation have a specific respiratory profile, close to normal values. However, there are few studies on this subject, and we suggest that more research be done.

Longitudinal assessment of lung area measurements by two-dimensional ultrasound in fetuses with isolated left-sided congenital diaphragmatic hernia.

OBJECTIVE: To evaluate lung growth in healthy fetuses and those with congenital diaphragmatic hernia (CDH) using two-dimensional (2D) ultrasound. METHODS: Fetal right lung measurements obtained by 2D ultrasound between 19 and 37 weeks' gestation were evaluated longitudinally in 66 healthy fetuses and 52 fetuses with isolated left-sided CDH. Right lung areas were determined by the 'tracing' and 'longest-diameters' methods and, subsequently, lung area-to-head circumference ratios (LHRs) were calculated. Functions fitted to these size parameters with respect to gestational age were evaluated for three sets of group-wise comparisons: (1) healthy vs CDH fetuses; (2) different degrees of severity of CDH; and (3) CDH fetuses that survived vs those that died by 6 months postpartum. RESULTS: There was a significantly slower increase in right lung areas and LHRs with advancing gestational age in CDH fetuses than in healthy individuals (P < 0.05). Compared to those with milder forms of CDH, lung areas and LHRs of fetuses with more severe forms displayed a smaller increase (P < 0.05) and LHRs of fetuses with severe CDH did not increase during pregnancy (P > 0.05). Individuals who died postpartum did not show any increase in LHR (P > 0.05) throughout gestation. CONCLUSIONS: The right lung area and LHR, calculated using either the longest-diameters or tracing method, display reduced growth rates during gestation in cases of isolated left-sided CDH as compared with healthy fetuses. The growth curve characteristics of fetal lung areas and LHRs may be useful for predicting neonatal mortality.

Early fetoscopic tracheal occlusion for extremely severe pulmonary hypoplasia in isolated congenital diaphragmatic hernia: preliminary results.

OBJECTIVE: To evaluate the effect of early fetoscopic tracheal occlusion (FETO) (22-24 weeks' gestation) on pulmonary response and neonatal survival in cases of extremely severe isolated congenital diaphragmatic hernia (CDH). METHODS: This was a multicenter study involving fetuses with extremely severe CDH (lung-to-head ratio < 0.70, liver herniation into the thoracic cavity and no other detectable anomalies). Between August 2010 and December 2011, eight fetuses underwent early FETO. Data were compared with nine fetuses that underwent standard FETO and 10 without fetoscopic procedure from January 2006 to July 2010. FETO was performed under maternal epidural anesthesia, supplemented with fetal intramuscular anesthesia. Fetal lung size and vascularity were evaluated by ultrasound before and every 2 weeks after FETO. Postnatal therapy was equivalent for both treated fetuses and controls. Primary outcome was infant survival to 180 days and secondary outcome was fetal pulmonary response. RESULTS: Maternal and fetal demographic characteristics and obstetric complications were similar in the three groups (P > 0.05). Infant survival rate was significantly higher in the early FETO group (62.5%) compared with the standard group (11.1%) and with controls (0%) (P < 0.01). Early FETO resulted in a significant improvement in fetal lung size and pulmonary vascularity when compared with standard FETO (P < 0.01). CONCLUSIONS: Early FETO may improve infant survival by further increases of lung size and pulmonary vascularity in cases with extremely severe pulmonary hypoplasia in isolated CDH. This study supports formal testing of the hypothesis with a randomized controlled trial.

A randomized controlled trial of fetal endoscopic tracheal occlusion versus postnatal management of severe isolated congenital diaphragmatic hernia.

