PV Pradeep MS, DNB, MRCS (Edin), M.Ch
(Endocrine Surgery), FACS, FRCS (Glasg)
Baby Memorial Hospital, Kozhikode, Kerala, India. PIN:
673004
Address for Correspondence: Dr PV Pradeep MS, DNB, MRCS
(Edin), M.Ch (Endocrine Surgery), FACS, FRCS (Glasg), Senior
Consultant, Endocrine Surgery, Baby Memorial Hospital,
Kozhikode, Kerala, India. PIN: 673004. E-mail:
pradeepputhenveetil@yahoo.co.in
Abstract
Endocrine emergencies related to diabetes is very common.
There are many non-diabetic emergencies in the field of
Endocrine Surgery which can be quite challenging. An attempt
is made here to look at these common endocrine emergencies.
Keywords: acute endocrinology, non-diabetic
Endocrine emergencies related to diabetes is very common.
There are many non-diabetic emergencies in the field of
Endocrine Surgery which can be quite challenging. An attempt
is made here to look at these common endocrine emergencies.
Acute adrenal insufficiency
First described by Thomas Addison in 1855. Prevalence is
35-120 per million. The usual etiology of primary adrenal
failure includes autoimmune conditions, tuberculosis,
metastasis, drugs like antifungals, HIV infection and adrenal
hemorrhage. Secondary adrenal insufficiency is due to the
pituitary disease and results only in glucocorticoid
deficiency. Adrenal crisis is usually precipitated by sepsis.
Abrupt stoppage of steroids can also precipitate a crisis. Can
also happen in patients who undergo bilateral adrenalectomy
and do not take the steroid supplements. Clinical features may
be shock with unexplained hypoglycemia and hyponatremia.
Patient may have associated abdominal pain. Hyperpigmentation
is a cardinal sign of primary adrenal failure. The basic
assessment includes measurement of electrolytes, cortisol,
ACTH. Short synacthen tests helps differentiate primary and
secondary adrenal failure. Treatment includes fluid
resuscitation with normal saline, IV hydrocortisone; later
shift to oral steroids and Fludrocortisone.
Acute hypercalcemia
Primary hyperparathyroidism (PTH mediated) and malignancy
account for 90% of these cases. Non PTH mediated hypercalcemia
is commonly due to osteolytic bone metastasis (PTHrP related),
Hypervitaminosis D (Chronic granulomatous diseases), drugs
(Thiazides), lithium and Vitamin A toxicosis. Hyperthyroidism,
acromegaly and pheochromocytoma are also associated with
hypercalcemia. Acute hypercalcemia presents with non
specific symptoms like weakness, confusion, anorexia,
vomiting, polyuria. ECG may reveal short QT interval,
bradycardia, AV block. Calcium levels > 12mg% is an
indication for prompt intervention. In such a scenario the
underlying etiology can be evaluated later. Treatment includes
rehydration with IV saline. Bisphosphonates (Palmidronate/
Zoledronate) are used to lower the high calcium. The action of
bisphosphates begins in 24-48 hours and it may take up to 4
days for calcium to normalize. The effect of IV
bisphosphonates may last a few weeks. Calcitonin has rapid
onset of action within 2-6 hours of administration and results
in fall in calcium up to 2 mg%. However overall effect of
calcitonin may be inconsistent, partial and transient.
Glucocorticoids, calcimimetic agents and diuretics also used
in appropriate cases.
Acute hypocalcemia
This is usually due to a low PTH. Neck surgery especially
thyroid, parathyroid surgery, autoimmune conditions, chronic
renal disease and vitamin D deficiency. Paresthesia, muscle
cramps, tetany are the clinical features. Diagnostic tests
include estimation of serum calcium, phosphorus, PTH, Vitamin
D, renal function and serum magnesium. The treatment of
hypocalcemia depends on the severity. Serum calcium less than
7.5mg% with associated tetany needs IV calcium either as
infusion or by bolus injections. Infusion can be started at
2mg/Kg/hr and tapered every 12 hours and stopped. Biochemical
hypocalcemia without symptoms can be managed with oral calcium
carbonate. Associated vitamin D deficiency has to be corrected
with calcitriol or Vitamin D2. Associated hypomagnesia needs
to be corrected to achieve normocalcemia in resistant cases.
Thyroid storm
Life threatening condition. Has severe symptoms and signs of
thyrotoxicosis. The diagnosis is based on clinical features
which includes high fever, tachycardia, cardiac failure,
hypotension, gastrointestinal symptoms and neurological
manifestations like apathy, coma and stupor. Burch and
Wartofsky have formulated a rating system to identify whether
the patient is in thyroid storm or is having only
thyrotoxicosis. Physical examination in a suspected case of
thyroid storm may reveal a goiter with or without
ophthalmopathy.
