Constraints on Elastic Neutrino Nucleus Scattering in the Fully Coherent Regime from the CONUS Experiment.

We report the best limit on coherent elastic scattering of electron antineutrinos emitted from a nuclear reactor off germanium nuclei. The measurement was performed with the CONUS detectors positioned at 17.1 m from the 3.9 GW_{th} reactor core of the nuclear power plant in Brokdorf, Germany. The antineutrino energies of less than 10 MeV assure interactions in the fully coherent regime. The analyzed dataset includes 248.7 kg d with the reactor turned on and background data of 58.8 kg d with the reactor off. With a quenching parameter of k=0.18 for germanium, we determined an upper limit on the number of neutrino events of 85 in the region of interest at 90% confidence level. This new CONUS dataset disfavors quenching parameters above k=0.27, under the assumption of standard-model-like coherent scattering of the reactor antineutrinos.

[Ring chromosome 8: microcephaly, mental retardation and minor facial anomalies with adhesive behavioral phenotype]. / Cromosoma 8 en anillo: microcefalia, retardo mental y disformias leves con fenotipo conductual adhesivo.

INTRODUCTION: Since the first description of ring chromosome 8 [r(8)] in 1973, only a few patients have been reported. In this report we present a child with this anomaly, and we compare his clinical manifestations with previously reported patients. CLINICAL CASE: A 12 year-old boy presented with low birth weight, mental retardation, microcephaly, short stature, hypotonia, and minor facial anomalies: hypotelorism, bilateral epicanthic folds, long philtrum, thin lips, narrow palate, micrognathia and low-set ears. Digital anomalies were bilateral brachyclinodactily of the fifth finger, and cutaneous syndactyly between second and third fingers. The boy had a pleasant personality but exhibit attachment for people and things with unrestricted affect. Cytogenetic analysis on peripheral white blood cells showed a r(8) chromosome. Parental karyotypes were both normal. CONCLUSIONS: The major features in the boy studied by us were found also in the other cases reported with an r(8) chromosome, but all they were non-specific features, and do not support the existence of a readily recognizable r(8) chromosome syndrome . Follow-up data with special emphasis on the behavioral characteristics are needed for defining an specific behavioral phenotype.

IFAP syndrome "plus" seizures, mental retardation, and callosal hypoplasia.

Ichthyosis follicularis, congenital alopecia, and photophobia are typical features of a rare X-linked recessive disorder termed ichthyosis follicularis with atrichia and photophobia syndrome. A 3-year-old male with these findings and severe growth failure, mental retardation, generalized seizures, vascularizing keratitis, nail anomalies, inguinal hernia, and a normal chromosome constitution is presented. Two maternal male relatives were affected by the same condition. Magnetic resonance imaging revealed corpus callosum hypoplasia not described at present. Syndromes with alopecia, seizures, and mental retardation are analyzed on the basis of genetic and clinical results.

Phacomatosis pigmentokeratotica: another epidermal nevus syndrome and a distinctive type of twin spotting.

The name epidermal nevus syndrome could be applied to a group of clinically and histopathologically different entities as has been pointed out by Happle. Phacomatosis pigmentokeratotica is a further type of epidermal nevus syndrome distinguished by the presence of a sebaceous nevus and a contralateral speckled lentiginous nevus of the papular type, associated with skeletal or neurological abnormalities. Three new cases of this recently delineated syndrome are presented. A common origin may account for the temporal and spatial relationship between the epidermal and the speckled lentiginous nevus. The concept of melanocytic-epidermal twin spotting similar to the interpretation of vascular twin spotting could explain the pathogenesis of this entity.

Atrichia, ichthyosis, follicular hyperkeratosis, chronic candidiasis, keratitis, seizures, mental retardation and inguinal hernia: a severe manifestation of IFAP syndrome?

A boy with congenital atrichia, ichthyosis follicular, keratitis, cutaneous infections and a huge inguinal hernia, but without deafness is reported. We believe it represents a new case of a rare X-linked recessive syndrome known as ichthyosis follicularis, alopecia, photophobia syndrome (IFAP). The differential diagnosis from keratitis ichthyosis deafness is discussed. The cutaneous infections seen in our case suggest the possibility of considering a genetic link between these syndromes.

Effect of alcohol use on allograft rejection rates after liver transplantation for alcoholic liver disease.

Alcoholic liver disease is a major cause of liver disease and has become an ever-increasing indication for liver transplantation (LTx). Follow-up studies have reported a higher rate of alcohol recidivism in patients transplanted for alcoholic hepatitis, compared with those transplanted for endstage alcohol-associated cirrhosis. It is assumed widely that recurrent alcohol use is associated with reduced compliance with immune suppression and, as a result, an increased risk of graft rejection and loss. To assess this question, 209 alcoholic patients transplanted for either alcoholic hepatitis with cirrhosis or cirrhosis alone between January 1, 1986 and December 31, 1991 were followed, with a mean follow-up of 4.4 +/- 0.6 years. There were 175 episodes of acute cellular rejection (ACR) that occurred in 137 patients, for an overall rejection rate of 83.7% or at a rate of 1.25 episodes/patient with rejection. The rate of ACR was three times as great in those who remained alcohol-abstinent (2.24 episodes/patient), compared with those who admitted to continued alcohol use (0.75 episodes/patient) (p < 0.01). A total of 33 episodes of chronic rejection occurred in 26 patients, for an overall rate of 12.4%. As was the case for ACR, the chronic rejection rate was greater among those who were continuously alcohol-abstinent, compared with those who intermittently used alcohol after successful LTx. There were no differences in the mean FK 506 or cyclosporin A levels in the groups with and without a rejection episode at the time the rejection episode was documented by liver biopsy.(ABSTRACT TRUNCATED AT 250 WORDS)

Complications of laparoscopic cholecystectomy in HIV and AIDS patients.

