Fatal trio-aplastic anaemia,agranulocytosis and epistaxis

Aplastic anaemia, Agranulocytosis & Epistaxis—when combines in one patient together becomes fatal. Here three case reports are given, all of them suffered from these three diseases simultaneously. All died in spite of best treatment except bone marrow transplantation. It was not done because of non-availability of HLA typing & marrow donor.     

Response of the ENPP1-Deficient Skeletal Phenotype to Oral Phosphate Supplementation and/or Enzyme Replacement Therapy: Comparative Studies in Humans and Mice

Inactivating mutations in human ecto-nucleotide pyrophosphatase/phosphodiesterase-1 (ENPP1) may result in early-onset osteoporosis (EOOP) in haploinsufficiency and autosomal recessive hypophosphatemic rickets (ARHR2) in homozygous deficiency. ARHR2 patients are frequently treated with phosphate supplementation to ameliorate the rachitic phenotype, but elevating plasma phosphorus concentrations in ARHR2 patients may increase the risk of ectopic calcification without increasing bone mass. To assess the risks and efficacy of conventional ARHR2 therapy, we performed comprehensive evaluations of ARHR2 patients at two academic medical centers and compared their skeletal and renal phenotypes with ENPP1-deficient Enpp1^asj/asj mice on an acceleration diet containing high phosphate treated with recombinant murine Enpp1-Fc. ARHR2 patients treated with conventional therapy demonstrated improvements in rickets, but all adults and one adolescent analyzed continued to exhibit low bone mineral density (BMD). In addition, conventional therapy was associated with the development of medullary nephrocalcinosis in half of the treated patients. Similar to Enpp1^asj/asj mice on normal chow and to patients with mono- and biallelic ENPP1 mutations, 5-week-old Enpp1^asj/asj mice on the high-phosphate diet exhibited lower trabecular bone mass, reduced cortical bone mass, and greater bone fragility. Treating the Enpp1^asj/asj mice with recombinant Enpp1-Fc protein between weeks 2 and 5 normalized trabecular bone mass, normalized or improved bone biomechanical properties, and prevented the development of nephrocalcinosis and renal failure. The data suggest that conventional ARHR2 therapy does not address low BMD inherent in ENPP1 deficiency, and that ENPP1 enzyme replacement may be effective for correcting low bone mass in ARHR2 patients without increasing the risk of nephrocalcinosis. © 2021 American Society for Bone and Mineral Research (ASBMR).     

Care of lingual vessels during surgery over floor of mouth

The lingual vessels and its branches being superficial at under surface of tongue and floor if mouth may get injured during surgery over of floor of mouth.In two cases, lingual vessels were severed accdentally during surgery with excessive haemorrhage. This study was done to care the lingual vessels during surgery. Four main surgical dangerzone are found and emphasis is being given to be over cautious while operating over this surgical danger zones. Surgery over the floor of mouth is done in a number of conditions. Lingual vessels being very superficial, its sub-lingual branches are damaged during surgery if surgeon is not cautious and may create problems.To avoid this, thorough knowledge of correct surgical anatomy, course & relation of lingual vessels and floor of mouth is essential.     

Central motor conduction time in malnourished children

The functional status of the descending motor pathways was assessed in malnourished children using transcranial electromagnetic stimulation of the cortex. Twenty children with different severities of malnutrition and 20 control subjects were studied electrophysiologically. The circular coil of a Dentac MAG 2 magnetic stimulator was applied tangentially over the vertex to stimulate the cortex. The muscle evoked potential in the children's arms was recorded from the abductor pollicis brevis muscle and in their legs from the extensor digitorum brevis muscle on both sides of the body using surface electrodes. The muscle evoked potential of the abductor pollicis brevis and extensor digitorum brevis muscles was further obtained using root stimulation by applying the coil at the cervical and lumbar spine, respectively. The indices of cortical threshold, cortical latency, and central motor conduction time (ms) were evaluated in both arm and leg muscles on both sides. The results showed an increased cortical threshold (mean (SD) 1232.5 (134.06) in the study group v 1147.5 (99.31) in the control group) for the abductor pollicis brevis muscle and for the extensor digitorum brevis muscle (1325.00 (115.70) in the study group v 1190.0 (125.24) in the control group). Similarly, significant prolongation of the central motor conduction time (ms) (study group 6.67 (0.91) v control group 5.71 (0.74)) in the abductor pollicis brevis muscle was seen in malnourished children.     

Clinical course,diagnosis, and management of bilateral COVID-19 associated conjunctivitis: A case study

We present a rare case of COVID-19 associated conjunctivitis where patient presented with redness, foreign body sensation, watering, and pain. Symptoms started while patient was COVID-19 positive. On examination, severe conjunctival congestion was present along with follicles. Visual acuity was 6/6 in both eyes. The patient was started on topical antibiotics and showed improvement but again presented with aggravated symptoms. Conjunctival swab was sent for culture and sensitivity, which was negative for any organisms. The patient was then started on oral and topical steroid, which showed improvement. Even though COVID-19 associated conjunctivitis is self-limiting, here it showed a progressive course and resolved only after steroid.