Severe intracranial haemorrhage in neonatal alloimmune thrombocytopenia due to antibodies against human platelet antigen 1b: Case report and literature review.

Fetal/neonatal alloimmune thrombocytopenia (FNAIT) is a rare life-threatening disorder, leading to severe thrombocytopenia and potentially bleeding, with intracranial haemorrhage (ICH) being the most serious complication. We report on a FNAIT case with fourth-degree ICH that arose due to antibodies against human platelet antigen (HPA)-1b. The male infant, born to an otherwise healthy mother, presented with severe signs of ICH soon after delivery. Since only moderate thrombocytopenia was noted and there were no active signs of bleeding, the infant did not receive intravenous immunoglobulins (IVIg) or platelet transfusion. Spontaneous recovery of platelets was noted on the eighth day of life, but permanent neurological impairment remained as a consequence of ICH. We report the results of HPA and human leukocyte antigen (HLA) antibodies in the mother's and the infant's sera, the family's HPA genotype and the mother's HLA genotype, and summarise previously described cases of FNAIT due to anti-HPA-1b antibodies in the literature. FNAIT with severe ICH due to anti-HPA-1b antibodies is rarely diagnosed. An association between HLA genes and sensitization to HPA-1b antibodies was not demonstrated. The severity of FNAIT and the occurrence of ICH is often difficult to predict. In this case, the infant presented with moderate thrombocytopenia and ICH, with subsequent permanent consequences.

Continuous glucose monitoring in the treatment of obesity in patients with glycogen storage disease type Ia.

UNLABELLED: Glycogen storage disease (GSD) type I is characterized by impaired production of glucose from glycogenolysis and gluconeogenesis resulting in severe hypoglycaemia and increased production of lactic acid, triglyceride and uric acid. The most common type, glycogenosis type Ia, demands a balanced, sufficient carbohydrate intake to preserve normal 24-h glycaemia. Insufficient intake of carbohydrates can cause hypoglycaemia, as the missing glucose-6-phosphatase enzyme cannot free the glucose stored as liver glycogen and nor is gluconeogenesis possible. The principle means of handling this disorder is to avoid starving by taking regular meals during the day and night. Such a dietary regimen could lead to obesity. Herein, we present the case of an adult patient with glycogenosis type Ia suffering from hyperuricaemia, dyslipidaemia and arterial hypertension. The accumulation of these cardiovascular risk factors could lead to the early onset of atherosclerosis, which should be postponed by contemporary methods of surveillance and treatment. LEARNING POINTS: Continuous subcutaneous glucose monitoring may be of value in every adult patient with GSD type I to evaluate the actual prevalence of eventual hypoglycaemic and hyperglycaemic episodes.Good dietary management minimizes the metabolic abnormalities of the disease and decreases the risk of long-term complications.Treatment of obesity in patients with GSD reduces the risk of earlier atherosclerosis and cardiovascular disease.

Comparison between continuous ambulatory arterial blood pressure monitoring and standard blood pressure measurements among patients of younger and older age group.

The purpose of the study was to evaluate whether there is a difference between blood pressure measured in a physician's office and the average 24 hr continuous blood pressure monitored by hypertensive patients at home. If there is a difference between these two situations then is it possibly the result of a blood pressure response by the patient to the physician which is known as "white coat effect" or "white coat hypertension". We studied 80 hypertensive outpatients which were divided into two groups of 40 patients each--a younger patient group, with a mean age of 22.8 +/- 1.8 years, and an older patient group with a mean age of 50.3 +/- 5.7 years. They were selected because they had been diagnosed as essentially hypertension grade 1, according to 2007 ESH/ESC Guidelines, or the USA Joint National Committee Guidelines (JNC 7) (i.e., arterial blood pressure > 140/90 mm Hg and < 160/100 mmHg) and 35 were not having any antihypertensive treatment. All participants in the study went through a two-week "wash-out" period without medication. At the beginning of the study blood pressure was measured using the Riva-Rocci-Korotkoff method (mercury sphygmomanometer) after 5 minutes of rest and with the patient in the sitting position. The average of the two last measurements by sphygmomanometer was used in the analysis. The subsequent measurement was made by continuous ambulatory blood pressure monitoring (SpaceLabs 90207 device). Continuous ambulatory blood pressure monitoring revealed that 17 patients of the younger age group (42.5%) who were diagnosed hypertonic, according to mercury sphygmomanometeric measurement, were in fact normotonic. In the older age group only 7 (17.5%) of participants were normotonic during 24 hr blood pressure monitoring. The proportion of miss-diagnosed normotonic younger patients was directly related to elevated clinic blood pressure, which could be referred to as office hypertension or isolated clinic hypertension (white coat hypertension). This was statistically significant (chi2 = 5.95; p = 0.015). Hypertension diagnosed in younger patients based only on occasional doctor's office mesurements, using a mercury sphygmomanometer, could be miss-interpreted and treated as the start of arterial hypertension. This could sometimes have unwanted results due to the side effects of precipitate antihypertensive medication as well as the unnecessary cost of testing, cost of treatment, prevalence of white-coat hypertension at baseline, and the varying incidence of new hypertension after the initial screening. The results indicate a potential savings of 3-14% in the cost of care for hypertension, and a 10-23% reduction in treatment days when ambulatory blood pressure monitoring is incorporated into the diagnostic process. Therefore CABPM should be used as a legitimate method in the diagnosing of "white coat hypertension", particularly in young patients. The identification of white coat hypertensive"' patients should be followed by a search for metabolic risk or organ damage using the latest guidelines, and medication should start after an organ damage or cardiovascular risk assesement.

Improving asthma control through telemedicine: a study of short-message service.

Home peak expiratory flow (PEF) measurement is recommended by asthma guidelines. In a 16-week randomized controlled study on 16 subjects with asthma (24.6 6.5 years old, asthma duration small ze, Cyrillic 6 months), we examined Global System for Mobile Communications (GSM) mobile telephone short-message service (SMS) as a novel means of telemedicine in PEF monitoring. All subjects received asthma education, self-management plan, and standard treatment. All measured PEF three times daily and kept a symptom diary. In the study group, therapy was adjusted weekly by an asthma specialist according to PEF values received daily from the patients. There was no significant difference between the groups in absolute PEF, but PEF variability was significantly smaller in the study group (16.12 +/- 6.93% vs. 27.24 +/- 10.01%, p = 0.049). forced expiratory flow in 1 second (FEV1; % predicted) in the study group was slightly but significantly increased (81.25 +/- 17.31 vs. 77.63 +/- 14.80, p = 0.014) and in the control group, unchanged (78.25 +/- 21.09 vs. 78.88 +/- 22.02, p = 0.497). Mean FEV1 was similar in the two groups both before and after the study. Controls had significantly higher scores for cough (1.85 +/- 0.43 vs. 1.42 +/- 0.28, p < 0.05) and night symptoms (1.22 +/- 0.23 vs. 0.85 +/- 0.32, p < 0.05). There was no significant difference between the groups in daily consumption of inhaled medicine, forced vital capacity, or compliance. Per patient, per week, the additional cost of follow-up by SMS was Euros 1.67 (equivalent to approximately $1.30 per 1 Euro), and SMS transmission required 11.5 minutes. Although a study group of 40 patients is needed for the follow-up study to achieve the power of 80% within the 95% confidence interval, we conclude that SMS is a convenient, reliable, affordable, and secure means of telemedicine that may improve asthma control when added to a written action plan and standard follow-up.