Is there any requirement for celiac disease screening routinely in postmenapausal women with osteoporosis?

Screening studies indicate a prevalence of celiac disease (CD) of up to 1% in populations of European ancestry, yet the majority of cases remain undiagnosed. One of the common complication of CD is intestinal osteopathy or osteoporosis [bone mineral density (BMD) based diagnosis]. Available data regarding the prevalence of CD in the patients with osteoporosis are limited and controversial. The objective of this study was to perform serological testing to screen for CD among postmenopausal women with osteoporosis. We studied 192 postmenopausal women with low BMD with a mean age of 62.75 ± 8.58 years. Among the patients, a total of 137 had osteoporosis and 55 had osteopenia. Venous blood samples were obtained for serological screening of CD and evaluation of bone metabolism. The serological screening protocol consisted of determining serum level of IgA antigliadin antibodies (AGA), IgG-AGA, IgA endomysial antibody (EMA), IgG-EMA. Subjects who were positive for both IgA-AGA and IgA-EMA were classified as having CD. Bone metabolism was evaluated by serum calcium, phosphorus, alkaline phosphatase, parathyroid hormone, 25 (OH) vitamin D, osteocalcin, serum C-telopeptide cross-linked collagen type I levels. Of the 192 patients evaluated, only one (0.5%) was found to have positive for both IgA-AGA and IgA EMA tests and accepted as having CD. Prevelance of CD in postmenopausal women with low BMD (0.5%) did not differ from prevelance of CD in normal healthy population (0.3–1%). BMD values at proximal femur level were significantly lower in IgA-AGA (+) patients when compared to IgA-AGA (−) patients. However, the mean levels of bone metabolism markers were found similiar in both IgA-AGA (+) and (−) patients. In conclusion, the results of our study suggest that there is no need for routine screening of CD in postmenopausal women with osteoporosis.     

Idiopathic penile mondors' disease: A case report

A 34-year old man was admitted to us with a mild painful cord-like induration on the left dorsal side of the penis extending from corona of the glans to the base of the penis. The patient was treated with anti-inflammatory and anticoagulan agents. Cord-like-induration resolved and recanalized in 4th week, and venous flow was detected in Doppler examination.     

Pheochromocytoma in children

BACKGROUND/PURPOSE: Etiopathogenesis, management, and outcome of pediatric pheochromocytoma (PHEO) still is obscure because of limited number of cases. Therefore, a retrospective clinical study was performed to present an updated picture of the entire spectrum of pediatric PHEO based on the authors' 30 years' experience consisting of one of the largest noncollected series treated in a single medical center. METHODS: Records of patients treated for PHEO in the authors' unit from 1970 to 1999, inclusive, were reviewed retrospectively. Information recorded for each patient included age, sex, past medical and family history, clinical characteristics, diagnostic methods, treatment, pathologic findings, and outcome. RESULTS: Sixteen children with a mean age of 10.7 +/- 2.9 years consisting of 12 boys and 4 girls were treated for PHEO. Most of the tumors were right sided (n = 6) and bilateral (n = 6). Sporadic cases of PHEO accounted for 14 patients (88%), whereas 2 children had von Hippel-Lindau (VHL) disease and multiple endocrine neoplasia type 2b (MEN2b). Hypertension was the most common symptom followed by headache and sweating. The diagnosis of PHEO was made by laboratory and radiologic studies. Preoperative medical therapy was done in all patients. Laparotomy confirmed that 11 patients had localized, 4 patients had regional, and 1 patient had metastatic disease. The localized tumors were excised totally by bilateral (n = 4) and unilateral (n = 6) adrenalectomy. Surgical procedures performed for regional disease were total excision (n = 2), incisional biopsy (n = 1) and partial excision (n = 1). Incisional biopsy could be taken only from a patient with metastatic disease at presentation. Two patients with localized disease and 2 patients with regional disease had benign recurrences in right (n = 2) and left (n = 2) adrenal glands within 3 to 7 years after operation. Total excision of the recurrent tumors was done in all patients. Pathologic examination found apparently malignant features in 3 patients who presented with regional (n = 2) or metastatic (n = 1) disease and underwent incisional biopsy (n = 2) or partial excision (n = 1). Pathologic features suggestive of malignancy were noted in 4 patients presenting with regional (n = 2) and localized disease (n = 2). Apparently benign pathologic features were noted in the remaining 9 patients. There was not any operative mortality in our series. Adjuvant chemotherapy was commenced postoperatively in all patients with malignant and suggestive of malignant pathologic features. During the long-term follow-up for 16 years, 3 patients died (19%). One patient with VHL disease died of astrositoma 5 years after her recurrent PHEO was excised. Of the 3 patients with malignant disease, 2 patients in whom only incisional biopsies were done had distant metastases and died of disease within 2 years. Another patient with malignancy who had MEN2b was lost to follow-up. CONCLUSIONS: Early diagnosis and total excision are the most important aspects of accurate treatment for childhood PHEO. Pre- intra- and postoperative medical management is as important as the surgical procedure. Our surgical treatment policy is mainly minimizing the risk of recurrence while preserving adequately functioning adrenal medullar tissue. Incomplete excision and advanced-stage disease are the major determinants of poor outcome. None of the clinical, laboratory, or pathologic features are reliable predictors for recurrence and discrimination of malignancy. Because of the steadily increasing incidence of precancerous genetic syndromes related to adrenal glands and poor prognosis of advanced-stage PHEO, childhood cases of hypertensive disorders should receive a detailed and vigorous diagnostic evaluation and appropriate treatment as given to adults.     

