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Kwashiorkor

Protein energy malnutrition


Presentation

The following symptomatology is evident in acute or chronic phase of Kwashiorkor:

General appearance: Kwashiorkor patients show apathy and lethargy as acute signs. They may appear with signs of easy irritability which are all attributed to low energy levels due to lack of protein. They are generally thin with cachexia due to the absence of muscle mass with defective protein synthesis.

Integumentary system: Hair may appear with different shades of light and dark bands referred to as the “flag sign”. The different hues in the hair represents the different periods of impaired nutrition. The skin will appear flaky due incompetent connective tissues formed from a defective protein synthesis process.

Cardiovascular and respiratory system: The heart may present with mild murmur due to micronutrient deficiency anemia or impending heart failure in the late stages. Pericardial effusion may present with severe PEM in children [7]. Breathing may be shallow due to metabolic acidosis.

Abdomen: The abdomen may be protuberant or enlarged due to ascites. The liquid damming in the abdominal cavity is due to the hypoalbuminemia effect on osmosis. Hepatomegaly may ensue with chronic fatty liver disease. Intestinal parasitism may not be infrequent with severe malnutrition.

Extremities: The limbs will generally convey weakness because of the lack of energy from proteins. Bipedal non-pitting edema may occur in the legs due to the osmotic imbalance.

