SOAL PRE TEST SUB BAGIAN ENDOKRIN METABOLIK

PATOFISIOLOGI, DIAGNOSIS, DAN

PENATALAKSANAAN HIPERPARATIROID

HUSNA FAUZIAH
SUB BAGIAN ENDOKRIN METABOLIK
PROGRAM STUDI ILMU PENYAKIT DALAM
FAKULTAS KEDOKTERAN UNIVERSITAS ANDALAS
PADANG 2018
 A previously fit and well 21-year-old man
presented to the orthopaedic team with:
• Right hip pain that had been going on for 3 weeks, no
previous history of trauma.
• Able to bear weight but had an antalgic gait.
• Had a limp as a child that was self-limiting and no cause for
this was identified.
• No relevant family history and he lived alone.
• He was found to be very thirsty, and even at night he would
routinely drink 2 L of water.
• He was a non-smoker and drank alcohol socially.
 INVESTIGATION
• BLOOD SAMPLE:
– Hypercalcaemia (3.55 mmol/l)
– Anemia (Hb 8.6 mg/dL)
– Renal impairment (urea: 13.6 mmol/L, creatinine
170 μmol/L)
– Raised alkaline phosphatase (2534 U/L)
• Renal ultrasound: nephrocalcinosis
• Raised parathyroid hormone level: 1285ng/L
• Neck ultrasound scan and a sesta-mibi scan:
demonstrated the appearances in keeping
with a left lower parathyroid adenoma.
• X-rays of his pelvis and femurs: demonstrated
a right intracapsular fracture of the neck of
femur and a displaced transverse mid-shaft
fracture of left femur.
 Question
• What is the diagnosis of this patient?
• What is the clinical presentation of the
disease?
• What is the examination that can support the
diagnosis?
• What is the complication of the disease?
• What is the the treatment for the disease?
 Defenition

• Primary hyperparathyroidism is a common

disorder of mineral metabolism that is due to
excessive secretion of parathyroid hormone. The
clinical consequences of abnormally active
parathyroid tissue are typically hypercalcaemia
and concentrations of parathyroid hormone that
are either clearly elevated above the normal
range or inappropriately normal in the context of
hypercalcaemia
 Patophysiology
• The secretion and synthesis of parathyroid hormone is controlled by the
ambient circulating ionised calcium concentration. Under normal
conditions, an increase in circulating ionised calcium concentration 
instantly suppress parathyroid hormone secretion. Similarly, an
imperceptible reduction in serum calcium concentration will immediately
simulate parathyroid hormone secretion. This inverse sigmoidal
association between parathyroid hormone and serum calcium
concentration is regulated by the calcium-sensing receptor. The other
principal regulator of parathyroid hormone secretion is 1,25-
dihydroxyvitamin D concentration, which is also inversely associated with
parathyroid hormone concentration. Although not considered to be as
important as calcium or 1,25-dihydroxyvitamin D, phosphate
concentration does bear some association with parathyroid hormone
secretion, probably as an indirect factor by influencing calcium
concentration. Furthermore, a potential suppressive effect of fibroblast
growth factor 23 on parathyroid hormone secretion has been described.
 Clinical Presentation
• The clinical syndrome of primary
hyperparathyroidism can be easily
remembered as "bones, stones, abdominal
groans, and psychic moans
• Skeletal manifestations: primarily a selective
cortical bone loss. Bone and joint pain,
pseudogout, and chondrocalcinosis have also
been reported.
• Renal manifestations: polyuria, kidney stones,
hypercalciuria, and rarely nephrocalcinosis.
• Gastrointestinal manifestations: anorexia,
nausea, vomiting, abdominal pain, constipation,
peptic ulcer disease, and acute pancreatitis.
• Neuromuscular and psychologic manifestations: proximal
myopathy, weakness and easy fatigability, depression,
inability to concentrate, and memory problems or subtle
deficits that are often characterized poorly and may not be
noted by the patient.
• Cardiovascular manifestations: hypertension, bradycardia,
shortened QT interval, and left ventricular hypertrophy. [5]
• Physical examination: usually noncontributory. Examination
may reveal muscle weakness and depression. A palpable
neck mass may indicate parathyroid cancer. A previously
undiagnosed thyroid nodule is much more commonly the
source of a palpable nodule.
 Examination
• The combination of hypercalcemia and an
elevated or inappropriately normal parathyroid
hormone (PTH) level makes PHPT the most likely
diagnosis
• Measured total serum calcium should be
adjusted for albumin. If the corrected serum
calcium is normal and PTH is elevated, serum
ionized calcium should be measured, as PHPT can
present with an elevated ionized calcium despite
a normal albumin adjusted serum calcium
• An elevated serum PTH in the presence of
consistently normal albumin-adjusted calcium
and ionized calcium, normal serum 25-
hydroxyvitamin D (25OHD>20 ng/mL or 50
nmol/L), and well-maintained renal function
(eGFR > 60 mL/min/1.73 m2) supports this
diagnosis
• erology primary
– hypercalcemia
– ↑ PTH

• secondary
– hypocalcemia/normocalcemia
– ↑ PTH

• malignancy
– ↓ PTH
• ↑ alkaline phosphatase
• normal anion gap metabolic acidosis
– ↓ renal reclamation of bicarbonate
• Urinalysis primary
– hypercalciuria (renal stones)
– ↑ cAMP
• Radiograph cystic bone spaces ("salt and pepper")
– often in the skull
• loss of phalange bone mass
– ↑ concavity (see key image of this topic)
• EKG shortened QT
 Complication
• Peptic ulcer disease
– ↑ gastrin production stimulated by ↑ Ca2+
• Acute pancreatitis
– ↑ lipase activity stimulated by ↑ Ca2+
• CNS dysfunction
– anxiety, confusion, coma
– result of metastatic calcification of the brain
 Therapy
• Acute hypercalcemia IV fluids
• Loop diuretics
• Symptomatic hypercalcemia is treated
surgically treat with parathyroidectoy
• complications include post-op hypocalcemia
• manifests as numbness, tingling, and muscle
cramps
• should be treated with IV calcium gluconate
Primary hyperparathyroidism results most
often (75-80%) from the occurrence of one or
more adenomas in previously normal
parathyroid glands, although in 20% of cases
diffuse hyperplasia of all parathyroid glands
may be present or, rarely, parathyroid
carcinoma may be found (less than 1-2%)