An Intriguing Case of Amyloidosis Leading to PAGE Kidney in a Post Renal Transplant Recipient: A Case Report.

Amyloidosis is an infiltrative disease where amyloid fibrils get deposited in the organs like kidney, liver and spleen. Amyloid deposition in the kidneys classically meant deposition in the glomeruli and mesangium until 2008 when interstitial amyloid deposits were isolated and named as` Leukocyte cell-derived chemotaxin 2-associated amyloidosis. It is a progressive disease which clinically manifests as slowly progressive renal dysfunction and/or proteinuria. Our case 34 year old renal transplant recipient underwent graft biopsy post transplantation which revealed interstitial LECT-2 amyloid deposits. Unfortunately, he developed page kidney post biopsy which was managed conservatively with percutaneous drainage. Supplementary Information: The online version contains supplementary material available at 10.1007/s12291-022-01072-6.

Diagnostic Dilemma of Vanishing Bone Disease - A Case Report and Review of Literature.

Rationale: Vanishing bone disease (VBD) is a rare bone disorder in which progressive osteolysis may lead to complete disappearance of involved bones. The diagnosis of this disease requires a high degree of clinical suspicion. We present a case of progressive osteolysis of mandible in a patient. Patient Concerns: The patient had been without definitive diagnosis and treatment for over a year. Diagnosis: Diagnosis was made by exclusion of genetic, traumatic, inflammatory, infective, endocrine and neoplastic aetiologies and by carefully correlating clinical, imaging and histopathological findings of the patient. Treatment: Segmental resection of the advancing edge of the lesion was carried out. Outcome: The patient is disease free, with no evidence of further osteolysis, after six months of follow-up. Take-Away Lessons: This article describes the exclusion-based approach adopted to diagnose a case of VBD, aiming to standardise a workup for the diagnosis.

Evidence-Based Guidelines for the Management of Allergic Bronchopulmonary Aspergillosis (ABPA) in Children and Adolescents with Asthma.

BACKGROUND: Allergic bronchopulmonary aspergillosis (ABPA) frequently complicates asthma. There is urgent need to develop evidence-based guidelines for the management of ABPA in children. The Evidence Based Guideline Development Group (EBGDG) of the Indian Academy of Pediatrics (IAP) National Respiratory Chapter (NRC) addressed this need. METHODS: The EBGDG shortlisted clinical questions relevant to the management of ABPA in asthma. For each question, the EBGDG undertook a systematic, step-wise evidence search for existing guidelines, followed by systematic reviews, followed by primary research studies. The evidence was collated, critically appraised, and synthesized. The EBGDG worked through the Evidence to Decision (EtD) framework, to formulate recommendations, using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach. RESULTS: Seven clinical questions were prioritized, and the following recommendations formulated. (1) Children with poorly controlled asthma should be investigated for ABPA (conditional recommendation, moderate certainty of evidence). (2) Low dose steroid therapy regimen (0.5 mg/kg/d for the first 2 wk, followed by a progressive tapering) is preferable to higher dose regimens (conditional recommendation, very low certainty of evidence). (3) Oral steroid regimens longer than 16 wk (including tapering), should not be used (conditional recommendation, very low certainty of evidence). (4) Antifungals may or may not be added to steroid therapy as the evidence was neither in favour nor against (conditional recommendation, low certainty of evidence). (5) For clinicians using antifungal agents, the EBGDG recommends against using voriconazole instead of itraconazole (conditional recommendation, very low certainty of evidence). (6) No evidence-based recommendation could be framed for using pulse steroid therapy in preference to conventional steroid therapy. (7) Immunotherapy with biologicals including omalizumab or dupilumab is not recommended (conditional recommendation, very low certainty of evidence). CONCLUSIONS: This evidence-based guideline can be used by healthcare providers in diverse clinical settings.

"Bridging the Breach": Cranioplasties Using Different Reconstruction Materials-An Institutional Experience.