OBJECTIVE: Severe pulmonary hypoplasia and pulmonary arterial hypertension are associated with reduced survival in congenital diaphragmatic hernia (CDH). We aimed to determine whether fetal endoscopic tracheal occlusion (FETO) improves survival in cases of severe isolated CDH. METHODS: Between May 2008 and July 2010, patients whose fetuses had severe isolated CDH (lung-to-head ratio < 1.0, liver herniation into the thoracic cavity and no other detectable anomalies) were assigned randomly to FETO or to no fetal intervention (controls). FETO was performed under maternal epidural anesthesia supplemented with fetal intramuscular anesthesia. Tracheal balloon placement was achieved with ultrasound guidance and fetoscopy between 26 and 30 weeks of gestation. All cases that underwent FETO were delivered by the EXIT procedure. Postnatal therapy was the same for both treated fetuses and controls. The primary outcome was survival to 6 months of age. Other maternal and neonatal outcomes were also evaluated. RESULTS: Twenty patients were enrolled randomly to FETO and 21 patients to standard postnatal management. The mean gestational age at randomization was similar in both groups (P = 0.83). Delivery occurred at 35.6 ± 2.4 weeks in the FETO group and at 37.4 ± 1.9 weeks in the controls (P < 0.01). In the intention-to-treat analysis, 10/20 (50.0%) infants in the FETO group survived, while 1/21 (4.8%) controls survived (relative risk (RR), 10.5 (95% CI, 1.5-74.7), P < 0.01). In the received-treatment analysis, 10/19 (52.6%) infants in the FETO group and 1/19 (5.3%) controls survived (RR, 10.0 (95% CI, 1.4-70.6) P < 0.01). CONCLUSION: FETO improves neonatal survival in cases with isolated severe CDH.

Prediction and probability of neonatal outcome in isolated congenital diaphragmatic hernia using multiple ultrasound parameters.

OBJECTIVES: To evaluate the accuracy and probabilities of different fetal ultrasound parameters to predict neonatal outcome in isolated congenital diaphragmatic hernia (CDH). METHODS: Between January 2004 and December 2010, we evaluated prospectively 108 fetuses with isolated CDH (82 left-sided and 26 right-sided). The following parameters were evaluated: gestational age at diagnosis, side of the diaphragmatic defect, presence of polyhydramnios, presence of liver herniated into the fetal thorax (liver-up), lung-to-head ratio (LHR) and observed/expected LHR (o/e-LHR), observed/expected contralateral and total fetal lung volume (o/e-ContFLV and o/e-TotFLV) ratios, ultrasonographic fetal lung volume/fetal weight ratio (US-FLW), observed/expected contralateral and main pulmonary artery diameter (o/e-ContPA and o/e-MPA) ratios and the contralateral vascularization index (Cont-VI). The outcomes were neonatal death and severe postnatal pulmonary arterial hypertension (PAH). RESULTS: Neonatal mortality was 64.8% (70/108). Severe PAH was diagnosed in 68 (63.0%) cases, of which 63 died neonatally (92.6%) (P < 0.001). Gestational age at diagnosis, side of the defect and polyhydramnios were not associated with poor outcome (P > 0.05). LHR, o/e-LHR, liver-up, o/e-ContFLV, o/e-TotFLV, US-FLW, o/e-ContPA, o/e-MPA and Cont-VI were associated with both neonatal death and severe postnatal PAH (P < 0.001). Receiver-operating characteristics curves indicated that measuring total lung volumes (o/e-TotFLV and US-FLW) was more accurate than was considering only the contralateral lung sizes (LHR, o/e-LHR and o/e-ContFLV; P < 0.05), and Cont-VI was the most accurate ultrasound parameter to predict neonatal death and severe PAH (P < 0.001). CONCLUSIONS: Evaluating total lung volumes is more accurate than is measuring only the contralateral lung size. Evaluating pulmonary vascularization (Cont-VI) is the most accurate predictor of neonatal outcome. Estimating the probability of survival and severe PAH allows classification of cases according to prognosis.

African swine fever in Mozambique: review, risk factors and considerations for control.

