Intracranial Rosai–Dorfman disease in a child mimicking bilateral giant petroclival meningiomas: a case report and review of literature

Objectives and importance Rosai–Dorfman disease (RDD) is a rare but distinctive entity of unknown etiology; isolated intracranial RDD is uncommon. Of 37 reported intracranial RDD cases, only three were reported in children.Clinical presentation We report an unusual case of a 15-year-old boy presenting with 4 months history of raised intracranial pressure with visual deterioration. Computed tomography and magnetic resonance imaging revealed bilateral petroclival enhancing lesions with cavernous sinus extension mimicking meningioma. However, histological examination was diagnostic of RDD.Intervention The patient underwent extended right-sided middle fossa approach and near-total tumor removal from petroclival region and cavernous sinus on both sides in two stages 6 weeks apart.Conclusion Ours is the first case of pediatric isolated intracranial RDD presenting with giant bilateral petroclival masses successfully managed with bilateral extended middle fossa approach in two stages. An optimal treatment for RDD is not established, but complete surgical resection alone seems effective.     

Anterior encephaloceles: a report of 30 cases

Thirty cases of anterior encephalocele treated in our centre over an 18-year period (from 1973 to 1990) are presented. At the time of surgery over 60% of the patients were under the age of 2 years, and 40% were aged under 1 year. Only one child was over 10 years of age. Twenty-six patients had the frontoethmoidal type of defect, while two each had frontonasal- and nasoorbital-type lesions. Twenty-five children had varying degrees of hypertelorism. Four had an enlarged head and four microcephaly. In 26 patients one-stage repair of the encephalocele and reconstruction of the orbits was undertaken. A ventriculoperitoneal shunt was performed prior to definitive surgery in three patients with gross hydrocephalus. There was no post-operative mortality. Six patients had postoperative CSF rhinorrhoea, three of them requiring a lumboperitoneal shunt. This study highlights the role of one-stage repair of this defect.     

Optic nerve injury in children. A prospective study of 35 patients.

Thirty-five children under 15 years of age with optic nerve injury are prospectively studies for their clinical presentation, radiological findings, visual evoked potentials and outcome. Over 50% were under 10 years of age. In half of the children, injury was due to fall from a height. Fracture of the skull was recorded in a third of the patients, and optic canal fracture was seen only in three children. Visual Evoked Potentials (VEP) were record in 30 children and were repeated several times in first three weeks. All the patients received corticosteroids and optic canal decompression was not carried out routinely. Overall spontaneous visual recovery was observed in 12 patients. Among the 30 children in whom Veps were recorded, 17 children had repeatedly absent VEP, and none of the children showed wave formation, 10 (77%) had visual recovery. Only in 5 patients an optic canal decompression was carried out several weeks after injury. This study brings out the role of VEP in children with optic nerve injury.     

The changing “epidemiology” of pediatric head injury and its impact on the daily clinical practice

Aim  This article focuses on the developments that occurred during the last two decades in the management of pediatric head injury. It describes the changes in incidence, various advancements in diagnosis, management, prognosis, prevention and strategies required for better outcome, and control of head injury. Materials and methods  Thorough evaluation of various papers, research, and our experience revealed that in developed countries, there has been a decreasing trend in head trauma incidence and trauma-related deaths as compared to developing countries. Results  This is mainly attributed to the widespread implementation of preventive measures. The development in imaging facilities, better characterization and grading of severe trauma (see, for example, diffuse axonal injury), an advanced understanding of the pathophysiology of secondary brain injury, endocrinological disturbances, predictive factors of outcome, development in neurophysiological monitoring, management advances in critical care units, implementation of safely measures, etc. have brought a significant change in overall outcome and profile of pediatric head injury Conclusion  The further developments in field of brain plasticity, stem cell, rehabilitation, evolution of new drugs, preventive community measures, and global policies to deal with head trauma are expected to play a major role in days to come. The development of future pediatric trauma centers based on current evolutions (in order to achieve a good outcome), global and emphatic preventions of trauma will be required to establish equilibrium between developed and developing countries.     

Efficacy of Single Low-Dose Rasburicase in Management of Tumor Lysis Syndrome in Leukemia and Lymphoma Patients

BackgroundTumor lysis syndrome (TLS) is a metabolic emergency in hematology patients. The recommended dose of rasburicase for the management of TLS is 0.2 mg/kg per day for 5 days, which is cost prohibitive for many patients. We sought to determine the efficacy of single low-dose rasburicase in the prevention and treatment of hyperuricemia in TLS.Patients and MethodsWe planned a prospective study for the safety and efficacy of fixed (weight based) dose of rasburicase to manage TLS. Patients diagnosed with leukemia/lymphoma with laboratory or clinically confirmed TLS or presence of ≥ 2 high-risk factors and serum uric acid > 7.5 mg/dL were included. The primary endpoint was uric acid normalization (< 7.5 mg/dL) within 24 hours of rasburicase administration.ResultsFifty-five patients were recruited for this study. Pediatric patients (< 18 years) accounted for 43.6% of cases. Rasburicase was provided prophylactically to 43 patients (78.2%) and for treating TLS to 12 (21.8%). Mean ± standard deviation serum uric acid at baseline and 24 hours was 9.2 ± 1.8 mg/dL and 3.2 ± 2.1 mg/dL, respectively. There was significant reduction in the serum uric acid and creatinine (P < .001) within 24 hours of rasburicase administration. The response was maintained up to 72 hours. A single dose of rasburicase was effective in 94.5% of patients. Single low-dose rasburicase led to 95% direct cost savings compared to the recommended dose.ConclusionSingle-dose rasburicase with frequent laboratory monitoring is effective in the management of TLS and offers significant cost reductions.     

