Ehlers-Danlos type IV is a major concern to vascular surgeons because it is often associated with spontaneous hemorrhage from arteries containing decreased type III collagen. Five members of a family with Ehlers-Danlos type IV and a review of another family of five with Ehlers-Danlos type IV are reported. Evaluation of the recent family included clinical evaluation as well as assay of collagen production. The age range of the three involved females and two males was 7 to 52 years. The father of the affected family had a spontaneous colon perforation at age 39. His son, at age 27, had a spontaneous rupture of the iliac artery. Revascularization was accomplished with difficulty. His daughter had a large cerebral bleed. Two granddaughters, ages 7, have not had any bleeding or aneurysmal events. The amount of type III collagen was only 10% of normal in the patient with the iliac artery rupture. The three females all exhibited similarly low levels of type III collagen. The father's type III collagen level was not sufficiently low to confirm Ehlers-Danlos type IV, although he had a spontaneous colon perforation. In the other Ehlers-Danlos type IV family of five, the three surviving members had type III collagen levels as low as 5% of normal. Two family members died after spontaneous iliac rupture at ages 24 and 33. Both families exhibited an autosomal dominant inheritance pattern. Ehlers-Danlos type IV remains a challenging problem for vascular surgeons. It is transmitted as an autosomal dominant inheritance with a high degree of penetrance. Spontaneous arterial and intestinal perforations should alert the clinician to the possibility of Ehlers-Danlos type IV. Patients should be evaluated noninvasively. Arterial repairs may not be successful in these patients because the vessels are extremely friable. Assays of collagen production are advisable in establishing the diagnosis.
The policy of routine angiography (ANG) for all penetrating neck wounds results in a high rate of negative studies. The medical records of all patients who presented to Wishard Memorial Hospital and Methodist Hospital of Indiana with penetrating injuries to the neck from January 1992 to April 2001 were reviewed. All patients who were hemodynamically stable underwent four-vessel ANG to evaluate for vascular injury irrespective of findings on physical examination (PE). A total of 216 patients sustained penetrating neck injuries. Patients were divided according to positive or negative PE findings and the results of ANG. Of the 63 patients with a positive PE, 40 (68%) also had a positive ANG finding. Of the 89 patients with negative PE, only 3 had a positive ANG and none of these injuries required operative repair. PE therefore had a 93 per cent sensitivity (SEN) and a 97 per cent negative predictive value (NPV) for predicting the results of ANG. The SEN and NPV of PE for detecting vascular injuries requiring operative repair were both 100 per cent. In this series, no patient with a negative PE had a vascular injury that required operative repair, irrespective of zone of injury. Routine ANG may therefore be unnecessary for patients with penetrating neck injuries and a negative PE.
Five stars in the extreme outskirts (from ∼ 5 to ∼ 12 elliptical half-light radii, r h ) of the Ursa Minor (UMi) dwarf galaxy have been identified as potential new members using a Bayesian algorithm applied to Gaia EDR3 data. These targets were observed with the GRACES spectrograph, resulting in precise radial velocities and metallicities that confirm their association with UMi. For the brightest and outermost star (Target 1, at ∼ 12 r h ), the chemical abundances of α-(Mg, Ca, Ti), odd-Z (Na, K, Sc), Fe-peak (Fe, Ni, Cr), and neutron-capture process (Ba) elements have also been determined. We also discuss data from the literature and from APOGEE DR17. We find the chemical patterns in UMi are consistent with a star formation history that includes yields from core collapse supernovae, asymptotic giant branch stars, and supernovae Ia. Evidence for a knee in the [α/Fe] ratios near [Fe/H] ∼ −2.1 indicates a low star formation efficiency similar to that in other dwarf galaxies. Given the distance of Target 1 from the centre of UMi (R∼4.5 kpc), we show that UMi has a more extended structure than previously thought. This "stellar halo" around UMi could be a secondary feature resulting from tidal stripping after multiple orbits around the Galaxy, or maybe a primary UMi feature due to early hierarchical accretion activity or to strong gravitational fluctuations prompted by feedback in the early star formation phase. Also consistent with observations is a late-time merger-free scenario where outside-in star formation is accompanied by gradual supernovae Ia enrichment.
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