Notes

Abnormalities involving clean quest inside Parkinson's illness: A systematic evaluate.
To explore the spectrum of presentation, underlying monogenetic defects and outcome in very early onset inflammatory bowel disease (VEO-IBD).

The prospective, observational study was conducted at the Children's Hospital, Lahore, Pakistan, from January 2017 to December 2018, and comprised children developing features of inflammatory bowel disease aged <6 years. Data included demography, clinical presentation, diagnostic tools and outcome. Data was analysed using SPSS 21.

Of the 60 children with relevant symptoms, 26(43.3%) were diagnosed as having very early onset inflammatory bowel disease. Of them, 13(50%) had underlying monogenic defect, and 16(61.5%) had ulcerative colitis. There were 22(84.6%) males with median age of 1.5(11) months in monogenic inflammatory bowel disease versus 24(43) months for non-monogenic inflammatory bowel disease (p<0.05). In the monogenic group, isolated rectal bleeding was the major presentation 13(100%) versus non-monogenic who presented mainly with failure to thrive 13(100%). Upper and lower endoscopies with histopathology had good diagnostic yield and inflammatory infiltrates on the biopsied tissues were the major findings. Mutations detected among the subjects were XIAP, PRKDC, PIK3CD, RAG-1, LRBA, DOCK8, TTC7, MEFV and EPCAM. Mortality was significantly higher in the monogenic group 7(54%) than in the non-monogenic group 2(15%) (p<0.05).

Very early onset inflammatory bowel disease should be suspected when conventional management fails to rectify common disease mimickers. Testing for underlying immunological defect and genetic mutation would be helpful for managing these rare disorders.
Very early onset inflammatory bowel disease should be suspected when conventional management fails to rectify common disease mimickers. Testing for underlying immunological defect and genetic mutation would be helpful for managing these rare disorders.
To determine the clinical outcome and significance of pathological necrosis after neoadjuvant chemotherapy.

The retrospective study was conducted in Shaukat Khanum Memorial Cancer Hospital and Research Center and comprised data from January 2010 to December 2015 related to young with newly diagnosed Ewing sarcoma on histopathology. Data was collected on patients aged <20 years of either gender along with primary tumour site, metastatic status, tumour volume, mode of local therapy, degree of necrosis post-surgery, tumour margins after resection, outcome at the end of treatment and at last follow-up visit. Tumours were categorised as grade I = little or no necrosis, grade II = 50-90% necrosis, grade III = 90-99% necrosis, and grade IV = 100% necrosis. Data was analysed using SPSS 20.

Of the 124 patients, 89(72%) were non-metastatic; 35(28%) were metastatic; 37(29.8%) underwent surgery; 58(46%) received radiotherapy; 7(5.6%) received both surgery and radiotherapy; 22(17.7%) received no treatment. Histopathology report post-surgery showed little grade 1 necrosis in 10(8%) patients, grade II in 8(6.5%), grade III in 8(6.5%) and grade IV in 14(11%). Event-free survival in grade IV necrosis was 93% in 14 (11.3%) patients, EFS in grade III necrosis was 71% in 8(6.5%) patients , EFS in grade II necrosis was 22% seen in 9(7.3%) patients and EFS in grade- I necrosis was 35% seen in 14 (11.3%) patients. Overall survival in grade IV necrosis was 93% in 14 (11.3%) patients, OS in grade III 75% seen in 8(6.5%), OS in grade II 25% seen in 9(7.3%) and OS in grade I was 50% in 14(11.3%) patients. Event-free survival was 48 (38%) patients and overall survival of Ewing sarcoma patients was 52 (46.6%) patients respectively.

Tumour necrosis and histopathological changes post-surgery were found to have great impact on survival outcome in Ewing Sarcoma.
Tumour necrosis and histopathological changes post-surgery were found to have great impact on survival outcome in Ewing Sarcoma.
To identify the mental health problems among patients having symptoms of tinnitus and vertigo due to vestibulocochlear disturbances.

The cross-sectional study was conducted from May to September 2019 at the Hearts International Hospital and the Hearing and Balance Clinic, Rawalpindi, in collaboration with the Foundation University, Islamabad, and comprised vestibulocochlear disorder patients of either gender aged >18 years having chief symptoms of vertigo and tinnitus for at least the preceding 3 months. Psychiatric evaluation was conducted using the Tinnitus Handicap Inventory, the Dizziness Handicap Inventory and the Depression, Anxiety, Stress Scale. click here Audiological evaluations were carried out to gauge the severity of symptoms. Data was analysed using SPSS 20.

Of the 202 subjects, 60(30%) were females and 142(70%) were males. Patients experiencing vertigo and tinnitus symptoms had a strong tendency to develop psychiatric problems (p<0.05). Significant differences were noted among male and female patients regarding the representation of psychiatric symptoms (p<0.05).

Patients experiencing tinnitus and vertigo symptoms tended to have a deviant psychological profile analogous with other chronic diseases.
Patients experiencing tinnitus and vertigo symptoms tended to have a deviant psychological profile analogous with other chronic diseases.
To outline the genotype of Chikungunya virus strains and to form their phylogenetic tree.

