Gastroenteric parasite of wild Galliformes in the Italian Alps: implication for conservation management.

This study provides insights about the diversity, prevalence and distribution of alpine wild galliformes gastrointestinal parasite community, trying to fill a gap in the scientific information currently available in scientific literature. The analysis included three host species: 77 rock partridge (Alectoris graeca saxatilis), 83 black grouse (Tetrao tetrix tetrix) and 26 rock ptarmigan (Lagopus muta helveticus) shot during the hunting seasons 2008-2015. Parasites isolated were Ascaridia compar, Capillaria caudinflata and cestodes. The rock ptarmigan was free from gastrointestinal parasites, whereas the most prevalent helminth (37%) was A. compar in both black grouse and rock partridge. C. caudinflata occurrence was significantly higher in black grouse (prevalence = 10%, mean abundance = 0.6 parasites/sampled animal) than in rock partridge (prevalence = 1.20%, mean abundance = 0.01 parasites/sampled animal). Significant differences were detected among hunting districts. A. compar was found with a significant higher degree of infestation in the hunting districts in the northern part of the study area whereas cestodes abundance was higher in Lanzo Valley. Quantitative analysis of risk factors was carried out using a generalized linear model (GLM) only on the most common parasite (A. compar). Latitude was the only factors associated with infestation risk (OR = 52.4). This study provides information on the composition and variability of the parasite community in the alpine Galliformes species.

A novel HLA allele, HLA-A*02:393, identified in a two generation Italian family.

HLA-A*02:393 differs by a single nucleotide substitution from both A*02:26 (A524G) and from A*02:40:01 (T527A).

Long-term results of Rehbein's procedure: a retrospective study in German-speaking countries.

AIM: In contrast to other countries, no collective study of Rehbein's procedure in German-speaking nations has been performed. Therefore, our intention was, analogously to Goto and Ikeda's (10) Japanese study in 1984, Kleinhaus's (13) study on Swenson's procedure in 1979, Bourdelat's (2) French-Canadian investigation into Duhamel's technique in 1997 and Martuciello's (11) and Teitelbaum's (16) follow-up in the year 2000, to perform a follow-up study of Rehbein's technique of deep anterior resection. METHODS: The data of 200 patients from 22 German-speaking centers in Switzerland, Austria and Germany were collected. These data were gathered by questionnaire and the children were followed up in the individual participating hospitals for at least 3.5 years after the procedure. The procedure was performed between 1993 and 1997, over a 5-year period. The questionnaire contained 74 items including anamnestic data, diagnostic postoperative treatment and reoperations. RESULTS: Concerning the incidence of anastomotic leaks and resolving anastomotic strictures there was no significant difference between the results in our series and those of the collective analyses made by Hofmann von Kap-herr (7), Holschneider (9) and Sherman (18). In 6.6 % of the 191 patients an anastomotic leak and in 9.9 % a rectal stricture, which had to be dilated, was observed. Concerning late complications, 22.8 % of the children suffered from constipation, 4.3 % from encopresis, 10.6 % from enterocolitis and only 0.5 % from enuresis. The frequency of constipation diminishes over the years. A comparison of the different large series in the literature clearly shows that the incidence of constipation is higher after Rehbein's procedure and the frequency of urinary incontinence and encopresis higher following Swenson's, Soave's and Duhamel's techniques. The incidence of enterocolitis is less after Rehbein's procedure than after Swenson's, Soave's and Duhamel's techniques. CONCLUSIONS: The different results in the literature are due to the individual experience of the author, the very different follow-up methods and the date of follow-up. Therefore, the different results are hard to compare with our study. Nevertheless, Rehbein's anterior resection still could be presented as an adequate and important method to treat Hirschsprung's disease.

Outcome of a latex avoidance program in a high-risk population for latex allergy - a five-year follow-up study.

