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Albright's hereditary osteodystrophy (AHO) refers to the syndromes of pseudohypoparathyroidism (PHP) and pseudopseudohypoparathyroidism (PPHP). Both PHP and PPHP have the same phenotype, but PHP patients have hypocalcemia and fail to respond to parathyroid hormone. The PPHP patients have normal serum calcium values. Cases have been observed to change, over a period of time, from PHP to PPHP. This case of PHP demonstrates intracutaneous lamellar bone formation. The patient with AHO is short with shortened metacarpals and metatarsals, round facies, short broad nails, and soft tissue calcifications. They may also have cataracts, basal ganglia calcification, mental retardation, defective teeth, and multiple other skeletal abnormalities. There may also be tetany or muscle cramps. The mode of inheritance is unclear and may be sex-linked dominant. Differential diagnosis includes hypoparathyroidism, basal cell nevus syndrome, Gardner's syndrome, and sex-chromatinnegative Turner's syndrome.

The diminished absorption of calcium and the osteodystrophy which accompany chronic renal disease are often "resistant" to vitamin D. The basis for this resistance remains uncertain. Recent studies have emphasized the role of an abnormality in vitamin D metabolism in accounting for the alterations in calcium absorption and the apparent vitamin D resistance which occur in renal failure. The results of experiments in this laboratory with a uremic animal model strongly suggest that in addition to the proposed mechanism involving an interference with the metabolism of vitamin D, renal insufficiency also affects the cellular reactions mediating calcium transport in a manner which, while opposite in direction to that of vitamin D, is independent of a direct interaction with the vitamin or its metabolites.

WOMEN OMEN who lack one X chromosome (45 XO) are sex chromatin negative and have gonadal dysgenesis, usually associated with short stature and sexual infantilism. Such patients often suffer many other anomalies, as has been documented in numerous clinical and cytogenetic investigations. These reports cite several individuals whose appearance mimics that of another unusual clinical syndrome, pseudopseudohypoparathyroidism (PPH), 1 a clinical entity unrelated to known parathyroid dysfunction, for which several alternative names have been proposed, including Albright's hereditary osteodystrophy 2 and brachymetacarpal dwarfism. 3 PPH denotes normocalcemic individuals with a set of anomalies, primarily skeletal, which are sufficiently distinct to permit clinical recognition. When the same anomalies occur in association with hypocalcemia, the term pseudohypoparathyroidism (PH) applies. The classification and possible relationship of PH, PPH, and gonadal dysgenesis are unclear, and further knowledge of etiological factors is needed to allow more precise understanding. During the course of cytogenetic investigations at

RECENTLY we had the opportunity to study an unusual case of chronic renal failure, associated with changes in bone suggestive of osteitis fibrosa cystica. The patient had true diabetes mellitus rather than renal glycosuria, which has been described in previous cases. Additional unusual findings at postmortem examination, including polycystic disease of the pancreas, prompted us to present this case with a review of the literature. The triad of chronic nephritis, bony changes in the skeleton and physical dwarfism has been designated as renal rickets. 1 This is actually a misnomer in that the bony changes are not those characteristic of rickets and it is not even necessary that rickets be associated with this syndrome, 2 although it is sometimes associated with rickets. 3 A better term would be renal hyperparathyroidism with osteoporosis, as designated by Park and Eliot, 4 renal osteitis fibrosa cystica (Albright and others 5 ) or osteodystrophy (Norman

Pseudohypoparathyroidism and pseudopseudohypoparathyroidism are now considered variants of the same disease and are referred to jointly as Albright's hereditary osteodystrophy. Cutaneous ossification may be a presenting feature in Albright's hereditary osteodystrophy. The second case of cutaneous ossification in Albright's hereditary osteodystrophy occurred in a 39-year-old woman.

Hyperphosphatemia in hemodialysis (HD) patients is considered to be the silent killer. Adherence to phosphorus restricted diets by HD patients is too low in the literature. This study aims to reveal the NIMO trial plan and the baseline characteristics of the study sample Adult stable patients (n=750) were selected from 12 hospital-based HD units. Baseline patient characteristics were assessed during the 6 months prior to study initiation. HD units were randomly assigned to the study clusters: Cluster 1 (6 units, 370 patients) was divided into full intervention (A) and control groups (B) (as per shifts); where the full intervention received medical nutritional therapy (MNT) for 6 months as 2 hours/patient/month, delivered by externally recruited dietitians, fully dedicated to the unit. The hospital dietitian (blinded to study protocol) continued providing dietetic services as per existing practice to all patients. As for cluster 2 (6 units, 380 patients): partial intervention (C), where the MNT was delivered by the hospital dietitian after HD-specific nutritional training. It was not possible to have a control in cluster 2 for ethical reasons. Dietitians delivering the study protocol in group A and C received the same training by study coordinator based on the nutritional guidelines of Kidney Disease Outcomes Quality Initiative and trans-theoretical model. Key results at baseline were: Nutritional knowledge (%) of Group A, B, C was 40.14±12.39, 38.2±12, 38.92±12.74 respectively. Serum P (mg/dl) of Group A, B, C was 5.58±1.53, 5.39±1.5, 5.17±1.44 respectively. The serum P of Group A was significantly higher than the other groups. Actual consumption of protein compared to needs (%/d) of Group A, B, C was 59, 64, 58 respectively. Frequency of hospital dietitian’s visits was