Paroxysmal hypertension with reversible posterior leukoencephalopathy syndrome in a child: an unresolved riddle

Nine year old girl was presented with paroxysmal episodes of hypertensive emergency. She was asymptomatic with normal blood pressure without antihypertensives in between the episodes. MRI brain was suggestive of reversible posterior leukoencephalopathy.' Acute episodes were managed with IV labetalol infusion and amlodipine. She was evaluated extensively to find out the etiology of hypertension. Cardiac and renal causes were ruled out. Work up for pheochromocytoma, hyperaldosteronism, porphyria and vasculitis were negative. The case is reported in view of the rare presentation and the leukoencephalopathy noted in this case.

Glycemic Control and Long-term Complications in Pediatric Onset Type 1 Diabetes Mellitus: A Single-center Experience from Northern India

Objective: To study glycemic control, mortality and long-termcomplications in children with type 1 diabetes (T1D).Design: Cross-sectional study.Setting: Referral centre at a government teaching hospital.Participants: Patients with T1D with age ≤18 years at onset.Methods: We retrospectively collected demographic data fromcomputer records from 1991 to 2015. Prospective study foroutcomes was conducted between 2012 and 2016.Main outcome measures: Mortality rate, glycosylatedhemoglobin (HbA1c), and microvascular complication rate.Results: The proportion of T1D patients (n=512) <5 years of ageat onset was 18.6% between 1995 and 2004, and 24.2% in2005-2014 (P<0.001). Twenty eight patients had died out of 334whose living status was known (mortality 1.1 per 100 patient-years over 2549 patient-years follow up). Median (range)HbA1c (n=257) was 8.3% (5.1-15.0%). At least one episode ofsevere hypoglycemia (coma/seizure/inability to assist self) hadoccurred in 22.8% patients over two years. Hypertension waspresent in 11.7% patients. Microvascular complications screenin 164 eligible patients [median (range) age 20 (8-45) y andduration of diabetes 9.1 (5-30) y] showed diabetic nephropathy in3.0%, proliferative retinopathy in 3.6% and LDL cholesterol >100mg/dL in 34% patients.Conclusion: The mortality rate and prevalence of hypertensionwere high, given the short duration of diabetes of the patients.The proportion of patients with age ≤5 years at onset of diabeteshas increased at our center.

Perlecan (basement membrane heparan sulfate proteoglycan) and its role in oral malignancies: An overview.

Perlecan means pearl-like structures. Perlecan is a large proteoglycan (400-500 kDa) present in virtually all vascularized tissues with a distribution that is primarily confined to basement membranes including those of oral mucosa. It is a basement membrane-type heparan sulfate proteoglycan. Perlecan is synthesized by basal cells and fibroblasts adjacent to the basal lamina . Perlecan is also synthesized by vascular endothelial and smooth muscle cells present in the extracellular matrix. It has been demonstrated in recent years that perlecan is distributed in the stromal space of various pathophysiological conditions. The complex pleiotropy of perlecan suggests that this gene product is involved in several developmental processes, at both early and late stages of embryogenesis, as well as in cancer and diabetes. In the oral cavity, perlecan expression is reported to basal cells in normal mucosa and its expression increases in precancer and cancerous conditions. It is also expressed in various odontogenic tumors such as ameloblastoma, keratocyst odontogenic tumor, and also salivary gland tumors such as adenoid cystic carcinoma, mucoepidermoid carcinoma, etc.