Poly-l-lactic acid for HIV-1 facial lipoatrophy: 48-week follow-up

Objectives

Poly‐l ‐lactic acid (PLA) injections modestly increase objectively assessed facial thickness but not facial soft tissue volume (FSTV) over 24 weeks. The durability of this response has not been well defined objectively.

Methods

HIV‐infected lipoatrophic adults were randomized to four open‐label PLA treatments administered every 2 weeks from week 0 (immediate group, n=50) or from week 24 (deferred group, n=50). Endpoints included FSTV assessed by computed tomography, facial lipoatrophy severity, quality of life (QoL) and safety. Analyses were by intention to treat.

Results

Between weeks 24 and 48, soft tissue thickness increased modestly in injection planes, at the maxillary [mean 0.9 mm; 95% confidence interval (CI) 0.3–1.5 mm; P=0.007] and base of nasal septum levels (mean 0.4 mm; 95% CI 0.1–0.8; P=0.021), but not in untreated areas (P=0.79 and P=0.24). PLA durability assessed at week 48 in immediate group participants showed a mean change in FSTV of 14 cm³ (95% CI?1 to 29 cm³; P=0.060) and increased tissue depth at the maxillary (P<0.0001), base of nasal septum (P<0.0001) and mandibular (P=0.0035) levels. At week 48, clinicians and patients subjectively assessed facial lipoatrophy severity as reduced in immediate participants (83 and 91%, respectively), and the Mental Health scale score of the Short Form‐36 Health Survey improved significantly in immediate participants relative to deferred participants (P=0.027). Subcutaneous injection‐site nodule incidence at 48 weeks was 10%.

Conclusions

PLA treatment benefits were durable, with objectively assessed modest increases in facial volume and tissue thickness sustained over 48 weeks in injection planes but not in other facial areas. Improvements in some QoL domains were maintained.

     

Diagnostic accuracy of trigeminal reflex testing in trigeminal neuralgia

The authors prospectively studied 120 consecutive patients with trigeminal neuralgia (TN) to identify the clinical and laboratory features that most accurately distinguished symptomatic from classic TN. After a standardized evaluation, they identified 24 patients with symptomatic TN. Age, sensory examination, and affected division were not useful in the differential diagnosis. In contrast, electrophysiologic testing of trigeminal reflexes accurately distinguished symptomatic from classic TN (sensitivity 96%, specificity 93%).     

"Epileptic encephalopathy" of infancy and childhood: electro-clinical pictures and recent understandings

There is growing interest in the diagnosis of cognitive impairment among children with epilepsy. It is well known that status of seizures control has to be carefully investigated because it can be sufficient "per se" to cause progressive mental deterioration conditions. Subclinical electroencephalographic discharges may have subtle effects on cognition, learning and sleep patterns, even in the absence of clinical or sub-clinical seizures. In this respect, electroencephalographic monitoring (long-term and nocturnal recording) and in particular an all night video-polysomnography (V-NPSG) record can be crucial to detect the presence of unrecognized seizures and/or an inter-ictal nocturnal EEG discharge increasing. Epileptic encephalopathies (EE) are a group of conditions in which the higher cognitive functions are deteriorate as a consequence of epileptic activity, which, in fact, consists of frequent seizures and/or florid and prolonged interictal paroxysmal discharges, focal or generalized. AEDs represent the first line in opposing the burden of both, the poor seizures control and the poor interictal discharges control, in the cognitive deterioration of EE affected children. Thus, to improve the long-term cognitive/behavioural prognosis in these refractory epileptic children, it should be taken into account both a good seizures control and a strict sleep control, choosing carefully antiepileptic drugs which are able to control not only seizures clinically recognizable but even the EEG discharges onset and its increasing and spreading during sleep. Here, we review the efficacy and safety of the newer AEDs that, to date, are used in the treatment of EE in infancy and childhood.     