African swine fever (ASF) is the most important disease that constrains pig production in Mozambique. Until 1994 it was apparently restricted to the central and northern provinces, but since 1994 outbreaks have been experienced throughout the country. ASF causes severe economic losses both in the small commercial sector and among the large numbers of small-scale producers in the family sector in rural and peri-urban areas. The history of ASF in Mozambique since its first confirmation in 1960 is briefly reviewed, recent outbreaks are reported, and the available information on the virus genotypes that have been responsible for some of the outbreaks is presented. Epidemiological factors that contribute to ASF outbreaks and strategies for limiting the negative effects of the disease in the different pig farming sectors in Mozambique, including raising farmer and community awareness, are discussed.

Multiple sclerosis: long-term remission after a high dose of cyclophosphamide.

The objective of this case report is to document the possibility that immunoablative doses of cyclophosphamide may provide a long-term remission of multiple sclerosis (MS). We report the case of a 48-year-old woman with definite MS diagnosed in 1994 who has been in complete remission since a dose of 3800 mg of cyclophosphamide was accidentally given intravenously in early 1997. For 7 years there have been no signs of disease activity on history, physical examination, or on high-quality magnetic resonance imaging (MRI) with appropriate contrast-enhancement methodology. This case includes information on the possibility that less aggressive chemotherapy than that used with stem cell transplantation may be effective in the long-term control of MS.

Prognostic factors associated with congenital cystic adenomatoid malformation of the lung.

This study presents 18 cases of prenatally diagnosed congenital cystic adenomatoid malformation (CCAM) to identify potential factors that could predict prognosis. Comparisons of prenatal parameters were made between fetuses that survived and those that died perinatally. It was found that microcystic lesion, bilateral lung involvement and hydrops were each highly correlated with poor prognosis, while neither polyhydramnios nor mediastinal shift was significantly associated with had outcome. Fetal interventions were indicated only in two of the surviving cases: a thoracocentesis and a cysto-amniotic shunt. A therapeutic amniocentesis was performed in one case of polyhydramnios. The diagnosis of CCAM was histologically confirmed in all cases by necropsy or by postnatal lobectomy.

[Postoperative mortality in infective endocarditis: determinant factors]. / Fatores determinantes da letalidade após cirurgia para endocardite infecciosa.

The factors leading to high postoperative mortality in active infectious endocarditis (IE) are poorly defined. We studied patients operated at an University Hospital between March 1978 and April 1992. We hypothesized that the summation of potential adverse factors would strongly increase mortality after surgery. We studied 39 patients (28 men), age range 13-70 years (mean +/- SD = 32 +/- 16) operated during active IE (time from onset 52 +/- 48 days). Predisposing factor: rheumatic valvar disease in 14 cases, intravenous drug use in 5. Affected valves: aortic in 14, mitral in 10, tricuspid in 8, multiple structures in 7. In most cases, S aureus (12) or Streptococcus sp (10) was isolated in blood cultures. Surgery was indicated in most patients because of heart failure (30), multiple embolic complications (17) or treatment failure (14). The possible adverse influence of specific demographic characteristics, clinical features and surgical variables was assessed by the Student t test or the chi 2 test. Also, multiple regression analysis was performed in order to identify independent adverse factors for increased mortality. Positive correlations were further investigated with the chi 2 test to assess whether an increasing number of adverse factors could identify a special subset of patients with markedly elevated death risk. Fourteen patients (36%) died after surgery. Emergency surgery (p = 0.001), the presence of coma 6 hours after surgery (p = 0.0015) and S. aureus infection (p = 0.023) were all associated with increased mortality. The presence of neurological complications was correlated with a high mortality (54% vs. 27%). However this increase was of dubious statistical significance (p = 0.097). Multiple regression analysis confirmed S. aureus and emergency surgery as independent adverse factors for increased mortality. When put together, an increasing number of adverse factors was highly predictive of a fatal outcome, even after exclusion of that evaluated after surgery (level of consciousness). Patients with two or three adverse factors had a very high mortality rate (> 76.9%). Mortality following surgery for active IE is increased in patients operated on an emergency basis especially if the infection is caused by S. aureus. The presence of neurological complications may also be associated with worse outcome. Early consideration of surgery should reduce the high mortality in patients with active IE.

