[Facial edema as an earlier presenting sign of giant cell arteritis. Possible relationship with angioedema]. / Gesichtsödem als Frühsymptom einer Riesenzellarteriitis. Mögliche Beziehung zum Angioödem.

Giant cell arteritis (GCA) is a chronic granulomatous vasculitis of unknown etiology occurring in the elderly. New-onset headache, scalp tenderness, jaw claudication, temporal artery abnormalities on physical examination, visual symptoms and associated polymyalgia rheumatica represent the most typical and frequent features of the disease. However, facial edema is being more commonly recognized as a presenting symptom that may herald the disease. We present a case with facial edema as initial symptom and discuss if this rare symptom of GCA is due to hereditary or acquired angioedema.

Acute renal failure due to leukaemic infiltration in chronic lymphocytic leukaemia: case report.

A 73-year-old woman was presented with altered mental status and disorientation. She was diabetic and hypertensive, and she had experienced an ischemic cerebrovascular accident 3 years ago. Physical examination revealed the findings of chronic obstructive pulmonary disease, cor pulmonale and congestive heart failure. Hepatomegaly, splenomegaly and ascites were found and might be associated with postsinusoidal portal hypertension secondary to congestive heart failure. Laboratory tests showed uremia, lymphocytosis and thrombocytopenia. Neurologic findings were related with uremia and hypoxia. Multiple pathologic lymphadenopathies were seen in abdominal ultrasonography and thoracic computed tomography. Bone marrow histology indicated chronic lymphocytic leukaemia (CLL). The reason for acute renal failure was leukaemic infiltration of the kidneys due to CLL that was shown with renal biopsy. Blood urea nitrogen (BUN) and serum creatinine responded well to cyclophosphamide and methyl prednisolone treatment. In CLL, direct renal involvement is frequently seen in autopsy studies especially in advanced disease, however, renal failure due to leukaemic infiltration is extremely rare.

Osteodystrophy in posthepatitic cirrhosis.

This study investigated the incidence and severity of hepatic osteodystrophy in patients with posthepatitic liver cirrhosis, and the role of hepatocellular injury in bone loss. Twenty-four patients (15 females and 9 males, mean age 49 +/- 13 years) with posthepatitic cirrhosis were enrolled in this study. The control group consisted of 22 healthy age and sex matched adults. The bone mineral density (BMD) was evaluated by dual energy x-ray absorptiometry of the L1-L4 vertebral bodies. A detailed questionnaire was used to assess the epidemiological findings. A statistically significant decrease in BMD of the patients was observed. There were no significant differences in the alkaline phosphatase, parathyroid hormone, calcitonin, 25-hydroxyvitamin D, osteocalcin, free testosterone, luteinizing hormone, follicle stimulating hormone, and estradiol levels, oral calcium intake, urinary calcium, phosphorus and hydroxypyroline excretion between patients and controls. The control group smoked more cigarettes, consumed more coffee and meat, and were exposed the sun light for a longer period than the study group. Multiple regression analysis showed that osteopenia depends significantly on the extent of liver disease. The data shows that the patients with posthepatitic cirrhosis had osteopenia, and that cirrhosis was a direct and independent risk factor.

A case of paroxysmal nocturnal hemoglobinuria presenting with intra-abdominal bleeding due to splenic rupture, developing renal infarct.

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare disorder characterized by intravascular hemolysis, hemoglobinuria, and thrombosis. Thrombotic attacks are life threatening and are responsible for nearly 50% of PNH-related deaths. Compared with thrombotic events, bleeding related to thrombocytopenia in PNH is quite rare. This report describes an atypical clinical presentation with problems in the diagnosis and management of a woman who presented with a splenic infarct followed by massive intra-abdominal bleeding due to splenic rupture. She also developed a renal infarct during hospitalization after diagnosis.

Technetium-99m 2-methoxy-isobutyl-isonitrile uptake scintigraphy in detection of the bone marrow infiltration in multiple myeloma: correlation with MRI and other prognostic factors.

