The true identity of \u3cem\u3eScorpio\u3c/em\u3e (\u3cem\u3eAtreus\u3c/em\u3e) \u3cem\u3eobscurus\u3c/em\u3e Gervais, 1843 (Scorpiones, Buthidae)

The true identity of the scorpion Scorpio (Atreus) obscurus Gervais, 1843 is discussed based on new data. The original type material was recently discovered in the collections of the Muséum national d’Histoire naturelle in Paris, which confirms that this old species, originally described by Gervais based on two female specimens from French Guiana, does indeed belong to the genus Tityus C. L. Koch, 1836. Furthermore, an analysis of all morphological characteristics of Tityus (Atreus) obscurus verifies its position as a senior synonym of both Tityus paraensis Kraepelin, 1896 and Tityus cambridgei Pocock, 1897. In this paper, we designate the two female syntypes of Scorpio (Atreus) obscurus as a female lectotype and a female paralectotype for Tityus (Atreus) obscurus (Gervais, 1843), and synonymize Tityus paraensis Kraepelin, 1896 with Tityus obscurus (Gervais, 1843)

Further considerations on the species of the genus \u3cem\u3eOrthochirus\u3c/em\u3e Karsch, 1891 from Africa, with description of three new species (Scorpiones: Buthidae)

New considerations are proposed regarding the African species of the genus Orthochirus Karsch, 1891. Two species, Orthochirus aristidis (Simon, 1882) and Orthochirus innesi Simon, 1910 have been the subject of several publications in the past decades; however, doubts remain about their exact identity and range of geographical distribution. In this note, their taxonomic status is reinvestigated. The type material is revised and the lectotype and paralectotypes are designated for O. aristidis. Revised diagnoses and illustrations are proposed for both species, and these are confirmed as valid. Three new species are described from Algeria, Morocco, and Mauritania. The total number of African species is raised to five

One more new species of the genus \u3cem\u3eOrthochirus\u3c/em\u3e Karsch, 1891 from Africa (Scorpiones: Buthidae)

Following the recent considerations proposed on the African species of the genus Orthochirus Karsch, 1891, one new species is described from the south of Morocco. The total number of African species is now raised to six

A new species of the genus \u3cem\u3eButhus\u3c/em\u3e (Scorpiones: Buthidae) from northern Cameroon

A new species belonging to the genus Buthus Leach (Scorpiones: Buthidae) is described from northern Cameroon in Central Western Africa. The new species can be included in the “Buthus occitanus” complex of species, and probably can be associated with the “Buthus occitanus” from the former French West Africa (AOF) previously reported by Vachon from this large region. This is the first record of a Buthus species from Cameroon, and with the description of Buthus prudenti sp. n., the status of one more population of Buthus spp. from the sub-Saharan region of Africa is clarified

A new species of \u3cem\u3eAndroctonus\u3c/em\u3e Ehrenberg, 1828 from Morocco (Scorpiones: Buthidae)

A new species of scorpion belonging to the genus Androctonus Ehrenberg, 1828 (family Buthidae C. L. Koch, 1837), is described on the basis of one male and one female specimens collected at Sidi Smaïl in the Atlantic Coast of Morocco. With the description of Androctonus maroccanus sp. n., the total number of Androctonus species known to Morocco is raised to seven

The genus \u3cem\u3eChaerilus\u3c/em\u3e Simon, 1877 (Scorpiones, Chaerilidae) in the Indian Ocean Islands and description of a new species

A new species is described belonging to the genus Chaerilus Simon, 1877. Chaerilus andamanensis sp. n. was discovered in the Island of Little Andaman in the Indian Ocean. The new species shows affinities with Chaerilus variegatus Simon, 1877 and Chaerilus borneensis Simon, 1880, respectively described from Java and Borneo. This s the first record of the genus Chaerilus in the Andaman Islands

High resolution MRI for preoperative work-up of neonates with an anorectal malformation: a direct comparison with distal pressure colostography/fistulography

Objective: To compare MRI and colostography/fistulography in neonates with anorectal malformations (ARM), using surgery as reference standard. Methods: Thirty-three neonates (22 boys) with ARM were included. All patients underwent both preoperative high-resolution MRI (without sedation or contrast instillation) and colostography/fistulography. The Krickenbeck classification was used to classify anorectal malformations, and the level of the rectal ending in relation to the levator muscle was evaluated. Results: Subjects included nine patients with a bulbar recto-urethral fistula, six with a prostatic recto-urethral fistula, five with a vestibular fistula, five with a cloacal malformation, four without fistula, one with a H-type fistula, one with anal stenosis, one with a rectoperineal fistula and one with a bladderneck fistula. MRI and colostography/fistulography predicted anatomy in 88 % (29/33) and 61 % (20/33) of cases, respectively (p = 0.012). The distal end of the rectal pouch was correctly predicted in 88 % (29/33) and 67 % (22/33) of cases, respectively (p = 0.065). The length of the common channel in cloacal malformation was predicted with MRI in all (100 %, 5/5) and in 80 % of cases (4/5) with colostography/fistulography. Two bowel perforations occurred during colostography/fistulography. Conclusions: MRI provides the most accurate evaluation of ARM and should be considered a serious alternative to colostography/fistulography during preoperative work-up. Key Points: • High-resolution MRI is feasible without the use of sedation or anaesthesia. • MRI is more accurate than colostography/fistulography in visualising the type of ARM. • MRI is as reliable as colostography/fistulography in predicting the level of the rectal pouch. • Colostography/fistulography can be complicated by bowel perforation

