Porovnajte primárnu, sekundárnu a terciárnu hyperparatyreózu (príčina, kalcium, fosfát).
Najprv si odpoveď povedz nahlas alebo v duchu – až potom ju odkry.
🫀 Interná medicína · 73
Choroby prištítnych teliesok
Definícia
- • Medzi hlavné choroby prištítnych teliesok patrí hyperparatyreóza – primárna (nadmerná sekrécia PTH pri ochorení prištítnych teliesok), sekundárna (chronická hypersekrécia PTH pri hypokalciémii z iných príčin) a terciárna (autonómna sekrécia PTH pri dlhotrvajúcej sekundárnej forme). Hypoparatyreóza je nedostatok PTH, charakterizovaný hypokalciémiou a hyperfosfatémiou.
Etiológia a patogenéza
- • Primárna hyperparatyreóza: histologicky asi v 85 % adenóm prištítneho telieska, asi v 15 % hyperplázia ≥ 2 teliesok, karcinóm v < 1 % prípadov.
- • Sekundárna hyperparatyreóza vzniká najčastejšie pri pokročilej chronickej chorobe obličiek, keď znížená tvorba aktívneho vitamínu D v obličkách a ďalšie faktory vedú k hypokalciémii a chronickej stimulácii sekrécie PTH.
- • Terciárna hyperparatyreóza: sekrécia PTH sa stáva nezávislou od kalciémie, zvyčajne pri dlhotrvajúcej sekundárnej hyperparatyreóze (napr. roky trvajúce terminálne zlyhanie obličiek).
- • Hypoparatyreóza môže vzniknúť napr. po odstránení alebo poškodení viacerých prištítnych teliesok pri tyreoidektómii či paratyreoidektómii alebo pri dedičných či autoimunitných ochoreniach.
Klinický obraz
- • Primárna hyperparatyreóza sa najčastejšie prejavuje asymptomatickou hyperkalciémiou; častá je aj nefrolitiáza, najmä pri hyperkalciúrii z dlhotrvajúcej hyperkalciémie.
- • Príznaky, ak sú prítomné, vyplývajú z hyperkalciémie: napr. chronická únava, zápcha, anorexia, nauzea a vracanie, poruchy koncentrácie, zmätenosť a depresia.
- • Nadbytok PTH stimuluje osteoklasty a môže viesť k osteitis fibrosa cystica (rarefakcia kosti s fibróznou degeneráciou, cystami a fibróznymi uzlíkmi).
- • Hypoparatyreóza: príznaky hypokalciémie – mravčenie v rukách a okolo úst, svalové kŕče, v ťažkých prípadoch tetánia; latentnú tetániu odhalí Chvostekov a Trousseauov príznak.
Diagnostika
- • Primárna hyperparatyreóza: hyperkalciémia so zvýšeným PTH, u menšiny s vysoko-normálnym PTH, ktoré je pri zvýšenom kalciu neprimerané; nízky sérový fosfát (najmä so zvýšenou fosfatúriou) podporuje diagnózu.
- • Familiárnu hypokalciurickú hyperkalciémiu (FHH) odlišuje od primárnej hyperparatyreózy nízka kalciúria.
- • Zobrazovacie vyšetrenia (napr. USG, 4D-CT, sestamibi scintigrafia) slúžia na predoperačnú lokalizáciu, nie na stanovenie diagnózy ani na rozhodnutie o operácii.
- • Hypoparatyreóza: nízke alebo aj nízko-normálne PTH pri hypokalciémii; ďalej vysoký sérový fosfát a normálna alkalická fosfatáza.
Liečba
- • Pri symptomatickej alebo progredujúcej hyperparatyreóze je indikovaná paratyreoidektómia.
- • Pri asymptomatickej primárnej hyperparatyreóze sa operácia odporúča napr. pri kalciu o 1 mg/dl (0,25 mmol/l) nad hornou hranicou normy, hyperkalciúrii alebo nefrolitiáze, klírense kreatinínu < 60 ml/min, zlomenine stavca alebo T-skóre −2,5 a nižšom a pri veku < 50 rokov.
- • Konzervatívny postup možno zvoliť pri asymptomatickej primárnej hyperparatyreóze u pacientov s vysokým operačným rizikom; pri samotnej miernej hyperkalciémii možno podať cinakalcet.
- • Hypoparatyreóza: kalcium a vitamín D, pri nedostatočnej odpovedi liečba náhradou PTH (v EÚ je na chronickú hypoparatyreózu dospelých schválený palopegteriparatid); pri tetánii 10 ml 10 % kalcium glukonátu i.v. počas 10 minút.
Komplikácie a prognóza
- • Nefrolitiáza – hyperkalciémia často spôsobuje hyperkalciúriu s tvorbou obličkových kameňov.
- • Osteoporóza v dôsledku nadmernej kostnej resorpcie pri primárnej hyperparatyreóze.
