Monday, 19 February 2007

Kwashiorkor--no way!!

The following case has been anonymised for patient confidentiality.

A few months ago a female patient was seen at a peripheral hospital in her 30s who was admitted with weight loss over several years. She denied all symptoms including loss of appetite, fevers, night sweats, cough, sputum, abdominal pain and diarrhoea.

However, on direct questioning she admitted to having some recent constipation, easy satiety with little food and leg swelling.

During her admission she was noted to have recurrent hypoglycaemia which occurred when she was taken off of the intravenous glucose infusion. During hypoglycaemia she developed dizziness and pre-syncopal symptoms but no sweating, shaking or hunger.

She was noted to have abnormal liver function but she denied drinking alcohol and had no history of risks for hepatitis B or C infection.

Her periods had stopped suddenly several years before, but before the weight loss, she admitted to only ever having 3 periods a year.

She had been investigated previously with a gastroscopy and colonoscopy to rule out malignancy and all had been normal.

Previous medical history was otherwise unremarkable.

When I reviewed this patient she was extremely low in body weight being only 30kg. She had diffuse muscle wasting and temporalis muscles were wasted.

She had cool hands and her nails showed Koilonychia (spoon nails) consistent with Iron Deficiency Anaemia. Her pulse was regular and approx 60 per minute. Her skin was thin and flakey and there were signs of multiple bruising all over her body. The knuckles of her hands showed callosities and her teeth showed some mild dissolution.

JVP was not raised. There was a normal thyroid on palpation and no evidence of lymph nodes.
Heart sounds were loud but no murmur was evident.

Chest examination revealed intercostal recession and percussion was difficult due to the patient's frailty. Chest sounds were normal with no signs of crackles or wheeze.

Abdominal examination showed a thin abdomen and on percussion the liver edge was below the costal margin by approximately two fingers. The liver was non-tender. There were no signs of chronic liver disease and bowel sounds were increased.

Legs were extremely oedematous and leaking. She had sores on her knees.

Clinical Impression

1) Anorexia nervosa (extremely emaciated, callosities on he hands, dissolving teeth, cool peripheries, oedema, secondary amenorrhoea, hypoglycaemia)

2) Likely multivitamin deficiency including signs of Scurvy (fragility of capillaries leading to multiple bruising)

3) Hypoproteinaemic state

4) Possible early liver cirrhosis due to malnutrition resulting in abnormal liver function and recurrent hypoglycaemia ( impaired gluconeogenesis, glycogenolysis and impaired glucogenesis)

5) Possible Iron Deficiency Anemia as a result of decreased nutritional intake.

Blood results revealed a low insulin and C-peptide level, borderline hypothyroidism, an albumin of 3.1, negative anti-insulin antibodies. Cortisol collection result was above normal with a high ACTH level. FSH and LH levels were decreased. GH level was normal.

Abdominal Echo showed some ascites but a normal pancreas. CT showed no evidence of malignancy. Cardiac echo revealed some mild pericardial effusion.


It was suggested that the insulin and C-peptide be repeated during an episode of hypoglycaemia and for a glucagon level to also be drawn. Moreover, I also suggested that urine sulphonylurea levels be checked to rule out self-administration.

The patient might have been developing cirrhosis due to severe malnutrition and hence, a liver biopsy would be an approprite next step.

In view of the severe low body weight and hypoglycaemia It was also suggested that the patient should have NG or PEG feeding as it might be the only way to ensure that the patient had sufficient and guaranteed calorie intake especially as the patient had shown signs of self-induced vomiting.

In view of the probable multi-vitamin deficiency, the patient would need replacement including ascorbic acid (Vitamin C).

This patient saw nothing wrong with being so under weight and often patients consider that they are over weight when they are fact severely under weight and hence, she might have an abnormal recognition of self-image. A psychiatric opinion would also be helpful although congnitive retraining can be difficult especially in older patients, and from the history of infrequent periods, I suspect that she has had an eating disorder most of her life.

Unfortunately, the secondary amenorrhoea, hypothyroidism and other features of this illness only abate when sufficient weight has been put on.

The patient clearly had anorexia nervosa and from the severe features of the disorder including hypoglycaemia, oedema, ascites and moderate liver dysfunction It was considered that she might have Kwashiorkor.

This disorder is seen in states of severe protein malnutrition and the features are the same as described in this patient. Anorexia nervosa is a recognised cause of Kwashiorkor.

With restoration of proper nutrition the features are reversible except those of liver disease if cirrhosis has developed.

This was an extremely interesting case for me and the history, physical and blood tests gave me the answer!

For thinking of causes of Hypoglycaemia, remember EXPLAIN.

EX- Exogenous e.g. alcohol, insulinl, sulphonylureas
P- Pituitary failure
L- Liver failure
A- Adrenal Failure
I- Immune e.g. insulin receptor antibodies
N- Neoplasia (rare e.g. insulinoma, haemangiopericytoma, retroperitoneal fibrosarcoma)

Always rule out drugs by taking urine screens, insulin levels with C-peptide.

For a more indepth explanation of hypoglycaemia and its investigation, please see a more detailed text.

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