Malnutrition
  • This suggests a decreased CYP1A2 activity in all categories of malnutrition.[ncbi.nlm.nih.gov]
  • Patients with any chronic disease liable to cause malnutrition were excluded.[ncbi.nlm.nih.gov]
  • Malnutrition in childhood continues to be one of the most important risk factor for secondary immunodeficiency in the world; therefore one should think of existence of malnutrition in a child suffering of frequent infections, not only in developing country[ncbi.nlm.nih.gov]
  • Advanced cases of malnutrition are rare in the United States. Since it is rarely suspected, a correct diagnosis of malnutrition, even in extreme form, may easily be missed.[ncbi.nlm.nih.gov]
  • Kwashiorkor is part of the spectrum of protein-energy malnutrition. The condition results from a lack of nutritional protein coupled with carbohydrate excess.[ncbi.nlm.nih.gov]
Anemia
  • Initially, recovery was associated with rapid loss of weight and anemia and subsequently with weight gain and return to good health.[ncbi.nlm.nih.gov]
  • The child presented at 10 months of age with decreased weight for height, rash, hepatomegaly, edema, hypoproteinemia, hypoalbuminemia, anemia, hypoglycemia, and evidence of hepatic sequestration of lipids.[ncbi.nlm.nih.gov]
  • Mediated by some complex mechanism, children suffering from sickle cell anemia are predisposed to PEM and Kwashiorkor.[symptoma.com]
  • […] of kwashiorkor : severe malnutrition chiefly affecting young children especially of impoverished regions that is characterized by failure to grow and develop, changes in the pigmentation of the skin and hair, edema, fatty degeneration of the liver, anemia[merriam-webster.com]
  • Until she began developing the symptoms of Kwashiorkor, including general fatigue, edema of the face and extremities, anemia, alopecia, and weight loss, she had been leading a normal life post-gastrectomy.[ncbi.nlm.nih.gov]
Fatigue
  • Until she began developing the symptoms of Kwashiorkor, including general fatigue, edema of the face and extremities, anemia, alopecia, and weight loss, she had been leading a normal life post-gastrectomy.[ncbi.nlm.nih.gov]
  • Early symptoms include fatigue, irritability, and lethargy. As protein deprivation continues, one sees growth failure, loss of muscle mass, generalized swelling (edema), and decreased immunity. A large, protuberant belly is common.[amitahealth.adam.com]
  • Symptoms include: Changes in skin pigment Decreased muscle mass Diarrhea Failure to gain weight and grow Fatigue Hair changes (change in color or texture) Increased and more severe infections due to damaged immune system Irritability Large belly that[nlm.nih.gov]
Anorexia
  • BACKGROUND: Customary blood protein markers for malnutrition are of limited value in the diagnosis of protein-energy malnutrition or anorexia nervosa in children and in the follow-up to refeeding in such children.[ncbi.nlm.nih.gov]
  • We present the case of a woman aged 48 years, diagnosed with anorexia nervosa (AN) at the age of 12.[ncbi.nlm.nih.gov]
  • Kwashiorkor is a form of severe protein–energy malnutrition characterized by edema, irritability, anorexia, ulcerating dermatoses, and an enlarged liver with fatty infiltrates.[osmosis.org]
  • List items include Diarrhea, Anorexia and more. Many of the symptoms of Kwashiorkor may be related to other illnesses or conditions, so if you're experiencing any of these symptoms don't automatically assume you have Kwashiorkor.[ranker.com]
Weight Loss
  • The greater the weight loss, the lower the serum carnitine concentration amongst the kwashiorkor children (r 0.46; p 0.01). There was a linear relationship between serum free carnitine and hiolein oxidation (r 0.89; p 0.001).[ncbi.nlm.nih.gov]
  • Systemic manifestations include peripheral edema, liver disease, neurologic changes, diarrhea, weight loss, and hypoalbuminemia.[ncbi.nlm.nih.gov]
  • Until she began developing the symptoms of Kwashiorkor, including general fatigue, edema of the face and extremities, anemia, alopecia, and weight loss, she had been leading a normal life post-gastrectomy.[ncbi.nlm.nih.gov]
  • The combination of Malawian diet and kwashiorkor microbiome produced marked weight loss in recipient mice, accompanied by perturbations in amino acid, carbohydrate, and intermediary metabolism that were only transiently ameliorated with RUTF.[ncbi.nlm.nih.gov]
  • It leads to dehydration and weight loss. Starvation is a form of this disorder.[healthline.com]
Diarrhea
  • Systemic manifestations include peripheral edema, liver disease, neurologic changes, diarrhea, weight loss, and hypoalbuminemia.[ncbi.nlm.nih.gov]
  • Here, we report an infant who presented with diarrhea and skin signs of kwashiorkor, and duodenal biopsy was consistent with Crohn's disease.[ncbi.nlm.nih.gov]
  • We report a case of a 8-month-old male infant in the suburban Detroit, MI, USA who presented with diffuse edema, erythroderma and desquamation, a "bull-dog" face, diarrhea, and irritability, consistent with kwashiorkor as a result of severe dietary restriction[ncbi.nlm.nih.gov]
  • They may take a stool sample to rule out other issues related to diarrhea if diarrhea is a symptom. Your doctor may also test your urine or your blood to help identify a nutrition deficiency.[healthline.com]
