A Rare Case of Anti-TIF-1γ Antibody Positive Dermatomyositis in Adulthood.

Dermatomyositis (DM) presents with inflammatory myopathy and distinct skin manifestations, often linked to specific autoantibodies. Anti-transcriptional intermediary factor-1 gamma (TIF-1γ) antibodies (Abs) are typically linked to DM in older patients and malignancy in 15% to 40% of cases. We highlight a case of a 24-year-old female who presented with weakness of proximal muscles, periorbital edema, heliotrope rash, erosions on oral mucosa, and painful scaly rash on the lower extremities. Transcriptional intermediary factor-1 gamma Abs were positive, confirming inflammatory myopathy. Treatment with steroid pulse therapy and immunoglobulin led to improvement. Evaluation for malignancy yielded unremarkable results. This case underscores the importance of recognizing and managing DM with TIF-1γ Ab positive, even in atypical demographics, and highlights the need for comprehensive malignancy evaluation.

Primary Circumscribed Meningeal Melanoma Involving the Meckel's Cave: A Report of a Rare Case and Review of Literature.

Primary intracranial meningeal melanomas are rare. Diagnosing primary meningeal melanomas mostly involves comprehensive assessment through clinical and radiological means. This evaluation should encompass a detailed dermal and ophthalmic examination. Any suspicious lesion must be biopsied and examined microscopically. This is crucial not only to differentiate primary intracranial melanoma from other brain tumors but also to rule out metastases from potential sources of primary cutaneous or non-cutaneous melanomas. Surgery is considered the mainstay of treatment. Despite melanomas being generally considered radio- and chemo-resistant tumors, adjuvant radiotherapy and chemotherapy still play a crucial role in their management. The treatment landscape for primary meningeal melanoma is continually evolving, with ongoing research aiming to improve outcomes for patients with this challenging disease.

Clinical and Genetic Description of Hereditary Chronic Pancreatitis in Pakistani Children.

BACKGROUND/AIMS: The purpose of this study was to identify the spectrum and frequency of pathogenic variants as well as the clinical and genetic insight of hereditary chronic pancreatitis in Pakistani children. MATERIALS AND METHODS: The deoxyribonucleic acid of affected probands of 44 unrelated Pakistani families, having hereditary chronic pancreatitis-affected children, were subjected to massive parallel sequencing for candidate reported genes (SPINK1, PRSS1, CFTR, CPA1, CTRC, CBS, AGL, PHKB, and LPL). Data were analyzed using different bioinformatics tools for the variants and in-silico analysis. All the identified variants were validated by direct sequencing of the targeted exons in the probands and their parents. RESULTS: There were 50 patients included in this study with confirmed hereditary chronic pancreatitis. Nine known mutations in SPINK1, PRSS1, CFTR, CTRC, CBS, and AGL genes, and 10 novel variants in LPL, CFTR, CTR, and PHKB genes were identified. The identified variants were found in heterozygous, compound heterozygous, and trans-heterozygous forms, with rare allele frequency in the normal population. The novel variants were [c.378C>T(p.Lys126Asn) and c.719G>A(p.Arg240Gln) in CTRC, c.586-3C>A and c.763A>G(p.Arg255Gly) in CPA1, c.1160_1161insT(p.Lys387Asnfs*26), c.784C>T(p.Gln262*), c.1139+1G>A, c.175G>A(p.Gly59Arg) in LPL, c.388C>G(p.leu130val) in CFTR, and c.2327G>A(p.Arg776His in PHKB)]. The phenotypic characteristics were variable and correlated with the relevant variant. CONCLUSIONS: The genetic composition plays a significant role in the predisposition of hereditary chronic pancreatitis. The clinical presentation varies with the genetic determinant involved. This information would help in building up a diagnostic algorithm for our population that can be used for genetic screening services in affected cohorts.

Comparative Evaluation of Novel Herbal Rice Husk Mouthwash with Kidodent against Streptococcus mutans: A Parallel Double-blinded Randomized Control Trial.