Clinical and pharmacological aspects of inflammatory demyelinating diseases in childhood: an update

Inflammatory demyelinating diseases comprise a spectrum of disorders affecting the myelin of the central and peripheral nervous system. These diseases can usually be differentiated on the basis of clinical, radiological, laboratory and pathological findings. Recent studies have contributed to current awareness that inflammatory demyelinating diseases are not restricted to the adult age group, but are more common in pediatric age than previously believed. Some of pediatric inflammatory demyelinating diseases carry an unfavorable long-term prognosis but appropriate treatments can improve the outcome. The possibility of physical and cognitive disability resulting from these diseases, highlights the urgent need for therapeutic strategies for neurorehabilitation, neuroregeneration, and neurorepair. This review discusses characteristics of primary demyelinating diseases more frequently observed in childhood, focusing on epidemiology, clinical aspects and treatments.     

Exposure of distal internal carotid artery through mandibular vertical ramus osteotomy

Exposure of the distal internal carotid artery at the level of the second cervical vertebra required manoeuvers such as division of digastric muscle or mandibular subluxation. These increase the exposure but may not provide adequate access and are associated with significant cranial nerves or temporal mandibular joint complications. Vertical Ramus Osteotomy (VRO) provided access of the internal carotid artery (ICA) up to the base of the skull, with low incidence of cranial nerve injury temporo-mandibular joint (TMJ) pain and no preincision preparation. We report two cases in which vertical division of the mandibular ramus provided access of the ICA up to the base of the skull. Preoperative Duplex Scan examination and in the second case the arteriography revealed ICA preocclusive stenosis within 1.5 cm of the skull base. VRO was performed trouhgh a standard neck incision and miniature titanium plates were used to reapproximate the mandible after vascular procedure. There were no death, cranial nerve injury, mandibular nonunion, malocclusion or TMJ pain. We found that VRO is useful when carotid artery pathology extends beyond the usual field of exposure, avoiding nerve injury or TMJ lesion and requires no additional pre-incision preparation.     

Precipitants and aetiology of cyclic vomiting syndrome

Thirty-two patients aged 2-22 y with cyclic vomiting syndrome (CVS), and 64 age- and gender-matched controls were assessed to determine the nature, severity, precipitants and associated features of attacks and the incidence of potential aetiological factors. The mean age of onset was 3.5 y. Patients experienced a mean of nine attacks per year, of average duration 2.4 d, and two-thirds missed more than 10 d of school per year. Patients were more likely to have migraine and co-ordination difficulties, a past history of forceps delivery and gastroesophageal reflux than controls. Compared with controls, subjects had a higher incidence of psychological symptoms (38% compared with 19%) and migraine (37% compared with 9%). CVS is a chronic, disabling condition and is a migraine variant, with attacks usually precipitated by stress and intercurrent infections.     

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. These results indicate that BMD continues to increase 12 months after ceasing GH therapy in adolescents with GHD, but that unfavourable alterations in body composition occur.     

Oral treatment for constitutional delay of growth and puberty in boys: a randomised trial of an anabolic steroid or testosterone undecanoate

Thirty three boys (mean 14.6 years old, range 12.8-16.2 years) with constitutional delay of growth and puberty were randomised into two groups to determine which form of oral treatment would give the better anthropometric response. The two drugs were administered by mouth (one tablet/day) for a mean of 3.5 months (range 3-7 months). At randomisation, 17 boys received testosterone undecanoate (40 mg/day) and 16 oxandrolone (2.5 mg/day). At the start of treatment they were prepubertal or in early puberty, their height SD score was -1.97 in boys treated with testosterone and -2.21 in those treated with oxandrolone, and their growth rates were 4.3 and 4.2 cm/year respectively. Both sex steroid and anabolic steroid treatments induced a significant growth acceleration in all patients except four (three treated with testosterone and one with oxandrolone). When treated with the alternative sex steroid, all four non-responders had a significant anthropometric response. In all boys the induced growth acceleration was sustained when treatment was interrupted. There was no significant difference in the induced growth spurt and bone maturation between the two groups. Spontaneous progress into puberty was achieved in all boys with an increase in testicular volume from a mean of 4.6 to 8.5 ml. The rate of development in secondary sexual characteristics was also similar in the two groups. These data suggest that oral testosterone and oxandrolone are equally effective in the treatment of growth delay in boys with constitutional delay of growth and puberty.