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Dysphagia is described as a geriatric syndrome that occurs more frequently with aging. It is associated with the deterioration in functionality however, it is usually ignored. Frailty is a geriatric syndrome that is recognized more with its well-known adverse consequences. Very recently, dysphagia has been suggested to accompany frailty in older adults. We aimed to investigate the association between dysphagia and frailty in the community dwelling older adults.Prospective, cross-sectional study.Geriatric outpatient clinic.Older adults aged ≥60 years.Dysphagia was evaluated by EAT-10 questionnaire and frailty by FRAIL scale. Handgrip strength (HGS) was evaluated by hand-dynamometer. Gait speed was evaluated by 4-meter usual gait speed (UGS). Nutritional status was assessed by mini-nutritional assessment-short form (MNA-SF).1138 patients were enrolled. Mean age was 74.1±7.3 years. EAT-10 questionnaire was answered by all and FRAIL-scale by 851 subjects. EAT-10 score15 points was regarded as significant dysphagia risk. The participants with EAT-1015 points were older when compared to the participants with EAT-10=15 points (p=0.002). Among participants with EAT-1015 points, women gender and neurodegenerative diseases were more prevalent (p=0.04, p=0.002; respectively); number of chronic diseases, number of drugs and FRAIL score were higher (p=0.001 for each), and HGS, UGS, MNA-SF scores were lower (p=0.002, p=0.01, p0.001; respectively). In multivariate analyses, the factors independently associated with presence of EAT-10 score15 were FRAIL score and the number of drugs.Dysphagia is associated with frailty irrespective to age, presence of neurodegenerative diseases, number of chronic diseases and drugs. To our knowledge, this is the largest serie in the literature providing data on independent association of dysphagia with frailty.

Intravascular large B-cell lymphoma (IVLBCL) is a very rare subtype of extranodal large B-cell lymphoma. It may involve various organ systems such as skin, liver, lung or kidney. Isolated kidney involvement of IVLBCL is also very rare. Herein we report a very rare case of isolated renal IVLBCL presented with fever of unknown origin, acute kidney injury and nephrotic syndrome. Diagnosis was suspected with isolated high renal (18)F fluorodeoxyglucose uptake in positron emission tomography and confirmed with renal biopsy. Complete remission was obtained with combined chemotherapy including rituximab. We reviewed the English literature in terms of IVLBCL with renal involvement and we could only find 16 such cases. Accordingly, fever, AKI and nephritic syndrome are the most common presenting symptoms in renal intravascular lymphoma.

Ectopic secretion of growth hormone-releasing-hormone (GHRH) is a rare cause of acromegaly-representing less than 1% of patients. A 25-year-old woman was admitted to the hospital with acromegaly and a 6 x 6 cm infrahepatic mass. Sellar magnetic resonance imaging indicated diffuse pituitary enlargement consistent with hyperplasia. The infrahepatic mass was resected, and the histopathological diagnosis was a well-differentiated invasive neuroendocrine carcinoma of the duodenum with metastases to local lymph nodes. The tumor cells contained cytoplasmic immunoreactivity for GHRH. Because increased IGF-1 concentrations persisted after the operation, the patient was treated with octreotide long-acting repeatable (LAR) injections of 20 mg/month. Growth hormone and IGF-1 levels normalized. After 6 years of surveillance, a left paraaortic mass was detected by uptake of indium 111 octreotide. Surgical exploration revealed metastatic neuroendocrine carcinoma in a 2.5-cm lymph node. Postoperatively, the IGF-1 concentration was mildly elevated. Octreotide LAR therapy is being continued at 10 mg/month. This case suggests that octreotide treatment may have a beneficial effect on disease course and can be maintained for as long as 7 years in a patient with acromegaly due to a GHRH-secreting neuroendocrine carcinoma.

There are few reports concerning Mycobacterium tilburgii infection in humans because this bacterium is non-cultivatable. Herein, using new molecular techniques, we report the case of an immunocompromised patient with fatal disseminated lymphadenitis that was caused by M. tilburgii.26 years old Caucasian HIV negative female patient presented with abdominal pain. Her clinical assessment revealed disseminated lymphadenitis, that was acid fast bacilli positive. Further molecular evaluation showed the causative agent as M. tilburgii. Despite anti mycobacterial therapy and careful management of intervening complications patient died because of an intraabdominal sepsis. This is the first fatal M. tilburgii infection in the literature. This case points the importance of careful management of patient's immune status and intervening infections besides implementation of effective drug treatment.

