Congenital insensitivity to pain and anhydrosis due to a rare mutation and that is complicated by inflammatory bowel disease and amyloidosis: a case report.

Patients with congenital insensitivity to pain and anhydrosis syndrome are at risk for renal amyloidosis and inflammatory bowel disease. Physicians caring for such patients should be aware of these complications.

A family with five siblings affected with nephronophthisis.

Nephronophthisis is an autosomal recessive disease that leads to end-stage renal disease. These days, molecular genetic analysis is used pre-emptively for making a definitive diagnosis in patients who have clinical and radiological data suggestive of the disease. Herein, we are reporting a 12-year-old girl who was genetically diagnosed to have juvenile nephronophthisis, which explained the mystery of the chronic kidney disease in her four affected siblings.

Benign pheochromocytoma presented 6 years after kidney transplantation.

Hypertension is very common in kidney transplant patients; however, severe and resistant cases should raise suspicion of secondary causes. Pheochromocytomas are rare but serious tumors because of their lethal hypertensive and possible malignant nature. The diagnosis is occasionally elusive, but prompt diagnosis and localization is essential for definitive surgical management. We report a case of a patient with benign pheochromocytoma presenting largely asymptomatically, but with severe resistant hypertension, 6 years after kidney transplantation. To the best of our knowledge, this is the first case report of this type of tumor after kidney transplantation.

Spectrum of glomerulonephritis in adult Jordanians at Jordan university hospital.

We retrospectively reviewed the records and histopathological findings of 64 adequate native kidney biopsies performed at the Jordan University Hospital from January 2002 through December 2006. The nephrotic syndrome (NS) was the main reason for biopsy in 51.6% of the cases and deterioration of kidney function in 31%. Primary glomerulonephritis (GN) was diagnosed in 59.4% of the biopsies, and focal segmental glomerulosclerosis (FSGS) was the most common pathology detected (17.2%). Systemic lupus erythematosis was found in 17 patients (26.6%), and it was the commonest secondary GN pathology.

Brucella glomerulonephritis resulting in end-stage renal disease: a case report and a brief review of the literature.

Brucella glomerulonephritis is a rare condition with only a few reported cases. We review the literature, and describe a 24-year-old female who presented with edema and proteinuria. Blood grew Brucella melitensis. Renal biopsy showed diffuse proliferative glomerulonephritis. The patient progressed to end-stage renal disease despite antibiotic and steroid therapy.

The spectrum of bone disease in Jordanian hemodialysis patients.

OBJECTIVE: To evaluate the spectrum of mineral abnormalities and bone disease (BD) in hemodialysis patients at Jordan University Hospital (JUH), Amman, Jordan. METHODS: A cross-sectional study was conducted among 63 patients (38 males and 25 females), mean age 44.19 years (range 17-76 years), with chronic kidney disease (CKD) on regular hemodialysis at JUH between November 2004 and April 2005. All patients have undergone complete blood count, chemistry profile, alkaline phosphatase, serum albumin, intact parathyroid hormone (iPTH) and plain x-rays. RESULTS: Bone disorders were identified in 45 patients on x-rays (70%). Osteopenia was found in 43 patients (68.3%), subperiosteal resorption in 24 patients (38.3%) and metastatic calcification in 22 patients (35%). Hypocalcemia was found in 28.6% and hypercalcemia in 7.9%. All patients were taking calcium carbonate, and 55.5% of patients were on vitamin D supplements. The calcium levels in 63.5% and the phosphorus levels in 50.8% of patients were within the recommended guidelines of the National Kidney Foundation Kidney Disease Outcomes Quality Initiative (K/DOQI). Serum i-PTH level was above 300 pg/ml high turnover bone disease in 24.6% of patients, 21.3% had iPTH of 150-300 pg/ml target, and 44.3% had i-PTH levels below 100 pg/mL suggesting a dynamic bone disease. Patients with severe bone disease had a statistically significant higher iPTH levels (p<0.005). CONCLUSION: Bone disease and mineral abnormalities are common in hemodialysis patients at JUH. Earlier detection of bone disease and better overall management strategy may reduce the frequency and severity of bone disease in CKD patients in Jordan.