Intranasal midazolam compared with intravenous diazepam in patients suffering from acute seizure: a randomized clinical trial

Acute seizure attack is a stressful experience both for health care personnel and parents. These attacks might cause morbidity and mortality among patients, so reliable methods to control the seizure preferably at home should be developed. This study was performed to measure the time needed to control seizure attacks using intranasal midazolam compared to the common treatment [intravenous diazepam] and to evaluate its probable side effects. This study was conducted as a not blind randomized clinical trial among 60 patients coming to Imam Ali Hospital, Zahedan, Iran. The patients were 2 months to 15 years old children coming to our emergency department suffering from an acute seizure episode. Intranasal midazolam was administered 0.2 mg/kg equally dropped in both nostrils for case group and intravenous diazepam was administered 0.3mg/kg via IV line for control group. After both treatments the time needed to control the seizure was registered by the practitioner. Pulse rate and O[2] saturation were recorded at patients' entrance and in minutes 5 and 10 after drug administration. The time needed to control seizure using intranasal midazolam [3.16 +/- 1.24] was statistically shorter than intravenous diazepam [6.42 +/- 2.59] if the time needed to establish IV line in patients treated by intravenous diazepam is taken into account [P<0.001]. The readings for O[2] saturation or heart rate did not indicate a statistically significant difference between two groups of patients either at entrance or 5 and 10 minutes after drug administration. Considering the shorter time needed to control acute seizure episodes compared to intravenous diazepam and its safety record, intranasal midazolam seems to be a good candidate to replace diazepam, as the drug of choice, in controlling this condition

case of concurrent proteus syndrome and hemophilia A

Proteus syndrome is a very rare condition with less than 100 confirmed cases reported worldwide. We report a case of Proteus syndrome in a two-year-old male who has hemophilia A comorbidity. A two-year-old male patient was admitted with the chief complaint of severe bleeding in mouth cavity after trauma for two weeks. At admission he was found to have petechiae on buccal mucosa and fecal discoloration due to GI bleeding. We noted multiple abnormalities in his musculoskeletal system and skin. He had lymph edema in left leg, hemihypertrophy, macrodactyly in both foots and macrocephaly. With the history of severe bleeding and recurrent blood product transfusion, we suspected a hemorrhagic disorder. The reduced level of Factor VIII activity confirmed the diagnosis of hemophilia A. Considering patient's various musculoskeletal abnormalities according to the diagnostic criteria and after ruling out similar disorders the diagnosis of Proteus syndrome was established. Because of the variability of clinical features, Proteus syndrome can be confused with other disorders of multiple tissue overgrowth. Our case of Proteus syndrome, who had hemophilia A comorbidity outlines the challenges in diagnosis of such rare combination of diseases

T2-star [T2*] magnetic resonance imaging for assessment of kidney iron overload in thalassemic patients

Improved survival in thalassemic patients has lead to the manifestation of morbidities such as renal dysfunction. This involvement suggests the need for a reliable and non-invasive method to assess the degree of kidney iron overload. We conducted the present study to evaluate the relationship between serum ferritin levels, liver, heart, and kidney MRI gradient echo [T2*] relaxation times in thalassemic patients, as a step to evaluate the feasibility of using MRI T2* to assess the degree of kidney iron overload. This was a prospective study of 120 [60 males, 60 females] regularly transfused thalassemic patients [mean age: 25.9 +/- 9 years] who suffered from major and intermediate thalassemia. Patients attended an adult thalassemia clinic located in Tehran, Iran. Cardiac, hepatic and renal MRI T2* were performed. Serum ferritin levels were measured. Our results indicated a moderate correlation between kidney MRI T2* relaxation time and serum ferritin [r = -0.446, P < 0.001]. Kidney MRI T2* relaxation time weakly correlated with liver MRI T2* relaxation time [r = 0.388, P < 0.001] and cardiac MRI T2* relaxation time [r = 0.338, P = 0.023]. The moderate correlation between kidney MRI T2* relaxation time and serum ferritin, and its weak correlation with liver and heart T2* relaxation times indicate that relying on liver and heart MRI T2*, as well as serum ferritin levels to predict the exact condition of kidney iron overload might not be a reliable approach. Our findings suggest the use of kidney MRI T2* as a noninvasive method for evaluating renal iron overload in thalassemic patients. Further studies to investigate the relation between kidney MRI T2* relaxation times and renal function, as well as the cost benefit of using this method, are suggested