Relationship between mobility, violence and HIV/STI among female sex workers in Andhra Pradesh, India

ABSTRACT: BACKGROUND: Violence and mobility have been identified as critical factors contributing to the spread of HIV worldwide. This study aimed to assess the independent and combined associations of mobility and violence with sexual risk behaviors and HIV, STI prevalence among female sex workers (FSWs) in India. METHODS: Data were drawn from a cross-sectional, bio-behavioral survey conducted among 2042 FSWs across five districts of southern India in 2005--06. Regression models were used to estimate odds ratios and 95% confidence intervals (CIs) for sexual risk behaviors and HIV infection based on experience of violence and mobility after adjusting for socio-demographic and sex work related characteristics. RESULTS: One-fifth of FSWs (19%) reported experiencing violence; 68% reported travelling outside their current place of residence at least once in the past year and practicing sex work during their visit. Mobile FSWs were more likely to report violence compared to their counterparts (23% vs. 10%, p < 0.001). Approximately 1 in 5 tested positive for HIV. In adjusted models, FSWs reporting both mobility and violence as compared to their counterparts were more likely to be infected with HIV (Adjusted odds ratio (adjusted OR): 2.07, 95% CI: 1.42--3.03) and to report unprotected sex with occasional (adjusted OR: 2.86, 95% CI: 1.76--4.65) and regular clients (adjusted OR: 2.07, 95% CI: 1.40--3.06). CONCLUSIONS: The findings indicate that mobility and violence were independently associated with HIV infection. Notably, the combined effect of mobility and violence posed greater HIV risk than their independent effect. These results point to the need for the provision of an enabling environment and safe spaces for FSWs who are mobile, to augment existing efforts to reduce the spread of HIV/AIDS.     

Primary small bowel volvulus in Nepal

Small bowel volvulus is a rare cause of intestinal obstruction. Primary small bowel volvulus occurs without any predisposing cause. It is rare in Western countries but common in Africa and Asia. It is the most common cause of bowel obstruction in Nepal (excluding incarcerated external hernias). Early diagnosis and management is essential to avoid infarction of bowel. Persistent central abdominal pain is characteristic. There is no single specific diagnostic clinical sign or abnormality in laboratory or radiological findings. There is no role of conservative management and, in suspected cases, early laparotomy should be done to avoid bowel infarction. In follow-up patients may present with stricture formation or recurrence of bowel volvulus.     

Clinical experience with a transdermal nitroglycerin system

Forty-seven patients with chronic stable angina pectoris entered a thirteen-week open-label study with a transdermal therapeutic system of nitroglycerin in order to evaluate its clinical efficacy, safety, and patient acceptance. In 19 patients, a beta-blocker and in 17 patients a calcium-channel blocker were continued throughout the study period without alteration of their doses. The study consisted of a two-week run-in period and an eleven-week active drug period. Acute titration was done with nitroglycerin patches on the basis of weekly patient diaries on frequency of angina and sublingual nitroglycerin consumption. Overall, reductions in frequency of angina and in nitroglycerin consumption were statistically significant (p less than 0.05). Adverse reactions were common but tolerable. The reported side effects were headache in 32, skin rash in 18, dizziness in 10, palpitation and itching in 9 each, nausea in 7, flushing in 3, and vomiting in 1 patient. In conclusion, the present study demonstrates that individual dose titration with nitroglycerin patches for obtaining significant antianginal effect is essential. The present therapeutic system is convenient to use and well tolerated and had acceptable side effects in our study population.     

Ramping Glucosuria for Management of Type 2 Diabetes Mellitus: An Emerging Cynosure

Chronic hyperglycemia is a characteristic feature of type 2 diabetes mellitus (T2DM). The kidney plays a vital role in maintaining blood glucose homeostasis by recovering glucose from glomerular filtrate which is controlled by SGLT2 cotransporters expressed mainly in proximal tubule. In T2DM patients, inhibition of SGLT2 normalizes glycemic levels by preventing glucose from being reabsorbed through SGLT2 and re‐entering the circulation. Thus, SGLT2 inhibition seems to be a logical approach and pose a novel insulin‐independent mechanism of action for management of T2DM by promoting urinary glucose excretion in the body. Canagliflozin is the first SGLT2 inhibitor approved by US Food and Drug Administration (US FDA) followed by dapagliflozin while empagliflozin is under FDA review. Various other drug candidates in late‐stage clinical developments are also expected to hit the global markets in the coming years. In this review, studies on various early‐ and late‐stage SGLT2 inhibitors have been investigated and recent clinical developments summarized.