We retrospectively evaluated the results of laparoscopic cholecystectomy in patients infected with the human immunodeficiency virus (HIV) with and without acquired immunodeficiency syndrome (AIDS). One thousand one hundred twenty-seven consecutive patients underwent laparoscopic cholecystectomy by our surgical group. Eighteen of these patients were known to be infected with the HIV virus; 6 were asymptomatic and 12 had AIDS. We reviewed the medical records of all HIV-positive individuals with regard to morbidity, mortality, and postoperative outcome following laparoscopic cholecystectomy. In the six HIV-patients without AIDS, five (83%) had improvement of symptoms postoperatively. There was one minor complication (17%). In contrast, only one of the 12 patients with AIDS had postoperative improvement of symptoms and eight (66%) had complications after surgery. There were four deaths (33%) within 30 days of surgery in this group. Only a small percentage of AIDS patients benefit from laparoscopic cholecystectomy. There is a significantly morbidity and mortality following this procedure in this group. Strategies to improve outcome are presented.

Trophic effects of neurotensin in massive bowel resection in the rat.

The trophic effect of the administration of exogenous neurotensin on the intestinal mucosa was studied in rats following an 80% bowel resection. Villus length and mucosal DNA content were assessed in the jejunal and ileal mucosa of the remnant intestine 14 days after resection. The data obtained in an 80% resected control group (80% group) and an experimental group receiving an infusion of neurotensin (300 micrograms/kg/day) for 14 days subcutaneously (80% + NT group) were compared. The results indicate that the administration of exogenous neurotensin (80% + NT) increases villus length (jejunum: 920 +/- 77 vs 861 +/- 25 microns and ileum length: 975 +/- 23 vs 875 +/- 99 microns) to an extent greater than that observed in the 80% resected group not receiving exogenous neurotensin. The levels of mucosal DNA per milligram of protein increased significantly in both groups but was paradoxically less in the 80% + NT group than in the 80% resection group (jejunum: 8.12 +/- 0.56 vs 10.18 +/- 0.80; ileum: 8.63 +/- 0.43 vs 10.05 +/- 0.46). These data suggest that the administration of exogenous neurotensin to the rat potentiates the growth of intestinal villi and accelerates the intestinal trophic response seen following massive bowel resection. The increase in circulating enteroglucagon levels noted after neurotensin administration (80% + NT: 547 +/- 48 pg/ml vs 80%: 341 +/- 41 pg/ml) suggests that some of the trophic effects of neurotensin may be mediated, at least in part, by enteroglucagon. These data also suggest a potential role for the use of neurotensin in the initial treatment of individuals with short bowel syndrome.

Plasma enteroglucagon levels in different models of intestinal resection in the rat.

To assess the influence of the different intestinal segments on the plasma enteroglucagon level, three models of intestinal resection in the rat were studied (jejunal, ileal, 90%). The basal values for this peptide and those obtained after an infusion of intraduodenal glucose were compared. The results obtained in basal/post-glucose infusion were: 50% proximal (jejunum): 220/728 pg/ml; 50% distal (ileum): 10/233 pg/ml; and the middle 90%: 108/297 pg/ml. The glucose infusion produced a maximal response, permitting a better evaluation of the differences among the three resection models. The highest levels corresponded to the group in which the entire ileum was conserved.

Liver transplantation in patients with a previous portasystemic shunt.

Over a six-year period, 4 patients who had undergone previous portasystemic shunt procedures received an orthotopic liver transplantation (OLTx). The types of shunt used were porta-caval (2 patients), distal splenoral shunt (1 patient), and the Sugiura procedure (1 patient). The mean interval between shunt and transplantation was 3.25 years. There was no statistical difference in blood, plasma, and crystalloid requirements, cold and warm ischemia, duration of the transplantation procedure or postoperative complications between these 4 patients and any of the others.

Liver transplantation for alcoholic liver disease: survival of patients transplanted with alcoholic hepatitis plus cirrhosis as compared with those with cirrhosis alone.

From January 1986 through December 1991, a total of 221 patients with alcoholic liver disease received liver transplantation. In 147 of these cases, complete pretransplant histopathologic, demographic, and laboratory data (minimum of CBC, AST, ALT, total bilirubin, albumin, and prothrombin time) were available for review. Forty-five (30%) of the 147 recipients had alcoholic hepatitis plus cirrhosis (AH), whereas 70% had cirrhosis (CIRR) alone. Age and sex were similar in the subgroups, but the patients with CIRR had a greater AST/ALT ratio, longer protime, and lower platelet count (all p < 0.01). Coexistent hepatitis B (4.7%) or hepatitis C (4.1%) was similar in both groups. Current survival is 80% for patients with AH and 84% for those with CIRR (NS). Overall, survivors were younger (43.4 +/- 1.7 years) than nonsurvivors (53.6 +/- 3.2) (p < 0.01), an age influence that was significant in the CIRR group (p < 0.01) but not in the AH group. Inexplicably, the AST/ALT ratio was greater in AH survivors (1.5 +/- 0.2) than it was in nonsurvivors (0.4 +/- 0.1) (p < 0.01). In patients with CIRR, the platelet count was greater in survivors (252 +/- 29 vs. 86 +/- 11 x 10(9) cells/liter). The data support the clinical impression that patients with chronic decompensated cirrhosis referred for liver transplantation had more severe complications of their liver disease than did those with AH. Survival in both subgroups was similar, but overall the survivors are nearly a decade younger than the nonsurvivors.(ABSTRACT TRUNCATED AT 250 WORDS)