Background & objectives:People with hemophilia constitute a significant proportion of the population and an oral health care professional faces a considerable challenge while treating them. This study aimed to assess the oral health and dentition status as well as fear of dental treatment in patients with hemophilia and compare it with age-matched healthy subjects. Patients & methods:This single-center, case-control cross-sectional study was performed on 100 subjects with hemophilia and 100 age-matched healthy controls. Oral health and dentition status was recorded for all the subjects and scored using the simplified oral hygiene index (OHI-S), plaque index, and the dmft/DMFT index.

A 4 year old girl with neurofibromatosis type 1 (NF1) was referred for hypertension. An aortogram showed narrowing of the left main renal artery. An angiogram three and a half years later showed coarctation of the abdominal aorta. She underwent aortoplasty but the stenosis recurred. Vascular involvement in NF1 may be progressive and requires long term follow up. (Arch Dis Child 1997;76:454-455) Keywords: neurofibromatosis; coarctation; renal artery stenosis; hypertension Vascular lesions are widely recognised to complicate neurofibromatosis type 1 (NF1) and include stenoses of the renal arteries, mesenteric artery, and aorta. We describe a child with hypertension complicating NF1 in whom renal artery stenosis was rapidly diagnosed but who later developed abdominal aortic coarctation. This case highlights the importance of measuring the blood pressure annually in children with NF1 and repeated meticulous catheter angiographic investigations in those who are hypertensive. Case reportA 4 year old girl suVered a minor head injury while playing. Two hours later she developed progressive loss of function in the left arm and left leg and was admitted to the local hospital where a diagnosis of a right extradural haematoma was made. She was hypertensive and was noted to have approximately 20 café au lait spots over her abdomen, back, and arm and pigmentation in her right axilla. Her sister, brother, mother, and maternal grandmother also had café au lait spots.On admission to this hospital she was well but her blood pressure was 147/100 in the left arm. Her left hemiparesis was resolving; no other abnormality was found. Renal function was normal, vanillylmandelic acid excretion was normal and echocardiography showed mild left ventricular hypertrophy and no structural abnormality. A renal ultrasound scan and dimercaptosuccinic acid scan showed two normal kidneys. Recumbent plasma renin samples were normal. A renal angiogram demonstrated a left renal artery narrowed at its origin from the abdominal aorta. The right renal, coeliac, and superior mesenteric arteries appeared normal.Hypertension persisted despite treatment with lisinopril. Two weeks after discharge she was admitted to her local hospital with severe frontal headache, twitching of her right arm, and increasing drowsiness. She was transferred to our care. Blood pressure control was improved by captopril and labetalol and her neurological status returned to normal.She was admitted 14 months after her original presentation because of poor blood pressure control and underwent balloon angioplasty of the left main renal artery stenosis. Her antihypertensive medication remained unchanged until repeat angiography 3.5 years after presentation that showed a 3 cm long stenosis of the abdominal aorta below the superior mesenteric artery at the level of the renal arteries. Lateral imaging (fig 1) demonstrated that the aorta was narrowed in the anterior-posterior diameter by 50% (normal aorta 5.9 mm, narrowed segment 2.7 mm). The main renal arteries appeared slightl...

Hemophilia is a genetic disease that impairs quality of life due to its chronicity of nature where the individual will experience spontaneous bleeding or bleeding after an injury which requires frequent visits to the hospital for treatment. The objective of this study was to find the Health-related quality of life (HRQoL) of children and adolescents with moderate and severe hemophilia between 6 years and 16 years using the Haemo-QoL questionnaire. A prospective survey was carried out among 107 children and adolescents from two hemophilia treatment centers in Karnataka state. Approval was taken from the ethical committee. The data was analyzed using SPSS version 16.0. The mean age was 11.00 ± 2.98 years. Among the 107 participants, 89.70% had hemophilia A and 10.30% had hemophilia B. Moderate hemophilia was found among 54.20% participants and 45.80% had severe hemophilia. Overall, the HRQoL scores (55.41) were higher in the age group of 6–7 years compared to 8–12 years and 13–16 years. The mean HRQoL in the domain of family were: 77.84 ± 23.12 among 6–7 years, 66.00 ± 17.34 among the 8–12 years and 60.38 ± 16.72 among 13–16 years. Children demonstrated poor HRQoL in the domains of family and friends. The results indicate a need for continuous monitoring of QoL to identify better treatment methods.

Introduction Thromboelastography (TEG) is a whole blood clotting assay largely used in major surgeries and trauma to monitor patients' in vivo hemostatic status. Standardization of kaolin‐activated citrated whole blood thromboelastography is not done in the Indian population. This study primarily aims to derive reference ranges of kaolin‐activated TEG for healthy volunteers in the Indian population. Secondarily, it aims to study the age‐ and gender‐related hemostatic changes in the study population. Methods A total of 120 healthy volunteers were enrolled (55 adult males, 32 adult females, and 33 children). The volunteers were interviewed for any bleeding history or drug intake which affects coagulation. Kaolin‐activated TEG was performed on citrated whole blood, and parameters including R‐time, K‐time, angle, MA, LY30, and CI were analyzed. Results Derived reference range for total volunteers irrespective of age and sex were as follows: R‐time: 3.8‐10.6, K‐time: 1.2‐3.1, angle: 44.9‐72.0, MA: 41.2‐64.5, LY30: 0‐9.9, and CI: −3.7 to 3.4. Statistically significant difference was observed in different age and sex groups for R‐time, K‐time, and angle. About 40% of the volunteers had at least one abnormal parameter according to the manufacturer's reference range which decreased to 12.5% when the derived reference ranges were considered. Conclusion Gender‐ and age‐related variances were observed in reference ranges of our population and which was also differed from the other ethnic population. Many of our healthy volunteers were categorized as coagulopathic when manufacturer's reference range was considered. So, it is important to derive the reference range of the target population before using the TEG into clinical practice.