The object of this study was to investigate the genotoxic effect of acute overexposure to combustion products originating from coal or wood stoves in patients presenting with acute carbon monoxide intoxication.In a prospective study, we analyzed the frequency of sister chromatid exchange and the carboxyhemoglobin concentration in 20 consecutive patients without a history of smoking or drug use who had been treated in the Emergency Care Unit of Istanbul Medical Faculty due to acute carbon monoxide intoxication. All of these cases were domestic accidents due to dysfunctioning coal or wood stoves. The results were compared with a control group of 20 nonsmoking, nondrug-using healthy individuals matched for age, sex, and absence of other chemical exposure.The mean sister chromatid exchange frequency per metaphase was significantly higher in the study group compared to the control group: 8.11 +/- 2.39 vs. 6.33 +/- 1.60 (p = 0.008). We found that there was no positive correlation between the blood carboxyhemoglobin concentration and sister chromatid exchange frequency.These results suggest that acute exposure to combustion products of wood or coal is genotoxic to DNA. Potential causes of genotoxicity include known mutagenic compounds present in coal or wood smoke and ash, oxygen radicals formed during combustion, as well as hypoxic and reperfusion injury mechanisms initiated by carbon monoxide intoxication. Additional studies on separate carbon monoxide exposure from smoke and ash are needed to understand individual genotoxic contributions and mechanisms.

Priapism is a rare symptom with diverse etiological factors. Although most cases in adults are secondary to drug use and intracavernosal injections, blood dyscrasias and hypercoagulable states, vasculitis, penile metastases, neurological conditions, spider bites, carbon monoxide poisoning, and total parenteral nutrition may also result in priapism. We report a case of recurrent and refractory priapism in a 61-year-old man which was diagnosed as multiple myeloma after emergence of hypercalcemia and renal failure due to progression of the underlying pathology. The value of the initial diagnostic approach is emphasized.

We analyzed the induction of sister chromatid exchange (SCE) by cyclosporin A (CsA) as a marker of genotoxic potential. In 30 patients undergoing renal transplantation, SCE induction was tested before the introduction of CsA and 3 months later. We found that SCE frequency increased significantly at the end of 3 months. To our knowledge, this is the first study demonstrating in vivo induction of SCE by CsA in humans. We conclude that CsA has a genotoxic potential on human lymphocytes.

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.

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.

Systemic lupus erythematosus (SLE) is a multisystemic disease which potentially involves various organs including the skin, joints, kidneys, liver, hematopoetic system, and serous membranes. It is rarely seen in elderly males. The most common cardiovascular involvement type is pericarditis. Anti-Ro antibodies may be associated with neonatal lupus which causes heart blocks. Recent literature indicates that anti-Ro antibodies may be associated with various rhythm and conduction disturbances in the adulthood. The most common finding associated with anti-Ro antibodies is prolonged corrected QT (QTc) interval. Herein, we present an elderly male patient with anti-Ro-positive SLE associated with prolonged QTc interval and AV blocks that significantly improved after corticosteroid treatment.

We investigated the clinical and laboratory findings of hypogonadism and feminization in male patients with viral or alcoholic cirrhosis to determine whether chronic liver disease plays a primary role in the development of sexual dysfunction and hormonal changes. Two groups of male patients with liver cirrhosis (23 alcoholic, 33 viral) age- and Child's grade-matched, and 20 age-matched healthy men, as a control group, were included in this study. Clinical signs of hypogonadism and feminization were examined in the cirrhotic patients. Follicle-stimulating hormone, luteinizing hormone, prolactin, testosterone, free testosterone, estradiol, androstenedione, dehydroepiandrosterone sulfate, and sex hormone-binding globulin were estimated in all groups. Seminal fluid was also analyzed in 7 alcoholic and 15 viral cirrhotics. Serum levels of estradiol, androstenedione, and sex hormone-binding globulin were significantly higher, and free testosterone and dehydroepiandrosterone sulfate levels were significantly lower in both groups of cirrhotics compared with the control group. Child's C patients in both groups of cirrhotics were found to have higher estradiol and lower free testosterone levels than child's A and B patients. Alcoholic and viral cirrhotics had markedly reduced sperm motility and density. The differences between alcoholic and viral cirrhotic patients in the clinical signs of hypogonadism, serum levels of sex steroids, and the results of seminal fluid analysis were not statistically significant. These findings suggest that liver cirrhosis per se, independent of etiology, causes hypogonadism and feminization, and that the degree of hypogonadism and feminization correlates well with the severity of liver failure.

