A systematic review of reported cases of pachyonychia congenita tarda.

Pachyonychia congenita (PC) describes a group of genodermatoses manifesting as thickened nails, palmoplantar keratoderma (PPK) and increased risk of cutaneous infections. PC tarda (PCT) describes late-onset PC, and associated genetic polymorphisms have been identified. There has been discussion that PCT may not be a distinct entity but rather misdiagnosed ectodermal dysplasia (ED) or PPK. Clarification of this is important for appropriate diagnosis, management and patient and genetic counselling. We aimed to conduct a systematic review of all reported cases of PCT in the published literature and collate evidence of genetic polymorphisms and clinical features to compare with known features of PC, ED and PPK. PubMed (1946 to 1 July 2018), Scopus (1955 to 1 July 2018) and Web of Science (1990 to 1 July 2018) databases were searched for case reports of PCT with no search restrictions on date or language. The search strategy included the terms pachyonychia congenita tarda OR pachyonychia congenita AND (late onset OR delayed OR PCT). In total, 13 reports describing 19 individual cases of PCT were identified. Of the three identified genetic polymorphisms, the earliest identified has been shown to be highly probably pathogenic, with the second likely to result in a benign amino acid change, while the third has since been shown to be nonpathogenic,. No epigenetic studies have been performed on any reported cases. Previous authors have suggested that a number of cases of PCT may be misdiagnosed ED or PPK. The findings of our review cannot refute this suggestion, and highlight the need for thorough clinical documentation of suspected cases of PCT and thorough genetic screening of kindred to identify causative genetic polymorphisms. Further high-quality datasets and reporting are needed to give further insight into the nature of PCT as a unique entity.

Validation of current procedural terminology codes for surgical stabilization of rib fractures.

INTRODUCTION: Current procedural terminology (CPT) codes for surgical stabilization of rib fractures (SSRF) are based solely on the number of ribs fixed, tricotomized at 1-3, 4-6, and ≥ 7. Our objective was to validate CPT codes against operative time at our institution, as well as further stratify complexity by rib fracture location and surgical approach. The purpose of this study is to validate the current CPT coding schema for SSRF, and to identify potential modifiers that are associated with increased case complexity. We hypothesized that operative time is associated with CPT code, number of fractures repaired, exposure technique, and fracture location. METHODS: Retrospective review of SSRF cases from October 2010 to March 2020. The primary outcome was the length of the operation (minutes). Predictor variables were CPT code, number of fractures repaired (grouped similarly to CPT codes), fractures repaired:ribs repaired ratio > 1, fracture location (sub-scapular vs. other), and positioning/exposure (supine, lateral, prone, and multiple). Kaplan-Meier time-to-event analyses were used to assess relationship with operative time. RESULTS: 188 patients underwent repair of 904 fractures. Operative time was significantly associated with both number of ribs repaired and number of fractures repaired (p<0.01). Although operative time varied significantly by CPT group (p<0.01), there was no significant difference between the 4-6 rib and the ≥ 7 rib groups (p = 0.33). By contrast, each group was significantly different from the others when organized by number of fractures repaired (p = 0.04). Operative time was significantly longer when the fractures repaired:ribs repaired ratio was > 1 (p<0.01), even after stratifying by number of ribs repaired. Both multiple positions/exposures (p<0.01), and repair of ≥ 1 sub-scapular fracture (p<0.01) were significantly associated with operative time. CONCLUSION: Number of fractures repaired provided a more accurate estimation of operative time as compared to number of ribs repaired. Based on these data, we recommend altering the CPT schema for SSRF to involve number of fractures repaired, with modifiers for both multiple positions/exposures and repair of sub-scapular fractures.

Responsibility of the obstetrician to the fetus. II. Influence of prepregnancy weight and pregnancy weight gain on birthweight.

The diet of pregnant women has been restricted in various ways since earliest history. Dietary restriction may have severely deleterious effects on the fetus. Our study revealed a positive correlation of prepregnancy weight and pregnancy weight gain to term infant birthweight. Their influences are independent and additive. It is of the utmost importance to the fetus and the future of the human race that the diet of pregnant women contain the caloric value and essential nutrients recommended by the National Research Council.

Foreign worker dependence in the Gulf, and the international oil companies: 1910-50.

