Articles

There are variations in therapeutic regimens of different liver diseases. The accurate diagnosis ensNusinersen treatment is a novel therapy for spinal muscular atrophy (SMA) type 1; consequently, the adverse reactions of the therapy, have not been well known, yet. The present study is a case report that declares a hyponatremia development after the nursinersen therapy. Since the therapy is quite new one and has limited practice, we hope that this rare complication will contribute to the scientific literature.

Airway hyperresponsiveness (AHR) is a hallmark of persistent asthma measured using direct or indirect airway bronchial challenge testing. The purpose of this study is to investigate the putative relationships between type 2 inflammatory biomarkers, airway geometry (FEV1 and FEF25-75) and specific IgE (RAST or skin prick) to AHR. We performed a retrospective analysis of our database (n = 131) of patients with asthma. Of these subjects, 75 had a histamine challenge and 56 had a mannitol challenge. Fractional exhaled nitric oxide (FeNO) and specific immunoglobulin E (IgE) but not blood eosinophils were significantly higher in patients with AHR to either histamine or mannitol. FEV1 % and FEF25 - 75 % were significantly lower in patients with AHR. Elevated Type 2 biomarkers including FeNO and specific IgE but not blood eosinophils were associated with AHR. Highlights: FeNO and specific IgE but not blood eosinophils are raised in patients with airway hyperresponsiveness.

Objectives: There are variations in therapeutic regimens of different liver diseases. The accurate diagnosis ensures prompt recovery from these diseases. The present study aimed to evaluate the underlying causes of unexplained signs and symptoms associated with liver diseases through biopsies. Methods: A retrospective study was conducted in a public child care specialty of Lahore, Pakistan. The data was collected from medical records of the patients who were index hospitalized with unexplained clinical presentation of liver disease between 1st July, 2017 and 31st December, 2017. Data were analyzed by using Statistical Packages for Social Sciences (IBM SPSS Statistics for Windows, Version 21.0. Armonk, NY: IBM Corp.), and Microsoft Excel (MS Office 2010). Results: Overall, the records of 53 patients were selected for the study. Most of them were 11 to 15 years of age. The patients were presented with unexplained hepatomegaly (60.4%) and jaundice (40.7%) during index hospitalization which made them eligible for liver biopsy (LB). The findings of LB revealed that the underlying causes of liver diseases in most of the cases were metabolic (33.9%) and inflammatory disorders (22.6%). Majority of the patients were ≤4 years of age, however cryptogenic cirrhosis (39.1%) was commonly found in >10 years of age. Although most of the patients were suffering from metabolic disorders (p-value=0.07) and liver cirrhosis (p-value=0.08) but these were not statistically significant. Conclusions: LB was beneficial in evaluating the etiologies of unexplained signs and symptoms of liver diseases. It was found that glycogen storage diseases and liver cirrhosis were the most common etiologies of liver diseases among pediatric patients. But etiologies like metabolic and inflammatory diseases were insignificantly associated with gender.

Disruptions in Maternal-infant Bonding are shown to be the mediating variable between maternal distress and the subsequent expression of childhood asthma. When the mothers’ bonding is repaired, their children’s asthmatic symptoms diminish or remit. This study evaluated 16 asthmatic children before and after their mothers were treated with Bonding Therapy. Fourteen improved on 11 measures, including reduction in the STEP classification system and medication use. Thirteen children were able to stop all medications. Surprisingly, all mothers scores on the Beck Depression Inventory improved through Bonding Therapy, suggesting that impaired bonding can lead to maternal depression or even Postpartum Depression. The link between bonding disruptions and airway inflammation are discussed. Bonding Therapy is described.

