Prenatal presentation and postnatal evolution of a patient with Jansen metaphyseal dysplasia with a novel missense mutation in PTH1R.

Wave-shaped ribs were detected at prenatal ultrasound in a 20(+1) week female fetus. At birth, skeletal radiographs showed marked hypomineralization and suggested hypophosphatasia. However, elevated blood calcium and alkaline phosphatase excluded hypophosphatasia and raised the possibility of Jansen metaphyseal dysplasia. Molecular analysis of the PTH/PTHrP receptor gene (PTH1R) showed heterozygosity for a previously undescribed transversion variant (c.1373T>A), which predicts p.Ile458Lys. In vitro evaluation of wild type and mutant PTH/PTHrP receptors supported the pathogenic role of the p.Ile458Lys substitution, and confirmed the diagnosis of Jansen metaphyseal dysplasia. This disorder may present prenatally with wavy ribs and in the newborn with hypomineralization, and may therefore be confused with hypophosphatasia. The mottled metaphyseal lesions typically associated with this disease appear only in childhood.

Credit rationing and crowding out during the Industrial Revolution: Evidence from Hoare's Bank, 1702-1862

Crowding-out during the British Industrial Revolution has long been one of the leadingexplanations for slow growth during the Industrial Revolution, but little empirical evidence exists to support it. We argue that examinations of interest rates are fundamentally misguided, and that the eighteenth- and early nineteenth-century private loan market balanced through quantity rationing. Using a unique set of observations on lending volume at a London goldsmith bank, Hoare s, we document the impact of wartime financing on private credit markets. We conclude that there is considerable evidence that government borrowing, especially during wartime, crowded out private credit.

The role of Mexico in the first oil shortage: 1918-1922, an international perspective

In 1921 Mexico produced a quarter of world s petroleum, making the country the secondlargest producer in the world, but by 1930 it only accounted for 3 per cent of world sproduction. To date the discussion has mostly relied on events taking place in Mexico forexplaining the decline of the industry. Very little attention has been placed todevelopments in petroleum industry elsewhere, except Venezuela. Practically noattention has been paid to the reasons for the rise of oil output in Mexico. This neglectsthe massive changes taking place in the petroleum industry worldwide during the GreatWar years and its aftermath, and overall ignores the shortage of oil that occurred in theworld s markets between 1918-1921. These are crucial events in order to understand theearly rise of the Mexican oil industry and set the basis for a better understanding of thesubsequent sudden decline.

Avaliacao dos resultados cirurgicos com a tecnica da resseccao do musculo de Muller-conjuntiva no tratamento da blefaroptose. [Evaluation of the surgery results of Muller's muscle-conjunctival resection in the treatment of blepharoptosis]

OBJECTIVE: To evaluate the results of Muller's muscle-conjunctival resection for correction of blepharoptosis and to discuss the advantages of this procedure. METHODS: 38 patients (39 eyelids) were submitted to Muller's muscle-conjunctival resection. Blepharoptosis varied from 1.0 mm to 3.0 mm (mean: 2.0 mm). The amount of eyelid elevation produced by phenylephrine guided the amount of tissue to be resected. RESULT: 33 eyelids (85%) treated with this procedure were cosmetically acceptable. CONCLUSIONS: Muller's muscle-conjunctival resection procedure is a relatively simple technique for blepharoptosis, with good levator function and positive 10% phenylephrine test. The advantages are: preservation of tarsus and predictable results.

Itä-Karjalasta Suomeen 1917-1922 tulleet pakolaiset

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