Successful medical treatment of two patients with eosinophilic oesophagitis.

BACKGROUND/PURPOSE: Significant oesophageal eosinophilia is associated with oesophagitis and gastroesophageal reflux (GER). Eosinophilic oesophagitis is a rare disease that causes thickening of the oesophageal wall, narrowing of the oesophageal lumen, and severe motor disturbance. METHODS AND RESULTS: Two 12 yr-old patients with eosinophilic oesophagitis were studied prospectively. Clinical and investigation details are presented. Elemental formula was administered until complete remission of disease. Final outcome was assessed after 3 months on regular diet with exclusion of specific allergic components. Both patients responded to the dietary manipulation. CONCLUSIONS: Eosinophilic oesophagitis must be included in the differential diagnosis when dealing with oesophageal strictures of unknown or unclear aetiology. Elimination diet therapy plays a crucial role in ameliorating the course of the illness. Blood eosinophilia correlates with therapeutic response and with improvement of the disease.

Severe short-bowel syndrome in children. Clinical experience.

INTRODUCTION: Innovative surgical and pharmacological therapeutic measures in short-bowel syndrome (SBS) are constantly changing the prognosis of this devastating condition. The aim of this paper is to present our most recent experience in the treatment of this disease, with particular emphasis on the impact of home parenteral nutrition (HPN) and the use of growth hormone (GH). METHODS: A group of 8 patients with severe SBS have been studied for the past 4 years. Intestinal length of less than 25% normal at the time of bowel resection was the criterion for inclusion in this study. RESULTS: Mean age at the time of diagnosis was 2 years (ranging from 1 day to 9 years). The etiology of the SBS was Hirschsprung's disease (n = 3), midgut volvulus (n = 2), gastroschisis (n = 1), omphalocele with ileal atresia and necrotizing enterocolitis (n = 1) and Crohn's disease (n = 1). Length of the residual bowel was 8 and 50 cm with ileocecal valve (ICV) preservation and 23, 27, 30, 50, 70, 100 cm without ICV. Sixty percent of the patients survived. Two patients died due to fulminant gram-negative sepsis and one due to cardiac malformation. Two patients are still on parenteral nutrition (PN) providing 30 and 60% of total calories. Human GH (0.3 U/kg/day) was used in two patients over a period of 28 days. In these patients, an increased tolerance to enteral feeding was observed. HPN was provided in 5 cases, allowing regular school attendance in 3 patients. In 3 cases, discontinuation of the PN was achieved at 24, 25 and 35 months respectively. CONCLUSIONS: Human GH can improve tolerance of enteral feeding. HPN has a beneficial effect on child behaviour. Intestinal transplantation must be considered when no other surgical or medical measures are available.

Microsurgery in gaining paediatric vascular access for haemodialysis.

One hundred and one surgical procedures performed in children for construction and maintenance of vascular accesses for haemodialysis were retrospectively analyzed. There were 86 operations performed to create a new fistula in patients without vascular access or with nonrecoverable failed angio-access. Fifteen surgical procedures were performed to treat fistula complications. The new fistulas were radiocephalic n = 60 (70%), ulnar-basilic n = 5 (5.8%), antecubital n = 9 (10.3%), and PTFE grafts n = 12 (14%). Microsurgical techniques were used in all cases, including PTFE graft fistulas. A microscope was used in 56 cases (55.4%) and magnifying loupes (x 2.5 magnification) in the rest of the operations. Early-failure rate for radiocephalic fistulas was 10%. Cumulative patency rates in radiocephalic fistulas were 79%, 75%, and 70% at 1, 2, and 5 years, respectively. No statistical differences were found from the cumulative patency curve of 730 radial-cephalic fistulas performed in adults during the same period of time. Radiocephalic fistulas can be constructed in most paediatric cases using microsurgical technique. Elbow fistulas can be the second-choice vascular access, and PTFE grafts can be reserved for children with exhaustion of autologous veins. Brachial-jugular PTFE grafts can be used in cases of subclavian vein stenosis.

[Effect of mechlorethamine in idiopathic nephrotic syndrome in childhood]. / Efecto de la mecloretamina en el síndrome nefrósico idiopático en la infancia.

Mechlorethamine has been assessed in 28 patients with idiopathic nephrotic syndrome previously treated with prednisone only. One patient was cortico-resistant (CR), 22 cortico-dependent (CD) and 5 patients had frequent relapses (FR). FR patients had not histological examination and CR patient had a global focal sclerosis (GFS). Among CD patients, 12 had minimal change nephropathy (MCN) and 10 had a diffuse mesangial proliferation (DMP). After prednisone treatment was started, 0.1 mg/kg/d of mechlorethamine for four days was administered iv, in hospital and repeated one month later. After mechlorethamine treatment, GFS evolution was unchanged. Among FR, all the patients improved, 3 with complete remission and 2 with infrequent relapses. MNC improved in 66.6% and DMP in 40%, with the best evolution in immunofluorescence negative patients. Among early side effects, we found gastrointestinal disorders in 11 cases (39.2%) and leukopenia in 8 (28.2%), that required discontinuation of treatment in only 2 cases. We have not assessed gonadal toxicity. We comment the evolution after treatment of the different histological patterns, comparing with other nitrogen mustards and we conclude that mechlorethamine is useful in FR and CD idiopathic nephrotic syndrome corresponding to MCN or DMP with negative inmunofluorescence. Results with other nitrogen mustards, especially chlorambucil, are better.

[Nephronophthisis: study of 10 cases. Incidence, natural history and associated pathology (author's transl)]. / Nefronoptisis: análisis de 10 casos. Incidencia, historia natural y patología asociada.

Nephronophthisis is a chronic interstitial nephropathy which in childhood may lead to terminal renal failure. Between January 1975 and July 1980, 41 children with terminal renal failure were seen in our service, of which 10 (21.9%) presented with nephronophthisis. Age of the patients ranged from 3.5-18 years, boys were in the majority (8/2). Three cases were isolated and 7 were familial (3 families). Onset was during the first year of life in 8 patients, and polydipsia-polyuria were the first symptoms. Retarded growth and anemia proportionate to the degree of renal failure were present in all patients. When diagnosed, 5 patients (50%) presented terminal renal failure, and the other 5 had renal failure of different degrees. Moderate proteinuria was found in 4 patients, without changes in urine sediment. Sodium depletion in urine was high in 5 cases and maximal urine osmolarity was less than 500 muOsm/l after hydropenia in all cases. Four had associated mental deficiency with cerebellar ataxia associated in two and congenital hepatic fibrosis (confirmed histologically) in one. The diagnosis was confirmed by biopsy in 8 and in two of these on frozen section during nephrectomy prior to kidney transplantation. At present, five of the patients are in maintenance hemodialysis, two died at home due to cardiovascular complications in terminal renal insufficiency and the remaining ones presented different degrees of renal insufficiency. Time elapsed between onset of the symptoms and inclusion in hemodialysis or death ranges form 6 months to 13 years (mean 6.7 years).