Nitric oxide (NO) has an important role in the inflammatory arthropathies. This study investigated NO levels in the synovial fluid and plasma of patients with primary osteoarthritis (OA) of the knee. Twenty-seven cases with primary knee OA and 13 controls were recruited for the study. Nitrate/nitrite levels of synovial fluid and plasma were measured by Griess reaction, and interleukin-1 beta (IL-1 beta) levels were measured quantitatively by a sandwich immunoassay technique. We found a significant increase in the synovial fluid nitrate/nitrite levels in cases with primary OA of the knee compared to controls (50.26+/-23.63 microg/l vs 32.49+/-10.05 microg/l, p=0.002) as well as increased plasma nitrate/nitrite levels (57.06+/-23.32 microg/l vs 39.98+/-16.36 microg/l, p=0.012). There was no difference in plasma and synovial fluid IL-1 beta concentrations between the study and control groups. These results may be considered as supporting evidence that NO might be one of the factors responsible for cartilage destruction in primary osteoarthritis of the knee.

We present a young woman with extrapulmonary sarcoidosis who had been treated for tuberculosis 15 years earlier. The initial diagnosis had been made on the basis of liver biopsy. The patient later developed bilateral visual loss secondary to uveitis. We identified noncaseating granulomatous lesions in the kidney, intra-abdominal lymph node, and previous liver biopsy specimens. Although rare, renal sarcoidosis can manifest itself as hypercalcemic nephropathy, granulomatous interstitial nephritis, renal tubular dysfunction, or glomerulonephritis. Corticosteroids are the treatment of choice.

Mucosa associated lymphoid tissue MALT lymphoma is a low grade malignancy that arises most commonly from the gastric mucosa. Small intestinal involvement is very rare. The causative relationship between Helicobacter pylori and the gastric MALT lymphoma is a well known issue, but recently there are several data suggesting the role of hepatitis C virus (HCV) infection in the pathogenesis of lymphoma including MALT lymphoma. Herein we present a rare case of duodenal MALT lymphoma with multiple intra-abdominal thromboses together with HCV infection that was confirmed by real-time polymerase chain reaction detecting HCV-RNA within the peripheral blood mononuclear cells.

Thrombotic thrombocytopenic purpura is a thrombotic microangiopathic disease. Associations with collagen vascular diseases, pregnancy, some drugs, surgical intervention, and infections are documented (known). Malignancy is also one of the known factors associated with thrombotic thrombocytopenic purpura. These malignancies are usually the disseminated solid organ tumors. Hematological malignancies constitute a rare association. Here, we present a patient with thrombotic thrombocytopenic purpura associated with multiple myeloma and discuss the pathogenesis. To our knowledge, this is the first case report of thrombotic thrombocytopenic purpura associated with multiple myeloma.

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.

Procalcitonin (PCT) represents a new marker of systemic inflammatory reactions to bacterial infections. The main aim in this study was to determine the diagnostic value of PCT in predicting the clinical severity of febril neutropenic attacks, compare it with that of C-reactive protein (CRP), and clarify its importance in culture-positive attacks.Between February 2001 and April 2002, 36 patients who were neutropenic due to various hematologic disorders and febrile were entered into the study. Blood samples were obtained on the first day of fever for the measurement of serum PCT and CRP levels.In clinically severe neutropenic fever attacks, means of serum PCT and CRP levels were measured as 0.93+/-1.33 ng/mL and 67+/-24 mg/L, while they were 0.37+/-0.23 ng/mL and 32+/-19 mg/L in clinically mild ones (p = 0.033 and p0.001). On the other hand, no statistical significance was found between culture-positive and negative attacks when either serum PCT or CRP levels were taken into consideration (p = 0.133 and p = 0.141). The specificity and positive predictive value of the serum PCT test for severe febrile neutropenia was higher than that of the serum CRP test (0.80 vs. 0.57 and 0.50 vs. 0.39). However, sensitivity and negative predictive value for CRP were higher than the values for PCT (1.00 vs. 0.40 and 1.00 vs. 0.73). Diagnostic value and positive likelihood ratio of CRP for severe febrile neutropenia were higher than those of PCT (71 vs. 67 and 2.32 vs. 2.00).PCT and CRP are comparable with each other in prediction of the clinical severity of febrile neutropenic attacks. Furthermore, serum CRP levels correlate with the duration of fever.