  • Pneumonia and diarrhea are almost always present.[clinicaladvisor.com]
Failure to Thrive
  • Failure to thrive may not be a result of organ disease, physical abuse, or intentional neglect. We describe an infant who developed kwashiorkor with a high-fat, low-protein, nondairy coffee creamer diet.[ncbi.nlm.nih.gov]
  • Failure to thrive (failure to put on height and weight) Loss of muscle mass Generalised swelling ( oedema ) Large protuberant belly (pot belly) Fatty liver Failing immune system so prone to infections and increased severity of normally mild infections[dermnetnz.org]
  • A child with Kwashiorkor looking miserable and having brownish hair color which are signs of Kwashiorkor Signs and Symptoms of Kwashiorkor Growth retardation (failure to thrive) Edema (swelling of the body) Muscle wasting Apathy (loss of interest) and[jotscroll.com]
Chronic Diarrhea
  • Eventually, Kumpheak’s entire body became swollen, he developed a full-body rash, fever, chronic diarrhea and uncontrollable vomiting, all of which are signs of kwashiorkor. Kwashiorkor is a severe type of protein-deficiency malnutrition.[angkorhospital.org]
  • The symptoms of marasmus include: weight loss dehydration chronic diarrhea stomach shrinkage You’re at an increased risk for marasmus if you live in a rural area where it’s difficult to get food or an area that has a food shortage.[healthline.com]
Abdominal Distension
  • History and Physical The clinical manifestations of kwashiorkor include the following: Peripheral pitting edema that begins in dependent regions and proceeds cranially Marked muscle atrophy Abdominal distension (with/without dilated bowel loops and hepatomegaly[ncbi.nlm.nih.gov]
Recurrent Diarrhea
  • Lack of exclusive breastfeeding may be an important cause of Kwashiorkor even before 1 year due to predisposition of the child to recurrent diarrhea and infections Kwashiorkor mostly occur in children that have been weaned suddenly or when weaning begins[jotscroll.com]
Hepatomegaly
  • The child presented at 10 months of age with decreased weight for height, rash, hepatomegaly, edema, hypoproteinemia, hypoalbuminemia, anemia, hypoglycemia, and evidence of hepatic sequestration of lipids.[ncbi.nlm.nih.gov]
  • ) Round face (prominence of the cheeks, or “moon facies”) Thin, dry, peeling skin with confluent areas of scaling and hyperpigmentation Dry, full, hypopigmented hair that falls out or is easily plucked Hepatomegaly (from fatty liver infiltrates) Growth[ncbi.nlm.nih.gov]
  • Exams and Tests The physical exam may show an enlarged liver ( hepatomegaly ) and general swelling.[health.ridgeviewmedical.org]
  • Hepatomegaly may ensue with chronic fatty liver disease. Intestinal parasitism may not be infrequent with severe malnutrition. Extremities: The limbs will generally convey weakness because of the lack of energy from proteins.[symptoma.com]
  • If hepatomegaly is found on physical exam, an abdominal ultrasound may be indicated. It is low in cost and doesn’t require exposure to radiation, and will provide an initial assessment of the hepatic anatomy.[cancertherapyadvisor.com]
Dermatitis
  • The presence of edema and "flaky paint" dermatitis should prompt a careful dietary investigation.[ncbi.nlm.nih.gov]
  • A 14-month-old girl and a 7-month-old boy each presented with a diffuse dermatitis, whole body edema, and hypoalbuminemia. The diets of both infants consisted almost entirely of Rice Dream, a rice-based, protein-poor beverage.[ncbi.nlm.nih.gov]
  • She was admitted to a highly specialised eating disorder facility with distended abdomen, muscular atrophy, ulcerative dermatitis, electrolyte derangements and low serum albumin.[ncbi.nlm.nih.gov]
  • We report a case of a 12-month old girl in Belo Horizonte, Minas Gerais, Brazil, who presented with diffuse edema, desquamation, and irritability misdiagnosed as atopic dermatitis.[ncbi.nlm.nih.gov]
  • This article describes a case of kwashiorkor in a child with food aversion that manifested as "flaky paint dermatitis." Our discussion will attempt to delineate underlying conditions that may predispose to kwashiorkor.[ncbi.nlm.nih.gov]
Alopecia
  • Cutaneous clues to the diagnosis of protein deficiency include the "flag sign," hypochromotrichia, alopecia, "crazy pavement" dermatosis, pallor, and glossitis.[ncbi.nlm.nih.gov]
  • Until she began developing the symptoms of Kwashiorkor, including general fatigue, edema of the face and extremities, anemia, alopecia, and weight loss, she had been leading a normal life post-gastrectomy.[ncbi.nlm.nih.gov]
  • Chronic iron deficiency may cause diffuse hair loss (alopecia).[britannica.com]
Dry, Brittle Hair
  • , brittle hair that falls out easily and may lose its colour failure to grow in height tiredness or irritability ridged or cracked nails Kwashiorkor can be fatal if it's left untreated for too long because children become very vulnerable to infections[nhs.uk]
Agitation
  • They were then told to add approximately 30 ml of water or just enough to dissolve the powder with vigorous agitation. Powder bottles contained a week's supply and went to carers at enrolment and then every four weeks.[doi.org]