Introduction: Cranioplasty is a time tested surgical procedure to restore the form and function of either congenital or acquired calvarial defects. Both autologous bone flaps and alloplastic substitutes have been surgically explored over time to achieve the pre-morbid contour and eliminate the existing and anticipated complications like the "Sinking flap Syndrome". Though autologous bone flap, if preserved, is generally considered the first choice for reconstruction, however its absence/large defect reconstruction calls for alternative options. The selection of the reconstructive material is governed by various patient factors and material-related issues. The current study is an institutional experience of calvarial reconstruction with different materials. Though the aim of the surgery is always maximum patient comfort and satisfaction, post-operative outcome may vary from case to case. Materials and Method: Patients with calvarial defects post decompressive craniectomy, referred to the Department of OMFS from the Department of Neurosurgery/Neurology at our tertiary care centre, were successfully managed for the same using autologous Bone flap, customized Titanium and PEEK patient specific implant (PSI). Post-op evaluation for aesthetics, ease of adjustment/workability, thermal conductivity and infection/exposure was done at 01 and 06 months. Results: Fifteen patients of cranial defects post decompressive craniectomy were taken up of calvarial reconstruction using autologous Bone flap (n = 5), Titanium (n = 5) and PEEK (n = 5) patient specific implant (PSI). Satisfactory restoration of cranial contour was seen in all cases immediately after surgery. One patient underwent flap necrosis in bone flap group, while thermal conductivity was reported in 2 out of 5 cases of titanium PSI. Ease of workability was relatively low in all cases of PEEK group while increased overall surgical duration was encountered in bone flap group owing to second surgical site in abdomen. Conclusion: A variety of reconstruction materials may be used for cranial contouring depending on the defect and surgeon's experience, however the results may vary from case to case thus mandating tailor-made treatment for each patient.

Evaluation of the efficacy of transcutaneous electrical nerve stimulation (TENS) on salivary flow rate in patients with xerostomia - A case control study.

Introduction: In oral cavity, saliva is a physiological fluid present in the oral cavity. Oral fluid is an absolute media exploring for health and disease surveillance. Saliva is a complex fluid. Xerostomia is the subjective complain of dryness in the oral cavity due to decreased salivary flow. Transcutaneous electrical nerve stimulation (TENS) is an established physical therapy, which is useful for the pain relief. Materials and Methods: A total of 25 subjects of xerostomia and 25 age and sex matched normal subjects visiting the Department of Oral Medicine and Radiology, Pacific Dental College and Hospital were included in the study. For patients with positive mirror stick test unstimulated saliva were collected with 'low forced spitting' method in a graduated test tube for 5 minutes. Individuals with unstimulated salivary flow equal to or less than 0.5 mL per 5 min were included in the study group (xerostomia) and individuals with salivary flow more than 0.5 mL per 5 min were included in the control group (age and sex matched). The TENS unit were then activated and intensity control switch were gradually increased to tolerable level of patient for 15 min. At this optimal intensity, stimulated saliva were collected for 5 min with same method in a separate graduated test tube and flow rate were compared with unstimulated salivary flow rate. Same were done with 25 control patients. Results: The data thus obtained was subjected to statistical analysis. The following findings were observed in the study. There was increase salivary flow rate in xerostomic individuals after TENS application. More improvement was found in inter appointment salivary flow than two follow-ups. Healthy group showed more improvement than xerostomia after TENS therapy Known cases of diabetes mellitus patients with xerostomia revealed more improvement than other etiologies of xerostomia included in this study. Conclusion: This present study has an important therapeutic value in the treatment of patients with xerostomia. TENS therapy was highly effective in stimulating whole salivary flow. The encouraging results of this present study indicate that TENS has the potential to increase salivary flow rate and can be an important alternative in the xerostomia treatment.

Isolated left side superior vena cava in a dialysis patient: Right direction, wrong turn!

Tunneled catheter insertion is a routine procedure undertaken by nephrologists world over. However, the presence of a venous anomaly can always test one's skills and can give them anxious moments. Persistent left superior vena cava (SVC) is the most common venous anomaly. We share our experience of successfully placing a hemodialysis central venous catheter in a very rare congenital anomaly wherein patient had persistent left SVC with agenesis of the right SVC.