"This article demonstrates that foreign worker dependence in the [Persian] Gulf dates from the establishment of the oil industry in the early twentieth century. The composition of labor inflows [was] mainly determined by political and strategic, rather than commercial, concerns. Contrasting patterns of labor force composition evolved between those areas under British control, which imported labor from the Indian sub-continent, and the independent Saudi Arabia where labor was drawn from more diverse sources including the Italian settlers in Eritrea."

Spatial aspects of population change in Libya.

PIP: Developments in the spatial distribution of Libya's population are analyzed for the years 1954-1980 using data from official and other published sources. Particular emphasis is given to the continuing concentration of population in the two major cities, Tripoli and Benghazi. Policies to influence the country's population distribution are considered. (summary in FRE)^ieng

Between Western Europe and the Middle East: changing patterns of Turkish labour migration.

The authors examine Turkish labor migration to Western Europe prior to 1973 and to the Middle East since that date. "The growth, organization and characteristics of these two phases in Turkish migration history are compared and contrasted. Particular emphasis is paid to the patterns of migrant worker origin and rates of emigration in the two periods. During the period 1961-73 the pattern of migration was dominated by the metropolitan and developed areas of Western Turkey. The re-direction of migration to Saudi Arabia, Libya and Iraq has been accompanied by a contraction and eastward shift in the main areas of migrant origins. Developments in the pattern of migrant worker origins are related to changes in the skill composition of the migrants and the mechanisms of the migration process. Opportunities for the employment of Turkish workers in the Arab world appear to have peaked and, although it has produced some short-term financial advantages, it has had little effect on Turkey's employment problems." (SUMMARY IN FRE AND TUR)

Progress in the human geography of the Maghreb.

PIP: Sutton and Lawless review a selection of the prolific literature on the Maghreb's human geography published from the late 1970s onward. The pedigree of the discipline in Maghreb was established by several notable geographers who lived, researched, and wrote there. Augustin Bernard's writings on rural settlement, his atlas, and study of Algeria are still cited. Jean Despois's regional study with Raynal and his work on Tunisia, together with the detailed rural research of Jean Poncet, provides a particularly rich coverage for that country. Jean Dresch's early work on Morocco exemplified an early critique of the contemporaneous uncritical colonial human geography. The Sahara's fascination also inspired pioneering studies by Robert Capot-Rey. The work of these notables and others provided a solid background of geographical literature on the Maghreb to be supplemented, criticized, and challenged by more recent researchers, sometimes pupils of the above individuals, and increasingly North African by birth. The tradition of general texts on the Maghreb has continued, though increasingly with systematic approaches replacing regional appraisals. The geography of the decolonization of the Maghreb by Isnard has been replaced by a study summarizing recent collaborative work by a team of French geographers. The Saharan extensions of the Maghreb states have often been linked in general works, sometimes being juxtaposed against the Maghreb as in the essays offered to Jean Despois. Lawless and Findlay's collaborative study juxtaposes geographical studies of each country's economic development with political scientists' evaluations of their sociopolitical development. This review limits itself largely to geographers and geographical journals, only occasionally including significant works from other subject areas, and to 1977-1978 publications, with a few notable exceptions. The spatial juxtaposition of contrasting approaches to economic development, liberal market economy approaches by Tunisia and Morocco arguably leading to dependency, and centrally-planned strategies by Algeria and Libya resulting in bureaucratic constraints serves to offer the Maghreb as a pertinent case-study region for the applied geographer of developing countries.^ieng

Some new trends in Mediterranean labour migration: the Middle East connection.

PIP: Changes in international labor migration in the Mediterranean region since the European economic recession of the early 1970s are examined. The authors note that labor migration to the oil-producing countries of the Middle East has increased and that this migration has differed from the previous movements to Europe, in that the migrants involved have been employed by contractors from their countries of origin or by other foreign companies. The probable future decline of this migration as infrastructure projects are completed is discussed, and the consequences are examined.^ieng

The Middle East: a new destination for Turkish labour migration.

PIP: The shifts in migration experienced by Turkish emigrants are discussed starting with the large-scale emigration to Western Europe in the early 1960s. Official figures illustrate that since the 1970s the major current has been toward the Middle East and North Africa, specifically to Libya, Saudi Arabia, and Iraq. Socioeconomic and political factors influencing Turkish migration are discussed. The earlier migrants to Western Europe and later migrants to the Middle East and North Africa are compared on the bases of sex ratio, settlement and duration of stay, remittances, motives, and individual migrants versus organized groups of workers. Turkish migration to Australia is also discussed.^ieng