Retroperitoneal Fibrosis Secondary to Different Etiologies (Hemilaminectomy and Hypothyroidism): Reports of Two Cases

Retroperitoneal fibrosis (RF) is a clinical entity characterized by the progressive proliferation of connective tissue that rarely forms a mass involving the periaortic area of the abdomen, which may be idiopathic as well as a result of an inflammatory process after aneurysmal dilatation of the aorta. This fibrotic tissue may cover both aorta and iliac arteries, reach the retroperitoneum and surrounding ureters, and cause serious obstructions and renal insufficiency in three-quarters of patients. Most of the patients are known to have atherosclerosis and local inflammation against the antigens of the plaques. A systemic autoimmune disease presenting with retroperitoneal fibrosis seems to be pronounced more frequently nowadays because of the elevated acute-phase reactant levels, positive autoantibodies, and concurrent autoimmune diseases affecting other organs in majority of the diagnosed patients. Ultrasonography, computed tomography, magnetic resonance imaging, positron emission tomography, and retroperitoneal biopsy are useful in diagnosing and assessing the full extent of the disease. Surgical interventions such as ureterolysis and aneurysm repair are frequently performed, but medical therapy including steroids and immunosuppressants is often needed because of the inflammatory and chronic-relapsing nature of the disease.

In this paper, we described two cases diagnosed with RF secondary to hemilaminectomy and hypothyroidism, and we summarized the literature related to RF.     

Treatment of two newborns with esophageal atresia and distal tracheoesophageal fistula complicated by gastric perforation: choosing the simple way

Abstract

Objective: Gastric distention and perforation are possible results in a preterm newborn with esophageal atresia and distal tracheoesophageal fistula, especially when there is a need for mechanical ventilatory support. The results of the reported cases treated with emergency thoracotomy and fistula ligation after gastrostomy are not very satisfactory. Sometimes simple temporary solutions can be useful for stabilization and allow safety for required surgical treatment for later.

Patient and methods: Two preterm newborns with esophageal atresia and distal tracheoesophageal fistula complicated by gastric perforation were reported.

Results: Both of the patients were initially treated with a simple peritoneal drainage and, then the definitive operations were performed without any problem in stabilized patients.

Conclusion: Performing fistula ligation or occlusion as an initial treatment in patients with impaired cardiac and respiratory functions may worsen the status of the patient. In such cases, it could be better to perform simple interventions first to facilitate subsequent treatments.     

Reliability and Validity of the Turkish Version of the Abiloco-Kids

ABILOCO-Kids is a scale that assesses the walking ability of children with Cerebral Palsy (CP) from the viewpoint of parental perception. The aim of this study was to translate the ABILOCO-Kids scale into Turkish and to establish its reliability and validity in children with CP. Turkish children were recruited in this study. ABILOCO-Kids is a scale developed by Caty and et al. This scale assess the walking ability of children with CP focusing on the activity domain of the International Classification of Functioning, Disability and Health (ICF). The ABILOCO-Kids scale was translated from English into Turkish using the forward-backward-forward method. The motor functions of the 63 children participating in the study were evaluated by the Gross Motor Function Classification System (GMFCS) and the Gross Motor Function Measurement-88 (GMFM-88). The ABILOCO-Kids scale was repeated in 30 children after one week to establish test-retest reliability. While internal and external construct validity were investigated using Rasch analysis and Spearman correlation coefficient, respectively; reliability was evaluated in terms of internal consistency by Cronbach’s alpha and Person Separation Index (PSI). All items of the ABILOCO-Kids were found to fit the Rasch Model (chi-square 14.35 (df?=?20), p?=?0.813). The internal construct validity was good, overall mean item fit residual was ?0.109 (SD: 0.719) and mean person fit residual was ?0.215 (SD: 0.817). The reliability was good with Cronbach’s Alpha of 0.98 and PSI of 0.99. When the test-retest was examined via Differential Item Functioning (DIF) by time, none of the items showed DIF. Spearman correlation coefficients of the ABILOCO-Kids scale in relation to the GMFM and GMFCS were r?=?0.824, p?<?0.001; r?=??0.788, p?<?0.001 respectively. The Turkish version of the ABILOCO-Kids scale is a valid, reliable and unidimensional scale for children with CP. This scale will allow the differences in the locomotion of children with CP to be evaluated from the perspective of the family.     

IgG4-related disease and ANCA positive vasculitis in childhood: a case-based review

Clinical Rheumatology - Autoimmune pancreatitis (AIP) type 1 is an IgG4-related disease (IgG4-RD), characterized by inflammatory pseudotumors and histologically by dense lymphoplasmacytic...     

A multicenter report of biologic agents for the treatment of secondary amyloidosis in Turkish rheumatoid arthritis and ankylosing spondylitis patients

In this multicenter, retrospective study, we evaluated the efficacy and safety of biologic therapies, including anti-TNFs, in secondary (AA) amyloidosis patients with ankylosing spondylitis (AS) and rheumatoid arthritis (RA). In addition, the frequency of secondary amyloidosis in RA and AS patients in a single center was estimated. Fifty-one AS (39M, 12F, mean age: 46.7) and 30 RA patients (11M, 19F, mean age: 51.7) with AA amyloidosis from 16 different centers in Turkey were included. Clinical and demographical features of patients were obtained from medical charts. A composite response index (CRI) to biologic therapy—based on creatinine level, proteinuria and disease activity—was used to evaluate the efficacy of treatment. The mean annual incidence of AA amyloidosis in RA and AS patients was 0.23 and 0.42/1000 patients/year, respectively. The point prevalence in RA and AS groups was 4.59 and 7.58/1000, respectively. In RA group with AA amyloidosis, effective response was obtained in 52.2 % of patients according to CRI. RA patients with RF positivity and more initial disease activity tended to have higher response rates to therapy (p values, 0.069 and 0.056). After biologic therapy (median 17 months), two RA patients died and two developed tuberculosis. In AS group, 45.7 % of patients fulfilled the criteria of good response according to CRI. AS patients with higher CRP levels at the time of AA diagnosis and at the beginning of anti-TNF therapy had higher response rates (p values, 0.011 and 0.017). During follow-up after anti-TNF therapy (median 38 months), one patient died and tuberculosis developed in two patients. Biologic therapy seems to be effective in at least half of RA and AS patients with AA amyloidosis. Tuberculosis was the most important safety concern.     

Topiramate‐induced nephrolithiasis

Nephrolithiasis is a less common side effect of the antiepileptic drug topiramate. We report the case of a 3‐year‐old boy who presented to the emergency department with abdominal pain; examinations revealed a large calcification in the left kidney. Regular ultrasound examinations are recommended in children using topiramate.