- • Pooperačná hypokalciémia – po paratyreoidektómii sa kalcium monitoruje niekoľkokrát denne počas 48–72 h; pri ťažšej alebo dlhotrvajúcej hyperparatyreóze, najmä pri sekundárnej hyperparatyreóze s ťažkou osteitis fibrosa cystica, môže byť hypokalciémia protrahovaná a symptomatická.
- • Ťažká hypokalciémia (< 7 mg/dl, t. j. < 1,75 mmol/l) môže spôsobiť hyperreflexiu, tetániu, laryngospazmus alebo generalizované kŕče.
Kľúčové pojmy
| primárna hyperparatyreóza | hyperparathyreosis primaria |
| sekundárna hyperparatyreóza | hyperparathyreosis secundaria |
| hypoparatyreóza | hypoparathyreosis |
| parathormón (PTH) | |
| adenóm prištítneho telieska | adenoma glandulae parathyroideae |
| hyperkalciémia | hypercalcaemia |
| hypokalciémia | hypocalcaemia |
| tetánia | tetania |
| osteitis fibrosa cystica | osteitis fibrosa cystica |
| paratyreoidektómia | parathyreoidectomia |
📎 Zdroje:
- Hyperparathyroidism – MSD Manual Professional Edition (Definícia)
Primary: Excessive secretion of PTH due to a disorder of the parathyroid glands Secondary: Hypocalcemia due to non-parathyroid disorders leading to chronic PTH hypersecretion Tertiary:
- Hyperparathyroidism – MSD Manual Professional Edition (Definícia)
Autonomous secretion of PTH unrelated to serum calcium concentration in patients with long-standing secondary hyperparathyroidism
- Hypoparathyroidism – MSD Manual Professional Edition (Definícia)
Hypoparathyroidism is characterized by hypocalcemia and hyperphosphatemia and often causes chronic tetany.
- Hypercalcemia – MSD Manual Professional Edition (Etiológia 1)
Histologic examination shows a parathyroid adenoma in approximately 85% of patients with primary hyperparathyroidism, although it is sometimes difficult to distinguish an adenoma from a normal gland. Approximately 15% of cases are due to hyperplasia of ≥ 2 glands.
- Hypercalcemia – MSD Manual Professional Edition (Etiológia 1)
Parathyroid cancer occurs in < 1% of cases.
- Hyperparathyroidism – MSD Manual Professional Edition (Etiológia 2)
Secondary hyperparathyroidism occurs most commonly in advanced chronic kidney disease when decreased formation of active vitamin D in the kidneys and other factors lead to hypocalcemia and chronic stimulation of PTH secretion.
- Hyperparathyroidism – MSD Manual Professional Edition (Etiológia 3)
Tertiary hyperparathyroidism results when PTH secretion becomes autonomous of serum calcium concentration. Tertiary hyperparathyroidism generally occurs in patients with long-standing secondary hyperparathyroidism, as in patients with end-stage renal disease of several years’ duration.
- Hypoparathyroidism – MSD Manual Professional Edition (Etiológia 4)
Hypoparathyroidism results from a parathyroid hormone (PTH) deficiency, which can be due to: Removal of or damage to several parathyroid glands during thyroidectomy or parathyroidectomy Inherited or autoimmune disorders
- Hyperparathyroidism – MSD Manual Professional Edition (Klinika 1)
Patients most frequently present with asymptomatic hypercalcemia. Nephrolithiasis is also common, particularly when hypercalciuria occurs due to long-standing hypercalcemia.
- Hyperparathyroidism – MSD Manual Professional Edition (Klinika 2)
Symptoms, when they occur, are due to hypercalcemia and include chronic fatigue, constipation, anorexia, nausea and vomiting, poor concentration, confusion, and depression.
- Hyperparathyroidism – MSD Manual Professional Edition (Klinika 3)
Excess parathyroid hormone stimulates osteoclastic activity, which over time can cause osteitis fibrosa cystica. In osteitis fibrosa cystica, increased osteoclastic activity causes rarefaction of bone with fibrous degeneration and formation of cysts and fibrous nodules.
- Hypoparathyroidism – MSD Manual Professional Edition (Klinika 4a)
Symptoms of hypoparathyroidism are due to hypocalcemia and include tingling in the hands or around the mouth and muscle cramps. In severe cases, tetany occurs.
- Hypocalcemia – MSD Manual Professional Edition (Klinika 4b)
Chvostek and Trousseau signs are easily elicited at the bedside to identify latent tetany.
- Hyperparathyroidism – MSD Manual Professional Edition (Diagnostika 1a)
Patients with primary hyperparathyroidism have an abnormally high PTH concentration in the absence of hypocalcemia. A minority of patients have a high-normal PTH concentration, which should be interpreted as inappropriate when the calcium concentration is elevated
- Hyperparathyroidism – MSD Manual Professional Edition (Diagnostika 1b)
Low serum phosphate concentration suggests hyperparathyroidism, especially when coupled with elevated renal excretion of phosphate.
