Financial costs for families of children with Type 1 diabetes in lower-income countries.

AIMS: To assess the direct costs of necessary consumables for minimal care of a child with Type 1 diabetes in countries where the public health system does not regularly provide such care. METHODS: Supply costs were collected between January 2013 and February 2015 from questionnaires submitted by centres requesting International Diabetes Federation Life for a Child Program support. All 20 centres in 15 countries agreed to the use of their responses. Annual costs for minimal care were estimated for: 18 × 10 ml 100 IU/ml insulin, 1/3 cost of a blood glucose meter, two blood glucose test strips/day, two syringes/week, and four HbA1c tests/year. Costs were expressed in US dollars, and as % of gross national income (purchasing power parity) per capita. RESULTS: The ranges (median) for the minimum supply costs through the private system were: insulin 10 ml 100 IU/ml equivalent vial: $5.10-$25 ($8.00); blood glucose meter: $15-$121 ($33.33); test strip: $0.15-$1.20 ($0.50); syringe: $0.10-$0.56 ($0.20); and HbA1c : $4.90-$20 ($9.75). Annual costs ranged from $255 (Pakistan) to $1,185 (Burkina Faso), with a median of $553. Annual % gross national income costs were 12-370% (median 56%). For the lowest 20% income earners the annual cost ranged 20-1535% (median 153%). St Lucia and Mongolia were the only countries whose governments consistently provided insulin. No government provided meters and strips, which were the most expensive supplies (62% of total cost). CONCLUSIONS: In less-resourced countries, even minimal care is beyond many families' means. In addition, families face additional costs such as consultations, travel and indirect costs. Action to prevent diabetes-related death and morbidity is needed.

Insulin storage in hot climates without refrigeration: temperature reduction efficacy of clay pots and other techniques.

AIM: Insulin loses potency when stored at high temperatures. Various clay pots part-filled with water, and other evaporative cooling devices, are used in less-resourced countries when home refrigeration is unavailable. This study examined the cooling efficacy of such devices. METHODS: Thirteen devices used in Sudan, Ethiopia, Tanzania, Mali, India, Pakistan and Haiti (10 clay pots, a goat skin, a vegetable gourd and a bucket filled with wet sand), and two identical commercially manufactured cooling wallets were compared. Devices were maintained according to local instructions. Internal and ambient temperature and ambient humidity were measured by electronic loggers every 5 min in Khartoum (88 h), and, for the two Malian pots, in Bamako (84 h). Cooling efficacy was assessed by average absolute temperature difference (internal vs. ambient), and % maximal possible evaporative cooling (allowing for humidity). RESULTS: During the study period, mean ambient temperature and humidity were 31.0°C and 32.0% in Khartoum and 32.9°C and 39.8% in Bamako. All devices reduced the temperature (P < 0.001) with a mean (sd) reduction from 2.7 ± 0.5°C to 8.3 ± 1.0°C, depending on the device. When expressed as % maximal cooling, device efficacy ranged from 20.5% to 71.3%. On cluster analysis, the most efficacious devices were the goat skin, two clay pots (from Ethiopia and Sudan) and the suspended cooling wallet. CONCLUSIONS: Low-cost devices used in less-resourced countries reduce storage temperatures. With more efficacious devices, average temperatures at or close to standard room temperature (20-25°C) can be achieved, even in hot climates. All devices are more efficacious at lower humidity. Further studies are needed on insulin stability to determine when these devices are necessary.

Bone mineral density of total body, spine, and femoral neck in children and young adults: a cross-sectional and longitudinal study.

Bone mineral density (BMD) of total body (TBMD), lumbar spine (L2-4), and femoral neck was measured in 266 normal subjects (136 males) aged 4-27 years (mean 13 years) using dual-energy x-ray absorptiometry (DXA). BMD of all sites increased significantly with age until 17.5 years in males and 15.8 years in females, except for femoral neck BMD in females, which peaked at age 14.1 years. Males had higher peak TBMD, which was attributed to greater weight and lean tissue mass. In contrast, despite a later timing, peak L2-4 BMD in males was not different from that in females. Before peak BMD, weight was the best predictor of TBMD and L2-4 BMD in both sexes (r2 ranged from 0.77 to 0.88), whereas femoral neck BMD was predicted equally by height and weight. Longitudinal information collected from 53 (25 boys) of these children, aged 4-16.9 years, showed that the average annualized gain in TBMD was 0.047 g/cm2 for boys and 0.039 g/cm2 for girls. No significant difference in the association between age and BMD (slopes) was found between cross-sectional and longitudinal data for either sex. We conclude that the timing for peak BMD was consistent for total body, lumbar spine, and femoral neck for each sex. The earlier peak BMD in females is most likely related to earlier puberty. The cross-sectional normative data of this study are useful in serving as a standard for serial assessment in health and disease states.

