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SLOesterol Powder

SLOesterol containerA tasteless, microencapsulated, powdered Medical Food for the dietary management of Smith-Lemli-Opitz Syndrome (SLOS). For individuals with sterol disorders who cannot obtain the recommended intake of cholesterol through regular diet. Not for the general population of consumers.

 

SLOesterol is a Medical Food specifically formulated using a proprietary microencapsulation process. SLOesterol represents a practical solution to the dosing of synthetic cholesterol for the SLOS patient.

 

SLOesterol contains a proprietary lipid micro-encapsulation process which offers the following advantages:

  • Protects shelf life and ensures product quality and consistency
  • Tasteless and odorless powder
  • Selective release of nutrients
  • Deliver functional ingredient
  • Provide ingredient stability
  • Improve delivery and target delivery mode
  • Prevent ingredient interaction
  • Act as or facilitate delivery
  • Simplifies transportation and travel restrictions

What is Smith-Lemli-Opitz Syndrome (SLOS)?

 

Smith-Lemli-Opitz Syndrome (SLOS) is a genetic disorder caused by a mutation that impairs or completely stops the last step of cholesterol production, leading to varying degrees of deficiency of cholesterol. The disease is homozygous recessive, meaning that a child needs to receive an abnormal gene from each parent. It is estimated that carriers of the gene may be as common as 1 in every 30 people with disease incidences on the order of 1 in 13,000 to 1 in 40,000. Some children with SLOS have no detectable cholesterol in their blood serum. Children or adults with SLOS have varying degrees of impairment that depend on the particular mutation in the gene that causes cholesterol deficiency of different degrees.

 

SLOS is a Metabolic Disorder caused by a mutation in the DHCR7 (7-dehydrocholesterol reductase) gene on chromosome 11

Chromosome 11

  • Incidence 1: 13,000 to 40,000 Newborns
  • Autosomal Recessive
  • Abnormal Cholesterol Levels / 7-DHC ↑
  • Varies in severity
  • Multiple symptoms
  • Limited treatment options
  • Treated by Metabolic / Genetic / Psychiatric Specialists

Reference Robert D Steiner, MD, Professor, Departments of Pediatrics and Molecular and Medical Genetics, Vice Chair for Research, Department of Pediatrics, Oregon Health & Science University; Director and Consulting Staff, Metabolic Bone Disease Clinic, Shriner's Hospital and Doernbecher Children's Hospital; Co-Director: Pediatric and Child Health Research, Oregon Clinical and Translational Research Institute (CTSA). http://emedicine.medscape.com/article/949125-overview 

 

How is SLOS diagnosed?

 

SLOS is confirmed by measurement of cholesterol and 7-dehydrocholesterol (7-DHC) by gas-chromatography mass spectrometry of blood serum. The gene defect leads to low total cholesterol and elevated amounts of the cholesterol precursors 7-DHC and 8-DHC.

 

Why does a person with SLOS need cholesterol?

 

Cholesterol is a critical building block needed for all tissues and cells as a part of the cell membrane. Cholesterol is especially high in the peripheral nerves and in the spinal cord and brain. Myelin, the protective and insulating material that surrounds the nerve cells, is composed of cholesterol. Dysfunction in the myelin of nerve fibers causes the interruption of smooth delivery of neurological information.

 

Cholesterol is Essential

 

Clinical Features of SLOS

 

General Cardiac
 Growth retardation Atrial and ventricular
 Failure to thriveseptal defects
 Intellectual disability Patent ductus arteriosus
 Developmental delay AV canal
  Hypertension
Craniofacial  
 Microcephaly Pulmonary
 Metopic prominence and Abnormal segmentation
bitemporal narrowing Ptosis Gastrointestinal
 Cataracts Poor feeding/suckling
 Broad nasal bridge Gastroesophageal reflux
 Anteverted nares Constipation
 Micrognathia Pyloric stenosis
 Broad aveolar ridges Malrotation
 Arched palate Colonic agangliosis
 Cleft palate/Bifid uvula Liver disease
  
Extremities Urogenital
 Rhizomelia Renal malformations
 Postaxial polydactyly Hypospadius
 Single palmar creases Cryptorchidism
 Short, proximally placed thumbs Ambiguous genitalia
 Retention of fetal pads Central Nervous System
 Syndactyly of the second and third toes  Holoprosencephaly Agenesis or partial agenesis of the corpus callosum
Skin  Hypotonia
 Photosensitivity 
 Cutis marmorata Behavior
 Dry skin/Eczyma Autism/Autistic features Hyperactivity Self-injurious behavior

Reference: Forbes D Porter, Section on Molecular Dysmorphology, Program on Developmental Endocrinology and Genetics, National Institute of Child Health andHuman Development, NIH, DHHS, Bethesda, MD, USA European Journal of Human Genetics (2008) 16, 535 – 541; doi:10.1038/ejhg.2008.10; published online 20 February 2008

 

Product Form

  • Powder (250 g of powder / 150 g of Cholesterol)

Indications

  • Smith Lemli Opitz syndrome (SLOS)
  • Diagnosed Sterol Disorders

 

Nutrition Information
Per 100 g
Energy, kcal  
360
Cholesterol, g  
60
Fat, g  
40
Carbohydrate, g  
0
Protein, g  
0
Vitamins  
Vitamin C, mg  
200
Vitamin E, mg  
80
   

*Conversion: 1 level scoop = 1 g cholesterol

 

Ingredients: Cholesterol, Soy Oil, Vitamin C, Vitamin E

 

Warning: Only for individuals with medical conditions requiring additional Cholesterol due to a defect in cholesterol synthesis, such as SLOS. Not to be used by individuals less than one (1) year of age. SLOesterol does not suspend well in liquids and may cause feeding tubes to block. Consult with a dietitian before attempting to tube feed SLOesterol.

 

Storage: Unopened bottles should be kept in a cool, dry, dark environment. Reseal opened bottles, store in a refrigerator and use within 3 months

 

 

SLOesterol - 888-8-765223

Reimbursement code (NDC)*: 57771-0002-01
HCPCS code: B4155

Contact Solace Nutrition
888-8-SOLACE
Fax: 401-633-6066
www.solacenutrition.com

 

 

*National Drug Codes (NDC) Format Codes: Products sold by Solace Nutrition are classified as Medical Foods by the US Food and Drug Administration (FDA). NDC format codes are product codes adjusted according to standard industry practice to meet the format requirements of pharmacy and health insurance computer systems.

 

 


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