IgG4-Related Neurological Disease: A Single Center Ambispective Study from South India.

IgG4-related disease (IgG4-RD) is an immune-mediated multi-system disorder. The nervous system (IgG4-RND) is rarely affected. We describe a short case series. We performed an ambispective analysis of IgG4-RND patients admitted at our centre between January 2016 and December 2022. Eight patients (M: F-2:6) were included with a mean age at presentation of 40.63 ± 17.88 years and disease duration of 5.16 ± 4.08 years. The common diseased sites were pachymeninges (7), orbits (4), paranasal sinuses (3), frontal lobe (1), hypophysis (1), leptomeninges (1), and middle ear (1). Common symptoms were headache and cranial neuropathy. The common nerves involved were the optic nerve, followed by the third, fifth, sixth, and seventh. Cerebrospinal fluid showed lymphocytic pleocytosis. Histopathology showed lymphoplasmacytic infiltrate (8), fibrosis (5), >10 IgG4 + cells (7), and IgG4/IgG >40% (6). Six had a relapsing course. The rituximab-based treatment regimen showed a favourable response.

Outcomes of Neonatal Resuscitation With and Without an Intact Umbilical Cord: A Meta-Analysis.

Around the world, very few babies require a more intensive resuscitative effort for stabilization. The optimal timing of an intact umbilical cord to help with resuscitation is controversial. Our objective in the review is to compare the outcomes of neonatal resuscitation with and without an intact umbilical cord. A search of six electronic database libraries was explored for data released between 2014 and 2023. A manual search of secondary references in relevant studies was also performed. Studies focused only on randomized controlled trials comparing the outcomes of neonatal resuscitation with and without an intact umbilical cord at any gestational age. Two reviewers retrieved data for relevant outcomes and independently evaluated trial quality and eligibility. Mortality rate and APGAR (appearance, pulse, grimace, activity, and respiration) scores were noted as common in the two studies. Four randomized control trials were assessed for the impact of delayed cord clamping on neonates. One study focused on neurodevelopmental outcomes and noted significant improvement. Other studies noted delayed clamping as beneficial for improving oxygen saturation, APGAR score, and mortality rate. The meta-analysis included three controlled trials with a total of 528 babies and tested the effects of clamping the umbilical cord either late (n = 264) or early (n = 264). The heterogeneity of mortality and APGAR score at 5 minutes were not significant, which may be because only two studies of each case were available to compare. We concluded that very few studies are available to identify a significant impact of delayed cord clamping in neonates. However, delayed clamping for up to 5 minutes is noted as beneficial to the newborn.

Proband and the Brother.

INTRODUCTION: Camurati-Engelmann disease (CED) is a rare autosomal dominant disease. It is characterized by hyperostosis of the long bones and the skull, Clinically patient will have limb pain, proximal muscle weakness a wide-based gait. The gene causing CED is located on chromosome 19, this region contains the gene encoding the TGF Beta -1. The diagnosis of CED is established in a proband with the characteristic radiographic findings and molecular genetic testing for TGF Beta-1 mutation. Treatment is with corticosteroids and Losartan. MATERIALS: A 40 year old lady presented with complaints of Left lower limb pain for 1 year duration. On examination there was tenderness of left greater trochanter, proximal and distal femur was present. Blood investigations showed high PTH and low Vitamin-D3. Imaging showed non specific sclerotic lesions in femur. As patient brother had limp since childhood genetic disorders were and a provisional diagnosis of sclerotic bone disease probable Progressive diaphyseal dysplasia was considered. PET-CT was done which revealed abnormal osteoblastic activity in both femurs, focal hyperostosis in humeral diaphysis suggestive of CED. She was tested Positive for TGF beta 1 mutation consistent with CED. He was started on LOSARTAN. On follow up patient is pain free. RESULT: Her brother was also evaluated in view of his limp and he was also diagnosed as CED. CONCLUSION: The diagnosis in this case was based on the clinical history, family history and characteristic radiological findings and genetic testing which confirmed TGF Beta-1 mutation. Family history is crucial in this case which led to diagnosis. References Van Hul W, Boudin E, Vanhoenacker FM, et al. Camurati Engelmann disease. Calcif Tissue Int 2019;104(5):554-560. Camurati-Engelmann Disease. NORD (National Organization for Rare Disorders); 2022.

The Right Leg that Troubled the Left Leg.