Aim: The purpose of the study is to evaluate and compare the effectiveness of rice husk extract mouthwash (RHM) and Kidodent mouthwash (KM) for reduction in salivary Streptococcus mutans count. Materials and methods: After approval from institutional review board and institutional informed consent, 45 children who met the inclusion criteria were divided into three groups. In group A, children received rice husk mouth wash whereas in group B placebo is specified and in group C, intervention being KM. The unstimulated saliva is collected at baseline, 7th, 10th, and 15th days and subjected to microbiological analysis. The data are statistically analyzed using one-way ANOVA and repeated measures analysis of variance (ANOVA). Results: Rice husk mouthwash showed equivalent, significant, and effective reduction in S. mutans count similar to KM (p < 0.05). Conclusion: The rice husk mouthwash showed potential therapeutic effect in reduction of S. mutans.Clinical trial registry india (CTRI No) : CTRI/2020/10/028594. How to cite this article: Havale R, Rao DG, Bemalgi N, et al. Comparative Evaluation of Novel Herbal Rice Husk Mouthwash with Kidodent against Streptococcus mutans: A Parallel Double-blinded Randomized Control Trial. Int J Clin Pediatr Dent 2022;15(3):356-361.

Very early onset inflammatory bowel disease: Spectrum of clinical presentation, diagnostic tools and outcome in children.

OBJECTIVE: To explore the spectrum of presentation, underlying monogenetic defects and outcome in very early onset inflammatory bowel disease (VEO-IBD). METHOD: 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. RESULTS: 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). CONCLUSIONS: 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.

Hepatic Glycogenosis In Children: Spectrum Of Presentation And Diagnostic Modalities.

BACKGROUND: Objectives of the study were to determine the clinical spectrum of presentation and various modalities helpful in the diagnosis of liver glycogenosis short of genetic analysis. METHODS: All patients under 18 years of age presenting to Paediatric Gastroenterology unit of Children's Hospital, Lahore with suspicion of hepatic glycogen storage disease (GSD) were enrolled over a period of 18 months. Demographic profile and various factors under observation were recorded. Collected data was analysed using SPSS version 22. RESULTS: Among 89 enrolled patients F:M ratio was (1.28:1). The most common GSD was type I (71, 79.7%) followed by III (13, 14.6%), II (3, 3.3%), IV (1, 1.1%) and IX (1, 1.1%). The Abdominal distension was the most common presentation in 89.5% followed by hepatomegaly in 86.5%, diarrhoea in 41.6%, doll's like appearance in 31.5% and vomiting, acidotic breathing with convulsions in about 20% of children in GSD I. Hepatomegaly (100%), failure to thrive (85%), developmental delay (69%) and splenomegaly (92.3%) were leading presentation in GSD III. Elevated triglycerides (77.5%) followed by transaminesemia (56%), hypercholesterolemia (63%), hyperuricemia (32%) and hypoglycaemia (14%) were significant biochemical findings in GSD I. Consistently raised liver enzymes (92%) and creatinine phosphokinase (100%) in addition to hypertriglyceridemia (69%) were seen in GSD III. The presence of enlarged hepatocytes with clearing of cells favour GSD1 showed in 79% of children while fibrosis and steatosis usually seen in GSD-III (14.6%). CONCLUSIONS: Hepatic glycogen storage diseases are serious health issues and should be excluded in any patient who present with hepatomegaly, short stature and hyperlipidaemia to decrease the disease mortality and morbidity.

Protein requirement and its intake in subjects with diabetic foot ulcers at a tertiary care hospital.

OBJECTIVE: To assess the protein intake and requirement among subject with type 2 diabetes having foot ulcers. METHODS: This study was conducted at Baqai Institute of Diabetology & Endocrinology (BIDE), a tertiary care diabetes centre of Karachi, Pakistan among people with type 2 diabetic foot ulcer attending foot clinic from January 2012 to March 2015. The baseline characteristics, dietary intake and laboratory investigations of the study participants were obtained through electronic hospital database "Health Management System" (HMS) based on the 24 hours dietary recall interview. Total grams of protein were calculated from each food group consumed by the subject. Protein intake of the subjects was recorded in mean grams and the protein requirement was calculated according to their body weight. The comparison of intake and requirement of protein choices was done through comparing the mean of both variables. SPSS version 13 was used for analysing the results. RESULTS: A total of 542 subjects were included in the study, 365 (67.2%) were males and 178 (32.8%) were females. Mena age of the subject was 54.61±10.51 (yrs) with the duration of diabetes and mean body mass index were 14.22±7.98 (yrs) and 26.65±5.38 (kg/m2), respectively. The dietary records showed the protein intake of subjects with diabetic foot ulcer is not appropriate when compared to daily requirement. Mean grams of protein intake is 76.87gms in males and 56.84gms in females. On the other-hand protein requirement is much higher than the intake, which is 219.5gms in males and 130.2gms in females. CONCLUSION: Dietary counselling should be a part of the treatment among subjects with diabetic foot ulcer to identify their nutritional needs and suggesting them better option to fulfil their protein requirement essential for wound healing process.