Natal and neonatal teeth: Clinically relevant findings in a retrospective analysis.

BACKGROUND: Teeth present at the time of birth are called natal teeth while the teeth which erupt within 30 days of birth are neonatal teeth. The natal and neonatal teeth are an uncommon occurrence causing extreme anxiousness among the parents because of various myths related to their occurrence in our society. METHODS: A retrospective analysis of 12 patients who reported to the dental center between 2012 and 2015 with natal or neonatal teeth was carried out. RESULTS: Our study sample comprised of 12 patients. The age group ranged from 4 to 27 days in which there were eight male and four female infants. Out of 12 cases, eight cases had history of a physiologic delivery, while four were delivered by C-section. All the teeth were white to yellowish white in color, grade III mobile, and were devoid of roots. Except difficulty in feeding, only one case had a complication because of the tooth which was the development of a small lingual ulcer. Eleven cases were managed by extraction of the natal or neonatal teeth, and in one case, the parents did not consent for extraction. Six infants below 10 days of age were administered inj Vit K prior to the extraction. On follow-up of all the patients families reported that the child was feeding much better after the treatment and there were no complications. CONCLUSION: Natal and neonatal teeth have been causing dilemmas in the minds of pediatricians and dentists world over. Thus, it is important to know the basics about such teeth so that proper guidance can be given to parents and a suitable case specific treatment plan can be formulated.

Application of Navigation Surgery in Temporomandibular Joint Ankylosis Case and Review of Literature.

The aim of this article is to develop a navigation-guided oral and maxillofacial surgery including surgical planning, simulation and navigation in temporomandibular joint ankylosis case practiced in tertiary care hospital. After getting the computed tomographic angiography of head and neck, the special software of Brain lab® is used to mark the arteries and veins of the patient, which was in close approximation to the mandibular condyle. Brain lab® navigation system was used during the surgery to ascertain the middle meningeal artery location, and osteotomy cut was given. Navigation technology use in the neurosurgery is not new. But its use in the subcontinent in the field of maxillofacial surgery is quite rare. First time, it has been used scientifically in temporomandibular joint ankylosis case. Navigation technology use in the maxillofacial surgery requires a holistic imaginative/creative approach to make the surgeries more predictive and safe.

Complications of Diagnostic TMJ Arthroscopy: An Institutional Study.

INTRODUCTION: The prevalence of using arthroscopy for the diagnosis of the TMJ disorders is increasing due to its superiority to conventional methods of imaging. Although considered to be safe, complications do occur. PATIENTS AND METHOD: A single operator single-institution retrospective study consisting of 50 patients taken up for diagnostic arthroscopy was analysed for complications. RESULTS: Lacerations of external acoustic meatus was found in 03 patients (6%); immediate partial hearing loss was seen in 01 patient (2%); transient facial nerve palsy was found in 05 patients (10%); sensory disturbances over the distribution of auriculotemporal nerve was evident in 01 patient (2%); haemorrhage as visualised by excessive bleeding through trocar skin puncture wound was seen in 05 patients (5%). Post-operative pain more than the pre-operative pain on assessment by visual analogue scale was noted in 05 patients (10%) on the immediate post-operative day. Reduction in spontaneous mouth opening was noted in 15 patients (30%). CONCLUSION: Though the complication rate was found to be higher than most of the other studies, they were minor which resolved without any intervention. The cases with complications were clustered at the beginning of the series which suggests the steep learning curve and the importance of surgeons' experience and skill involved in this procedure.

Evaluation of Total Alloplastic Temporomandibular Joint Replacement in TMJ Ankylosis.

