Currently, among the regional methods of anesthesia, caudal anesthesia is most popular in pediatric practice in young children. Objective: To improve the safety of patients and improve the effectiveness of anesthetic protection of young children with Hirschsprung disease in transrectal resection of the large intestine by comparing various methods of anesthesia and creating an effective and safe anesthesia regimen.

Material and methods of research: The study included 25 children with Hirschsprung disease who underwent transrectal resection of the large intestine. All investigated patients are divided into 2 groups depending on the chosen method of anesthesia. In I clinical group (n = 13) multicomponent endotracheal anesthesia with high doses of fentanyl was selected by anesthesia, in II clinical group (n = 12), operative interventions were performed under general anesthesia combined with caudal block.

Results of the study: The level of cortisol in patients of the II group at the 2 nd stage of the study significantly decreased by 22%, which indicated the adequacy of this technique of anesthesia. Also in patients of this group at the 4th stage of the study, the level of cortisol and glucose did not change. And in Group I patients who underwent multicomponent general anesthesia with high doses of fentanyl, at the 4th stage of the study there was a significant increase in the level of cortisol and glucose by 35% and 24%, respectively. And this indicates the appearance of severe pain 24 hours after the operation.

Conclusion: 1. Caudal anesthesia combined with general anesthesia provides a reliable analgesic effect and miorelaxation in transrectal resection of the colon in Hirschsprung disease in young children.

2. The combined administration of promedol with naropin into the caudal canal prolongs the analgesic effect up to 24 hours, which was confirmed by the stability of the indices of central hemodynamics and stress markers

Langerhans cells histiocytosis is a relatively common ailment in pediatric age group. Common presentations include bone pain, pathological fractures, skin and scalp eruptions, features of pancytopenia and lymphadenopathy. Sometimes there can be chronic cough and shortness of breath due to lung infiltration. Acquired meningocoele is a rare presenting feature for this disease. Here we present one such case of unifocalpolyostotic Langerhans cells histiocytosis in a 16 year old male patient who had acquired a meningocele. Investigations showed multiple punched out lesions in skull and lytic lesions in several bones but the lung field was cleared. Biopsy and histochemistry confirmed the diagnosis. However the child was found to be HIV positive for which treatment was started but he developed renal failure due to hypercalcemia and died.

Familial Mediterranean Fever (FMF) is an inherited autoinflammatory disease whose standard treatment is colchicine, which prevents attacks and inflammatory complications in 80 to 90% of cases. The overall rate of non-response is around 10-20%. Anti-cytokine therapies have been shown to be promising in the treatment of this disease in case of resistance to colchicine. We report the efficacy of an il-1 receptor antagonist (anakinra) in the control of febrile seizures in a child with colchicine-resistant fmf and the main reasons for considering colchicine resistance.

This is the first retrospective descriptive study, describing the clinical and paraclinical aspects of 257 Lebanese children presenting a mitochondrial disease. The patients were diagnosed over 7 years, with a suspicion of mitochondrial disorder confirmed by muscle biopsy. The clinical and paraclinical signs were studied in this population.

Hypotonia (51.3%), psychomotor delay (11.6%) and epilepsy (7.39%) were the most common presenting signs. With the progression of the disease, hypotonia was found in 80.9% of the patients, while 78.21% reached a condition with multiple disabilities. The brain imaging showed different lesions in different patients ranging from white matter lesions to basal ganglia lesions and cerebellar lesions. The most common finding on the muscle biopsy was a cytochrome C oxidase deficiency (52.1%) and red ragged fibers were found in 17.5% of the patients. 36.19% of the patients needed at least one time admission to the intensive care unit.

Conclusion: this is the first large scale study on metabolic disorders in Lebanon, it shows that the mitochondrial disorders are the most common metabolic disorders in Lebanon, with a huge variety of presenting signs and symptoms, and with a severe neurological deterioration accompanying the disease progression

Purpose: The objectives of this study are to determine the change in growth centile z-scores and to determine the growth velocity differences between intensive dietetic intervention and standard care over a 6-month period in children less than two years of age, medically diagnosed with Failure to Thrive (FTT) and seen by a dietitian.

Methods: This is a retrospective service evaluation of paediatric dietetic inpatient and outpatient services between January 2012 and January 2014 at the National University Hospital (NUH) in Singapore.

Findings: There was an improvement in mean weight and height z-scores over the 6-month period, with significant larger improvements in mean weight z-score for the intensive intervention compared to standard care. Multidisciplinary team intervention and coexisting medical disorders were associated with significant changes in weight velocity.

Conclusions: Dietetic intervention is beneficial, especially in children with medical co morbidities. Multidisciplinary team intervention and control of underlying medical illnesses may also provide additional improvement in growth