We aimed to evaluate the diagnostic values of soluble transferrin receptor (sTfR) concentration, transferrin-ferritin index (soluble transferrin receptor concentration/log ferritin), ferritin levels and other related parameters in geriatric patients with anemia of chronic disease (ACD) and iron deficiency (IDA). Forty-four elderly subjects (median age 73 [63-94]) and twenty healthy subjects (median age 49 [44-56]) were enrolled into this study, divided into four groups: twenty middle aged healthy subjects (group A), fifteen elderly patients with IDA (group B), fourteen elderly patients with ACD (group C) and fifteen nonanemic geriatric subjects (group D). Hemoglobin, mean corpuscular volume, serum iron concentration and transferrin saturation levels of the patients in IDA group were found significantly lower than those in both non-anemic group and healthy subjects. Serum sTfR concentrations of the patients in IDA group were significantly higher than those in non-anemic geriatric group, ACD group and healthy subjects. Transferrin-ferritin index of the patients with IDA was significantly higher than that in non-anemic geriatric and ACD group. Serum ferritin levels of the patients in IDA group did not show any differences when compared with the other groups. Serum ferritin was highly specific for IDA (95%) when compared with ACD, although its sensitivity was low (38%). STfR values were negatively correlated with both transferrin and ferritin levels (p = 0.042 r = -0.40; and p = 0.034 r = -0.41, respectively). In conclusion, serum soluble transferrin receptor and transferrin-ferritin index may be used together with serum ferritin to distinguish the iron deficiency state in the elderly.

In chronic hepatitis B, apoptotic rate of peripheral cytotoxic T cells may be related with hepatocyte injury. We aimed to investigate Fas (CD95) expression of peripheral cytotoxic T cells and to show the in vitro effect of interferon-alpha 2a on Fas expression and apoptosis.The study group consisted of 17 patients with chronic hepatitis B and control group consisted of 10 healthy subjects. Apoptotic cells were identified by flow-cytometric assay using Annexin V and propidium iodide. We used monoclonal antibodies stained by direct immunofluorescent method to show surface molecules. CH-11 monoclonal antibodies were used as anti-Fas antibodies.Basal expressions of CD95 and CD8+ CD95+ in the peripheral mononuclear cells were higher in chronic hepatitis B patients than controls. In vitro treatment with interferon-alpha 2a increased the percentage of CD8+ 95+ peripheral mononuclear blood cells in controls; this effect was less remarkable in patients with chronic B hepatitis, and the difference was not statistically significant. The number of apoptotic cytotoxic T cells also increased in 5 subjects of the study group and 3 subjects of controls; the percentage of CD95+ cells increased in 7 of 17 patients and 8 of 10 controls. The percentage of CD8+ 95+ cells and the apoptotic rate of CD8+ cells were not different between study group and controls after combined treatment with interferon-alpha 2a and monoclonal stimulating anti-Fas antibodies.We suggest that interferon-alpha 2a does not induce Fas-dependent apoptosis in CD8+ peripheral T cells of the patients with chronic hepatitis B, and CD95 molecules on peripheral cytotoxic T cells might be defective.

It is controversial whether hepatitis B or C viruses induce liver cancer through non-specific mechanisms (inflammation and cell renewal) or direct genotoxicity. Considering that both viruses infect peripheral lymphocytes, studying sister chromatid exchange frequency and mitotic index in peripheral lymphocytes is a reasonable experimental approach to investigate their genotoxic potential separately. In the present study we investigated sister chromatid exchange frequency and mitotic index in the peripheral lymphocytes of patients with cirrhosis and chronic carriers with positive serology for HBV or HCV infections.The study population consisted of 3 groups: group I involved 23 HBsAg positive chronic carriers; group II involved 30 HBsAg positive patients with cirrhosis and group III involved 9 HCV-positive patients with cirrhosis. The control group involved 30 healthy individuals.Sister chromatid exchange frequency was significantly higher in all the study groups than the control group (p0.05). The mitotic index was significantly lower in all the study groups than the control group (p0.05).The increased sister chromatid exchange frequency and low mitotic index may be reflecting a direct genotoxic effect of HBV and HCV in peripheral lymphocytes. We suggest that the same genotoxicity may also operate in the liver and contribute to hepatocarcinogenesis.