INTRODUCTION: Total joint reconstruction using autologous or alloplastic materials is one of the treatment options for the reconstruction of the affected joint in TMJ ankylosis. The clinical results of alloplastic TM joint replacement (Biomet Microfixation system) that was carried out for the treatment and reconstruction of TMJ ankylosis have been summarised. MATERIALS AND METHODS: A retrospective analysis of eight cases clinically and radiographically diagnosed as TMJ ankylosis with minimal facial asymmetry presenting beyond the growth period and willing for at least 12 months of follow-up who have been taken up for gap arthroplasty and reconstruction using Biomet Microfixation TMJ replacement system was carried out. Follow-up of these patients was carried out at regular intervals and assessed on the following criteria: maximal interincisal mouth opening, TM joint pain on the affected side (on VAS), lateral mandibular excursions to the unaffected side deviation on mouth opening, occlusal discrepancy and neuromotor deficit of peripheral branches of facial nerve. RESULTS: The mean maximal interincisal mouth opening pre-surgery and 1 year post-surgery was 2 and 31.8 mm, respectively. The mean lateral excursive movement to the unaffected side was found to be 5.5 mm with mean deviation on mouth opening to be 4.6 mm. No pain, occlusal discrepancy or neurological deficit existed at the end of 1 year. CONCLUSION: Biomet Microfixation system is a viable treatment option for reconstruction of TMJ in cases of ankylosis with no major complications.

Catheter Fracture of Subclavian Venous Chemoport Device at Costoclavicular Junction: Pinch-off Syndrome.

Chemoport is most commonly used venous access devices for instillation of chemotherapeutic drugs in cancer care. Mechanical complications like catheter fracture can lead to serious morbidity, albeit occurring rarely. We present a case of a 35-year-old lady, a case of carcinoma breast, who had spontaneous fracture of chemoport access device in subclavian vein at the level of clavicle after four successful cycles of chemotherapy. The fracture was suspected on chest x-ray and was subsequently confirmed on contrast linogram. The patient was successfully managed with endovascular interventional technique without suffering any ill effects. It is a rare presentation of pinch-off syndrome.

The use of intra-articular analgesics to improve outcomes after temporomandibular joint arthrocentesis: a review.

PURPOSE: The aim of this review was to assess the efficacy of intra-articular analgesics in improving outcomes after temporomandibular joint (TMJ) arthrocentesis. MATERIAL AND METHODS: An electronic search of PubMed, Scopus, and Google scholar databases was performed for papers in English published up to December 2017 reporting the use of intra-articular analgesics after TMJ arthrocentesis. Randomized controlled trials (RCTs), controlled clinical trials (CCTs), comparative studies, retrospective studies, and case series were included while case reports, technical reports, animal studies, cadaveric studies, and review papers were excluded. RESULTS: Of the six studies included in the review, three were RCTs, two were randomized comparative studies, and one was a retrospective study. Both opioids and non-steroidal anti-inflammatory drugs have been used after TMJ arthrocentesis. Morphine, tramadol, fentanyl, buprenorphine, tenoxicam, and COX-2 inhibitors are the drugs used till date. Placebo-controlled studies reported improved outcomes after TMJ arthrocentesis with morphine and fentanyl but no such results with buprenorphine and tenoxicam. Tramadol was found to be better than COX-2 inhibitor. The quality of literature was not high. CONCLUSIONS: There is inconclusive evidence in literature on the benefits of using intra-articular analgesics after TMJ arthrocentesis. Well-designed high-quality RCTs with standard protocol studying the effects of intra-articular opioids and NSAIDS after TMJ arthrocentesis would provide stronger evidence on its use.

Epithelioid osteoblastoma of maxilla: A rare and aggressive variant of a benign neoplasm at an uncommon site.

Osteoblastoma (OB) is an uncommon benign bone-forming tumor accounting for <1% of all bone neoplasms. Unlike conventional OB, its small subset variant "Epithelioid osteoblastoma (EO)" is characterized by its propensity for local invasion and recurrent behavior. This rare variant of an uncommon tumor when occurs in an atypical site can lead to diagnostic problems more so due to ambiguous clinico-radiologic presentation. This was what faced in the present case of 18-year-old female with a swelling in upper jaw. OB is usually more common in males and involves primarily the posterior element of the spine and the sacrum (40-55%). Less frequently, long bones of limbs are involved. Clinical, radiological and histopathological correlation in this case guided us to reach at right diagnosis of EO which helped the patient in getting correct treatment which involves surgical excision over conventional curettage. The purpose behind this case presentation is to improve the awareness about this recurrent tumor variant which has many close differentials including well-differentiated osteoblastic osteosarcoma.

