Our series shows improved results not only with survival but also in the number of patients that are off TPN. Multidisciplinary approach consisting of both medical and surgical expertise is necessary in the management of these patients. The authors advocate centralisation of short gut services to experienced centers with multidisciplinary expertise.
A systematic study of celiac disease in a defined population of Arab children has not been previously reported. We therefore performed a prospective study to determine the incidence and clinical presentation of celiac disease in Jordanian children. A total of 34 (12 boys and 22 girls) cases were diagnosed over a period of 36 months. Nine cases were clustered in three families. The incidence was calculated to be 1:2,800 live births. The mean age at presentation was 4.6 years (range 0.3-13), but it was 8.4 years at the time of diagnosis. Diarrhea was the main initial symptom in 15 (44%) patients. Other signs and symptoms included recurrent abdominal pain in five patients (14%), short stature in four (12%), abdominal distension in three (9%), constipation in three (9%), pallor and recurrent mouth ulcers in two patients each. At the time of diagnosis, 24 (71%) had hypochromic microcytic anemia, and nine (26%) had rickets. The relatively high incidence of celiac disease may be related to the large wheat consumption in this population (135 kg/head/year), and the late onset could be related to the widespread prevalence of prolonged breast-feeding in Jordan (12 months) and late introduction of cereals (6.2 months) in children with late presentation. The delay in diagnosis might be due to the presence of other diseases clinically resembling celiac disease in our community.
Outcomes for intestinal lengthening procedures are very good, and increasingly so. However, further analysis is required in order to fully understand the relative strengths and weaknesses of each procedure.
Clinical presentation and microbiology profiles of neutropenic paediatric oncology patients presenting with ecthyma gangrenosum (EG) were studied. Surgical strategies deployed for these critically ill children are reported. Between 1994 and 2005, all children with EG were identified. Case notes were reviewed. Hospital course and long-term outcome were documented. Ten patients were identified. Eight had acute lymphoblastic leukaemia, one child had acute myeloid leukaemia and another had rhabdomyosarcoma. Lesions occurred in the perineal region (n = 5), buttocks (n = 2), thigh (n = 2) and the face (n = 1). Seven children had positive blood cultures for Pseudomonas aeruginosa. Surgery included (1) radical debridement, and (2) debridement with covering colostomy for four of those with perianal lesions. Ecthyma gangrenosum is a rapidly spreading and potentially lethal condition. Paediatric oncology patients with neutropenia are at a high risk. Surgical excision is crucial for progressive lesions to prevent mortality.
Although advances have been made on the treatment of children with SBS, and improvements have been reached on home PN, this condition is still significantly affecting the quality of life of children and their families. The future quality control of medical care must have greater focus on psychosocial and emotional functioning, aiming for the best possible quality of life.
The treatment of short gut syndrome has evolved dramatically during the past decade. The combination of surgical techniques and medical management in the context of a multidisciplinary approach has improved the outcomes of these children. The authors describe in detail their technique of controlled tissue expansion of the bowel before lengthening procedures. Monitoring of the child and troubleshooting actions during the controlled tissue expansion program also are discussed.
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