BACKGROUND: Children with a shunted hydrocephalus are at highest risk for developing an immediate type allergy to latex. Limited data are available for preventive or therapeutical approaches. OBJECTIVE: To evaluate the effectiveness of latex avoidance, with special regard to status of sensitization and compliance. METHODS: In 1995, 131 children with a shunted hydrocephalus were screened for sensitization to latex by skin prick test and determination of specific IgE. Patients and parents were instructed on latex-avoiding strategies. Hospital physicians, family doctors and dentists were advised to perform further surgical and other medical interventions under latex-free conditions. In 2000, 100 of these 131 patients were re-evaluated according to the same testing procedures. Special attention was directed at the extent prophylaxis had been performed. RESULTS: In 1995, 30/100 patients re-evaluable in 2000 proved sensitized to latex, 70 had negative testing results. In 2000, 64/70 patients were still negative, six had meanwhile developed latex-specific IgE. Seven out of thirty subjects with former positive testing had changes within the same RAST-class, 20 showed a decline of at least one RAST-class, whereas in three cases an increase of latex-specific IgE was found. However, only 34 patients, mainly those being already sensitized, had thoroughly followed both medical and private prophylaxis. Within this group, 16 subjects (47.1%) had improved and another nine (26.5%) were still negative. Only three (8.8%) already previously sensitized patients presented with a further increase of latex-specific IgE. Medical prevention contributed more to the outcome than home prevention. No statistically significant correlation with latex-avoidance was observed, however, in previously unsensitized subjects. Underlying disease, atopy, number of operations, and age did not prove as significant variables. CONCLUSION: Secondary prevention results in a decrease of specific IgE in latex-sensitized patients with hydrocephalus. This is due to medical more than home prophylaxis. Sensitization obviously occurs mainly in early childhood, thus primary prevention remains to be the main target.

Spina bifida as an independent risk factor for sensitization to latex.

PURPOSE: Patients with spina bifida are at a high risk for having an immediate type allergy to latex products. The number of surgical interventions, atopy and catheterization are well known responsible factors, whereas the condition of spina bifida per se has not been established as an independent risk factor. MATERIALS AND METHODS: A total of 131 patients with a shunted hydrocephalus (48 with spina bifida and 83 of other origin) were investigated for sensitization to latex by skin prick tests and determination of specific IgE. We hypothesized that the diagnosis of spina bifida will increase the risk for latex sensitization while considering potential confounding factors. Thus, we performed a multiple logistic regression analysis to determine independent risk factors. RESULTS: Whereas 56.3% (27/48) of children with spina bifida proved sensitized against latex, this result was the case in only 16.9% (14/83) with another cause of hydrocephalus (p <0.001). The mean number of surgical interventions was 6.2 for patients with no latex sensitization and 9.3 for those with sensitization (p = 0.02). Of patient sensitized to latex 43.9% had a history of atopy compared to 15.5% of those not sensitized (p = 0.02). Sensitized and nonsensitized patients were comparable regarding gender and catheterization. In a multiple logistic regression analysis the cause of the hydrocephalus (odds ratio 6.76 for spina bifida), atopy (odds ratio 3.37) and the number of surgical interventions (odds ratio 1.14 per operation) were identified as independent risk factors. CONCLUSIONS: The increased risk of latex sensitization in patients with spina bifida seems to be disease associated. Possible explanations for this finding may be genetic, antigen mediated, early latex exposure and immunological reasons.

Long-term follow-up of children with prenatally diagnosed omphalocele and gastroschisis.

OBJECTIVE: The aim of this study was to follow up the 19 infants born in Tyrol province with abdominal wall defects between 1985 and 1996 whose malformation had been diagnosed prenatally, who were operated on immediately postpartum and who are alive today. METHOD: There were seven children in the omphalocele group and 12 in the gastroschisis group; 18 parents of affected infants took part in the study. RESULTS: Four out of seven children with omphalocele had major associated malformations (two Beckwith-Wiedemann syndrome, one porencephalic cyst, one with skeletal defects). These children presented handicaps related to the associated malformations but not to the abdominal wall defect. The three other children with omphalocele are developing normally. Five out of 11 children with gastroschisis had associated intestinal but no extraintestinal malformations. After discharge, ten of 11 children with gastroschisis were developing normally; one child shows signs of mental retardation. Of 14 mothers who had originally planned another pregnancy prior to the birth of the malformed child, nine decided against becoming pregnant again; the others delayed a further pregnancy for several years. CONCLUSIONS: In our group, associated malformations were the main factor affecting the long-term quality of life of children with omphalocele and gastroschisis. Although most of the children were developing normally, fear of a repetition of the malformation in a subsequent pregnancy dominated reproductive choices in all couples.

Foot deformities in adolescents and young adults with spina bifida.

