ABSTRACT
Leiner's disease (LD) is a rare and serious syndrome of infantile erythroderma of severe and progressive generalized seborrheic-like dermatitis, recalcitrant diarrhea, malabsorption and wasting, and recurrent local and systemic infections. The purpose of this study is to provide an updated review on management with a summarized review of available peer-reviewed articles on LD. The mechanisms underlying this disease process remain unclear. The diagnosis includes demonstration of deficient opsonic activity along with the clinical tetrad of erythroderma, persistent gastrointestinal disturbance, superimposed bacterial or candidal infection, and marked wasting. An important correlation between LD and defective yeast and Staphylococcus aureus opsonization has been established. For the familial form of LD, an association of either complement three deficiency or complement five dysfunction has been made. LD should be distinguished from other types of infantile erythroderma, including Omenn syndrome. Treatment includes fluid and nutrition replacement, antibiotics to control infection, and fresh-frozen plasma therapy. The prognosis is unclear; it depends on treatment. LD is a life-threatening condition that requires prompt identification and hospitalization. Affected infants who receive vigorous treatment not only have the prospect of surviving, but also generally lead a normal life after infancy.
Subject(s)
Complement C5/deficiency , Dermatitis, Exfoliative , Hereditary Complement Deficiency Diseases , Anti-Bacterial Agents/therapeutic use , Dermatitis, Exfoliative/diagnosis , Dermatitis, Exfoliative/etiology , Dermatitis, Exfoliative/therapy , Hereditary Complement Deficiency Diseases/diagnosis , Hereditary Complement Deficiency Diseases/etiology , Hereditary Complement Deficiency Diseases/therapy , Humans , InfantABSTRACT
EXPAREL® has been used successfully to prolong postoperative pain control when applied as a wound infiltrate. EXPAREL® has not yet been approved for use in regional anesthesia to prolong postoperative pain control. We conducted a clinical case series of 4 patients using EXPAREL® for sciatic blocks via the popliteal fossa approach. Our results suggested that there is a large degree of variability in response to the medication. These inconsistent results and the possibility of bimodal kinetics creating analgesic gaps as seen in two of our patients indicate that more studies with larger sample size are needed to better characterize these phenomena and determine if more consistent results can be obtained in a future clinical trial.
