Variant forms of the disorder may become apparent only later in infancy or childhood, with typically less severe symptoms that may only appear during times of fasting, stress or illness, but still involve mental and physical problems if left untreated.Sub-divisions of MSUD:
Classic MSUD
Intermediate MSUD
Intermittent MSUD
Thiamine-responsive MSUDGenerally, majority of patients will be classified into one of these four categories but some patients affected by MSUD do not fit the criteria for the listed sub-divisions and will be deemed unclassified MSUD. Prevention
There are no methods for preventing the manifestation of the pathology of MSUD in infants with two defective copies of the BCKD gene. However, genetic counselors may consult with couples to screen for the disease via DNA testing. DNA testing is also available to identify the disease in an unborn child in the womb. Treatment
Monitoring
Keeping MSUD under control requires careful monitoring of blood chemistry, both at home and in a hospital setting. DNPH or specialised dipsticks may be used to test the patients urine for ketones (a sign of metabolic decompensation), when metabolic stress is likely or suspected. Fingerstick tests are performed regularly and sent to a laboratory to determine blood levels of leucine, isoleucine, and valine. Regular metabolic consultations, including blood-draws for full nutritional analysis, are recommended; especially during puberty and periods of rapid growth. MSUD management also involves a specially tailored metabolic formula, a modified diet, and lifestyle precautions such as avoiding fatigue and infections, as well as consuming regular, sufficient calories in proportion to physical stress and exertion.
Sertaconazole, sold under the brand name Ertaczo among others, is an antifungal medication of the Benzothiophene class. It is available as a cream to treat skin infections such as athletes foot. It is also available in a vaginal tablet form. The most popular of these is Gyno-Dermofix. Medical uses
In randomized, double-blind, multicentre trials of 3 to 6 weeks duration (n=127-383), a significantly greater number of patients with tinea of the glabrous skin and tinea pedis receiving a topical 2% sertaconazole cream once or twice daily achieved a successful mycological cure compared with recipients of a placebo cream. Side effects
Side effects were rarely reported with sertaconazole therapy, but may include contact dermatitis, burning on application site and skin dryness. Pharmacology
Pharmacodynamics
Sertaconazole has several known mechanisms of action. It is considered fungistatic, fungicidal, antibacterial, antiinflammatory, antitrichomonal, and antipruritic.Like other imidazole antifungals, sertaconazole blocks the synthesis of ergosterol by inhibiting the 14α-demethylase enzyme. Ergosterol is a critical component of the fungal cell membrane. Inhibition of ergosterol synthesis prevents fungal cells from multiplying and impairs hyphae growth. Chemically, sertaconazole contains a benzothiophene ring which makes it unique from other imidazole antifungals. A benzothiophene ring is a sulfur analogue of the indole ring found in the amino acid tryptophan. Tryptophan is found in the fungal membrane in addition to lipids such as ergosterol. The benzothiophene ring in sertaconazole mimics tryptophan and increases the drugs ability to form pores in the fungal cell membrane.