Spina bifida is generally accompanied by a high incidence of foot deformities. The goal of management is to achieve a plantigrade foot. Deformities are related to the level of the lesion. With thoracic lesions, the most frequent deformity is an equinus lesion (55%), a club foot with mid-lumbar lesion (87%) and a calcaneal foot with sacral lesions (34%). No deformity was present in 56% of feet in sacral lesion children. Club foot surgery before the age of 2 years entails a high rate of recurrence (78%), necessitating redo surgery. A calcaneo-valgus deformity developed in 45% of ambulating patients with sacral lesions requiring operative stabilization of the foot. Patients with sacral lesions were almost the only ones who remained ambulators. Ambulation was not seen to be related to foot deformities in adolescents and young adults.

Hirschsprung's disease and intestinal neuronal dysplasia--a frequent association with implications for the postoperative course.

Between 1991 and 1993, 106 newly diagnosed cases of Hirschsprung's disease (HD) were prospectively analyzed for the association of HD and intestinal neuronal dysplasia (IND) at ten pediatric surgical departments in central Europe. Hirschsprung-associated IND (HaIND) was found in 40% of cases. IND was disseminated in one-third and localized in two-thirds of the patients. Initial clinical symptoms were related to the length of the aganglionic segment, but not to the presence of HaIND. An enterostomy performed in 72 cases (67.9%) was located in a segment of pathologically innervated bowel in 50% of all cases, but in 72% of cases of HaIND. The proximal margin of the resected bowel showed pathological innervation in 44% of cases. Supplemental biopsies from the intestine (apart from diagnostic suction biopsies and biopsies at the enterostomy site) led to the first identification or definition of length of associated IND in 17.9% of cases. Postoperatively, the presence of long-segment aganglionosis or associated IND implied a delay in the restoration or normal defecation. Persistent constipation was found in 40% of patients with associated disseminated IND at follow-up at 6 months, compared to 20.6% in patients with isolated HD. These children needed secondary interventions more often than patients with associated localized IND or isolated HD. HaIND thus has clinical implications for the postoperative course if IND is disseminated.

[Omphalocele and gastroschisis in Tirol-incidence and epidemiology 1985-1996]. / [Omphalozele und Gastroschisis in Tirol - Inzidenz und Epidemiologie 1985-1996]

There are basic differences between the Dutch and the Austrian systems of prenatal care, care during birth and postpartum care: whereas in the Netherlands every effort is made to keep care within the bounds of the eerste lijn - primary health care with midwives and general practitioners as primary providers - in Austria the federal 'family relief fund' and the 'mother and child passport' program have created an elaborate system in which care during pregnancy is not only free but providing antenatal care brings extra payments to the doctor and taking it brings extra money to the pregnant woman. The workings of both systems and the roles of midwives, doctors, hospitals and health insurers are explained and discussed.

Surgical treatment of bacillus Calmette Guérin lymphadenitis.

Although its protective effect is contested and the risk of contracting tuberculosis is rather low nowadays, BCG vaccination is frequently performed. Changes of strain repeatedly led to an increased complication rate. In Austria between 1990 and 1991, of 3386 newborn babies (Strain Pasteur) 116 developed lymphadenitis 3 to 28 weeks after vaccination. The affected children received four types of treatment: nothing specific, isoniazid, or surgery with and without isoniazid. Surgical treatment was found to be necessary in 96 cases. Bacilli were successfully grown in culture in 46% of cases up to week 20 after vaccination; but later than 20 weeks no culture became positive. All cultured bacteria were isoniazid-sensitive. From our data we drew the following conclusions: isoniazid therapy did not prove successful when inflamed lymph nodes exceeded a certain size. Suppurative lymphadenitis in lymph nodes exceeding 1.0 to 1.5 cm usually led to infiltration or even perforation of the skin. Surgery prevents these complications and significantly reduces healing time. Adjuvant isoniazid therapy cannot be recommended, except for generalized BCG tuberculosis.

Bupivacaine plasma concentrations during continuous epidural anesthesia in infants and children.