Workup

Patients with Kwashiorkor are often subjected to these following tests:

  • Complete blood count to determine state of anemia and inflammatory processes with infections.
  • Arterial blood gases to determine extent of metabolic acidosis of the patient.
  • BUN and Creatinine are accurate markers for the kidney’s health.
  • Serum potassium determination guides management of hypokalemia.
  • Total protein albumin and globulin determination to assess the levels of hypoalbuminemia and hypoglobulinemia.
  • Urinalysis will show ketone bodies due to improper protein metabolism.
Ketonuria
  • ケトン尿 ketonuria ケトン尿症 ketosis ケトーシス ketosis prone diabetes ketosis prone diabetes ketotic hypoglycemia ケトン性低血糖症 kidney biopsy 腎生検 kidney failure 腎不全 kidney transplantation 腎移植 knee jerk 膝蓋腱反射 knee reflex 膝蓋腱反射 kwashiorkor クワシオルコル[jds.or.jp]
Macrocytic Anemia
  • Thus, anemia is usually present, most often hypochromic microcytic anemia, but a concurrent deficiency of folates may lead to a mixed microcytic-macrocytic anemia.[humpath.com]

Treatment

The early intervention of fulfilling protein caloric deficiency in Kwashiorkor will greatly improve patient’s outlook. Dietary replacement therapy are arbitrarily dependent on the severity of the disease. Caloric replacement will usually start with carbohydrates and simple sugars until the patient is able to achieve adequate energy.

By then protein infusion may be necessary along with supplemental vitamins and minerals. Patients who underwent prolonged starvation may not be immediately infused with protein because it may trigger a Refeeding Syndrome (RS) which leads to the uncontrolled catabolic action of insulin [8]. Kwashiorkor patients with superimposed infections treated with antibiotics should be reevaluated for the kidney’s clearance of the drugs used because it is usually impaired in PEM [9].

Patients received in shock will benefit from the immediate transfusion with plasma expanders and fluids. Oral enzymatic replacement with lactase may be helpful in cases of lactose intolerance.

Prognosis

Kwashiorkor and other protein energy malnutrition is considered a metabolic emergency. Patients who get medical care early have a good outlook in the clinical course of the disease.

However, those treated at the later stage of the disease may still be alleviated but permanent conditions like mental and growth retardation may occur. Patients who demonstrated lipids and ketones in the urine may have a poorer prognosis with PEM [5].

Kwashiorkor and PEM carries a grim prognosis when it occurs in patients below 5 years of age and in elderly people beyond 55 years old [6].

Complications

The primary protein energy state in Kwashiorkor may give rise to these complications:

  • Sepsis: The dermatologic manifestation of generalized ulceration may invite a host of pathogenic bacteria to the ailing body. Defective protein synthesis will impair antibody production which may hamper significantly the body’s immune response against invading microorganism.
  • Coma: The state of continuous carbohydrate breakdown in the light of a limited supply of protein may lead to a system condition of metabolic acidosis. The rapid decline of pH and the increased acidity of the plasma may incapacitate the brain into a vegetative state of coma.
  • Hypovolemic shock: The incompetent fatty liver in Kwashiorkor will decrease the albumin production and decrease the osmotic gradient in the intracellular spaces. Progressive dehydration due to an incompetent osmotic gradient within the vessels may lead to hypovolemic shock.
  • Mental and physical disability: Uncontrolled metabolic imbalance that afforded chronic insults to the brain may result in a permanent mental retardation and physical incapacities.
  • Death: The severe forms of these PEMs may culminate with liver failure, heart failure or renal failure that can eventually lead to death.

Etiology

The basic etiology in Kwashiorkor is the limited or meager supply of food available in the community. This condition is greatly influenced by drought, famine, war, and political unrest which are common in some countries in Africa and Central America.

Kwashiorkor may also prevail in communities with no access to protein-rich foods like meat and poultry and are solely dependent on carbohydrates from grains and vegetables.

Epidemiology

The incidence of Kwashiorkor in the US is relatively low but PEM is somewhat prevalent among hospitalized patients. The World Health Organization in 2000 announced that there at least 181.9 million children suffering from severe malnutrition especially in developing countries. Statistics in Central Asia and Eastern Africa reveal that approximately half of the children have some form of growth retardation due to protein energy malnutrition.

About 5 million children deaths in the developing countries are related to PEM and Kwashiorkor. Mortality rates in Kwashiorkor decrease with increasing age of its onset. The dark skinned races have more incidence of Kwashiorkor due to the relatively poor socio-economic situation in their country that affects dietary input.

