Incontinentia Pigmenti with Persistent Hypercalcemia: Case Report

Incontinentia pigmenti (IP) is an X‐linked dominant disorder that in most cases is considered lethal in males. IP affects the skin, hair, teeth, nails, eyes, and central nervous system. We report a case of persistent hypercalcemia (10.6–11.3 mg/dL) in a 19‐month‐old Caucasian boy with clinical IP. The proposed mechanism for his hypercalcemia is discussed.     

Incontinentia Pigmenti Coxsackium

Late recurrences of first‐stage inflammatory vesiculobullous lesions of incontinentia pigmenti (IP) are uncommon but have been reported to occur in the setting of infections, fevers, and vaccinations. This phenomenon has not been described in the setting of atypical hand, foot, and mouth disease (HFMD). In addition, atypical HFMD often has a predilection for areas of previous inflammation or trauma, such as areas of atopic dermatitis (eczema coxsackium). We present a case of incontinentia pigmenti coxsackium in which a coxsackievirus infection triggered reactivation of IP and cutaneous viral lesions were concentrated in areas of IP.     

Clinical evaluation of brushing time and plaque removal potential of two manual toothbrushes

Abstract: Aim: To compare plaque removal efficacy of Oral‐B CrossAction (CA) used for 1 min with an American Dental Association (ADA) manual toothbrush used for 2 or 5 min in an examiner‐blind, three‐treatment, six‐period crossover study. Materials and methods: After refraining from all oral hygiene procedures for 23–25 h, subjects were randomly assigned to one of nine possible six‐period (visit) treatment sequences. Plaque was assessed at baseline (Rustogi Modified Navy Plaque Index). Post‐brushing scores were recorded after brushing with a marketed dentifrice and the assigned toothbrush for the specified duration. The same procedure was followed at each of six subsequent visits. Clinical measurements were carried out by the same examiner. Results: Forty subjects completed the study. All three treatments effectively removed plaque from the whole mouth, along the gingival margin and from approximal surfaces. Whole mouth and gingival margin plaque removal scores with CA for 1 min did not differ significantly from scores with the ADA toothbrush used for 2 min. The ADA brush used for 5 min showed significantly greater whole mouth (P < 0.001) and gingival margin (P < 0.001) plaque reduction than the two other treatments. Approximal plaque removal scores did not differ between the three treatments. Conclusions: Efficient plaque removal can be achieved after 1 min of brushing with CA. The amount of plaque removed did not differ significantly from that achieved with the ADA brush after 2 min of brushing. Greater whole mouth and gingival margin plaque removal scores were seen with the ADA brush after 5 min.     

Respiratory insufficiency in neuronopathic and neuropathic disorders

Twenty-nine patients with a neuronopathic or neuropathic disorder were referred for assessment of respiratory insufficiency between 1978 and 1994. Diagnoses included spinal muscular atrophy (6), chronic idiopathic demyelinating neuropathy (4), Vialetto-van Laere syndrome (3), hereditary motor and sensory neuropathy (3) and a miscellaneous group (5). We also describe seven patients with Guillain-Barre syndrome (GBS) who required long-term ventilatory support for over 6 months to 7 years after the initial illness. Respiratory insufficiency occurred as a consequence of respiratory muscle weakness, impaired bulbar function and restrictive lung defects. In some groups presentation was with progressive nocturnal hypoventilation culminating in acute respiratory failure. Five patients with GBS or chronic idiopathic demyelinating neuropathy were weaned from ventilatory support up to 18 months after the initial illness. The remaining 24 patients required continuous or nocturnal ventilatory support using intermittent positive-pressure ventilation (13), negative pressure ventilation (4), nasal-mask-delivered intermittent positive-pressure ventilation (4), nasal-mask-delivered continuous positive-pressure ventilation (3), mouthpiece-assisted ventilation by day (2) and rocking bed (1). None have been weaned from support after a period of ventilation ranging from one month to 10 years. Eight patients have subsequently died.      

Dose effects associated with rivastigmine transdermal patch in patients with mild-to-moderate Alzheimer's disease

Aim: The cholinesterase inhibitor rivastigmine is available in both oral and transdermal forms. The efficacy of oral rivastigmine appears to be dose‐dependent. The current analysis investigates the effect of dose on the efficacy of the rivastigmine transdermal patch. Methods: This was a retrospective analysis of a large, international, 24‐week, randomised, placebo‐ and active‐controlled trial (IDEAL, CENA713D2320) of rivastigmine in patients with mild‐to‐moderate Alzheimer’s disease (AD). Patients received the 9.5 mg/24 h rivastigmine patch, the 17.4 mg/24 h rivastigmine patch, 12 mg/day rivastigmine capsules or placebo. Changes from baseline at week 24 on the AD Assessment Scale–cognitive subscale (ADAS‐cog), AD Cooperative Study–Clinical Global Impression of Change (ADCS‐CGIC) and the AD Cooperative Study–Activities of Daily Living (ADCS‐ADL) scale were calculated based on the patient’s mode and last prescribed patch dose. The analysis included the 4.6 mg/24 h and 13.3 mg/24 h patch doses, for which efficacy data have not previously been reported. Results: Significant differences (p < 0.05 vs. placebo) were seen on the ADAS‐cog and ADCS‐ADL for all mode rivastigmine patch doses (except 4.6 mg/24 h) and all last prescribed rivastigmine patch doses (except 4.6 mg/24 h and 13.3 mg/24 h). Patients with a last prescribed/mode patch dose of 9.5 mg/24 h and 13.3 mg/24 h showed significant improvements (p < 0.05 vs. placebo) on the ADCS‐CGIC. Conclusion: Rivastigmine patch doses higher than 9.5 mg/24 h may offer additional benefits. The 13.3 mg/24 h patch is worthy of further investigation.     

Pediatric photosensitivity

Photosensitivity is a rarely encountered problem in the pediatric population. It may be caused by a diverse group of primary, idiopathic photosensitivity conditions, or may reflect photoexacerbation of an existing dermatosis. In addition, there are several genodermatoses, metabolic disorders, and connective tissue disorders that can present with photosensitivity, usually in addition to other extracutaneous clinical and laboratory findings. It is important that both dermatologic and pediatric practitioners be able to recognize the various causes of photosensitivity, as well as be familiar with the associated stigmata and necessary workup, if needed, of each particular disorder. This review offers an approach to the pediatric patient who presents with photosensitivity, with emphasis on arriving at the proper diagnosis, necessary evaluations, and management strategies.     

Biological competition: Decision rules, pattern formation, and oscillations

Competition solves a universal problem about pattern processing by cellular systems. Competition allows cells to automatically retune their sensitivity to avoid noise and saturation effects. All competitive systems induce decision schemes that permit them to be classified. Systems are identified that achieve global pattern formation, or decision-making, no matter how their parameters are chosen. Oscillations can occur due to contradictions in a system's decision scheme. The pattern formation and oscillation results are extreme examples of a complementarity principle that seems to hold for competitive systems. Nonlinear competitive systems can sometimes appear, to a macroscopic observer, to have linear and cooperative properties, although the two types of systems are not equivalent. This observation is relevant to theories about the evolutionary transition from competitive to cooperative behavior.