Whole-body scintigraphy with Technetium-99m 2-methoxy-isobutyl-isonitrile ((99m)Tc-MIBI) has been proposed as a useful method for demonstrating the areas of active bone marrow infiltration in multiple myeloma (MM). In this study, we compared the (99m)Tc-MIBI scan with magnetic resonance imaging (MRI), skeletal X-ray survey, and biochemical markers of disease activity in MM to determine its potential in predicting the extension of the disease. Twenty-four myeloma patients had undergone to the (99m)Tc-MIBI scan. Only two patients showed negative results in the (99m)Tc-MIBI scan; one had clinically active disease, and the other was on remission. MRI was performed to 18 clinically active patients, and 16 of them showed positive myelomatous bone marrow involvement. No significant difference was found between the (99m)Tc-MIBI scan and MRI in predicting the extension of bone marrow infiltration in MM (p = 0.11). (99m)Tc-MIBI scores were correlated with bone marrow neoplastic plasma cell ratio (p = 0.005), serum paraprotein level (p < 0.001), serum lactate dehydrogenase (p = 0.031), and beta-2 microglobulin (p = 0.045). The (99m)Tc-MIBI scan showed disease activity better than the skeletal X-ray survey (x2 = 5.299, p = 0.021). A significant decrease was found in posttreatment (99m)Tc-MIBI scores of the patients with positive overall response (p = 0.016). The (99m)Tc-MIBI scan is a noninvasive test that can show the extension of the disease in MM. It seems that the (99m)Tc-MIBI scan and MRI show extension and intensity of the myelomatous bone marrow infiltration equally well. The (99m)Tc-MIBI scan can be an alternative to MRI when it is not available or if there is any limitations for its usage.

Fever of unknown origin: a report of 57 cases.

Fever of unknown origin (FUO) is defined as the body temperature higher than 38.3 degrees C on several occasions and lasting longer than 3 weeks, with the aetiology remaining uncertain after 1 week of investigation. In this study, we reviewed 57 patients with FUO hospitalised in our inpatient department between 1998 and 2003. The median age of the patients was 44 years (17-84), and 26 were males (46%), while 31 were females (54%). In 24 patients (42%), infectious diseases such as tuberculosis (12 patients) and brucellosis (5 patients) were found under the aetiology of FUO. Inflammatory rheumatic diseases were the second most common cause of FUO (17 patients, 30%), while malignancies were found in 10 patients (18%). In six patients (10%), aetiology could not be identified. In conclusion, infectious diseases especially tuberculosis and brucellosis are the leading causes of FUO in our country.

Bilateral renal artery stenosis after abdominal radiotherapy for Hodgkin's disease.

A 49-year-old woman was admitted with fatigue, dyspnoea, pretibial oedema and decreased daily urination. Seven years ago she was treated with doxorubicin, bleomycin, vinblastine and dacarbazine, alternating with mechlorethamine, vincristine, procarbazine and prednisone and 80 Gy abdominal radiotherapy for Hodgkin's disease. Two years later, malignant hypertension was diagnosed. Angiotensin-2 antagonist and beta-blocker treatment was given. After increased serum creatinine levels were determined, renal angiography was performed and total obstruction in the left renal artery and near total obstruction in the right side was observed. She was admitted to our clinic with oliguria, and acute renal failure was diagnosed. Balloon angioplasty and stent implantation was performed to the right renal artery. After a polyuric period, serum creatinine reduced to near normal levels. Angiotensin-2 antagonist treatment worsened the course in this patient. Patients with resistant hypertension occurring years after abdominal radiotherapy should be evaluated for renal artery stenosis.

A case of Noonan syndrome with pulmonary and abdominal lymphangiectasia.

Noonan syndrome is characterised by a Turner-like phenotype and a normal karyotype. Although it is reported to be associated with abnormalities of the lymphatic system, involvement of the pulmonary lymphatics is rare. We present a case of Noonan syndrome where a whole body scintigraphy revealed lymphangiectasia of the lower extremities, abdomen and lungs.