Features of the metabolic syndrome after childhood craniopharyngioma.

Obesity and multiple pituitary hormone deficiency are common complications after surgery for childhood craniopharyngioma. We hypothesized that post craniopharyngioma surgery, children are at high risk for the metabolic syndrome, including insulin resistance due to excess weight gain and GH deficiency. This study characterized body composition (anthropometry and dual energy x-ray absorptiometry) and metabolic outcomes in 15 children (10 males and 5 females; age, 12.2 yr; range, 7.2-18.5 yr) after surgical removal of craniopharyngioma. In 9 subjects, outcomes were compared with those of healthy age-, sex-, body mass index-, and pubertal stage-matched controls. Insulin sensitivity was measured by 40-min iv glucose tolerance test. Seventy-three percent of subjects were overweight or obese. Sixty-six percent had normal growth velocity without GH treatment. Subjects had increased abdominal adiposity (P = 0.008) compared with controls. However, there was no significant difference in total body fat. Subjects had higher fasting triglycerides (P = 0.02) and lower high density lipoprotein cholesterol to total cholesterol ratio (P = 0.015). Insulin sensitivity was equally reduced for subjects and controls (P = 0.86). After craniopharyngioma removal, patients had more features of the metabolic syndrome compared with controls. This could be a result of hypothalamic damage causing obesity and GH deficiency. Further studies exploring predictors of the metabolic syndrome after craniopharyngioma surgery are required.

Body-composition assessment by dual-energy x-ray absorptiometry in subjects aged 4-26 y.

This cross-sectional study describes the body composition of 265 normal subjects (137 males and 128 females) aged 4-26 y determined by dual-energy x-ray absorptiometry (DXA). Lean tissue mass (LTM) and bone mineral content (BMC) increased with age in females until 13.4 and 15.7 y, respectively, and in males until 16.6 and 17.4 y, respectively. A strong relation between LTM and BMC was found for each sex (r = 0.98, P = 0.0001 for males; r = 0.98, P = 0.0001 for females). DXA percent body fat (%BFDXA) increased with age in females (r = 0.52, P < 0.001) but not in males and was higher in females than in males at all ages. Trunk to leg fat ratio (TLFR) was calculated as DXA trunk fat/leg fat. In post-pubertal age the TLFR was higher in males than in females (1.01 +/- 0.23 and 0.75 +/- 0.16, P = 0.001), but there was no sex difference in younger children. DXA weight underestimated scale weight by a mean of 0.83 kg. %BFDXA correlated with %BF by skinfold thickness measurement with good agreement for males but overestimated %BF by skinfold thickness for females. These normative data for body composition demonstrate significant sex differences in all body compartments after the pubertal years.

Changes in body composition and bone density after discontinuation of growth hormone therapy in adolescence: an interim report.

Growth hormone deficiency (GHD) in adults and children is associated with decreased lean tissue mass (LTM), increased fat mass and reduced bone mineral density (BMD). The changes in BMD and body composition, 6 and 12 months after ceasing GH treatment, were assessed using dual-energy X-ray absorptiometry in eight patients with GHD (age range, 13.8-17.5 years). Seven age-matched normal subjects who had completed growth were assessed at 0 and 12 months. Total body BMD was low at baseline (p < 0.05) in patients with GHD compared with the predicted values based on sex-specific regression equations, with height, weight and age taken into account. Total body, lumbar spine and femoral neck BMD increased in the patients and controls at 12 months. LTM decreased significantly by a mean of 1.37 kg in the patients with GHD at 12 months whereas there was a non-significant increase in LTM in the control group. The percentage of body fat increased in all patients with GHD at 6 and 12 months, from 27.2 +/- 11% (mean +/- SD) at baseline to 32 +/- 9.9% at 12 months (p = 0.009). There was no significant increase in mean percentage body fat in the control group. The ratio of android (trunk):gynoid (legs) fat was calculated using default settings of dual-energy X-ray absorptiometry. The mean android:gynoid fat ratio increased, though non-significantly, in patients with GHD at 12 months, with 6 of 7 showing an increase; no change was observed in the control group.(ABSTRACT TRUNCATED AT 250 WORDS)

Type 2 diabetes mellitus in Papua New Guinea--an historical perspective.