Secondary Ameloblastic Carcinoma of Mandible with Pulmonary Metastasis.

Ameloblastic carcinoma (AC) is a very rare odontogenic malignancy. Primary AC is more common in maxilla, while secondary AC is more common in mandible. Histologically, it is an ameloblastoma with features of cancer. Presence of metastasis is not necessary to establish the diagnosis. This tumor usually metastasises in bone or lungs. We report a case of aggressive secondary AC of mandible with lung metastasis. There are few cases in medical literature discussing management of AC with lung metastasis. In this case, a 42-year woman, who was diagnosed with ameloblastoma of right mandible, was treated with right hemi-mandibulectomy. However, 10 years later, the patient developed AC with pulmonary metastasis. Patient received six cycles of carboplatin-paclitaxel-based regimen. There was noted a partial response to the therapy. However, the patient went into respiratory failure, although she was provided adequate analgesic control as part of end-of-life care. Further, oncological or surgical management was not possible as patient had poor performance status.

Laryngeal cancer: Global socioeconomic trends in disease burden and smoking habits.

OBJECTIVES/HYPOTHESIS: To characterize health burden and determine the associated level of equality of laryngeal carcinoma (LC) burden at a global level. METHODS: One hundred eighty-four countries were organized by socioeconomic status using Human Development Index (HDI) categorizations provided by the United Nations Development Program. Disability-adjusted life years (DALYs), obtained from The Global Health Data Exchange, were calculated and compared between each HDI category for the period from 1990 to 2015. Equality of LC burden was then evaluated with concentration indices. RESULTS: Global LC burden, as measured by age-standardized DALYs, has improved significantly over the 25-year period studied. This burden has declined for very high, high, and medium HDI countries, whereas it has remained unchanged for low HDI countries. The majority of LC global burden was found in high socioeconomic countries before 2010 and has shifted toward low socioeconomic countries, as indicated by concentration indices. Over the last 25 years, Central and Eastern Europe continue to have the largest disease burden in the world. CONCLUSION: This is the first analysis that we are aware of investigating health disparities of LC at a global level. The global burden of the disease has declined, which is a trend corresponding with significantly reduced smoking behaviors in developed countries. Although the global inequality gap decreased between 2010 and 2015, there remain reasons for concern. Smoking continues to trend upward in low socioeconomic countries, which could increase LC burden in low socioeconomic countries in the near future. A new global initiative directed toward low socioeconomic countries may yield dividends in preventing subsequent disparities in the LC burden. LEVEL OF EVIDENCE: 4. Laryngoscope, 128:2039-2053, 2018.

Electrocardiographic Manifestations In Paediatric Wilson Disease.

BACKGROUND: Wilson disease (WD) is one of the most common metabolic liver diseases in older children. It has a strong genetic background with autosomal recessive inheritance. WD is a multisystem disorder with predominant hepatic and neurological manifestations and variable age of presentation. The data on cardiac manifestations in children is very limited and only few adult studies are available in the literature. This study was planned to determine the frequency and spectrum of Electrocardiographic (ECG) changes in pediatric WD. METHODS: This was an observational cross-sectional study conducted at The Children Hospital & the Institute of Child Health, Lahore, from January 2015 to January 2017. The children diagnosed as Wilson disease were enrolled for the recording of resting ECG. The ECG changes were seen and discussed with an experienced pediatric cardiologist who was involved and explained about the objectives of study. RESULTS: Total 55 patients were enrolled but record of ECG was missing for 4 patients and excluded from the study. Out of 51 patients 22 had at least one ECG abnormality. Most frequent findings seen were T wave abnormality in 18 patients (35.2%) followed by sinus tachycardia and sinus bradycardia in 12 and 8 patients respectively. Other abnormalities included bifid P waves, ST segment changes each of 2 patients, and one premature ventricular contraction. QRS details including axis, complex, amplitude ratio and QT interval was normal in all the patients. There was no mortality during the study period due to cardiac cause. CONCLUSIONS: ECG abnormalities are not uncommon in pediatric WD but of mild nature. These are presumably related to underlying cardiomyopathy due to deposition of copper in heart which can be quantified by cardiac magnetic resonance imaging (MRI) and echocardiography is required to confirm ECG abnormalities detected.