This study compares the diagnostic value of troponin T (TnT) and myoglobin with creatinine kinase (CK) for myocardial infarction (MI) in a tertiary care centre in a developing nation. The study group comprised 33 acute myocardial infarction patients and 27 healthy controls. Receiver operating characteristic curves for TnT, myoglobin and CK were drawn and areas under the curve calculated. At admission, myoglobin levels had greater diagnostic sensitivity than TnT or CK levels. After 2 h, myoglobin and TnT had equal sensitivity and specificity, whereas CK still had lower sensitivity than myoglobin and TnT. After 4 h there was no difference between the tests. It was concluded that myoglobin levels on admission and TnT at 2 h had the greatest diagnostic rate, whereas all the tests were similar after 4 h for MI.

Background The stomach is regarded as a rare site for metastasis. When a gastric mass is observed macroscopically, the presumed diagnosis is usually a primary gastric carcinoma. However, the stomach may be involved in metastatic malignant melanoma. Besides a possible macroscopical misdiagnosis, metastatic gastric melanoma may also be misdiagnosed microscopically as adenocarcinoma due to its protean histological characteristics. These features make metastatic gastric melanoma a challenging diagnosis in some cases. Case report We report a patient with metastatic gastric melanoma referred to us with an initial macroscopic and histopathological diagnosis of primary gastric adenocarcinoma. He was diagnosed as having metastatic gastric melanoma by further examination because of the peculiar metastatic involvement and normal gastrointestinal tumor marker levels. Conclusions The stomach may be involved in melanoma and melanoma metastasis to the stomach is a diagnosis that should be taken into account while evaluating any gastric mass lesion. It is likely to be encountered more commonly nowadays due to the significant increase in the melanoma incidence. A history of melanoma, an atypical metastatic pattern, and normal gastrointestinal tumor marker levels may contribute to its diagnosis.

Adult lipid storage disorders with pulmonary involvement are rare and usually diagnosed at autopsy. We report a patient with splenomegaly and reticulonodular pattern on lung computed tomography. Bronchoalveolar lavage was performed and revealed the presence of lipid-containing foamy cells, with the demonstration of both periodic acid-Schiff (PAS) and scharlach red stain positive vacuoles in the cytoplasm of alveolar macrophages. The same cells were found in bone marrow biopsy. As in other rare disorders, bronchoalveolar lavage may be of diagnostic value in lipid storage disorders with pulmonary involvement.

Peritoneal seeding after abdominal surgery is a well known route of metastasis in intra-abdominal solid tumours. Direct mechanical contamination, local peritoneal trauma and subsequent inflammation, postoperative immunosuppression, and laparoscopic surgery are the proposed predisposing factors for this type of metastasis. These factors probably result in enhanced adhesion or growth of tumour cells. However, this route of metastasis has not yet been reported for lymphomas. Here, we report the first case of peritoneal seeding of lymphoma cells after an abdominal surgery.

Background: High-dose therapy with melphalan and autoPBSCT induce remissions in multiple myeloma but are not able to cure this disease. Eventually, all patients relapse and require further treatment. One possibility suitable particularly for patients who had not been earlier treated with thalidomide is a combination of this drug with dexamethasone. Aims: To retrospectively assess the effectiveness of such treatment in a cohort of patients treated in a single center. The endpoints of the study were: response, EFS, OS and toxicity. Methods: Forty five multiple myeloma patients (23 female/22 male) with a median age of 54 years (range, 33-69 years) were included into the analysis between June 2005 and December 2007. Twenty seven patients were IgG, 10-IgA, 2-nonsecretory, 6-light chain. Clinical stage according ISS: 20 patients in stage 1, 9 in stage 2, 16 in stage 3. Patients have been initially treated with VAD protocol (vincristin/adriamycin/ dexamethasone) followed by cyclophosphamide mobilization and then by tandem high-dose melphalan with autoPBSCT. Relapsing patients were treated according to the following protocol: thalidomide 200 mg/day orally continuously until any sign of progressive disease or relapse, and dexamethasone 40 mg orally for 4 days every 3 weeks. Fifteen patients received enoxaparin and 30 patients acetylsalicylic acid prophylaxis. Response was evaluated using the International Myeloma Working Group Uniform Response Criteria. Moreover, overall survival (OS) and event-free survival (EFS) was assessed. Results: Altogether, 6 patients (13.3%) achieved complete response (CR), 7 patients (15.5%) very good partial response (VGPR), 18 patients (40%) partial response (PR), 11 patients (24.4%) stable disease (SD), and 3 patients (6.6%) progression disease (PD). The median time to response was 12 weeks (range, 12-28 weeks). Duration of treatment TD was 14.6 months (median; range, 3-48.6 months). The median EFS was 24 months (range, 14-41.3 months). The median OS has not been reached. The major adverse events of THALDEX were: peripheral neuropathy, 18 patients (40%); constipation, 12 patients (26.6%); somnolence, 5 patients (11.1%); deep-vein thrombosis, 1 patient (2.2%). Conclusion: THALDEX seem to be effective in multiple myeloma patients relapsing after high dose therapy and tandem PBSCT and well tolerated.

