Objectives: To determine the outcome of autonomic disturbances in childrenwith Guillain Barre Syndrome. Study Design: Case series study. Setting: Pediatric ICU andNeurology ward of Nishtar Medical University/hospital Multan. Period: 9th June 2014 to 8th June2015. Methods: 48 children having age less than 18 years. Clinical examination of centralnervous system was conducted by single examiner to see presence of acute flaccid paralysisand to detect autonomic dysfunction. Cerebrospinal fluid analysis for albumino-cytologicdissociation, electromyography and nerve conduction studies was done in every patient ofGuillain Barre Syndrome. Outcome was noted as expired, discharged or LAMA. Results: Meanage was 5.4 years and male female ratio was 1.2:1. Total 34 children were found to have clinicalevidence of autonomic instability. Among 48 children, 17 children (35.4%) received no therapy,28 children (58.3%) received Intravenous immunoglobulin (IVIG) therapy, and 3 children (6.3%)received plasmapheresis. In children with autonomic disturbance, 24 children (70.6%) weredischarged, 8 children (23.5%) expired and 2 children (5.9%) LAMA. Of the total 14 childrenwithout autonomic disturbance, 11 children (78.6%) were discharged, 3 children (21.4%)expired and no child LAMA (p-value >0.05). Mean duration of stay for patients with autonomicinstability was 130 days versus 63 days in patients without autonomic instability (p-value<0.005). Demyelination variety was seen in 35 children (72.9%) and axonal degenerationin 13 children (27.1%). Mortality was high in children who had axonal degeneration but withinsignificant p-value of >0.05. Conclusions: Autonomic instability is common in patients ofGBS and is associated with increased risk of morbidity. Prompt recognition and treatment ofautonomic instability can improve the outcomes of children with GBS.
AimsPatients with Trisomy 18 have a poor prognosis – only 5%–10% of children survive beyond the first year; however various clinical features of Trisomy 18 such as gastrointestinal, cardiac and central nervous system anomalies may benefit from surgery. Providing intensive care including surgery must be carefully considered. We aimed to review surgical intervention in babies with Trisomy 18 and its outcome.MethodsA retrospective case note review was undertaken of babies with a confirmed diagnosis of Trisomy 18 admitted to NICU over a 5 year period (01/09/2011 to 31/08/2016).Results15 babies with Trisomy 18 were identified, 12 male and 3 female. Mean birth weight was 1.91 kg. Babies were born at a mean gestation of 39 weeks (range 31–41 weeks) (Tables 1, 2 and 3).Out of the 8 babies who had surgical pathology, 6 were stable enough to receive surgical intervention. 1 infant with a TOF/OA and 1 with complex cardiac problems were too unstable for surgery and died in hospital. Of the patients operated on, 1 baby died in theatre but the remainder were discharged home.Gastrointestinal problems were the main indications for surgery. 3 operations were undertaken for tracheo-oesophageal fistula, 2 for intestinal obstruction and 1 myelomeningocoele repair. Three of the 6 operations took place before a diagnosis of Trisomy 18 was confirmed.ConclusionPatients with Trisomy 18 who had surgical lesions that were amenable to intervention received surgery and had good short term outcomes with the majority of these patients discharged home. Surgery was performed both before and after the diagnosis of Trisomy 18 was confirmed, although it was suspected in all but one case. We therefore consider that a diagnosis of Trisomy 18 should not be a contraindication to surgical intervention. Our data shows that despite the poor long term prognosis of Trisomy 18, surgery should be considered to enable good quality palliative care, enable discharge home and improve the quality of a short life.Abstract G511(P) Table 1Abstract G511(P) Table 2Abstract G511(P) Table 3ReferencesBurke AL, Field K, Morrison JJ. Natural history of fetal trisomy 18 after prenatal diagnosis. Arch Dis Child Fetal Neonatal Ed. 2013; 98 (2): F152Burns D, Campbell. Twenty two survivors over the age of 1 year with full trisomy 18: presenting and current medical conditions. Am J Med Genet A. 2014 Mar; 164A (3)Prager K, Hardart G. Ethical challenges posed by trisomy 18 infants. Isr Med Assoc J. 2012 Aug;14(8):498–500.
To determine the outcome of autonomic disturbances in children with Guillain Barre Syndrome. Study Design: Case series study. Setting: Pediatric ICU and Neurology ward of Nishtar Medical University/hospital Multan. Period: 9 th June 2014 to 8 th June 2015. Methods: 48 children having age less than 18 years. Clinical examination of central nervous system was conducted by single examiner to see presence of acute flaccid paralysis and to detect autonomic dysfunction. Cerebrospinal fluid analysis for albumino-cytologic dissociation, electromyography and nerve conduction studies was done in every patient of Guillain Barre Syndrome. Outcome was noted as expired, discharged or LAMA. Results: Mean age was 5.4 years and male female ratio was 1.2:1. Total 34 children were found to have clinical evidence of autonomic instability. Among 48 children, 17 children (35.4%) received no therapy, 28 children (58.3%) received Intravenous immunoglobulin (IVIG) therapy, and 3 children (6.3%) received plasmapheresis. In children with autonomic disturbance, 24 children (70.6%) were discharged, 8 children (23.5%) expired and 2 children (5.9%) LAMA. Of the total 14 children without autonomic disturbance, 11 children (78.6%) were discharged, 3 children (21.4%) expired and no child LAMA (p-value >0.05). Mean duration of stay for patients with autonomic instability was 130 days versus 63 days in patients without autonomic instability (p-value <0.005). Demyelination variety was seen in 35 children (72.9%) and axonal degeneration in 13 children (27.1%). Mortality was high in children who had axonal degeneration but with insignificant p-value of >0.05. Conclusions: Autonomic instability is common in patients of GBS and is associated with increased risk of morbidity. Prompt recognition and treatment of autonomic instability can improve the outcomes of children with GBS.
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