Mediated by some complex mechanism, children suffering from sickle cell anemia are predisposed to PEM and Kwashiorkor [1].

Sex distribution
Age distribution

Pathophysiology

Kwashiorkor as a protein energy malnutrition state in children happens with adequate carbohydrate nutrition but devoid of protein sources.

This condition will lower the body’s drive to produce its own visceral protein leading to poor energy supply. In Kwashiorkor, low protein substrates (raw materials) can lead to hypoalbuminemia that causes the extravasation of fluid to the extracellular space manifesting as edema.

Consequently, this low protein state may impair the production of B-lipoproteins that results in a fatty liver [2]. The inadequate production of transfer proteins may lead to micronutrient deficiency like zinc.

The concurrent zinc deficiency is implicated as the main cause of skin diseases like ulcerations in Kwashiorkor patients. Kwashiorkor is seen to affect glucose clearance in the blood causing a dysfunction in the beta-cells of the pancreas [3]. Pregnant mothers with PEM may affect inherent metabolic physiology of the fetus that my result to marasmus or Kwashiorkor when the baby is born [4].

Prevention

  • Antioxidants may be able to curb excessive free radical activity and prevent the development of kwashiorkor in susceptible children.[ncbi.nlm.nih.gov]
  • CONCLUSIONS: Antioxidant supplementation at the dose provided did not prevent the onset of kwashiorkor.[ncbi.nlm.nih.gov]
  • Role of antioxidants in preventing kwashiorkor The “negative” results of this study made us speculate if a larger antioxidant dose is needed to prevent kwashiorkor.[doi.org]
  • Primary Prevention Successful prevention strategies for kwashiorkor do not exist.[online.epocrates.com]

References

Article

  1. Hyacinth HI, Adekeye OA, Yilgwan CS. Malnutrition in Sickle Cell Anemia: Implications for Infection, Growth, and Maturation. J Soc Behav Health Sci. Jan 2013; 1:7(1).
  2. Bandsma RH, Spoelstra MN, Mari A, Mendel M, van Rheenen PF, Senga E, van Dijk T, Heikens GT. Impaired glucose absorption in children with severe malnutrition.J Pediatr. 2011; 158(2):282-7.e1 
  3. Spoelstra MN, Mari A, Mendel M, et al. Kwashiorkor and marasmus are both associated with impaired glucose clearance related to pancreatic beta-cell dysfunction. Metabolism. Mar 2 2012;
  4. Forrester TE, Badaloo AV, Boyne MS, Osmond C, Thompson D, Green C, et al. Prenatal factors contribute to the emergence of kwashiorkor or marasmus in severe undernutrition: evidence for the predictive adaptation model. PLoS One. 2012; 7(4):e35907.
  5. Badaloo AV, Forrester T, Reid M, Jahoor F. Lipid kinetic differences between children with kwashiorkor and those with marasmus. Am J Clin Nutr. 2006; 83(6):1283-8 
  6. Demling RH. The incidence and impact of pre-existing protein energy malnutrition on outcome in the elderly burn patient population. J Burn Care Rehabil. Jan-Feb 2005; 26(1):94-100.
  7. Ahmad S, Ellis J, Nesbitt A, Molyneux E. Pericardial effusions in children with severe protein energy malnutrition resolve with therapeutic feeding: a prospective cohort study. Arch Dis Child. 2008; 93(12):1033-6 
  8. Walmsley RS. Refeeding syndrome: screening, incidence, and treatment during parenteral nutrition. J Gastroenterol Hepatol. Dec 2013; 28 Suppl 4:113-7.
  9. Oshikoya KA, Sammons HM, Choonara I. A systematic review of pharmacokinetics studies in children with protein-energy malnutrition. Eur J Clin Pharmacol. 2010; 66(10):1025-35 
  10. Dorner TE, Lackinger C, Haider S, Luger E, Kapan A, Luger M, et al. Nutritional intervention and physical training in malnourished frail community-dwelling elderly persons carried out by trained lay "buddies": study protocol of a randomized controlled trial. BMC Public Health. Dec 2013; 13(1):1232.

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Last updated: 2019-07-11 22:25