Type 2 diabetes mellitus is an increasing problem in Papua New Guinea (PNG). Five dominant themes emerge from the literature on diabetes in PNG: 1) The concept of the 'thrifty genotype' predisposing Papua New Guineans, like many other traditional populations, to type 2 diabetes, 2) Some coastal ethnic groups (particularly with Wanigelans and Tolais) appear to be more predisposed than others, 3) Prevalence of type 2 diabetes markedly rises with urbanization and adoption of a more western lifestyle, 4) Morbidity and mortality associated with the disease is high, and 5) Type 2 diabetes is overwhelmingly the most common type, with other types being rare. The literature is reviewed within the framework of these themes.

A survey of diabetes services in hospitals in Papua New Guinea.

AIM: To determine the number and distribution of diabetic persons in Papua New Guinea (PNG), and to document available services. METHODS: Survey of the 19 hospitals thought to be the most significant centres for care of diabetes. RESULTS: 16 hospitals were successfully surveyed. Type 1 diabetes is very rare, with 18 known cases in the country (6 less than 15 years old). Known type 2 cases totalled around 4600 with almost all cases in coastal centres, particularly Port Moresby, East New Britain and Manus. There were very few cases in the highlands. Insulin is generally available, along with oral hypoglycaemic drugs. Testing for complications is very limited, and glycosylated haemoglobin (HbA1c) testing is available in only one centre. Only 3 trained diabetes educators are available in the country, with 1 dietitian and no specialist endocrinologists. CONCLUSIONS: The vast majority of persons with type 2 diabetes in PNG are undiagnosed and are not receiving treatment: based on the lower of two country prevalence estimates, less than 3% of the diabetic persons in the country are seen at health facilities. Services are limited, with only a handful of health professionals specially trained in diabetes. Expansion of services and awareness and prevention programs are urgently needed.

Type 1 diabetes mellitus in children in Papua New Guinea.

Anecdotal and published information suggest a low incidence and prevalence of type 1 diabetes in Papua New Guinea (PNG). Incidence and prevalence were followed prospectively from July 1996, using the PNG Paediatric Surveillance Unit (PSU). No children were receiving insulin in Papua New Guinea at the start of the period. Over the next 4.5 years, 8 cases were reported--an annual incidence of 0.08/100,000 children aged < 15 years and a prevalence of 0.28/100,000 aged < 15 years. These figures are as low as any reported elsewhere. Three cases were from the small expatriate population. All cases in Melanesian children posed significant management problems, with two children dying during the study period.

Tropical distal renal tubular acidosis: clinical and epidemiological studies in 78 patients.

BACKGROUND: Distal renal tubular acidosis (dRTA) caused by mutations of the SLC4A1 gene encoding the erythroid and kidney isoforms of anion exchanger 1 (AE1 or band 3) has a high prevalence in some tropical countries, particularly Thailand, Malaysia, the Philippines and Papua New Guinea (PNG). Here the disease is almost invariably recessive and can result from either homozygous or compound heterozygous SLC4A1 mutations. METHODS: We have collected and reviewed our own and published data on tropical dRTA to provide a comprehensive series of clinical and epidemiological studies in 78 patients. RESULTS: Eight responsible SLC4A1 mutations have been described so far, four of them affecting multiple unrelated families. With the exception of the mutation causing South-East Asian ovalocytosis (SAO), none of these mutations has been reported outside the tropics, where dRTA caused by SLC4A1 mutations is much rarer and almost always dominant, resulting from mutations that are quite different from those found in the tropics. SLC4A1 mutations, including those causing dRTA, may cause morphological red cell changes, often with excess haemolysis. In dRTA, these red cell changes are usually clinically recessive and not present in heterozygotes. The high tropical prevalence of dRTA caused by SLC4A1 mutations is currently unexplained. CONCLUSION: A hypothesis suggesting that changes in red cell metabolism caused by these mutations might protect against malaria is put forward to explain the phenomenon, and a possible mechanism for this effect is proposed.

