Unusual case of chronic recurrent multifocal osteomyelitis.

BACKGROUND: Chronic recurrent multifocal osteomyelitis (CRMO) is a rare auto-inflammatory bone disorder that primarily affects young girls, with a mean age of 10 years at onset. Generally, it is a self-limited disease. However, recent data indicate that more than 50% of patients have a chronic persistent disease and about 20% a recurring course of this condition. Also, there are more cases reported with associated auto-inflammatory and autoimmune diseases. In this case report, we present a rare case of sporadic CRMO in which the patient eventually developed C-ANCA (cytoplasmic anti-neutrophil cytoplasmic antibodies)-associated renal vasculitis and hyperparathyroidism. CASE PRESENTATION: A 14 year old female patient was brought to the emergency department with a sudden onset of left leg pain and oedema. After physical evaluation and initial investigation, she was diagnosed with femoral and pelvic deep vein thrombosis. While searching for possible thrombosis causes, osteomyelitis of the left leg was identified. Additional CT and MRI scans hinted at the CRMO diagnosis. Due to the multifocal lesions of CRMO, endocrinological evaluation of calcium metabolism was done. The results showed signs of hyperparathyroidism with severe hypocalcaemia. Moreover, when kidney damage occurred and progressed, a kidney biopsy was performed, revealing a C-ANCA associated renal vasculitis. Treatment was started with cyclophosphamide and prednisolone according to the renal vasculitis management protocol. Severe metabolic disturbances and hyperparathyroidism were treated with alfacalcidol, calcium and magnesium supplements. Secondary glomerulonephritis (GN) associated hypertension was treated with ACE (angiotenzine converting enzyme) inhibitors. Anticoagulants were prescribed for deep vein thrombosis. After 1.5 years of treatment, the patient is free of complaints. All microelement and parathormone levels are within normal range. Kidney function is now normal. To date, there are no clinical or diagnostic signs of deep vein thrombosis. CONCLUSIONS: This case report presents a complex immunodysregulatory disorder with both auto-inflammatory and autoimmune processes. We hypothesize that the long lasting active inflammation of CRMO may induce an autoimmune response and result in concomitant diseases like C-ANCA-associated vasculitis in our patient. Any potential specific pathogenic relationships between these two rare pathologies may need to be further studied. Furthermore, there is a lack of specific biomarkers for CRMO and more studies are necessary to identify CRMO's characteristic patterns and how to best monitor disease progression.

Doppler flowmetry of the superior mesenteric artery and portal vein: impact for the early prediction of necrotizing enterocolitis in neonates.

PURPOSE: Necrotizing enterocolitis (NEC) is a serious gastrointestinal disorder in newborns. Early diagnosis and rapid treatment is essential for seeking good outcome for neonates. The aim of our study was to evaluate intestinal blood flow in superior mesenteric artery (SMA) and portal vein (PV) in neonates with suspected or confirmed NEC and investigate the prognostic cut-off values to develop NEC. METHODS: Doppler flowmetry of SMA and PV was performed for 62 newborns. Resistive (RI) and pulsatility (PI) indexes were measured in SMA as well as Volumetric blood flow (Vflow) in PV. ROC curves were applied to estimate sensitivity and specificity and to identify cut-off values. RESULTS: There were 93.5% preterm neonates. 29 patients (46.8%) were diagnosed with NEC and 33 (53.2%) formed a control group. 96.3% NEC patients had RI >0.75 with sensitivity of 96.3% and specificity of 90.9% (OR 260). 88.9% NEC patients had PI >1.85 with sensitivity of 88.9% and specificity of 78.8% (OR 29). Portal Vflow lower than 37 ml/min was present in 89.7% patients with NEC (OR 11.7). CONCLUSION: Intestinal blood flow Dopplerography can be a useful tool for diagnosing and predicting NEC.

Recurrent urinary tract infection in girls: do urodynamic, behavioral and functional abnormalities play a role?

BACKGROUND: To determine urodynamic, behavioral and functional abnormalities predisposing to recurrent urinary tract infection in 5- to 17-year-old girls. METHODS: A prospective case-control study was carried out. A total of 148 girls met inclusion criteria. They received a careful evaluation including complete history, voiding-drinking diary, bowel questionnaire, physical investigation, sonography, voiding cystourethrogram and urodynamic investigation. RESULTS: In a multivariate model, independent risk factors for recurrent urinary tract infection included age 20 mL (OR=1.1; 95% CI, 1.0-1.1). CONCLUSIONS: Independent risk factors for recurrent urinary tract infection were age

The accuracy of different imaging techniques in diagnosis of acute hematogenous osteomyelitis.