The development and validation of a cerebral ultrasound scoring system for infants with hypoxic-ischaemic encephalopathy

BACKGROUND: Hypoxic-ischaemic encephalopathy (HIE) is an important cause of morbidity and mortality in neonates. When the gold standard MRI is not feasible, cerebral ultrasound (CUS) might offer an alternative. In this study, the association between a novel CUS scoring system and neurodevelopmental outcome in neonates with HIE was assessed. METHODS: (Near-)term infants with HIE and therapeutic hypothermia, a CUS on day 1 and day 3-7 after birth and available outcome data were retrospectively included in cohort I. CUS findings on day 1 and day 3-7 were related to adverse outcome in univariate and the CUS of day 3-7 also in multivariable logistic regression analyses. The resistance index, the sum of deep grey matter and of white matter involvement were included in multivariable logistic regression analyses. A comparable cohort from another hospital was used for validation (cohort II). RESULTS: Eighty-three infants were included in cohort I and 35 in cohort II. The final CUS scoring system contained the sum of white matter (OR = 2.6, 95% CI 1.5-4.7) and deep grey matter involvement (OR = 2.7, 95% CI 1.7-4.4). The CUS scoring system performed well in cohort I (AUC = 0.90) and II (AUC = 0.89). CONCLUSION: This validated CUS scoring system is associated with neurodevelopmental outcome in neonates with HIE

Comparable low-level mosaicism in affected and non affected tissue of a complex CDH patient

In this paper we present the detailed clinical and cytogenetic analysis of a prenatally detected complex Congenital Diaphragmatic Hernia (CDH) patient with a mosaic unbalanced translocation (5;12). High-resolution whole genome SNP array confirmed a low-level mosaicism (20%) in uncultured cells, underlining the value of array technology for identification studies. Subsequently, targeted Fluorescence In-Situ Hybridization in postmortem collected tissues demonstrated a similar low-level mosaicism, independently of the affected status of the tissue. Thus, a higher incidence of the genetic aberration in affected organs as lung and diaphragm cannot explain the severe phenotype of this complex CDH patient. Comparison with other described chromosome 5p and 12p anomalies indicated that half of the features presented in our patient (including the diaphragm defect) could be attributed to both chromosomal areas. In contrast, a few features such as the palpebral downslant, the broad nasal bridge, the micrognathia, microcephaly, abnormal dermatoglyphics and IUGR better fitted the 5p associated syndromes only. This study underlines the fact that low-level mosaicism can be associated with severe birth defects including CDH. The contribution of mosaicism to human diseases and specifically to congenital anomalies and spontaneous abortions becomes more and more accepted, although its phenotypic consequences are poorly described phenomena leading to counseling issues. Therefore, thorough follow-up of mosaic aberrations such as presented here is indicated in order to provide genetic counselors a more evidence based prediction of fetal prognosis in the future

Long-term cognitive follow-up in children treated for Maroteaux-Lamy syndrome

Background: It remains unclear to what extent the brain is affected by Maroteaux-Lamy syndrome (MPS VI), a progressive lysosomal storage disorder. While enzyme replacement therapy (ERT) elicits positive effects, the drug cannot cross the blood–brain barrier. We therefore studied cognitive development and brain abnormalities in the Dutch MPS VI patient population treated with ERT. Methods: In a series of 11 children with MPS VI (age 2 to 20 years), we assessed cognitive functioning and brain magnetic resonance imaging prospectively at the start of ERT and at regular times thereafter up to 4.8 years. We also assessed the children’s clinical characteristics, their siblings’ cognitive development, and their parents’ educational levels. Results: The patients’ intelligence scores ranged from normal to mentally delayed (range test scores 52–131). In 90 %, their scores remained fairly stable during follow-up, generally lying in the same range as their siblings’ test scores (median for patients = 104, median for siblings = 88) and comparing well with the parental educational levels. Native-speaking patients had higher intelligence test scores than non-native-speaking patients. Two patients, both with high baseline glycosaminoglycan levels in their urine and severe mutations in the arylsulfatase B gene, scored clearly lower than expected. Patients with pY210C performed best. Brain abnormalities were aspecific, occurring more in patients with severe symptoms. Conclusion: Our study shows that cognitive development in MPS VI patients is determined not only by familial and social-background factors, but, in patients with a severe form of the disease, also by the disease itself. Therefore in patients with severe disease presentation cognition should be monitored carefully