Angiosarcoma of the heart.

The case of a woman with angiosarcoma of the right atrium is reported. The tumor was inoperable, and the patient was treated with chemotherapy. There was no response to treatment.

Ioxaglate in paediatric angiocardiography.

It is generally agreed that low osmolar contrast media are better tolerated than conventional media. This study examined both tolerance and image quality of ioxaglate in a group of paediatric patients undergoing angiocardiography for congenital heart disease. A consecutive series of 50 patients (mean age 5.47 years; range 4 days-14 years) were examined. The mean dose of ioxaglate administered per patient was 2.93 mL/kg. In general, ioxaglate was well tolerated. Three patients became febrile and another developed eosinophilia. Serum creatinine rose by a mean of 10 mumol/L. Significant renal dysfunction occurred in 12 patients with an increase in creatinine of 20-30 mumol/L. In no patient, however, were these effects a significant clinical problem. Diagnostic image quality was generally considered to be good with both cine-angiography and digital subtraction angiographic techniques.

Renal effects of growth hormone. II. Electrolyte homeostasis and body composition.

Growth hormone (GH), either directly or through insulin-like growth factor-1 (IGF-1), has a wide spectrum of physiological and renal effects. This review concentrates on the effects of GH (derived from either pituitary or recombinant technology) and IGF-1 in three main areas: (1) sodium and water homeostasis; (2) calcium and phosphate balance, bone density and interactions with mineral regulating hormones; (3) fat and lean body mass. Observations of physiological changes in states of GH deficiency and excess in humans and animal models are presented. The lack of long-term toxicological data indicates that GH treatment for short stature in non-GH deficient children, with or without renal disease, should proceed with caution.

Renal effects of growth hormone. I. Renal function and kidney growth.

Growth hormone (GH) affects renal function and kidney growth. Pituitary-derived or recombinant human GH (rhGH), acting via insulin-like growth factor-1 (IGF-1), increases glomerular filtration rate (GFR) and renal plasma flow (RPF) in GH-deficient as well as in normal adults. Furthermore, GFR and RPF are low in hypopituitarism and elevated in acromegaly. These effects of GH on GFR and RPF have not been demonstrated in moderate renal insufficiency. IGF-1 is implicated in compensatory renal hypertrophy. Markedly elevated levels of serum GH accelerate glomerular sclerosis in rodents, although the significance of these findings for GH treatment in humans is uncertain. rhGH therapy offers great promise to children with short stature from various aetiologies. Preliminary reports on the use of rhGH in children with renal disease and after renal transplantation have not shown any consistent change in kidney function, although follow-up times are short. The long-term impact of rhGH therapy on kidney function in short children needs further evaluation.

Effects of growth hormone treatment for short stature on calcium homeostasis, bone mineralisation, and body composition.

We investigated the effect of growth hormone (GH) treatment on mineral and vitamin D homeostasis, bone mineralisation, and body composition in short-statured children without GH deficiency (GHD). 11 children received GH (0.50 +/- 0.08 IU/kg/week) for 24 weeks. 1,25-Dihydroxyvitamin D3 levels (mean +/- SD in pmol/l) rose from a baseline of 73.7 +/- 39.2 to 114.0 +/- 32.7 at 8 weeks (p < 0.05) and 111.9 +/- 39.7 at 24 weeks (p < 0.01). Body composition evaluation using dual-energy X-ray absorptiometry revealed increased lean tissue mass and a reduction in fat tissue. As a percentage of total body mass, fat decreased from 19.0 +/- 11.8% at baseline to 17.3 +/- 11.5% at 8 weeks (p < 0.005) and 16.8 +/- 11.5% at 24 weeks (p < 0.05). L2-L4 bone mineral density was 0.637 +/- 0.155 g/cm2 at baseline and 0.666 +/- 0.160 g/cm2 at 24 weeks (NS). We conclude that recombinant human GH treatment of short children without GHD has significant effects on vitamin D homeostasis and body composition.