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1/3. Primary palpebral and orbital ossification in pseudo-pseudohypoparathyroidism.

    PURPOSE: To present a case of ossification of the eyelid, episclera and orbit in a patient with pseudo-pseudohypoparathyroidism (pPHP). methods: A 20-year-old woman diagnosed with pseudo-pseudohypoparathyroidism underwent clinical and histopathological examination of calcified plaques of the right eyelid and orbit. The patient presented with a round face, tousled short hair and retarded speech. She had been diagnosed with pPHP at 3 years of age. During her first decade, calcified plaques developed in the right eyelid and orbit. Gradually, she developed horizontal diplopia, pseudo-ptosis and periorbital pain. Vertical eye movements were reduced to 10 mm, although levator function remained intact. Computer tomography scans of the orbit showed three separate dense structures. Radiographic findings also showed bilateral shortening of the fourth metacarpus and a calcified subcutaneous plaque in the left thigh. The patient's blood status revealed an elevated level of thyroid stimulating hormone, but was otherwise normal. The patient was treated with eltroxin and shortly afterwards regained normal hair and normal speech function. RESULTS: The calcified structures were removed surgically and almost normal eye movements were re-established. Histological examination of the excised tissue demonstrated bone formation. CONCLUSION: This is the first reported case of ossification of the eyelid and orbit in a patient with pseudo-pseudohypoparathyroidism.
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2/3. Pseudohypoparathyroidism: a difficult diagnosis in early childhood.

    We have studied one adult and three children with pseudohypoparathyroidism and observed that the physical character of short metacarpal bones is not evident in the first 4-5 years of life, that hypocalcaemia and hyperphosphataemia may be absent in the first years of life, but that the renal unresponsiveness to parathyroid hormone can still be demonstrated. Our data confirm earlier observation that in evaluating the renal responsiveness to parathyroid hormone, urinary cyclic amp is a better parameter than urinary phosphorus. Thus in early childhood, it may be difficult to differentiate between a normal child, a child with pseudohypoparathyroidism and a child with pseudo-pseudohypoparathyroidism unless the renal parathyroid hormone responsiveness is studied.
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3/3. pseudopseudohypoparathyroidism associated with idiopathic growth hormone deficiency. role of treatment with biosynthetic growth hormone.

    A case is presented of a prepubertal girl with the characteristic somatic features of Albright's hereditary osteodystrophy, including severe short stature, cataracts and shortening of all metacarpals and metatarsals and of the second middle hand phalanges, whose diagnosis of pseudopseudohypoparathyroidism (PPHP) was confirmed by laboratory evaluation (normocalcemia, normophosphatemia, normal levels of circulating PTH and normal response to exogenous PTH). Since an isolated idiopathic GH deficiency has been diagnosed at the age of 9.7 yr, by an abnormal GH response to standard provocation tests, a poor spontaneous nocturnal GH secretion and a blunted response to GHRH test, our patient was treated with biosynthetic GH during a 3.5-year period. Although a good improvement of growth velocity was obtained when comparing pretreatment height velocity (4 cm/yr) with growth velocity evaluated during GH treatment (6.6, 6.2 and 5.9 cm/yr in the first, the second and the third year of therapy, respectively), bone age advanced more rapidly than chronological age, so that it is uncertain whether the growth acceleration promoted by GH administration really improved final height, which remained below the third centile. Our patient is the first described case of PPHP associated with idiopathic GH deficiency, and the second report of long-term GH treatment in a subject with PPHP. Further observations are necessary to define the frequency and significance of GH deficiency and the role of GH replacement therapy in pseudohypoparathyroidism- and PPHP-associated short stature.
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