The cross-sectional study was conducted at the Armed Forces Institute of Pathology, Rawalpindi, Pakistan, from August 2016 to September 2017, and comprised suspected Chikungunya samples. The viral ribonucleic acid was extracted and amplified. The purified product was sequenced using Sanger dideoxy method. Phylogenetic tree was constructed. Sequences generated were checked for alignment with sequences reported globally. Structural prediction and analysis was performed using Phyre2 and the models were visualised in PyMol2.2.

In the 11 suspected samples, 7(63.6%) were found to be positive for Chikungunya. Of them, 5(71.4%) sequences generated were found to be aligned with other full structural polyprotein sequences, having 99% similarity with amino acid sequences.

The Chikungunya strains in the study belonged to the East/Central/South African genotype.
The Chikungunya strains in the study belonged to the East/Central/South African genotype.
To compare the characteristics of connective tissue disease-associated interstitial lung disease with idiopathic pulmonary fibrosis at a tertiary care hospital.

The retrospective study was conducted at the Aga Khan University Hospital, Karachi, and comprised demographical, clinical and radiological data of patients with interstitial lung disease between October 2016 and October 2017 accessed through the outpatient data registry. Data was compared in terms of characteristics and key features of patients with connective tissue disease-associated interstitial lung disease with those of idiopathic pulmonary fibrosis. Statistical analysis was done using STATA 12.

Of the 184 patients, 52(29.3%) had connective tissue disease-associated interstitial lung disease and 62(35%) had idiopathic pulmonary fibrosis. The most prevalent conditions among connective tissue disease-associated interstitial lung disease patients were rheumatoid arthritis 22(42.3%) and scleroderma 13(25%). Compared to patients with idiopathic pulmonary fibrosis, those with connective tissue disease-associated interstitial lung disease were predominantly younger (p<0.001) and female (p<0.001). History of gastroesophageal reflux disease was also significantly lower in connective tissue disease-associated interstitial lung disease (p=0.05).

Connective tissue disease-associated interstitial lung disease patients were found to be younger and predominantly female compared to patients of idiopathic pulmonary fibrosis.
Connective tissue disease-associated interstitial lung disease patients were found to be younger and predominantly female compared to patients of idiopathic pulmonary fibrosis.
To evaluate the effect parental attitudes have on the dental caries experience of children.

The cross-sectional observational study was conducted at Yeditepe University, Istanbul, Turkey, from March to September 2019, and comprised mothers of children who came to the Department of Paediatric Dentistry for treatment of children. Multi-variable poisson regression model was run to predict prevalence of dental caries. Mothers' attitudes in family were measured using the parental attitude research instrument scores. Data was analysed using Stata 15.

There were 258 mothers of as many children aged 6-15 years. Among the children, 150(58%) were boys and 108(42%) were girls. 'Rejection of traditional housewife role' and 'level of discipline' had a statistically negative correlation (p<0.05), while 'level of marital conflict' had a statistically positive correlation with the prevalence of caries.

Healthy family environment and empowering parental style had a positive effect on the oral health of children.
Healthy family environment and empowering parental style had a positive effect on the oral health of children.
To compare improvement in symptoms following Heller's myotomy with Dor fundoplication and endoscopic pneumatic dilatation for the treatment of achalasia cardia.

The prospective comparative study was conducted at the Department of Upper Gastroenterology and Minimally Invasive Surgery, Civil Hospital, Karachi, from February 2016 to January 2019, and comprised patients diagnosed with achalasia cardia on oesophageal manometry. The subjects were randomised into endoscopic pneumatic dilatation group A and laparoscopic Heller's myotomy with Dor fundoplication group B. Eckardt scores were compared between the groups at one-year follow-up. Data was analysed using SPSS 22.

Of the 42 patients, 21(50%) were in each of the two groups. The mean age of patients in group A was 37±12.87 years compared to 34±8.59 years in group B. Treatment success in group A was 11(52%) compared to 21(76%) in group B. Eckardt scores between the groups were significant (p<0.001). Patient satisfaction was significantly more in group B (p<0.05).

The efficacy of Heller's myotomy with Dor fundoplication was found to be greater than endoscopic pneumatic dilatation for improvement in dysphagia and overall patient satisfaction at one-year follow-up.
The efficacy of Heller's myotomy with Dor fundoplication was found to be greater than endoscopic pneumatic dilatation for improvement in dysphagia and overall patient satisfaction at one-year follow-up.
To determine the occurrence and characteristics of lepra reactions in leprosy patients.

The retrospective study was conducted at the Marie Adelaide Leprosy Centre, Karachi, and comprised data of patients admitted between January 1, 2013, and December 31, 2015, for the management of lepra reactions. Data was noted on a detailed proforma and was analysed using Microsoft Excel and applying chi-square test.

Of the 130 cases, 95(73%) were males and 35(27%) were females. Mean age at onset of the first episode was 39±14 years. Borderline lepromatous was the most common classification 76(58%), with 40(53%) of them having type 1 reaction as the first episode and 36(47%) having a type 2 reaction Risk factors associated with recurrence were skin lesions, fever, lymphadenopathy and type of reaction (p<0.05).

Healthcare providers need to be aware of the clinical manifestations of lepra reactions in order to diagnose them early.
Healthcare providers need to be aware of the clinical manifestations of lepra reactions in order to diagnose them early.
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