Evaluation of impact of steroid replacement treatment on bone health in children with 21-hydroxylase deficiency.

There are conflicting data regarding the potential impact of chronic glucocorticoid (GC) therapy on the bone mineral density of patients with congenital adrenal hyperplasia (CAH). Previous studies performed by dual-energy X-ray absorptiometry reported conflicting results. The purpose of this study was to assess the impact of chronic GC replacement treatment in children with classical and non classical CAH due to 21-hydroxylase deficiency (21-OHD) by quantitative ultrasonometry (QUS), an easy, cheap, and radiation-free technique. The study population consisted of nineteen 21-OHD patients (nine males) on lifelong GC treatment. Anthropometric, hormonal, and treatment data were recorded for each patient, and bone quality was assessed by QUS measurements. QUS findings (amplitude-dependent speed of sound and bone transmission time) were normal in 21-OHD patients and did not correlate with duration of treatment, daily, total, and yearly hydrocortisone dose. Furthermore, no significant correlation was found between QUS findings and 17α-hydroxy progesterone, Δ4-androstenedione, and testosterone levels. In conclusion, our results provide reassurance that currently used replacement doses of GC do not have a major impact on bone in patients with CAH. QUS seems to be a reliable tool for screening of bone health in children with 21-OHD.

Recurrent hemorrhage from an invaginated Meckel's diverticulum in a 78-year-old man.

Complications arising from Meckel's diverticulum are uncommon in adults and are seldom, if ever, seen in the elderly. When they do occur in adults, intestinal obstruction or inflammation is the usual mode of presentation, hemorrhage being much less common. The patient described in this case report was 78 yr old, presented initially with iron deficiency anemia and, later, developed severe acute hemorrhage. The cause of the hemorrhage was ulceration at the tip of an invaginated Meckel's diverticulum. The ulceration was not peptic in origin, as is usually the case in similar presentations in children, no ectopic oxyntic mucosa being detected in the diverticulum of our patient. In previous reports, invaginated Meckel's diverticula have always been accompanied by intussusception, and abdominal pain has been an important part of the symptom complex in such patients. Our patient had no abdominal pain, and no intussusception was noted at surgery. This case emphasizes the need for considering a Meckel's diverticulum as the source of acute or chronic hemorrhage, irrespective of the patient's age. The utility of radionuclide blood pool imaging in arriving at a diagnosis in these cases is discussed.

Dialysis-related amyloidosis during peritoneal dialysis.

Patients treated with long-term hemodialysis may develop carpal tunnel syndrome, cystic bone lesions, and/or an arthropathy. This syndrome has been called hemodialysis-related amyloidosis (HRA) and appears to be the result of the accumulation of polymerized Beta-2 microglobulin (B2M). The risk to patients treated with peritoneal dialysis is unknown. Diphosphonate scans have been advocated as a useful noninvasive test for HRA. The authors report a well-documented case of HRA established during hemodialysis and progressing during peritoneal dialysis. In addition, this patient's HRA was not identified by a diphosphonate scan. This is the second well-documented case of HRA associated with peritoneal dialysis.