- Hyperparathyroidism – MSD Manual Professional Edition (Diagnostika 2)
FHH can present with hypercalcemia and normal or mildly elevated parathyroid hormone but is differentiated from primary hyperparathyroidism by the presence of low urinary calcium.
- Hyperparathyroidism – MSD Manual Professional Edition (Diagnostika 3a)
Imaging studies of the parathyroid(s) may be done preoperatively to localize an abnormal gland; imaging is not done to establish the diagnosis or determine whether surgery is needed.
- Hyperparathyroidism – MSD Manual Professional Edition (Diagnostika 3b)
Contrast-enhanced 4-dimensional (4D) CT MRI High-resolution ultrasound Digital subtraction angiography Technetium-99 sestamibi scanning
- Hypoparathyroidism – MSD Manual Professional Edition (Diagnostika 4a)
Low or even low-normal PTH concentration in patients with hypocalcemia is inappropriate and suggests hypoparathyroidism.
- Hypoparathyroidism – MSD Manual Professional Edition (Diagnostika 4b)
Hypoparathyroidism is further characterized by high serum phosphate and normal alkaline phosphatase.
- Hyperparathyroidism – MSD Manual Professional Edition (Liečba 1)
In patients with symptomatic or progressive hyperparathyroidism, surgery is indicated.
- Hyperparathyroidism – MSD Manual Professional Edition (Liečba 2a)
Many experts recommend surgery in the following circumstances
- Hyperparathyroidism – MSD Manual Professional Edition (Liečba 2b)
Serum calcium 1 mg/dL (0.25 mmol/L) greater than the upper limits of normal Hypercalciuria (> 250 mg/day [> 6.25 mmol/day] in females or > 300 mg/day [>7.5 mmol/day] in males) or the presence of nephrolithiasis
- Hyperparathyroidism – MSD Manual Professional Edition (Liečba 2c)
Creatinine clearance < 60 mL/minute (< 1 mL/second) Vertebral bone fracture or bone density at the hip, lumbar spine, or radius that is 2.5 standard deviations below controls (T score = −2.5) Age < 50 years
- Hyperparathyroidism – MSD Manual Professional Edition (Liečba 3a)
Conservative management with methods to ensure that serum calcium concentrations remain low or in the normal range may be used for patients who have asymptomatic primary hyperparathyroidism who are at high risk for surgery due to unrelated medical problems.
- Hyperparathyroidism – MSD Manual Professional Edition (Liečba 3b)
Patients with mild hypercalcemia alone can be managed with cinacalcet.
- Hypoparathyroidism – MSD Manual Professional Edition (Liečba 4a)
Hypoparathyroidism that does not respond adequately to calcium and vitamin D supplementation may require treatment with recombinant parathyroid hormone (rhPTH)
- Yorvipath – European Medicines Agency (EPAR) (Liečba 4c)
Yorvipath is a parathyroid hormone (PTH) replacement therapy indicated for the treatment of adults with chronic hypoparathyroidism
- Yorvipath – European Medicines Agency (EPAR) (Liečba 4c)
Yorvipath contains the active substance palopegteriparatide.
- Hypocalcemia – MSD Manual Professional Edition (Liečba 4b)
For tetany, calcium gluconate 10 mL of 10% solution IV over 10 minutes is given.
- Hyperparathyroidism – MSD Manual Professional Edition (Komplikácie 1)
Hypercalcemia frequently causes hypercalciuria with resultant nephrolithiasis, and patients with hypercalcemia can present with pain due to passage of a kidney stone.
- Hyperparathyroidism – MSD Manual Professional Edition (Komplikácie 2)
Primary hyperparathyroidism causes hypercalcemia, hypophosphatemia, and excessive bone resorption (leading to osteoporosis).
- Hyperparathyroidism – MSD Manual Professional Edition (Komplikácie 3a)
Serum calcium must be monitored several times a day in the immediate 48 to 72 hours postoperatively.
- Hyperparathyroidism – MSD Manual Professional Edition (Komplikácie 3b)
In patients with more severe or prolonged hyperparathyroidism, particularly secondary hyperparathyroidism with severe osteitis fibrosa cystica, prolonged, symptomatic hypocalcemia may occur postoperatively.
- Hypocalcemia – MSD Manual Professional Edition (Komplikácie 4)
Severe hypocalcemia with serum calcium < 7 mg/dL (< 1.75 mmol/L) may cause hyperreflexia, tetany, laryngospasm, or generalized seizures.
Precvičiť tému (6)
Kartičky
Vymenujte indikácie paratyreoidektómie pri asymptomatickej primárnej hyperparatyreóze.
Najprv si odpoveď povedz nahlas alebo v duchu – až potom ju odkry.
Aké sú príčiny, laboratórny obraz a liečba hypoparatyreózy?
Najprv si odpoveď povedz nahlas alebo v duchu – až potom ju odkry.