Gastric outlet obstruction due to corrosive ingestion: incidence and outcome

A retrospective clinical study was performed to determine the incidence, management, and outcome of gastric outlet obstruction (GOO) caused by caustic ingestion in children. Of 220 patients who sustained caustic substance ingestion and were treated at our unit between 1976 and 1996, 168 ingested alkaline substances; of these, 9 children (5.3%) developed GOO in addition to esophageal strictures. The remaining 52 patients ingested acid agents, and 2 of them (3.8%) presented with GOO without esophageal strictures. The overall incidence of corrosive GOO was 5% (n = 11). The mean age of the patients with GOO was 5.7 ± 2.8 years (range 2–14) with a female:male ratio of 6:5. Sodium hydroxide (n = 6), potassium hydroxide (n = 3), and hydrochloric acid (n = 2) were the ingested caustic agents. The patients were subdivided into two groups according to serial endoscopic and radiologic findings: group I: moderate (dense superficial and spotty ulcerations with intact mucosa) mucosal injury with partial pyloric obstruction; and group II: severe (deep ulcerations, extreme hemorrhagic erosions, eschar formation with white plaques) mucosal injury with complete pyloric obstruction. Group I consisted of 5 patients who ingested alkali agents while group II included 6 who presented with ingestion of alkaline (n = 4) and acid (n = 2) agents. Surgical treatment included Billroth I (n = 6) operations performed in group II and Finney (n = 3) and Heineke-Mikulicz (n = 2) pyloroplasty procedures done in group I. All patients are alive without any complaints. Fiberoptic endoscopy should be the preferred method of evaluating a patient with ingestion of a corrosive agent. It determines the presence of injury and assesses the extent of damage, establishing the diagnosis and allowing therapy to be instituted immediately. Our experience revealed that substantial damage has occurred early after ingestion, and early surgical intervention has decreased the morbidity and mortality. The extent of the mucosal injury and status of the pylorus and antrum determined the type of surgical treatment. A Billroth I procedure recommended for severely injured mucosa with complete pyloric obstruction, and pyloroplasty for moderate mucosal injury associated with partially obstructed but still viable pylorus. In contrast to the current belief, alkali ingestion also has a high risk of corrosive gastric injury causing GOO, which should be considered during assessment of the injury. We emphasize that a detailed evaluation of radiologic and especially endoscopic findings is very important for determining the timing, necessity, and type of appropriate surgical treatment. Accepted: 17 June 1998     

Waardenburg syndrome associated with bilateral renal anomaly

A 1-year-old girl with Waardenburg syndrome type I presented with double collecting system of left kidney accompanied by nonobstructive hydronephrosis of lower pole and by ureteropelvic junction obstruction of right kidney. Renal involvement in Waardenburg syndrome was reported once in a 4-month-old boy with unilateral duplication of the renal collecting system and in a 16-day-old girl who had right multicystic dysplastic kidney and hydronephrosis in the left kidney. The third case of renal involvement in Waardenburg syndrome is presented here, with special emphasis on early diagnosis and management of renal anomaly. The authors conclude that urinary system anomalies should also be considered in the wide spectrum of Waardenburg syndrome clinical features to avoid life-threatening complications.     