Cystic fibrosis (CF) is a hereditary syndrome composed of exocrine gland dysfunction involving multiple systems which if untreated may result in chronic respiratory infections, pancreatic enzyme deficiency and failure to thrive. The association between CF and other inherited diseases or congenital anomalies is rare. We describe a rare case of CF with concomitant congenital adrenal hyperplasia (CAH). 21- Hydroxylase deficiency accounts for most CAH cases. Varity in clinical phenotypes depends on the amount of enzymatic activity which in turn depends on different combination of gene mutations. The genes of CAH and CF are located in different locations. The chance of these diseases coexisting in our patient would be a rare combination. However, such a case will be more frequent in our population than others because of consanguineous marriage and common ancestors. There are diagnostic difficulties, similarities and contradictions between two diseases and they are pointed out.

Treatment of various inflammatory processes, including acute pneumonia(АP), over the past decades is identical and does not reflect the specifics of a particular disease. The basis of such treatment is «antibiotics alone». The need for additional therapeutic efforts is realized by the use of General therapeutic techniques, regardless of the diagnosis. This does not take into account the important fact that the localization of inflammation not only determines its clinical picture,but,above all,the mechanisms of influence on other organs and systems of the body.

Introduction: There is currently no strategy for identifying chronic obstructive pulmonary disease (COPD) patients whose pulmonary function could benefit from inhaled corticosteroids. We investigated whether a 28-day regime of inhaled corticosteroids improved pulmonary function test results among COPD patients with a fractional exhaled nitric oxide concentration > 35 parts per billion. Methods: This single-centre one-arm pre–post trial included COPD patients with a fractional exhaled nitric oxide concentration > 35 parts per billion treated at our institution from September 2018 to August 2019. Patients were administered budesonide (200 μg, 8 puffs daily) for 28 days. The primary outcome measure was the difference between the forced expiratory volume in 1 s (FEV1) at baseline and after 28 days of inhaled corticosteroid treatment. Secondary outcomes included differences in COPD Assessment Test scores, %FEV1, and that between the percent forced vital capacity (%FVC) at baseline and after 28 days of treatment. Results: Twenty patients completed the 28-day inhaled corticosteroid regime. The mean difference in FEV1 between day 1 and day 28 was 340 mL (95% confidence interval: −100 to 770 mL; p = 0.122). The mean differences in secondary outcomes were: %FVC, −0.16% (95% confidence interval [CI]: −2.84 to 2.53%; p = 0.905); %FEV1, 1.63% (95%CI: −4.56 to 7.81%; p = 0.589); COPD Assessment Test score, −2.50 (95%CI: −5.72 to 0.72; p = 0.121). Conclusion: The 28-day course of inhaled corticosteroids yielded no significant difference in FEV1 for COPD patients with a fractional exhaled nitric oxide concentration > 35 parts per billion. Trial registration: University Hospital Medical Information Network Center, UMIN000034005. Registered 3 September 2018. https://upload.umin.ac.jp/cgi-open-bin/ctr/ctr_view.cgi?recptno=R000038557

Background: Intestinal obstruction (IO) is defined as a partial or complete blockage of the bowel that results in the failure of intestinal contents to pass through. It is a common cause of emergency surgical problems. IO has been the leading cause of acute abdomen in several African countries. Objective: To assess surgical management outcome of intestinal obstruction by IESO professionals and its associated factors of intestinal obstruction in surgically treated patients at South Wollo zone. Method: A cross-sectional study was done on 216 patients ‘the data was collected from medical cards of the patient by using a pretested data abstraction format. Three nurses were involved in the process of data collection. The collected data was cleaned, coded and analyzed by SPSS version 23 statistical package. First descriptive statics was done for categorical and analyzed using frequencies and percentage. Multivariable logistic regression models was used to determine the association factors on the management outcome of intestinal obstruction when p - value < 0.05 and the strength of statistical association was measured by adjusting odds ratio and 95% confidence interval. Statistical significance was taken at p - value < 0.05. Results: From all study participants about 177 (82%) of them had good surgical outcome. Study participants who were managed by 1-3 years of work experience of IESO {(p = 0.004, AOR (95% CI) = 7.2[1.89, 27.68]}, preoperatively diagnosed as small bowel obstruction {(p = 0.001, AOR (95% CI) = 4.5[1.91, 10.40], Surgery conducted at day time {(p = 0.03, AOR (95% CI) = 2.8[1.06, 7.16]} had shown positive association with management outcome of intestinal obstruction conducted by IESO professionals. Conclusion and recommendation: Majority patients with intestinal obstruction had good surgical outcome done by IESO professionals. Year of experience of IESO Workers, preoperative diagnosis and time of surgery of the respondents had shown positive association for the occurrence of chronic liver disease whereas. In this study we can conclude that surgeries of intestinal obstruction conducted by IESO professionals are as good as intestinal obstruction surgeries conducted by physicians so there is a need to train more IESO professionals to deliver decentralized surgical service for rural areas.