McCune–Albright syndrome is a genetic disorder characterized by polyostotic fibrous dysplasia, precocious puberty, and cafeau-lait spots. The main defect is a missense mutation in the GNAS1 gene, which results in the constitutive activation of the a-subunit of the Gs protein leading to overproduction of cAMP. Clinical presentation is variable due to the mosaic pattern of involvement of the tissues. Herein, we present a case of McCune–Albright syndrome (MAS) displaying the radiologic and biochemical features of Paget’s disease of bone. A 24-year-old woman was referred to our hospital due to skeletal deformities and facial dysmorphism. Her history included regular menses beginning at the age of 5 months, and bone deformities becoming evident at the age of about 5 years. Recently, she had been operated on due to a maxillary tumor; its pathological assessment yielded a diagnosis of fibrous dysplasia. On physical examination she was measured to be 90 cm in height. Facial asymmetry with depression of the left maxillary area was noted. She had extreme kyphoscoliosis and her right leg (tibia) had a bowing deformity. Routine blood chemistry showed a calcium level of 9.3 mg/dL, a phosphorus level in the lownormal range (2.9 mg/dL, normal 2.7–4.5), and a high alkaline phosphatase level (834 U/L, normal 42–98). Plasma levels of T3, T4, TSH, LH, FSH, estradiol, DHEAS-S, testosterone, free testosterone, growth hormone, prolactin, cortisol, and ACTH were all in the normal range. The parathyroid hormone level was 59.5 pg/mL (normal 9–55). Urinary hydroxyproline excretion was 392.3 mmol/mL creatinine (normal ,30). Radiological examination of the cranium revealed diffuse sclerosis of the skull base, thickening of the calvarium, and radiolucent areas (Figure 1). These changes were thought to be most compatible with Paget’s disease of bone. Both femurs showed widening of the bone shaft, ground glass appearance, and cortical thinning. Whole-body scintigram revealed dense uptake of the radiolabel in an asymmetric pattern (Figure 2). MRI scan of the sella revealed no pituitary adenoma. The presence of pathologically confirmed polyostotic fibrous dysplasia and a history of precocious puberty was considered decisive for diagnosis of MAS. The absence of cafe-au-lait spots does not exclude such a diagnosis, considering the mosaic nature of the disease. The most striking feature of the case was radiological findings closely mimicking those of Paget’s disease of bone. In the cranial radiographs, calvarial thickening, radiolucent areas resembling osteoporosis circumscripta, and sclerotic changes were considered compatible with Paget’s disease. Although these two disorders can cause thickening of the bones, X-ray findings of polyostotic fibrous dysplasia mimicking those of Paget’s disease have been emphasized very infrequently. Coexistence of both clinical situations has also been reported. Histologically, prominent osteoblasts on the surface of bone trabeculae and abnormal lamellar bone are characteristics of Paget’s disease and distinguish it from fibrous dysplasia. However, differentiating between Paget’s disease and polyostotic fibrous dysplasia should not be difficult on clinical grounds considering the very early onset of symptoms in the latter, particularly in the present case where the bony deformities had become evident at the age of about 5 years, which has not been seen in Paget’s disease. The high urinary hydroxyproline excretion and high serum alkaline phosphatase observed in our case may reflect the high bone turnover associated with polyostotic fibrous dysplasia, a feature also shared by Paget’s disease of bone. These findings suggest Address for correspondence and reprints: Dr. Sukru Palanduz, Fevzi Pasa Caddesi, Testereci Sokak, 1/10, Karagumruk, Fatih, 34230 Istanbul, Turkey. Figure 1. Lateral radiograph of the cranium showing calvarial thickening, radiolucent areas resembling osteoporosis circumscripta, and striking osteosclerosis involving particularly the skull base. Bone Vol. 24, No. 2 February 1999:157–158

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.