Diabetic ketoacidosis admissions at Middlemore Hospital: observational study of cause and patient demographics.

AIM: To analyse data on diabetic ketoacidosis (DKA) admissions to better understand characteristics of those presenting with DKA and identify high-risk groups. METHOD: Study population consisted of people with type 1 diabetes discharged from Middlemore Hospital between 01 July 2015 and 30 June 2016 with the diagnosis of DKA. Basic demographic data and socioeconomic status as defined by 2013 New Zealand deprivation index quintiles were obtained, in addition to the cause of DKA. RESULTS: There were 69 DKA admissions from 57 people; 35% were Pasifika and 23% Maori. Fifty-six percent were from quintile 5, the quintile with the lowest socioeconomic status. The most common cause of DKA was non-adherence to insulin (59%), followed by infection (16%) and new diagnosis of type 1 diabetes (14%). There was greater proportion of Pasifika and Maori population in those with non-adherence as the cause. CONCLUSION: Non-adherence is a major cause of DKA admissions at Middlemore Hospital. When compared to the regional census data, there is over-representation of Pasifika and Maori population and those of lower socioeconomic status in those admitted with DKA. Similar pattern was seen in those with non-adherence as the cause of DKA and those with recurrent DKAs.

Observations on the Natural History of Camurati-Engelmann Disease.

Camurati-Engelmann disease (OMIM 31300) is a rare cranio-tubular bone dysplasia characterized by osteosclerosis of the long bones and skull caused by dominantly-inherited mutations in the transforming growth factor beta 1 (TGFB1) gene. A wide variation in phenotype has been recognized, even within families carrying the same mutation. In addition, aspects of the natural history of the disorder, in particular whether it is always progressive or can remit spontaneously, remain uncertain. In a large kindred carrying a TGFB1 gene mutation (c.653G > A; p.R218H) we have attempted to clarify the extent of phenotypic variability and the natural history of the disease through detailed individual histories of symptoms, and skeletal imaging by both radiography and scintigraphy. Only one subject had the classical childhood onset with bone pain in the legs and gait disturbance. Eight subjects reported the onset of leg pain in their teenage years that, by their early 20s, had either resolved or persisted at a low level. Two of these eight later developed cranial nerve palsies. There was a wide variation in the radiographic appearance in adults, but disease extent and activity in long bones, as assessed by scintigraphy, was inversely correlated with age (p < 0.025). In younger subjects the radiographic and scintigraphic appearances were concordant, but in older subjects the scintigram could be quiescent despite florid radiographic changes. Sequential scintigrams in two subjects showed reduced activity in the later scan. One subject had suffered meningoencephalitis in early childhood that resulted in paresis of one arm. The affected arm showed markedly less disease involvement, implicating mechanical or growth factors in its etiology. Our data suggest that the natural history of Camurati-Engelmann disease can be benign, and that disease activity commonly attenuates in adulthood. Severe cases of childhood onset and/or with cranial nerve involvement, may occur only in a minority of mutation carriers. © 2019 American Society for Bone and Mineral Research.