Venous bupivacaine plasma concentrations were measured in six neonates and infants aged 4 days to 3.9 mo (mean, 2.1 mo) and 10 infants and children aged 9 mo to 6 yr (mean, 3.1 yr) after administration of an initial bolus of 0.5 mL/kg bupivacaine 0.25%, followed by a continuous infusion of local anesthetic (0.25 mL.kg-1.h-1) over a period of 4 h (first hour: bupivacaine 0.25%, then reduced to 0.125%). Plasma concentrations of local anesthetic measured at 180 min and 300 min after beginning of bupivacaine administration were significantly higher in younger infants when compared to older infants and children (180 min: 0.67 +/- 0.24 micrograms/mL [0.25-0.97] vs 0.27 +/- 0.11 micrograms/mL [0.19-0.55], P < 0.01; 300 min: 0.86 +/- 0.36 micrograms/mL [0.35-1.25] vs 0.34 +/- 0.12 micrograms/mL [0.18-0.57], P < 0.01). The results of our study show that despite applying the same dosage of epidural bupivacaine significantly higher plasma concentrations were seen after short periods of continuous infusion in infants up to 4 mo than in children older than 9 mo.

Twenty-three years of follow-up in patients with total colonic aganglionosis.

The main purpose of our study was to evaluate the outcome of patients with total colonic aganglionosis diagnosed and treated in our hospital. Seven of our twelve patients died within 6 month after birth due to infectious complications or underlying other diseases. 5 patients are alive after subtotal colectomy and ileo-rectal anastomosis, and were investigated for late complications, social integration, stool behavior, local status and laboratory parameters 12 and 23 years after surgery. All patients developed almost normally and were socially and professionally integrated. Although all had an accelerated passage of stools, no signs of malnutrition were found. One patient developed a recto-sacral fistulae and sacral osteomyelitis eight years after surgery and needed an ileostomy. After ileo-rectostomy all patients were continent with a grown rectal stump, had no strictures and a normal appearance of the mucosa.

Pallister-Hall syndrome and McKusick-Kaufmann syndrome: one entity?

The Pallister-Hall syndrome is characterised by specific facial anomalies, postaxial polydactyly, imperforate anus, and brain anomalies including a diencephalic hamartoblastoma. The hallmarks of the McKusick-Kaufmann syndrome are hydrocolpos owing to vaginal atresia, postaxial polydactyly, imperforate anus, and congenital heart defects. We report a patient with the unique features of hydrocolpos, postaxial polydactyly, and hypothalamic hamartoblastoma and discuss the different aetiological considerations of both syndromes and implications for clinical management.

HLA-DR5 antigen. A genetic factor influencing the outcome of hepatitis C virus infection?

BACKGROUND: Prognosis may be quite different among individuals infected with hepatitis C virus (HCV): a chronic liver disease is believed to occur in half the patients while in the other half there are no signs of histologic progression of liver damage. The host immune response might play an important role in such different outcomes. A relationship has been shown between HLA genes and immune response to viral hepatitis B, but to our knowledge, no evidence of an association with HCV has been reported so far. We investigated whether HLA class II alleles might influence the outcome of HCV infection. METHODS: Eighty-seven individuals, positive for anti-HCV by second-generation enzyme-linked immunosorbent assay and recombinant immunoblot assay tests, enrolled from May 1, 1991, to June 31, 1992, were evaluated. Thirty-six were symptom-free subjects found to have HCV antibodies when screened for blood donation: they all had normal results of liver function tests, normal results of physical examination, and normal hepatobiliary ultrasonography. Fifty-one were patients diagnosed as having a chronic liver disease by percutaneous liver biopsy specimen; histologic assessment was chronic persistent hepatitis in 15, chronic active hepatitis in 28, and liver cirrhosis in eight. A group of 231 donors of platelets and bone marrow, negative for anti-HCV, was used as a control population. All participants were typed for HLA class II antigens (DR and DQ) using National Institutes of Health recommended microlymphocytotoxicity test and were followed up by means of alanine aminotransferase and HCV testing for at least 1 year. RESULTS: Frequency of HLA-DR5 antigen was higher in symptom-free anti-HCV-positive individuals (52.8%) than among HCV-related patients with chronic liver disease (13.7%). The difference was statistically significant (corrected P value = .005; 95% confidence interval, 19.6% to 58.6%); between DR5 and long-term evolution of hepatitis C, there was a negative association (relative risk = 0.142). Moreover, frequency of HLA-DR5-positive subjects appeared to be inversely proportional to severity of liver disease (52.8% in symptom-free patients, 26.6% in patients with chronic persistent hepatitis, 10.7% in patients with chronic active hepatitis, and 0% in patients with liver cirrhosis, P < .001). CONCLUSIONS: Our results point to a strict relationship between HLA haplotype and ability of immune response to influence the outcome of HCV infection. Presence of HLA-DR5 antigen appears as a protective factor against a severe outcome of chronic infection, being correlated with a benign evolution of the infection, often asymptomatic, or a less severe chronic liver disease.