Ultra-rapid catalytic degradation of 4-nitrophenol with ionic liquid recoverable and reusable ibuprofen derived silver nanoparticles.

This study reports a one-pot and eco-friendly method for the synthesis of spherical ibuprofen derived silver nanoparticles (IBU-AgNPs) in aqueous media using ibuprofen analgesics drug as capping as well as reducing agent. Formation of AgNPs occurred within a few min (less than 5 min) at room temperature without resorting to any harsh conditions and hazardous organic solvents. Synthesized AgNPs were characterized with common analytical techniques. Transmission electron microscope (TEM) images confirmed the formation of spherical particles having a size distribution in the range of 12.5 ± 1.5 nm. Employment of IBU analgesic aided the control of better size distribution and prevented agglomeration of particles. Such AgNPs solution was highly stable for more than two months when stored at ambient temperature. The IBU-AgNPs solution showed excellent ultra-rapid catalytic activity for the complete degradation of toxic 4-nitrophenol (4-NPh) into non-toxic 4-aminophenol (4-APh) within 40 s. AgNPs were recovered with the help of water insoluble-room temperature ionic liquid and reused with enhanced catalytic potential. This method provides a novel, rapid and economical alternative for the treatment of toxic organic pollutants to maintain water quality and environmental safety against water pollution. It is extendable for the control of other reducible contaminants in water as well. Furthermore, this catalytic activity for an effective degradation of organic toxins is expected to play a crucial role for achieving the Sustainable Development Goal 6 set by United Nations.

Phosphomolybdate-doped-poly(3,4-ethylenedioxythiophene) coated gold nanoparticles: synthesis, characterization and electrocatalytic reduction of bromate.

Phosphomolybdate, H3PMo12O40, (PMo12)-doped-poly(3,4-ethylenedioxythiophene) (PEDOT) coated gold nanoparticles have been synthesized in aqueous solution by reduction of AuCl4(-) using hydroxymethyl EDOT as a reducing agent in the presence of polystyrene sulfonate and PMo12. The resulting PMo12-doped-PEDOT stabilized Au nanoparticles are water soluble and have been characterized by UV-visible spectroscopy, scanning electron microscopy and electrochemistry. Glassy carbon electrodes modified with these Au nanoparticles show excellent stability and catalytic activity towards the reduction of bromate in an aqueous electrolyte solution containing 10mM H2SO4 and 0.1M Na2SO4.

Ultra-fast catalytic reduction of dyes by ionic liquid recoverable and reusable mefenamic acid derived gold nanoparticles.

We synthesized mefenamic acid (MA) derived gold nanoparticles (MA-AuNps) in aqueous solution (MA-Au sol). Scanning Electron Microscopy (SEM) and Transmission Electron Microscopy (TEM) of the sol at 1, 5, 15 and 60 min showed changes in size and shape of formed AuNps. Fourier Transform Infrared (FTIR) Spectroscopy revealed the interaction between AuNps and MA. Each Au sol exhibited exceptional catalytic activity for the reduction of Methylene Blue (MB), Rose Bengal (RB) and Eosin B (EB) dye individually as well as collectively. However, complete reduction of dye(s) was accomplished by Au sol of 5 min in just 15s. The catalytic performance of Ma-Au sol was far superior to that adsorbed on glass. AuNps were recovered with the help of water insoluble room temperature ionic liquid and reused with enhanced catalytic potential. This finding is a novel, rapid and highly economical alternative for environmental safety against pollution by dyes and extendable for control of other reducible contaminants as well.