Persistent hypercalciuria and elevated 25-hydroxyvitamin D3 in children with infantile hypercalcaemia

The aim of the study was to characterize abnormalities of calcium-phosphate and vitamin D₃ metabolism in children with a past history of “mild” Lightwood-type idiopathic infantile hypercalcaemia. Seventeen seemingly healthy children aged 2 – 12 years, with long-term idiopathic hypercalcaemic syndrome since infancy were studied. Two reference groups were also included (vitamin D₃ intoxication/healthy and Williams groups). Despite a long-term milk-restricted diet and a restricted vitamin D₃ intake, urinary calcium excretion in the study group was 0.117±0.07 mmol/kg per 24 h. Compared with the reference groups (0.047±0.029 and 0.067±0.06 mmol/kg per 24 h, P<0.05), there was significant hypercalciuria in the children with idiopathic hypercalcaemia since infancy. Serum concentrations of 25-hydroxyvitamin D₃ in the study group were also elevated compared with the reference groups (57.4±15.5 vs. 34.6±9.3 and 22.7±10.5 ng/ml). 1,25-Dihydroxyvitamin D₃ levels were at the upper limit of normal (45.9±13.1 vs. 35.0±8.1 and 30.0±13.7 pg/ml). Non-progressive, clinically silent nephrocalcinosis was visible on ultrasound examinations. The disturbances of vitamin D₃ and calcium-phosphate metabolism persistent in the normocalcaemic phase of idiopathic infantile hypercalcaemia may be a primary metabolic defect of the condition. The mechanisms leading to elevation of metabolites of 1,25-dihydroxy- and 25-hydroxyvitamin D₃ and the relationship between this and persistent hypercalciuria and nephrocalcinosis need pathophysiological explanation. Received September 22, 1995; received in revised form May 3, 1996; accepted May 7, 1996     

Use of perfluorocarbon solutions in the surgical treatment of posterior luxation of the lens]

Posterior lens dislocations represent 20 to 40% of all lens dislocations. It can occur after trauma. Visual prognosis can be good, but most often there are complications such as glaucoma, retinal detachment and uveitis. A few years ago, surgery was difficult, and surgery was delayed until the development of specific complications. A new technique, using perfluorocarbon liquids (PFCL), should be recommended because it is simple, and constantly effective. After vitrectomy, perfluorodecalin is injected into the vitreous cavity. As PFCL have a density twice the density of water, it allows the lens to float on the surface of the PFCL, behind the iris. Lens extraction can be associated with trabeculectomy, and anterior chamber lens implantation. We present a prospective study of 6 cases operated according to this technique. Postoperative results were good: visual acuity was preserved and ocular tension was controlled in cases of preoperative hypertonia. We believe this technique can be recommended after posterior lens dislocation, to prevent complications and to preserve visual acuity.     

Angiolymphoid Hyperplasia With Eosinophilia: A Classic Clinical Presentation With Histologic Features of Angiosarcoma

BACKGROUND: Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare disease manifested by the proliferation of morphologically distinct endothelial cells. OBJECTIVE: To illustrate by a case report the clinical and varied histopathologic findings of ALHE. METHODS: A 29-year-old woman presented with a clinical picture of ALHE but had several histologic features of angiosarcoma. RESULTS: Management of this patient included repeat biopsies of the lesions, excision of the involved areas, careful histologic examination of the entire specimen, and appropriate follow-up. CONCLUSION: ALHE may present with various histologic features. Knowledge of the spectrum of benign and malignant vascular neoplasms helps manage these challenging cases.     

Beyond three: MCH nurses' assessment leads to early interventions.

This study reports the results of a one year trial of a comprehensive developmental assessment, including vision and hearing screening, of three to four year olds in one municipality. Initial results indicated that over 50% of the 790 children assessed had no identifiable problems, this increased a further 15% after retesting. One hundred and eighty-seven children were referred to a variety of agencies, mainly for vision and audiological reassessment. One hundred and thirty-five referrals for further investigation were for children whose problems were confirmed and appropriately managed.     

Gd-DOTA-enhanced MR imaging and color Doppler US of renal allograft necrosis.

In 21 recipients of renal transplants suspected of allograft necrosis, the authors correlated the results of imaging to pathologic and histologic data in order to describe the magnetic resonance (MR) imaging and color Doppler ultrasonographic (US) characteristics of infarction. All patients underwent MR imaging performed with and without gadolinium tetraazacyclododecanetetraacetic acid (DOTA) and color Doppler US. Nonenhanced T1-weighted images showed no obvious changes, whereas nonenhanced T2-weighted images demonstrated a slight increase in signal intensity in areas of ischemic necrosis and low or heterogeneous signal intensity in areas of hemorrhagic necrosis. Gd-DOTA-enhanced MR images showed no contrast material uptake in infarcted areas. Color Doppler US characteristics of infarction included absence of Doppler signal and alteration of the cortical echogenic structure, particularly in cases of ischemic necrosis. Color Doppler US allows measurement of vascular resistance and assessment of intrarenal vasculature and the renal pedicle. Gd-DOTA-enhanced MR imaging is useful in confirming the diagnosis of infarction and provides an accurate evaluation of the extent of the infarct.     