OBJECTIVE: The aim of this study was to establish and compare diagnostic accuracy (sensitivity, specificity, and diagnostic odds ratio) of plain x-ray, ultrasonography, bone scintigraphy, computed tomography (CT), and magnetic resonance imaging (MRI) in pediatric acute hematogenous osteomyelitis. MATERIAL AND METHODS: Analysis of patients' data, hospitalized at the Department of Pediatric Surgery with suspected acute hematogenous osteomyelitis in the period of 2002-2008, was carried out prospectively. Inclusion criteria were age of 1-18 years, pain in bone area, fever, functional disorder, and (or) signs of infection. Plain x-ray, ultrasonography, bone scintigraphy, computed tomography, and magnetic resonance imaging were performed. The recommendations of Standards for Reporting of Diagnostic Accuracy were used in study design. RESULTS: A total of 183 patients were included into the study. Acute hematogenous osteomyelitis was diagnosed in 156 (85%) patients, and 27 (15%) had other diseases. A total of 169 early plain x-rays (median on the first day of hospital stay), 142 late x-rays (15th day of hospital stay), 82 ultrasonographies (second day), 76 bone scintigraphy (third day), 38 MRI scans (seventh day), and 17 CT (15th day) were performed. The sensitivity of ultrasonography was 0.55 (95% CI, 0.43-0.67); specificity, 0.47 (95% CI, 0.24-0.7); and diagnostic odds ratio, 1.08 (95% CI, 0.3-3.84). The sensitivity of CT was 0.67 (95% CI, 0.38-0.88); specificity, 0.5 (95% CI, 0.01-0.98); and diagnostic odds ratio, 2.0 (95% CI, 0.02-172.4). The sensitivity of early x-ray was 0.16 (95% CI 0.1-0.23); specificity, 0.96 (95% CI, 0.78-1.0); and diagnostic odds ratio, 4.34 (95% CI, 0.63-186.3). The sensitivity of MRI was 0.81 (95% CI, 0.64-0.93); specificity, 0.67 (95% CI, 0.22-0.96); and diagnostic odds ratio, 8.67 (95% CI, 0.91-108.5). The sensitivity of late x-ray was 0.82 (95% CI, 0.75-0.88); specificity, 0.92 (95% CI, 0.62-1.0); and diagnostic odds ratio, 51.17 (95% CI, 6.61-2222.0). The sensitivity of bone scintigraphy was 0.81 (95% CI, 0.68-0.90); specificity, 0.84 (95% CI, 0.60-0.97); and diagnostic odds ratio, 22.30 (95% CI, 4.9-132.7). CONCLUSIONS: Our analysis showed that late x-ray is the most valuable radiologic method in the diagnosis of acute hematogenous osteomyelitis, but bone scintigraphy and magnetic resonance imaging are the most valuable tests at the onset of the disease.

Percutaneous transhepatic biliary stenting: the first experience and results of the Hospital of Kaunas University of Medicine.

Malignant biliary obstruction may be caused by cholangiocarcinoma and other nonbiliary carcinomas. At the time of diagnosis, 90% of patients with malignant obstructive jaundice may benefit from palliative treatment only. The objective of palliation is to relieve jaundice-related symptoms, prevent cholangitis, prolong survival, and improve quality of life. Percutaneous transhepatic biliary stenting is a well-established procedure used in patients with malignant obstruction of intra- and extrahepatic bile ducts. Twelve patients (9 women, 3 men; mean age, 68 years; range, 44-88 years) with inoperable malignant biliary obstruction were selected for percutaneous transhepatic biliary stenting with metallic stents in the period from January to December 2007. Technical and clinical success rate in this patient series was 83% and 80%, respectively. Minor and major complications occurred in 17% and 8% of cases, respectively, which is in the range reported by the others. This is our first experience of percutaneous transhepatic biliary stenting at the Hospital of Kaunas University of Medicine and, to our knowledge, the first reported patient series in Lithuania. These first results encourage expanding effective palliation by the employment of the percutaneous transhepatic biliary stenting in patients with nonresectable malignant biliary obstruction or in case of a recurrent disease after curative surgery. The cost effectiveness of percutaneous transhepatic biliary stenting against percutaneous transhepatic biliary drainage has yet to be evaluated in a prospective manner. However, immediate clinical benefits and positive short-term outcomes are unequivocal.