Low birth weight is a conditioning factor for podocyte alteration and steroid dependance in children with nephrotic syndrome.

BACKGROUND: Low birth weight (LBW) is associated with reduced nephron endowment. Clinical-pathologic features of post adaptive focal segmental glomerulosclerosis (FSGS) have been observed in subjects with prematurity and very LBW. METHODS: We aimed to investigate the correlation between LBW and outcome in a cohort of 89 children with idiopathic nephrotic syndrome (NS) (2-12 years-old at onset, followed for > 3 years), of whom 21 with LBW (birth weight < 10th percentile for gestational age, gender, ethnicity, and maternal parity or birth weight < 2500 g). RESULTS: Children with NS and LBW were found to have FSGS more frequently than children with normal birth weight (NBW) [8/21 = 38% vs. 4/68 = 6%; odds ratio, OR 7.754 (95% confidence interval, CI 2.184-27.525); χ2 = 9.817; p < 0.003]. Children with LBW and cortico-sensitive NS had a greater risk of cortico-dependence (CD) than those with NBW [10/13 = 76.9% vs. 28/63 = 44.4%, OR 4.744 (1.188-18.936); χ2 = 4.158; p < 0.05]. Moreover, children with LBW and CDNS needed a greater dose of immunosuppressive drugs than those with NBW [OR 4 (1.153-13.877); χ2 = 3.842; p = 0.05]. CONCLUSIONS: LBW children developing NS had higher risk of FSGS and CD, and needed heavier immunosuppressive therapy than those with NBW. These data might suggest a conditioning role for hemodynamic and podocyte changes due to reduced nephron mass in LBW.

Dense deposit disease in a child with febrile sore throat.

Dense deposit disease or membranoproliferative glomerulonephritis type II is a rare glomerulopathy characterized on renal biopsy by deposition of abnormal electron-dense material in the glomerular basement membrane. The pathophysiologic basis is uncontrolled systemic activation of the alternate pathway of the complement cascade. C3 nephritic factor, an autoantibody directed against the C3 convertase of the alternate pathway, plays a key role. In some patients, complement gene mutations have been identified. We report the case of a child who had persistent microscopic hematuria, proteinuria, and hypocomplementemia C3 for over 2 months. Renal biopsy confirmed the diagnosis of dense deposit disease.

High mobility group box 1: Biomarker of inhaled corticosteroid treatment response in children with moderate-severe asthma.

BACKGROUND: High mobility group box 1 (HMGB1) is abnormally expressed in serum and sputum of patients with allergic asthma. OBJECTIVE: The aim of this study was to investigate the role of HMGB1 as guidance for treatment management of children with asthma. METHODS: Thirty children with asthma and 44 healthy children were enrolled. The patients were classified according to Global Initiative for Asthma Guideline disease severity criteria. Sputum HMGB1 levels and lung function index (percentage forced expiratory volume in 1 second [FEV1%]) were recorded in the cohort study at baseline (T0) and after 3 (T3) and 6 (T6) months of inhaled corticosteroids (ICS) treatment. RESULTS: Sputum HMGB1 levels were significantly higher in all the patients with asthma (p < 0.001). An inverse correlation between sputum HMGB1 levels and pulmonary function parameters was observed only in the children with moderate asthma (T0: FEV1%, r = -0.9891, p < 0.001; T3: FEV1%, r = -0.6763, p < 0.001; T6: FEV1%, r = -0.5419, p < 0.05) and in the children with severe asthma (T0: FEV1%, r = -0.8696, p < 0.001; T3: FEV1%, r = -0.6477, p < 0.05; T6: FEV1%, r = -0.8627, p < 0.001). After ICS treatment, a significant decrease of sputum HMGB1 levels was noted in moderate (T0 [93.44 ± 20.65 ng/mL] versus T3 [77.96 ± 1.81 ng/mL] versus T6 [67.75 ± 3.01 ng/mL]; p < 0.0001) and in the children with severe asthma (T0 [130.3 ± 7.48 ng/mL] versus T3 [156.9 ± 1.09 ng/mL] versus T6 [116.08 ± 4.77 ng/mL]; p < 0.0001) data are mean ± standard deviation, respectively. The area under the receiver operating characteristic curve, performed to define the diagnostic profile of sputum HMGB1 levels in identifying the children with asthma, was 0.713. CONCLUSION: In addition to the findings that HMGB1 is a sensitive biomarker of allergic asthma in children, our data demonstrated a significant correlation between the decrease of HMGB1 levels and a successful treatment response.

Intestinal Behçet and Crohn's disease: two sides of the same coin.

Behçet's disease (BD) and Crohn's disease (CD) are chronic immune-mediated, inflammatory disorders affecting many different systems (joints, skin, eyes, gastrointestinal and biliary tracts). Both disorders have fluctuating courses and when gastrointestinal symptoms are prevalent, differential diagnosis can be difficult. BD involves the gastrointestinal tract in 10-15% of cases with localized lesions in the ileocecal region. The clinical picture is heterogeneous with various clusters of disease expression. CD is a chronic inflammatory disorder, which can affect any part of the intestinal tract, as well as extra-intestinal tissue. Factors that contribute towards the pathogenesis of both disease include the host's genetic profile, and immune system, and environmental factors such as the gut microbiota. The aim of this manuscript is to provide a narrative review of clinical features of BD and CD, highlighting the importance of differential diagnosis and therapeutic approach, especially in the presence of gastrointestinal involvement. A comprehensive search of published literature using the Pubmed ( http://www.ncbi.nlm.nih.gov/pubmed/ ) database was carried out to identify all articles published in English from 1999 to October 2016, using 4 key terms: "Behçet Disease", "Intestinal Behçet's Disease", "Crohn's Disease" and" Inflammatory Bowel Disease".

