RESUMO
BACKGROUND: Diagnosis of brain death relies on clinical and electroencephalographic (EEG) criteria. Waiting for 24 hours is mandatory to make definitive diagnosis of the condition in the Iranian protocol. Although it has been previously shown that oscillatory or spiked systolic or reversed diastolic flow patterns in transcranial Doppler sonography (TCD) are associated with faster brain death confirmation, it has not yet been approved in our protocol. Thus, the aim of this study was to assess the applicability of this method to our organ donation system. MATERIALS AND METHODS: This study was performed in Masih Daneshvari Organ Procurement Unit from July to December 2009. TCD from the middle cerebral and basilar arteries was attempted in 35 patients who fulfilled the clinical and EEG criteria for brain death. Extensive skull defects and hypotension (blood pressure < 80 mmHg) were the exclusion criteria. Examinations were made for about 30 minutes via temporal and occipital windows as soon as possible after diagnosis of brain death. RESULTS: The mean age of cases was 31.9±14.78 years and 18 (51.4%) were males. The most prevalent cause of brain death was trauma (in 19 or 54.2% of cases). We were unable to detect any intracranial artery in 2 (5.7%) cases. There were no false negative or false positive results in the remaining ones. Detected ultrasonic patterns of cerebral vascular flow were systolic spike and oscillating signal in 29 (87.9%) and 4 (12.1%) donors, respectively. CONCLUSION: Our study showed that TCD results in brain dead cases were concordant with clinical and EEG criteria. Therefore, TCD, as a confirmatory test, can be applied for rapid diagnosis of brain death.
RESUMO
OBJECTIVE: Muscle biopsy is a very important diagnostic test in the investigation of a child with suspected neuromuscular disorder. The goal of this study was to review and evaluate pediatric muscle biopsies during a 2-year period with focus on histopathology diagnosis and correlations with other paraclinic studies. MATERIALS & METHODS: We investigated 100 muscle biopsies belonging to patients with clinical impression of neuromuscular disorder. These patients have been visited consecutively by pediatric neurologists during 2010 to 2012. Samples were investigated by standard enzyme histochemical and immunohistochemical techniques. RESULT: Sixty-nine (69%) males and 39 (39%) females with a mean age of 5.7 years were evaluated. Major pathologic diagnoses were Muscular dystrophy (48 cases), Neurogenic atrophy (18 cases), nonspecific myopathic atrophy (12cases), congenital myopathy (6 cases), storage myopathies (4 cases) and in 6 cases there was no specific histochemical pathologic finding. EMG was abnormal in 79 cases. Degree of correlation between EMG and biopsy result was significant in children ≥ 2 years of age. CONCLUSION: This study confirms the high diagnostic yields of muscle biopsy especially only if standard and new techniques such as enzyme study and immunohistochemistry are implemented. Also, we report 11 cases of Merosin negative congenital muscular dystrophy. This is the largest documented case series of Merosin deficient congenital muscular dystrophy reported from Iran.
