Efficacy of hemodiafiltration in a child with severe lactic acidosis due to thiamine deficiency.

We report the case of a child in whom severe lactic acidosis (LA) and hyperammonemia developed after twenty days of total parenteral nutrition (TPN) for diffuse esophageal damage due to caustic ingestion. The revision of TPN preparation revealed that thiamine was never included and the hypothesis of thiamine deficiency was later confirmed measuring the serum thiamine level. Because severe metabolic acidosis the dialytic treatment with hemodiafiltration (HDF) and bicarbonate infusion were performed: the patient very quickly recovered with dramatic reestablishment of the acid-basic balance. Thiamine administration restored lactate metabolism. We emphasize that HDF is a useful and prompt treatment for LA to get over the critical phase of neurological and cardiological damage.

Progression of bone lesions in a child with primary hyperoxaluria type 1: evaluation by roentgenology and MRI.

We report the radiological and MRI findings of bone oxalosis and their rapid and extensive progression in a child with primary hyperoxaluria (PH) type 1. We emphasize the spectrum of skeletal changes and their differences from renal osteodystrophy and the role played by imaging in the diagnosis and follow-up of oxalosis.

[An echocardiographic study of the left ventricular functional indices in pediatric patients on hemodialysis and in treatment with recombinant human erythropoietin (r-HuEPO)]. / Studio ecocardiografico degli indici di funzione ventricolare sinistra in pazienti pediatrici emodializzati e in trattamento con eritropoietina umana ricombinante (r-HuEPO).

Recombinant human erythropoietin (r-HuEPO) is efficient in the treatment of anaemia in chronic renal failure on hemodialysis. We investigated the changes in cardiac function under r-HuEPO therapy using echocardiography. Seven patients with severe renal anaemia (Ht 19%) were treated with 50 U/kg r-HuEPO EV three times/week. Echocardiographic studies were performed in the anemic state and when hematocrit values were stable at levels (Ht 30%). Left ventricular function as showed by fractional shortening (D%) improved, the thickness of the interventricular septum and left ventricular hypertrophy were reduced. Our data indicate that the correction of renal anaemia by r-HuEPO can improve myocardial function in patients on maintenance hemodialysis.

[Influence of iron metabolism on the efficacy of r-HuEPO (recombinant human erythropoietin) treatment of anemia in children on hemodialysis]. / Influenza del metabolismo del ferro sull'efficacia del trattamento dell'anemia con r-HuEPO (recombined human erythropoietin) in pazienti embodializzati in età pediatrica.

Recombinant Human Erythropoietin (r-HuEPO) is efficient in the treatment of anaemia in terminal renal failure under dialysis. Five pediatric patients, who were under periodic hemodialysis, were treated and the interaction between the metabolism of iron and the response to r-HuEPO was studied in particular. In two patients it was noticed that a significant reduction of hematic ferritin levels occurred, while an efficient erythropoietic activity was maintained. On the contrary, three patients showed iron deficiency characterized by a reduced percentage of total transferrin saturation in the plasma, in the presence of high levels of ferritin in the blood. Also discovered was a missing increase or even a fall of the hemoglobin values that were obtained till now. In these cases, the increase of the hormone dose didn't lead to an improvement, that could only be obtained by the oral or parenteral administration of iron. The Authors in conclusion affirm that iron deficiency is the first cause to be searched for and to be corrected in the presence of missing hemoglobin increase even with adequate doses of r-HuEPO.

[Cystic renal disease in childhood]. / La malattia cistica renale nell'infanzia.

In the last five years, in the Surgery Pediatric Department and in the Pediatric Nephrology and Dialysis Service of the Florence University, have been observed 13 patients with cystic renal diseases. In every single case, for the diagnosis we have considered the age of the patients and the examinations made to them. We payed attention mostly to the echography considered the main diagnostic examination, even in prenatal age.

[Osteodystrophy in children with chronic renal insufficiency in dialysis therapy]. / Osteodistrofia nel bambino con insufficienza renale cronica in trattamento dialitico.

One of the most important complication of patients with chronic renal failure is osteodystrophy. This causes skeletal deformities, growth failure, bone pain and decreased physical activity. Osteodystrophy is more frequent among children than uraemic adults. In fact, 50-80% of children with chronic renal failure may occur in metabolic bone disease and the incidence tends to be higher in those children who have been in uraemic state for a long time before starting chronic haemodialysis. Osteodystrophy is a result of: 1) lesions of rickets; 2) lesions of osteitis fibrosa: 3) osteosclerosis. In contrast to adult, metastatic calcifications are virtually never observed in uraemic children. Hyperphosphoraemia, that is secondary to the reduction of G.F.R., may be the principal responsible of hyperparathyroidism that is the main cause of osteodystrophy. Hyperparathyroidism is also maintained and increased by deficit of 1,25(OH)2D3 which is responsible for lesions of rickets. Haemodialysis may markedly improve osteitis fibrosa and it is efficacious in reversing the mineral defect. Dialysate calcium concentration should be maintained at approximately 3,5 mEq/l. In this case we can raise serum calcium. On the contrary dialysate has to be lacking in phosphorus to correct hyperphosphoraemia. It must be noted that we have to prepare a dialysate with deionized water lacking in aluminum to avoid encephalopathy compliance.