Intestinal and neurological involvement in Behcet disease: a clinical case.

BACKGROUND: Behcet's disease (BD) is a chronic immune-mediated, inflammatory disorder which may affect a number of different systems (oral and genital mucosa, eyes, skin, vascular district, joints, gastrointestinal tract and nervous system). Neurological manifestations are present in 5-10%, and gastrointestinal tract involvement in 10-15% of cases. The simultaneous involvement of two systems, neurological and gastrointestinal tract, is very rare and represents the aim of our case report. CASE PRESENTATION: We describe a case of a 12-year-old girl with neurological (endocranial hypertension, papilledema, retinal vasculitis) and gastrointestinal tract (terminal ileum and cecum inflammation) involvement and with a history of recurrent oral aphthosis; therefore, according to both International Criteria for Behcet's Disease (ICBD) and Paediatric Behcet's Disease criteria (PEDBD) the diagnosis of BD was confirmed. CONCLUSIONS: This case report is one of the few described in literature with simultaneous involvement of the two systems, neurological and gastrointestinal tract, in paediatric BD. The diagnosis is really difficult because there is no specific diagnostic test. We think that our clinical case should help clinicians to suspect a BD with an unusual onset.

Management of pain on hallux valgus with percutaneous intra-articular Pulse-Dose Radiofrequency.

AIM: The purpose of our study was to investigate the role of intra-articular pulse-dose radiofrequency in management of painful hallux valgus refractory to conservative therapies. METHOD: Between November 2010 and April 2012, 51 patients (15 male, 36 female) with a median age of 71.4 years were included in our clinical trial. Under fluoroscopic guidance we introduced a 22 gauge 10 cm length cannula by a percutaneous access in the first metatarsophalangeal joint and its tip was placed intra-articularly. After removing the spindle, a radiofrequency needle with a 5 mm active tip was introduced. The following parameters were used: 1200 pulses at high voltage (45 V) with 20 msec duration followed by 480 msec silent phases. RESULTS: A great reduction in pain intensity was documented at 1 week, 1 month and 3 months after procedures. Pain intensity increased between 5 and 8 months after treatments, so we performed a second procedure in all patients between 7 months and 9 months since the first treatment. Also in this case we obtained a great reduction of pain intensity in the first 3 months after the procedure. Pain intensity returned at preprocedural values after 9 months after second procedure. No complications were observed. CONCLUSION: Our experience shows pulse-dose radiofrequency is a safe, repeatable and effective technique for managing patients with symptomatic hallux valgus in the short and medium term. Pulse-dose radiofrequency may improve pain control and quality of life in patients with hallux valgus refractory to conservative therapies.

Clinical evaluation of percutaneous vertebroplasty in a patient with paraplegia and immobilization syndrome: a case report.

We will discuss a potential role of percutaneous vertebroplasty (PVP) in the management of a patient with immobilization syndrome due to paraplegia and vertebral osteoporotic fractures. While PVP is commonly used for the treatment of osteoporotic thoracolumbar vertebral compression fractures, its role in vertebral stabilization in patient with immobilization syndrome has not been reported in the literature. A 73-year-old woman affected by immobilization syndrome due to paraplegia and vertebral osteoporotic fractures was treated with PVP of vertebrae D12, L1, and L4. After PVP, the patient did not need any antalgic therapy, and there was a significant improvement regarding mobilization, performance of physiological functions, daily management of personal care, and treatment of decubitus ulcers, increasing life quality and psychological well-being.

Primary extramedullary plasmacytoma of the heart: a rare manifestation of plasmacellular tumor.

Extramedullary plasmacytoma involving the heart is extremely rare. Primary extramedullary localizations are most commonly found in the head and neck region, with no radiologic evidence of additional skeletal lesions and normal bone marrow examination, but can occur in many other locations. They rarely occur in the heart and are commonly associated with multiple myeloma diagnosis. Here, we describe a case of primary extramedullary plasmacytoma of the heart in a 62-year-old man who presented with nocturnal dyspnea, arthralgia, and weakness. The symptoms can sometimes result from the mass effect on cardiac flow. The diagnosis and management require the same range of clinical and laboratory expertise as for patients with multiple myeloma. Their management is particularly challenging due to the lack of evidence or the presence of nonspecific symptoms. The case is presented as a learning point to remember to include plasmacytic tumors in the differential diagnosis of anaplastic tumors, even in unusual locations, such as the heart.

General versus spinal anaesthesia for elective caesarean sections: effects on neonatal short-term outcome. A prospective randomised study.

OBJECTIVE: To compare neonatal short-term outcome in patients who underwent spinal, general anaesthesia and conversion from spinal to general anaesthesia. METHODS: One hundred seventy-nine pregnant women undergoing elective caesarean section were allocated randomly to general (n=89) or spinal anaesthesia (n=90) and compared with 63 patients who required conversion to general anaesthesia. Umbilical cord artery pH, Apgar score as well as its individual parameter and need for assisted ventilation were evaluated. RESULTS: No differences were found in pH values (p=0.35), while the need for assisted ventilation differed significantly (p=0.001). The rate of depressed newborns was 1.1% in the spinal group, 25.9% in the general group and 12.7% in the conversion group with a significant difference for all comparisons. At 5-min, all newborns were vigorous. At 1 min, a higher score for each parameter was found in spinal group with respect to general group, while 'activity', 'grimace' and 'respiration' showed a higher score in conversion group than in general group. At 5 min, a difference was found only for 'activity'. CONCLUSIONS: All kinds of anaesthesia seem to be safe, but loco-regional blockade shows more advantages on the neonatal outcome also when a conversion is necessary.