Pharmacokinetics of oral cyclosporine (Neoral) in heart transplant recipients during the immediate period after surgery

Neoral cyclosporine has better absorption characteristics than the original Sandimmun formulation. This has allowed Neoral to be administered orally in circumstances where Sandimmun had been ineffective, including the postoperative phase of liver transplantation. Sampling strategies, such as the measurement of drug concentration 2 h after oral administration, have been used in a variety of settings to estimate systemic exposure to Neoral (measured as the area under the blood concentration curve (AUC) of the drug) in blood. We conducted a pilot study to determine whether Neoral could be administered orally immediately after heart transplantation and to determine which pharmacokinetic parameters reflect systemic drug exposure in this setting. Eight male patients (mean age 50 years) undergoing a first heart transplant were studied. Neoral was administered orally before surgery and at 12-h intervals via a nasogastric tube after surgery. Twelve-hour pharmacokinetic profiles were obtained on postoperative days 1, 3 and 5. Cyclosporine concentrations were measured with the Dade Behring Emit assay, which is specific for the parent drug. Drug concentrations were dose-normalised and drug exposure was measured by the AUC. Drug exposure following administration (AUC(0-12)) was low on day 1 but increased by 99% between postoperative day 1 and day 5 ( P<0.05), indicating more complete absorption of cyclosporine; exposure in the first 4 h post-dose (AUC(0-4)) increased by 126% ( P<0.01), reflecting more rapid cyclosporine absorption, and the maximum blood concentration observed increased by 137% ( P<0.05) during the same period. The correlation between the cyclosporine trough concentration and AUC(0-12) was low on all days. Due to the changing pattern of cyclosporine absorption, concentration measurements at a single time point could not accurately predict 12-h exposure to the drug on all study days. However, the drug concentration at 2 h post-dose had a high correlation with drug exposure during the first 4 h (correlation of C(2) to AUC(0-4): r(2)>0.93 on all days). Absorption of Neoral was low immediately after heart transplantation but improved substantially during the first 5 days after surgery. No single timed measurement of drug concentration reflected cyclosporine exposure; however, the 2-h concentration did provide an accurate measure of the early phase of drug absorption (AUC(0-4)). Oral administration of Neoral may result in inadequate immunosuppression immediately after heart transplantation unless it is supplemented either by intravenous cyclosporine or by the use of an induction agent.     

Uptake of influenza vaccine among Aboriginal and Torres Strait Island adults in north Queensland, 2002.

Since 1999, the Commonwealth has provided free annual influenza vaccine for all at-risk Aboriginal and Torres Strait Islander adults. The uptake of the vaccine in this population in north Queensland in 2002 was determined using the state-wide computerised immunisation register. Although 59.4 per cent of Aboriginal and Torres Strait Islander adults aged 50 years or over were vaccinated, the uptake in this age group exceeded 80 per cent in only the Cape York and Torres Strait and North Peninsula Area Health Service Districts (HSDs). Assuming that a third of Aboriginal and Torres Strait Islander adults 15-49 years of age had a medical risk factor, it was estimated that 85 per cent of those at-risk were vaccinated. There was considerable variation between HSDs, ranging from 159 per cent uptake in the Torres Strait and North Peninsula Area Health Service District to 48.7 per cent in the Cairns HSD.     

Motor flexibility problems as a marker for genetic susceptibility to attention-deficit/hyperactivity disorder.

BACKGROUND: Since many children with attention-deficit/hyperactivity disorder (ADHD) have fine visuomotor problems that are already evident at a young age, motor dysfunctioning is investigated in family-genetic perspective. We hypothesized that if fine motor problems may be a marker for genetic susceptibility to ADHD, nonaffected siblings of ADHD probands would experience motor problems similar to those of their ADHD siblings. METHODS: Twenty-five carefully phenotyped ADHD probands with a family history of ADHD, their nonaffected siblings (n = 25), and 48 normal control subjects (aged 6 to 17) completed a motor fluency task and a motor flexibility task. The motor fluency task involved completion of a familiar, automatized trajectory, whereas the motor flexibility task required continuous adjustment of movement to complete an unpredictable random trajectory. RESULTS: On the motor fluency task, the performance of the nonaffected children was significantly better than that of the ADHD probands; strikingly, on the motor flexibility task, they performed as well as their ADHD siblings. CONCLUSIONS: Nonaffected siblings experience complex motor problems similar to their ADHD siblings but only in nonautomatized movements that require controlled processing. The results suggest that higher-order controlled motor deficits in ADHD may be associated with genetic susceptibility for ADHD.