Colonoscopy preparation in children: safety, efficacy, and tolerance of high- versus low-volume cleansing methods.

BACKGROUND: The use of large-volume electrolyte balanced solutions as preparation for colonoscopy often results in poor patient compliance and acceptance. The tolerance, safety, and efficacy of high-versus low-volume colon-cleansing methods as preparation for colonoscopy in children were compared by randomized operator-blinded trial. METHODS: Twenty-nine children ages 3.6-14.6 years had either high-volume nasogastric balanced polyethylene glycol electrolyte lavage (20 ml/kg/ h) until the effluent was clear (n = 15), or two oral doses of sodium phosphate solution (22.5-45 ml) separated by oral fluid intake (n = 14). RESULTS: Both preparations were equally effective. The low-volume preparation was better tolerated and caused less discomfort that the high-volume preparation, judging by serial nurse observations. The incidence of abdominal symptoms, diarrhea, sleep disturbance, and vomiting was not significantly different between the two groups. Both groups had a small reduction in mean hematocrit and serum calcium levels. The sodium phosphate preparation caused increases in mean serum sodium concentrations from 140 to 145 mmol/L and serum phosphate concentrations from 1.41 to 2.53 mmol/L. Ten hours after the commencement of the preanesthetic fast, these concentrations had returned to normal. CONCLUSIONS: There are advantages in terms of tolerance, discomfort, and case of administration with acceptable colonic cleansing with the use of the less-invasive oral sodium phosphate low-volume colon-cleansing preparation in children. Safe use requires ensuring an adequate oral fluid intake during the preparation time and avoidance of use in patients with renal insufficiency.

(1,3)-beta-D-glucan synthase activity in mycelial and cell wall-less phenotypes of the fz, sg, os-1 ("slime") mutant strain of Neurospora crassa.

The cell wall-less fz, sg, os-1 ("slime") triple mutant of Neurospora crassa lacks (1,3)-beta-D-glucan synthase activity. fz, sg, os-1 segregants from slime x wild-type crosses initially germinate as a plasmodium (slime-like), but develop hyphae in a few hours and acquire a stable mycelial phenotype (mycelial intermediate). The cell wall-less phenotype (stable slime) can be reisolated from mycelial intermediates by filtration-enrichment selection in medium of high osmolarity. Pairs of mycelial intermediate and stable slime obtained from a single slime-like segregant were comparatively studied. Mycelial intermediate strains synthesize a cell wall with normal amounts of (1,3)-beta-glucan, chitin, and other polysaccharides and possess (1,3)-beta-glucan synthase activity with apparently normal properties (i.e., association with membranes, stability, Km app, Vmax, stimulation by GTP). The enzyme was dissociated by treatment with Tergitol NP-40 and NaCl into a membrane-bound catalytic center and a soluble factor which activates the enzyme in the presence of GTP. Heterologous reconstitution assays demonstrated that stable slime spheroplasts had normal activity of the soluble activating factor, but were severely deficient in membrane-bound activity. The genetic composition of the viable progeny of stable slime or mycelial intermediate x wild-type crosses failed to show differences between the two extreme phenotypes of slime. However, the analysis of heterokaryons demonstrated that the stable slime homokaryotic progeny of stable slime/wild-type heterokaryons were not viable. In contrast, the behavior of mycelial intermediate/wild-type heterokaryons was normal. Apparently, stable slime strains differed from the original mycelial intermediate in a mutation(s) which arose spontaneously during the filtration-enrichment selection applied to mycelial intermediates in order to obtain the cell wall-less phenotype. This new trait impaired conidial germination and might be the actual cause of the loss of (1,3)-beta-glucan synthase activity and cell wall.

Cell wall deficiency in "slime" strains of Neurospora crassa: osmotic inhibition of cell wall synthesis and beta-D-glucan synthase activity.