INTRODUCTION: Oncogenic osteomalacia or tumor induced osteomalacia (TIO) is a rare acquired paraneoplastic syndrome characterized by defective bone mineralization secondary to release of Fibroblast Growth Factor-23 (FGF-23), a phosphaturic protein - released from small, slow-growing mesenchymal tumors. Here, we report such a case and how it was investigated. MATERIALS: A 31 year old female presented with history of left leg pain and difficulty in walking since 1 year. General and systemic examination were found to be within normal limits and initial diagnostic workup revealed elevated alkaline phosphatase. X-ray bilateral hip and legs showed pseudo fractures of femur and tibia. Hence a probable diagnosis of metabolic bone disease was considered and further workup showed isolated hypophosphatemia. Patient was worked up for hypophosphatemic osteomalacia and further investigations showed low Tmp-GFR with a high FGF23 level. Hence a diagnosis of oncogenic osteomalacia was considered and a whole body PET scan was done which showed evidence of mesenchymal tumor in the right lower limb. Removal of the tumor resulted in resolution of symptoms and hence the diagnosis of oncogenic osteomalacia was confirmed. RESULT: Hypophosphatemia Normal S. Calcium and S. Vitamin D3 levels Conclusion: Oncogenic osteomalcia is a rare paraneoplastic form of renal phosphate wasting that results in severe hypophosphatemia and has excellent prognosis as surgical removal of the causative tumor results in dramatic improvement. High index of suspicion combined with prompt investigations can result in early diagnosis of the causative tumor and proper surgical treatment which will improve outcomes. Reference Chong WH, Molinolo AA, Chen CC, et al. Tumor-induced osteomalacia. Endocr Relat Cancer 2011;18(3):R53-R77.

SARS-CoV-2 seroprevalence in the city of Hyderabad, India in early 2021.

Background: COVID-19 emerged as a global pandemic in 2020, spreading rapidly to most parts of the world. The proportion of infected individuals in a population can be reliably estimated via serosurveillance, making it a valuable tool for planning control measures. Our serosurvey study aimed to investigate SARS-CoV-2 seroprevalence in the urban population of Hyderabad at the end of the first wave of infections. Methods: This cross-sectional survey, conducted in January 2021 and including males and females aged 10 years and above, used multi-stage random sampling. 9363 samples were collected from 30 wards distributed over six zones of Hyderabad, and tested for antibodies against SARS-CoV-2 nucleocapsid antigen. Results: Overall seropositivity was 54.2%, ranging from 50% to 60% in most wards. Highest exposure appeared to be among those aged 30-39 and 50-59 years, with women showing greater seropositivity. Seropositivity increased with family size, with only marginal differences among people with varying levels of education. Seroprevalence was significantly lower among smokers. Only 11% of the survey subjects reported any COVID-19 symptoms, while 17% had appeared for COVID-19 testing. Conclusion: Over half the city's population was infected within a year of onset of the pandemic. However, ∼ 46% of people remained susceptible, contributing to subsequent waves of infection.

Delayed Spontaneous Thrombosis of Neglected Direct Carotid-Cavernous Fistula: A Case Report.

Direct carotid-cavernous fistula (CCF) refers to direct communication between the cavernous portion of the internal carotid artery (ICA) and the cavernous sinus due to rent in the ICA, most commonly secondary to trauma. These are generally high-flow fistula and rarely resolve spontaneously. We report a case of a young male who developed features of direct CCF after trauma, was denied any treatment for 4 years, and then presented with spontaneous thrombosis of the fistula and a residual large pseudoaneurysm of the cavernous segment of the right ICA, which was subsequently managed with parent vessel occlusion.

Isolation and genetic characterization of mutans streptococci associated with dental caries in rural field practice of a dental institution: In vivo study.

BACKGROUND: Streptococcus mutans is well-known causative microorganism in the development of dental caries because they drop the plaque pH and produce acids from carbohydrates and survive in the acidic environment. It is now evident that knowledge of the bacteria enforces empirical approach to therapy, then specific antimicrobial therapy that might allow more conservative treatment options. Over the past few decades, there has been a remarkable increase in the prevalence rate of dental caries among children and the elders. Genotypic methods help in the detection and manipulation of nucleic acids which allows microbial genes to be examined directly. AIM: The aim of this study is to isolate and characterize S. mutans from rural population and to obtain genomic DNA and screen DNA band pattern. METHODOLOGY: A total of 80 plaque samples were collected from the buccal surfaces of maxillary and lingual surfaces of mandibular first molar with carious teeth in patients at a rural outreach center in Chikkaballapur district, Karnataka. Among these, 48 clinical isolates of S. mutans were recovered. Further, genomic DNA was extracted from all the positively isolated strains including the standard strain (microbial type culture collection 497), and stored at 4°C in tris EDTA buffer (TE). To analyze the molecular heterogeneity of the clinical strains, polymerase chain reaction (PCR), and restriction fragment length polymorphism was performed using restriction enzymes Hind III and Hae III. Using agarose gel electrophoresis, genomic DNA band pattern was analyzed. RESULTS: Statistically significant difference was seen in the "dex" gene collected from sample DNA and standard DNA in three different parameters (S. mutans 497). CONCLUSION: Genomic DNA of S. mutans was successfully isolated from the rural population. Dex gene was successfully amplified using PCR. Hae III enzymes successfully digested PCR amplicons and the fragments exhibited visible heterogeneity.