The thyroid profile will reveal the biochemical evidence of
hyperthyroidism. The treatment is a multimodality therapy.
Supportive care, correction of precipitating event and
targeting all the therapeutically accessible points in the
thyroid hormone synthesis, secretion and peripheral action
pathways. Inhibition of thyroid hormone synthesis is achieved
with thionamides (methimazole/ carbimazole), inhibition of
thyroid hormone release by lithium carbonate or iodine
therapy, inhibition of T4 to T3 (active form) conversion by
PTU, corticosteroids, non selective beta blockers. In
resistant cases plasmapheresis is done. In cases where the
patient has to undergo surgery a rapid blockade can be done;
however even with rapid blockade patient is safe to be
operated by 6th to 8th day. Beta blocker (Propranolol) 60-80mg
6th hourly, Dexamathasone 2mg 6th hourly, saturated solution
of potassium iodide (SSKI, 38mg iodide/drop) 5 drops 6th
hourly is used for rapid preparation. After clinical
manifestations of thyroid storm are, controlled long term anti
thyroid medications/ radio iodine therapy/ thyroidectomy can
be planned.
Myxedema coma
A medical emergency with high mortality (50-60%). Defined as a
severe form of hypothyroidism with depressed mental status,
hypothermia and reduced function of other organs. Most of
these patients are elderly women. A hypothyroid individual can
develop myxedema coma due to many exacerbating and
precipitating factors. These include hypothermia, congestive
cardiac failure, GI bleed, myocardial infarction, burns,
trauma and medications. History of these patients may reveal
prior thyroidectomy, radio iodine therapy or past history of
thyroxine therapy. In 5% of these patients myxedema may be
part of central hypothyroidism. Physical examination reveals
the classical features of hypothyroidism like dry skin,
hoarseness, macroglossia, delayed tendon reflexes etc.
Hypothermia is a clinical clue to the state of myxedema in a
hypothyroid subject. Cardiac manifestations include ECG
changes, recurrent pericardial effusion and congestive heart
failure. Evaluation of TSH/ T4 will clinch the diagnosis but
there can be associated hypoglycemia and hyponatremia.
Hypoadrenalism should also be ruled out in these patients.
On admission to ICU initiation of mechanical ventilation
should not be delayed so as to prevent the hypoxia and
hypercapnia. Adequate care is needed to treat the associated
hypothermia, hypotension and hyponatremia. Glucocorticoid
therapy 100mg IV 8th hourly is initiated. IV T4 and T3 is
initiated. T4 200-400 micrograms is given daily. T3 is
provided at 10 micrograms thrice daily.
Pheochromocytoma crisis
Patient with already diagnosed or undiagnosed pheochromocytoma
presents with life threatening hypertensive crisis or
cardiogenic shock. A detailed history in such patients reveal
a past history of episodic attacks of headache, sweating and
palpitations. Few of these patients may also be on more than
two anti-hypertensive agents. Diagnosis is confirmed by
estimating the plasma metanephrines or the 24 hour urinary
metanephrines. Confirmation can be done by CT scan or MRI scan
of the adrenal gland. IV sodium nitropruside is used to
control the hypertensive crisis. Phenoxybenzamine produces
irreversible, non-competitive, alpha 1 and 2 blockade
and in the acute setting a dose of 1mg/Kg in 250ml of 5%
dextrose is used over 4 hours. Once patient is stable
patient can be started on Prazocin to control the hypertension
and later operate.
Pituitary apoplexy
Sudden hemorrhage into the pituitary gland. This can occur
into a pre-existing pituitary adenoma. Clinical presentation
may vary from mild symptoms to adrenal crisis, coma and sudden
death. Sudden onset of severe headache, diplopia and symptoms
of hypopituitarism indicates the diagnosis. In the acute
setting cortisol deficiency has to be promptly corrected.
Apart from these, insulinomas are known to present in various
forms of emergencies due to the hypoglycemia. Frequent history
of loss of consciousness, coma, epileptic attacks and
neuropsychiatric symptoms.
To conclude, the endocrine emergencies, even though uncommon
are life threatening. Although endocrine emergencies usually
occur in patients known to have endocrine pathology, its
important not to miss them in the de novo presentations.
Aggressive supportive care, steroid replacement and other
deficient hormones have shown to decrease the morbidity and
mortality associated with endocrine emergencies.
References
1. G Van den Berge et al (Editors). Acute Endocrinology: From
cause to consequence. 2008; Humana Press New York, NY.
2. Kavalapalli S.
http://www.apiindia.org/medicine_update_2013/chap68.pdf
3. Burch HB, Wartofsky L. Graves ophthalmopathy: Currents
concepts regarding pathogenesis and management. Endocr Rev
1993;14:747-93.