Gestational malnutrition, hyperemesis gravidarum, and Wernicke's encephalopathy: What is missing?

Hyperemesis gravidarum (HG), or the severe nausea and vomiting of pregnancy, is one of the most dreaded complications of gestation, affecting between 1.5% and 3.0% of pregnant women. From the late 1800s to the mid-1980s, the etiology was frequently cited to have psychological and/or-later-perhaps hormonal origins, which have numbered at least 10. Current research has unearthed a genetic basis for HG that implicates growth differentiation factor 15, insulin-like growth factor binding protein 7, and hormone receptors (namely, glial cell line-derived neurogenic factor family receptor alpha-like and the progesterone receptor). Whatever the origins of this disease, it has caused immeasurable physiological and psychological damage to women, their fetuses, and their families. The psychological trauma includes a high rate of suicidal ideation as well as posttraumatic stress disorder. Whereas the healthcare costs are substantial for the mother with HG, the lifetime costs to the neonate include that which accompanies reduced employment earnings related to cognitive compromise. Another devastating outcome of severe HG can be Wernicke's encephalopathy (WE), which has a high fetal and maternal mortality rate. Our study explored 18 current reports of HG and WE. We highlighted additional presenting features we believe also accompany, and sometimes replace, the classically taught triad components of WE: ataxia, confabulation, and nystagmus. We agree with the conclusion made by Sheehan and Ironside in 1939 that thiamin alone may not reverse WE, and we offer possible explanations. Lastly, we offer suggestions for remediation.

Breast milk is conditionally perfect.

Breast milk is the universal preferred nutrition for the newborn human infant. New mother have been encouraged to exclusively breastfeed by health care professionals and consumer-advocacy forums for years, citing "breast milk is the perfect food". The benefits are numerous and include psychological, convenience, economical, ecological and nutritionally superior. Human milk is a composite of nutritional choices of the mother, commencing in the pre-conceptual era. Events influencing the eventual nutritional profile of breast milk for the neonate start with pre-conceptual dietary habits through pregnancy and finally to postpartum. Food choices do affect the nutritional profile of human breast milk. It is not known who coined the phrase "breast milk is the perfect food" but it is widely prevalent in the literature. While breast milk is highly nutritive, containing important immunological and growth factors, scientific investigation reveals a few short-falls. Overall, human breast milk has been found to be low in certain nutrients in developed countries: vitamin D, iodine, iron, and vitamin K. Additional nutrient deficiencies have been documented in resource-poor countries: vitamin A, vitamin B 12, zinc, and vitamin B 1/thiamin. Given these findings, isn't it more accurate to describe breast milk as "conditionally perfect"? Correcting the impression that breast milk is an inherently, automatically comprehensive enriched product would encourage women who plan to breastfeed an opportunity to concentrate on dietary improvement to optimizes nutrient benefits ultimately to the neonate. The more immediate result would improve pre-conceptual nutritional status. Here, we explore the nutritional status of groups of young women; some of whom will become pregnant and eventually produce breast milk. We will review the available literature profiling vitamin, mineral, protein and caloric content of breast milk. We highlight pre-existing situations needing correction to optimize conception and fetal development. While alternative forms of infant nutrition carry standard product labels of nutrient adequacy, this information does not apply universally to all breast milk. Infant formulas are fortified with various amounts of vitamins, minerals, supplemental protein concentrates, nucleic factors, omega 3 fatty acids and any important new nutritional finding. Infant formulas are manufactured to be consistent in composition and are monitored closely for quality. Not true for human breast milk. Any nutrient deficiency existing in pregnancy will ultimately be carried forward via lactation. It is a biological impossibility for a lactating woman to transfer nutrients via breast milk she does not have!

Management of a Woman With Maple Syrup Urine Disease During Pregnancy, Delivery, and Lactation.

Maple syrup urine disease (MSUD) is an inherited disorder of metabolism of the branched-chain amino acids leucine, isoleucine, and valine. Complications of acute elevation in plasma leucine include ketoacidosis and risk of cerebral edema, which can be fatal. Individuals with MSUD are at risk of metabolic crisis throughout life, especially at times of physiological stress. We present a case of successful management of a woman with MSUD through pregnancy, delivery, postpartum, and lactation, including nutrition therapy using modified parenteral nutrition.

Hyperemesis gravidarum: a case of starvation and altered sensorium gestosis (ASG).

Of the problems that complicate child-bearing, hyperemesis gravidarum (HG), or severe nausea and vomiting of pregnancy (NVP), is likely one of the most painful with unrelenting retching and vomiting that can lead to measurable injuries such as Mallory-Weiss Syndrome and esophageal rupture, and/or subtle maternal cognitive impairments related to starvation and dehydration. Recognized hallmarks of HG include dehydration, ketonuria, weight loss over 5%, and electrolyte abnormalities not attributable to other causes. Historically providers regarded the hyperemetic as a difficult to treat patient with potentially underlying psychological problems. Sick patients who experience pain and suffering present challenges to care, not excepting NVP. Ill patients can be demanding and agitated. Agitation can be one of the early signs of delirium or altered mental status (AMS). AMS can include previously diagnosed psychiatric conditions as well as new onset of Wernicke's encephalopathy, deliria, insomnia, hallucinations and autoscopy, resulting from various etiologies including and not limited to medications, pain including pain from hunger, vomiting and retching, constipation, dehydration, altered electrolytes, hypoglycemia, malnutrition and sleep deprivation. AMS may have a subtle waxing and waning trajectory, making the condition difficult to diagnosis in early stages. What have not been well elucidated in AMS are subjective images and/or experiences. Whether all AMS experiences are similar is unknown. We believe there may be a transient alteration of cognitive status or "altered sensorium gestosis" (ASG), attributed to the direct insults of hyperemesis gravidarum which will be discussed herein. How prevalent ASG might be is unknown and needs further investigation.

Maternal vitamin K deficient embryopathy: association with hyperemesis gravidarum and Crohn disease.

Chondrodysplasia punctata (CDP) is an etiologically heterogeneous disorder characterized by the radiographic finding of stippled epiphyses (punctate calcifications). It is often accompanied by a characteristic facial appearance, known as the Binder phenotype, which is attributed to hypoplasia of the nasal cartilages; abnormal distal phalanges (brachytelephalangy) are a common component manifestation as well. We report eight patients with a Binder phenotype with or without CDP who all shared a known or suspected maternal deficiency of vitamin K. We suspect that this phenotype is probably under recognized, and we hope to increase awareness about the maternal risk factors, especially hyperemesis gravidarum, which lead to nutritional deficiency.