The roles of HRCT and clinical parameters in assessing activity of suspected smear negative pulmonary tuberculosis

BACKGROUND: Smear-negative pulmonary tuberculosis (SNPTB) constitutes a major problem in countries with a moderate or high TB prevalence. The value of high-resolution computed tomography (HRCT), chest x-ray and other clinical findings in determining activity of SNPTB were investigated. METHODS: The study population consisted of 85 patients with suspected SNPTB, of whom 52 were confirmed as active pulmonary TB according to either culture positivity for Mycobacteriun tuberculosis or demonstration of caseous granulomatous inflammation. The remaining 33 patients accepted inactive TB sequel. RESULTS: Cough and expectoration were significantly frequent in inactive group whereas chest pain was detected higher in active patients. Sensitivity, specificity, positive predictive value, negative predictive value and diagnostic accuracy of HRCT in detecting disease activity were 88, 88, 92, 83 and 88%, respectively. Centrilobular nodules, other non-calcified nodules, consolidation and cavity were significantly higher on HRCT in active group. Chest x-ray scores that were graded 1 to 3 showed a linear trend for the disease activity. CONCLUSIONS: HRCT has good diagnostic value in detecting activity of SNPTB, and some clinical findings may help in predicting the activity.     

Relationship between sleep complaints and proinflammatory cytokines in haemodialysis patients

BACKGROUND: Sleep complaints are common in end-stage renal disease. We aimed to investigate the relationship between sleep-related complaints and inflammatory cytokines in haemodialysis (HD) patients, and also the effects of HD on sleep patterns and cytokine levels. METHODS: Predialysis serum interleukin-1beta (IL-1beta), interleukin-6 (IL-6) and tumour necrosis factor-alpha (TNF-alpha) levels in nine patients with sleep complaints were compared with those of nine patients without sleep complaints and nine healthy controls. Patients with sleep complaints underwent polysomnography the night after HD and the following night. RESULTS: Patients with sleep complaints had significantly higher predialysis IL-1beta levels compared with those without and healthy controls (P=0.004 and P=0.000, respectively). They also had higher predialysis IL-6 and TNF-alpha levels than those without sleep complaints; however, the difference was not significant. Patients without sleep complaints had higher mean IL-6 and TNF-alpha and similar mean IL-1beta levels compared with healthy controls (P=0.001, P=0.024, P=0.26, respectively). Obstructive sleep apnoea syndrome (OSAS) was found in six out of nine (66%) patients with sleep complaints. Sleep architecture and cytokine levels did not differ between the two nights. The mean serum IL-1beta, IL-6 and TNF-alpha levels did not differ in the pre- and post-polysomnographic samples. There was no correlation between IL-1beta, IL-6 or TNF-alpha levels and the apnoea-hypopnoea index. CONCLUSIONS: Proinflammatory cytokines, IL-1beta in particular, might be associated with sleep complaints in HD patients. OSAS is not uncommon in HD patients with sleep-related complaints and sleep architecture does not appear to be effected by the HD procedure itself.     

MRI findings of myositis in Behçet disease

We report the magnetic resonance imaging findings in a case of localized myositis in a 23-year-old man with long-standing Behçet disease.     

Calcifying fibrous pseudotumor of lung: a previously unreported entity

Calcifying fibrous pseudotumor (CFP) is one of the most unusual benign tumors of childhood and is located mostly in soft tissues, pleura, and peritoneum. The first case of pulmonary CFP in a 7-year-old boy is presented. The clinicopathologic features of this rare entity are discussed with special emphasis on histopathologic features and prognosis including a brief literature review. There are no presumptive clinical and laboratory findings, including tumor markers and imaging techniques, that distinguish CFP from other space-occupying lung lesions. The most important diagnostic aid is to bear this entity in mind when a child presents with an unexplained pulmonary mass. Total excision is mandatory for the appropriate diagnosis and treatment.