Background: Pulmonary fibrosis is a clinical problem with an enigmatic etiology with no effective therapy. Current therapies for lung fibrosis are ineffective for progression of lung fibrosis and preventing respiratory failure. Objectives: The aim of this study is to explore the expression of Desmin, α-smooth muscle actin (α-SMA) and the telomerase subunit: human telomerase reverse transcriptase (h-TERT) in a spectrum of lung tissue samples consist of lung fibrosis, lung cancer, and healthy controls. Materials and Methods: The expression of Desmin, α-SMA and hTERT were studied in samples of 15 pulmonary fibrosis samples, 16 samples of lung cancer and 14 healthy controls investigated. We evaluated Desmin, α-SMA as well as the expression of components of telomerase (TERT), by methods: RNA Extraction and cDNA synthesis, Real-Time quantitative PCR, Immunohistochemistry, all prepared from lung tissue paraffin blocked. Results: α-SMA marker detected 1(8.3%) of healthy control and 11(91.7%) of lung fibrosis samples. The difference between groups was significant (p<0.001). Also the difference between healthy control 1(6.7%) and lung cancer 14 (93.3%) for α-SMA marker was a significant (P<0.001). It was a significant difference between healthy control and lung cancer for TERT expression (P=.005). TERT was not positive in any sample of neither healthy control nor lung fibrosis. For TERT, it was a significant difference between lung fibrosis and lung cancer by Fisher’s Exact Test (P=.004). Expression of TERT and α-SMA between small cell lung cancer (SCLC) and non-small cell lung cancer (NSCLC) was not statistically significant (P=.700, P=0758), respectively. Conclusions: We recommend more investigation to regard α-SMA, Desmin in patients with lung fibrosis and follow them for possible cancer risk. Also, more study is needed to regard TERT as a marker in lung cancer. Assessment of these markers may have future implication to explain the same way of pathogenesis and carcinogenesis of fibrosis and cancer and for prevention or treatment

Familial Mediterranean fever (FMF) is an autosomal recessive autoinflammatory disease caused by mutation(s) in the Mediterranean fever (MEFV, pyrinmarenostrin) gene [1,2]. FMF is characterized by recurrent fever crises combined with serosal, synovial, or cutaneous inflammation and, in some individuals, by the eventual development, in the long-term, of systemic amyloidosis [3,4]. FMF mainly affects peoples living along eastern Mediterranean Sea (Turks, Sephardic Jews, Armenians) and it is not a rare disease in other Mediterranean areas such as Greeks, Italians and Iranians [4,6]. Until now, more than 304 sequence variants have been recorded [6]. In Italy M694V, V726A, M680I, M694I and E148Q are the most frequent FMF-associated mutations [7]. Here, we describe a recent case of mild FMF, characterized by all the clinical manifestations indicative of FMF described in the literature, according to Tei-Hashomer criteria [4] and by the analysis of MEFV gene, characterized by polymorphism c1588-69G>A. This is in agreement with previous our observations in a wider sample collected in the years. We are training to define the relations among gene mutations and clinical forms of FMF.