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Shwachman-Diamond syndrome (SDS) is a multisystem disorder characterized by exocrine pancreatic insufficiency and bone marrow failure. There is considerable variation in the phenotypes of SDS. We present a case of an infant presenting with SDS and left-sided ptosis.We report a case of an infant who presented with 2 episodes of severe sepsis and cytopenia, without overt symptoms of exocrine pancreatic deficiency or skeletal abnormalities. Persistent left-sided ptosis was noted in both presentations. Genetic testing confirmed the diagnosis of SDS. The left-sided ptosis was diagnosed as congenital myogenic ptosis.The association of ptosis and other congenital bone marrow failure syndromes is well established, but this is the first description of SDS with ptosis. This association may expand our understanding of SDS phenotypes if similar cases are reported in the future.

Recent national surveys in The United Republic of Tanzania have revealed poor standards of hygiene at birth in facilities. As more women opt for institutional delivery, improving basic hygiene becomes an essential part of preventative strategies for reducing puerperal and newborn sepsis. Our collaborative research in Zanzibar provides an in-depth picture of the state of hygiene on maternity wards to inform action. Hygiene was assessed in 2014 across all 37 facilities with a maternity unit in Zanzibar. We used a mixed methods approach, including structured and semi-structured interviews, and environmental microbiology. Data were analysed according to the WHO ‘cleans’ framework, focusing on the fundamental practices for prevention of newborn and maternal sepsis. For each ‘clean’ we explored the following enabling factors: knowledge, infrastructure (including equipment), staffing levels and policies. Composite indices were constructed for the enabling factors of the ‘cleans’ from the quantitative data: clean hands, cord cutting, and birth surface. Results from the qualitative tools were used to complement this information. Only 49% of facilities had the ‘infrastructural’ requirements to enable ‘clean hands’, with the availability of constant running water particularly lacking. Less than half (46%) of facilities met the ‘knowledge’ requirements for ensuring a ‘clean delivery surface’; six out of seven facilities had birthing surfaces that tested positive for multiple potential pathogens. Almost two thirds of facilities met the ‘infrastructure (equipment) requirement’ for ‘clean cord’; however, disposable cord clamps being frequently out of stock, often resulted in the use of non-sterile thread made of fabric. This mixed methods approach, and the analytical framework based on the WHO ‘cleans’ and the enabling factors, yielded practical information of direct relevance to action at local and ministerial levels. The same approach could be applied to collect and analyse data on infection prevention from maternity units in other contexts.

The article justifies the conception that innovation development of economy requires its breaking-up by production sectors and deep study of tendencies of development of each of them, namely: 1) sector of production of means of production; 2) sector of production of commodities; 3) sector of production and household services; 4) sector of formation of human capital and creation of intellectual values; and 5) sector of state administration. The article reveals and analytically describes tendencies of development of the sectors for the past 10 years. It shows, using example of development of the sector of production of means of production, that the past decade has faced the permanent process of accumulation of the deficit of production of means of production. Thus, for example, the deficit in 2010 was UAH 776 milliard. One cannot agree with the tendency of growth of a number of personnel of state administration in the age of computerisation and revolution in record keeping. Personnel increased by 195.7 thousand people only for the past five years, besides, the major part of it belongs to the state administration. Growth of the state machinery does not assist in the growth of economic efficiency.В статье обосновано понимание того, что инновационное развитие экономики требует ее расчленения по производительным секторам и глубокого изучения тенденций развития каждого из них, а также выделены: 1) сектор производства средств производства; 2) сектор производства предметов потребления; 3) сектор производственных и бытовых услуг; 4) сектор формирования человеческого капитала и создания интеллектуальных ценностей; 5) сектор государственного управления. Выявлены и аналитически описаны тенденции развития секторов за последние 10 лет. На апробированном примере развития сектора производства средств производства показано, что в последнем десятилетии происходит постоянный процесс наращивания дефицита производства средств производства. Так, например, в 2010 г. дефицит составлял 776 млрд грн. Нельзя согласиться с тенденцией роста числа персонала государственного управления в век компьютеризации и революции в делопроизводстве. Только за последние пять лет персонал увеличился на 195,7 тыс. человек, причем значительную часть составляет общегосударственное управление. Рост государственного аппарата не способствует росту экономической эффективности.

To determine the association between carotid femoral pulse wave velocity (PWVCF); augmentation index (AI); and high-sensitivity C reactive protein (hs-CRP) with metabolic syndrome (MetS), and to determine the influence of ethnicity on PWVCF and AI, and the association between high hs-CRP and increased PWV, and AI in MetS.A cross-sectional study was conducted at Universiti Kebangsaan Malaysia Medical Center, Kuala Lumpur, Malaysia from September 2009 to September 2011. Three hundred and eighty men (Chinese and Malays) were recruited from the study. The PWVCF and AI were measured by Vicorder (SMT Medical, Wuerzburg, Germany). The hs-CRP level was also determined. We defined MetS using the International Diabetes Federation (IDF) and harmonized criteria.Malays had higher AI compared to the Malaysian Chinese. Patients with MetS had higher PWVCF (IDF criteria: 8.5 [8.3-8.7] versus 8.2 [8.0-8.4] m/s, p=0.03; harmonized criteria: 8.5 [8.4-8.7] versus 8.2 [8.0-8.4] m/s, p=0.007) and hs-CRP (IDF criteria: 0.9+/-2.0 versus 0.4+/-1.1 mg/L, p=0.0007; harmonized criteria: 0.8+/-1.9 versus 0.4+/-1.1 mg/L, p=0.002) compared to non-MetS. In subjects with MetS, those with high hs-CRP (3 mg/L) had higher PWVCF.Augmentation index values were significantly higher in Malays compared with Malaysian Chinese. Metabolic syndrome was associated with increased PWVCF and hs-CRP. Patients with MetS and high hs-CRP were associated with higher PWVCF. The measurement of hs-CRP reflects the degree of subclinical vascular damage in MetS.

To estimate the frequency of walking to school among primary school children and examine associated factors.In-class survey of Years 1-6 children attending Dunedin primary schools, November 2004, and a take home, written questionnaire for parents and caregivers.On study day, 34.5% of children walked to school and 36.8% intended to walk home. Overall, 1157 completed caregiver questionnaires were returned (68%) indicating that 47.5% of children walked to or from school less than three times per week. The strongest predictor of walking was proximity to school (1 km OR 29.3, 1-2 km OR 7.7, 2-3 km OR 3.0,3 km OR 1.00). Other predictors were not having a car in the household (OR 10.9), attending a (low socioeconomic) decile 2 to 4 school (2.35), having three or more adults in the household (1.85), being in a higher school year (1.72), having non-New Zealand European ethnicity (1.41), having a parent who had walked to school (1.35), and being male (1.33).This study established a baseline for the percentage of Dunedin primary school children walking to school. Key potentially modifiable predictors of walking were proximity to school and not having a car in the household. These findings have implications for health, transport and educational policies.