1. The RCP-3 S/H mutant of Neurospora crassa was obtained by vegetative selection in medium of high osmolarity of a mycelial form of an fz, sg, os-1 ("slime"-like) segregant. The mutant exhibits spheroplast-hyphal dimorphism conditioned by the osmolarity of the culture medium (Pietro et al. (1990). Journal of General Microbiology, 136: 121-129). The carbohydrate composition of the cell wall of the mutant was different from that of the wild type in the absence of an alkali-soluble galactosaminoglycan polymer. Furthermore the mutant cell wall had a somewhat lower content of beta-glucan relative to that of chitin. 2. Increasing concentrations of sorbitol in the culture medium of the mutant inhibited by 10-fold the formation of cell wall relative to total biomass. The cell wall of the mutant cultured in the presence of sorbitol lacked mannose- and galactose-containing polymers, and also showed progressively lower amounts of beta-glucan relative to chitin. 3. The activity of membrane-bound (1-3)-beta-D-glucan synthase from the mutant grown in the absence of sorbitol shared several properties with the wild type enzyme (i.e., Km app., Vmax, stability at 30 degrees C, activation by GTP gamma S, and dissociability by treatment with NaCl and Tergitol NP-40 into a membrane-bound catalytic center and a GTP-binding activating protein). On the other hand, the enzyme from the mutant but not that from the wild type was inactivated by about 15% by treatment with NaCl and detergent. 4. At high concentrations of sorbitol (1.0 M) the RCP-3 S/H mutant exclusively produced spheroplasts devoid of (1-3)-beta-D-glucan synthase activity. The defect was at the level of the membrane-bound catalytic center. The activity of the GTP-binding activating factor was apparently normal in these cells. 5. These results suggest that the definitive loss of cell wall in the N. crassa "slime" RCP-3 S/H mutant was due to a defect in (1-3)-beta-D-glucan synthase activity which was exaggerated in the presence of high osmolyte concentrations.

[Liver transplantation in children: initial experience of the Instituto da Criança of the Hospital das Clínicas of Universidade de São Paulo]. / Transplante de fígado em criança: experiência inicial do Instituto da Criança do Hospital das Clínicas da Universidade de São Paulo.

The initial experience at the Instituto da Criança do Hospital das Clínicas--School of Medicine of São Paulo University with liver transplantation in children is presented. A staff experienced in the management of children, including pediatric surgeons, hepatologists, critical care specialists, anesthesiologists, and other has been joined to draw therapeutic protocols. Afterward, more than 100 experimental liver transplant were performed in animals of medium size (dogs and pigs). From September, 1989 to July 1991, 12 liver transplants were performed on 9 children (3 retransplants) ranging in age from 2.5 to 17 years, being 5 boys and 4 girls. The donors had been selected according to the ABO blood group and body weight. Just once a blood A+ recipient received a liver from a blood O+ donor. The regular postoperative immunosuppression consisted of triple therapy with cyclosporin, prednisone and azathioprine. The postoperative stay in the Intensive Care Unit ranged from 3 to 24 days, according to the necessity of ventilatory support. These was no intraoperative mortality, arterial or venous thromboses, or early biliary complications. The overall survival is 78% (7/9). Primary non-function of the graft was the cause of death in two of our children. Although the number of cases is still small our results are comparable to those of the best liver transplant centers in the world.

[Epileptic crisis induced by food intake: report of a case]. / Crises epilépticas evocadas pela ingestão de alimentos: registro de um caso.

The case of one 23 year-old girl who had epileptic manifestations is reported. At first, generalized tonic seizures; afterwards, epileptic seizures precipitated by eating. The electroencephalograms showed left temporal lobe dysfunctions. Different types of drugs were used with no success. The best results were obtained by association of sodium valproate, clonazepam and phenobarbital. Comments are made about clinic and etiopathogenesis, believing the authors in the hypothesis of nervous structures chronic hyperactivity. To Walker the hyperactivity was reached by hormones production under neural control of specific cerebral centers. The continuous bombardment of epileptic discharges to hypothalamic centers is the probably responsible by epileptic seizures precipitated by eating.