Tuning of IMC based PID controllers for integrating systems with time delay.

Design of Proportional Integral and Derivative (PID) controllers based on IMC principles for various types of integrating systems with time delay is proposed. PID parameters are given in terms of process model parameters and a tuning parameter. The tuning parameter is IMC filter time constant. In the present work, the IMC filter (Q) is chosen in such a manner that the order of the denominator of IMC controller is one less than the order of the numerator. The IMC filter time constant (λ) is tuned in such a way that a good compromise is made between performance and robustness for both servo and regulatory problems. To improve servo response of the controller a set point filter is designed such that the closed loop response is similar to that of first order plus time delay system. The proposed controller design method is applied to various transfer function models and to the non-linear model equations of jacketed CSTR to demonstrate its applicability and effectiveness. The performance of the proposed controller is compared with the recently reported methods in terms of IAE and ITAE. The smooth functioning of the controller is determined in terms of total variation and compared with recently reported methods. Simulation studies are carried out on various integrating systems with time delay to show the effectiveness and superiority of the proposed controllers.

Awareness of the association between periodontal disease and adverse pregnancy outcome among the general female population.

BACKGROUND: Preterm low birth weight (PTLBW) is a public health issue which needs to be dealt with by educating the general female population. One of the major contributing factors is periodontal disease and treatment of the same could reduce the occurrence of PTLBW babies. Therefore, awareness of this among the female population is highly essential. Hence, this survey was planned with the aim to explore the awareness of the general female population regarding the association of periodontal disease and PTLBW. MATERIAL AND METHODS: This survey consisted of nine close-ended questions answered by 1284 females. RESULTS: Younger individuals had higher knowledge scores as compared to the older individuals (r = -0.161) and the more educated group had a higher knowledge score as compared to the less educated groups (r = 0.322). Furthermore, married women seemed to be more aware of the relation of PTLBW to periodontal disease as compared to unmarried women. CONCLUSION: Younger and educated females had better awareness of the association between periodontal diseases and PTLBW. Hence, efforts to educate the general female population on this association could contribute toward the reduction of the risk of PTLBW.

Gastric pull up reconstruction after pharyngo laryngo esophagectomy for advanced hypopharyngeal cancer.

Hypopharyngeal cancers are uncommon. The management of advanced hypopharyngeal carcinomas has been a difficult problem. Surgical resection has been more successful. While many surgical methods have been used and reported pharyngolaryngo esophagectomy with gastric pull up remains the best option. This study documents our experience with patients who underwent total pharyngolaryngoesophagectomy with immediate gastric pull-up for advanced carcinoma hypopharynx. The clinical data of 17 patients treated with pharyngo-laryngo-esophagectomy for advanced carcinoma of the hypopharynx between 2001 and 2004 was analyzed. All patients had advanced disease and required a gastric pull-up for reconstruction. Data obtained included age, sex, site, stage, post op complication, duration of follow up, recurrence & survival. Average age was 37.7 years and ranged from 27 to 56 years. There were 13 female patients and 4 male patients .13 patients presented with postcricoid tumours and 4 with posterior pharyngeal wall tumors, 13 patients presented with stage 3 tumors and 4 patients presented with stage 4 tumors. Wound infection was present in 2 patients (11.7%), anastomotic leak in 1(5.8%), hypocalcemia in 1(5.8%) and malignant pleural effusion in 1 patient (5.8%). Local recurrence occurred in 1(5.8%) while nodal recurrence occurred in 5(29.4%) patient. The average over all survival in our study was 19.5 months and ranged from 2 to 101 months. The gastric pull-up operation is a useful and effective method for the immediate reconstruction of the advanced hypopharyngeal malignancy.