El Khatib, "Hyperparathyroidism-jaw tumour syndrome detected by aggressive generalized osteitis fibrosa
cystica," Clinical Cases in Mineral and Bone Metabolism, vol.
cystica: the case of a nine year old girl who also exhibitis precocious puberty, multiple pigmentation of the skin and hyper thyroidism.
En el ano 1991 Rothmund publico un estudio que concluyo que la paratiroidectomia total con autoimplante, es la mejor opcion, para estos pacientes, cuyas indicaciones mas especificas son: progresion acusada de la osteitis fibrosa
a pesar de un adecuado tratamiento medico, hipercalcemia persistente asociada con otros sintomas como hipertension severa hiperfosforemia intensa, junto a evidencia histologica y radiologica de hiperparatiroidismo, prurito intenso intratable asociado con hiperparatiroidismo significativo, calcificaciones de tejidos blandos graves y persistentes asociadas a hiperfosforemia y evidencia de hiperparatiroidismo, necrosis cutanea y diseminada idiomatica, osteopenia de vertebras, marcada deformidad esqueletica y dolor oseo intenso (9,10).
cystica: the case of a nine year old girl who also exhibits precocious puberty, multiple pigmentation of the skin and hypert-hyroidism.
In early renal failure a shortfall of Calcitriol synthesis is an important factor; as renal failure progresses, the lack of Calcitriol become more pronounced as suggested by Lorenzo, who reported that because of classic sequence of events in renal failure that begins with a deficit of Calcitriol synthesis and retention of phosphorus results in serum calcium decreases and compensatory hyperplasia of the parathyroid gland result in increase parathyroid hormone, causing high turnover bone disease known as osteitis fibrosa
Recovery pattern of patients with osteitis fibrosa
cystica in primary hyperparathyroidism after successful parathyroidectomy.
Syndrome characterized by osteitis fibrosa
disseminate, areas of pigmentation and endocrine dysfunction with precocious puberty in females.
At the time he had no idea of the aetiology of this condition, which he named osteitis fibrosa
cystica is a markedly rare disease, present in only 2% of individuals diagnosed with primary hyperparathyroidism, which accounts for 90% of instances of the disease.
'Groans' refers to gastrointestinal symptoms of constipation, abdominal pain, indigestion and vomiting, whilst'bones' refers to complications of the bones in the form of osteitis fibrosa
cystica, osteopenia, osteoporosis and pathological fractures.
Table 3 KDIGO[R] Classification of Renal Osteodystrophy/Renal Bone Disease Renal Osteodystrophy Bone Bone Disorder Turnover Mineralization Osteomalacia Low Abnormal Adynamic bone disease Low Normal Mild hyperparathyroid- Medium Normal related bone disease and to high osteitis fibrosa
Mixed uremic High Abnormal osteodystrophy Renal Osteodystrophy Bone Disorder Volume Osteomalacia Low to medium Adynamic bone disease Low to normal Mild hyperparathyroid- Variable, depending related bone disease and on the duration of osteitis fibrosa
the disease Mixed uremic Normal osteodystrophy Source: Adapted from Moe et al.
In the differential diagnosis chondromyxoid fibroma and osteitis fibrosa
cystica were considered (Figs 1 a & b).