Comparison of articulating paper markings and T Scan III recordings to evaluate occlusal force in normal and rehabilitated maxillofacial trauma patients.

BACKGROUND: Prosthodontic Rehabilitation of Treated Maxillofacial Trauma Cases by Evaluating Occlusal Force Distribution Using Computerized Occlusal Analysis. METHOD: 30 patients were selected for the study. 15 normal and 15 treated trauma patients were subjected to T Scan analysis and evaluated for the occlusal force distribution. RESULTS: The results take into consideration the two parameters. Firstly the largest articulating paper mark (photographed) and secondly the T scan of the same patient. Comparison was made between the largest articulating paper mark and highest force tooth in the quadrant using T Scan. The matches and no matches were then tabulated for statistical analysis assessing the frequency of the matches to the no matches. CONCLUSION: The ultimate advantage of a T Scan III analysis is that it can detect the amount of force as well as location of the highest intensity contacts of a single tooth which is very specific.

Rehabilitation of a Patient with Central Giant Cell Granuloma of Mandible by Iliac Graft, Bone Distraction and Implant Retained Telescopic Prosthesis: a Two Year Follow Up.

Giant cell granulomas of the jaws are lesions that arise either peripherally in periodontal ligament, mucoperiosteum, or centrally in the bone. Histologically, both peripheral and central giant cell granuloma are characterized by the presence of numerous multinucleated giant cells in a prominent fibrous stroma. Traditional treatment has been local curettage, although aggressive sub-types have a high tendency to recur. This case report describes the rehabilitation procedure of a patient with central giant cell granuloma of left side of mandible. Marginal resection of the lesion was done followed by reconstruction of the resected mandible with iliac graft. Distraction of bone was done since there was partial uptake of the fibula graft. Five implants were placed once the distraction was complete. The patient was rehabilitated with implant retained removable telescopic prosthesis.

Relationship of serum thyroid stimulating hormone with body mass index in healthy adults.

OBJECTIVE: To investigate any possible relationship between serum thyroid stimulating hormone (TSH) with body mass index (BMI) in healthy adults. MATERIALS AND METHODS: A total of 417 subjects aged 18-60 years who volunteered to get screened for thyroid illness with serum TSH have been enrolled from November 2012 to July 2013. Patients were divided into four groups based on BMI value: Underweight (BMI <18 kg/m(2)), normal (BMI: 18-22.9 kg/m(2)), overweight (BMI: 23-24.9 kg/m(2)), and obese (BMI ≥25 kg/m(2)). RESULT: In our study we found a significant variation (P < 0.001) in TSH with increasing BMI. As the BMI increased, mean TSH in the BMI range also increased. The individuals with higher BMI had higher TSH and this trend continued from underweight to Obese. The mean TSH of underweight group was 1.6036 mIU/L, normal weight group 2.1727 mIU/L, overweight group 2.2870 mIU/L and obese group 2.6416 mIU/L. CONCLUSION: In this study we found a significant relationship between serum TSH and BMI and mean TSH increased as BMI increased. Further large scale data from the population is required to confirm our findings.

Failure of siblings to thrive beyond 5 years of age.

We describe a family in which parents had consanguinity, being children of real sisters. They had given birth to five children. In their family, children remained healthy from birth to pre-school age and then started having symptoms around the age of 5 years and two of them succumbed to this illness. Polyglandular autoimmune syndrome Type-1 is a rare sporadic autosomal recessive disease. It is characterized by the existence of two or more endocrinal disorders. Patients may require lifelong hormone replacement therapy for survival.