Teratoma of the umbilical cord. Case report with review of the literature.

Teratomas of the umbilical cord are very rare lesions. We found only five cases in the literature from 1887 to 1993, the latest reported in 1985. We report a case of a 10 x 7 x 5-cm mass located just at the end of an omphalocele in the umbilical cord of a full-term baby. The mass exhibited something like a cranial and a caudal pole, and tissues of all three germinal layers could be found, but there were no skeletal structures. Therefore, this lesion was diagnosed as a teratoma of the umbilical cord. We review the literature and discuss the relationship between the teratoma of the umbilical cord and the holoacardius amorphus.

Analysis of 309 cases of esophageal atresia for associated congenital malformations.

Three hundred nine cases of esophageal atresia were retrospectively investigated. One hundred and sixty-two (52.4%) had associated congenital malformations. The total number of malformations was 357. The eight groups of malformations in decreasing order of frequency were as follows; cardiac, 72 cases (23.3%); musculoskeletal, 55 cases (17.8%); anointestinal, 50 cases (16.2%); genitourinary, 45 cases (14.6%); head and neck, 31 cases (10.0%); mediastinal, 26 cases (8.4%); chromosomal, 17 cases (5.5%); pulmonary, 6 cases (1.9%). Of the 162 cases with associated malformations, 83 (51.2%) had a single group of malformations, 41 (25.3%) had two groups of malformations, and 38 (23.5%) had three or more groups of malformations. More than 50% of pulmonary (83.3%), head and neck (77.4%), chromosomal (58.8%), and mediastinal malformations (53.8%) were found in association with cardiac malformations. The frequency of associated malformations in esophageal atresia cases was found to be increased significantly during the investigated interval. Early gestational age and lower birthweights were significantly correlated with higher rates of malformations. The survival rates of patients with esophageal atresia was also found to be significantly increasing over time. The mean survival rates over 32 years for groups with and without malformations were 38.3% and 70.7%, respectively, and were statistically significant. The mean survival rates of the same groups in the last 5 years were found to have changed dramatically, rising to 62.9 in the group with malformations and to 100% in the group without malformations; again, the difference is significant.

Recent evaluation of prognostic risk factors in esophageal atresia--a multicenter review of 223 cases.

In this study, 223 cases of esophageal atresia (Type IIIb: 85.7%; Type II: 5.8%; Type IIIc: 4.0%; Type IIIa: 2.2%; Type IV: 2.2%) from 6 pediatric surgery centers of Austria, were retrospectively examined for the following parameters and their influence on the prognosis: Birth weight (2494.7 +/- 702.0 g), gestation week (range 27-42 weeks; mean 37.3 +/- 3.1 weeks), sex (male: n = 128; female: n = 95), long-gap atresia (> or = 2 cm: n = 33), Tracheomalacia (n = 16), associated malformations (n = 122; cardiac 27.4%, renal 17.9%, skeletal 17.0%, anal: 10.3%, intestinal 9.9%, mediastinal 7.6%, chromosomal 2.2%), preoperative aspiration (n = 92), pneumonia (n = 96), anastomotic insufficiency (n = 45), empyema (n = 5), mediastinitis (n = 8), sepsis (n = 32), other medical complications (n = 122, in 80 infants), other surgical complications (n = 57). The mortality rate was 41.3% overall, from 1975 to 1991; however, it was 25% from 1987 to 1991 and 0% in 1991. A statistically significant correlation was found between prognosis and the following factors: Cardiac malformations (p = 0.0001), medical complications except aspiration and pneumonia (p = 0.0001), empyema (p = 0.0081), mediastinitis (p = 0.0214), and sepsis (p = 0.0295). These 5 significant factors were given different points and a prognostic score was calculated by the addition of these points. This score was predictive for survival in 90.6% of cases and for mortality in 94% of cases.(ABSTRACT TRUNCATED AT 250 WORDS)