[Pediatric nephrology in developing countries]. / Néphrologie pédiatrique dans les pays en développement.

Pediatric nephrology is not a priority medicine in developing countries, but it should improve along with the development of both preventive and curative simple measures since the mortality rate is still important. This can be applied to the management of urinary tract infection, acute nephritis and some other cases of acute renal failure. The identification and management of chronic kidney diseases is mainly based on blood pressure measurement, proteinuria screening and plasma creatinine assessment. However renal replacement therapy (i.e., dialysis and transplantation) is highly expensive and its access is therefore limited to selected growing countries. The improvement in the care of renal children therefore requires better medical knowledge, nurse training and population information.

[Early hypomagnesemia, hypercalciuria and nephrocalcinosis: two cases in a family]. / Hypomagnésémie, hypercalciurie et néphrocalcinose de révélation précoce: deux observations familiales.

BACKGROUND: Hypomagnesemia-hypercalciuria and nephrocalcinosis is a rare inherited syndrome which is characterized by persistent hypomagnesemia despite supplementation, hypercalciuria, nephrocalcinosis and progressive renal failure. OBSERVATIONS: Case 1. A girl was referred at the age of 18 months because of polyuria, polydipsia and vitamin-resistant rickets. There was hypomagnesemia, hypercalciuria and mild renal insufficiency; ultrasonography showed nephrocalcinosis. For two years, she received hydrochlorothiazide and the course of the disease was marked by a significant reduction of urine output and hypercalciuria, recurrent urinary tract infections and a progression toward chronic renal failure. Case 2. The brother of this child was investigated at the age of nine months because of polyuria and polydipsia. He also had hypomagnesemia, hypercalciuria and nephrocalcinosis. Renal function was initially normal. After two years on continuous treatment with hydrochlorothiazide, hypercalciuria decreased without deterioration of renal function. No signs of rickets were noted and nephrocalcinosis remained stable. CONCLUSION: To our knowledge, these two patients are the youngest reported in the literature. The long-term deterioration of renal function is hazardous but rickets may be avoided by early administration of hydrochlorothiazide.

[Ultrasonographic evaluation of the kidney, liver and spleen size in children]. / Evaluation échographique de la taille des reins, du foie et de la rate chez l'enfant.

BACKGROUND: The aim of our study was to establish a standard growth curve of renal, hepatic and splenic dimensions with respect to age, and to find if any relationship existed between these different curves. PATIENTS AND METHODS: One hundred and fifty abdominal ultrasound studies were obtained on 62 male and 88 female normal children free of any chronic disease, whose ages ranged from 0 to 15 years. Hepatic, splenic and renal dimensions were obtained in a similar and reproducible fashion for all patients. A statistical study of the measurements obtained compared to the age was performed by dividing the subjects into five groups according to age, and after calculating the mean size and variance. RESULTS: Standard growth curves for the liver, kidney and spleen were constructed. Compared to age, splenic size follows the same growth as that of the kidneys, with a constant ratio. The growth pattern of the liver parallel the renal curve with a mean difference of 2.72 cm. CONCLUSION: A moderate enlargement of spleen and liver is difficult to evaluate only by clinical examination. Ultrasound may detect it by using the kidney size as a reference.

[Microcatheterization with gadolinium and glue embolization for post-biopsy arteriovenous fistula in a patient with renal failure]. / Microcathétérisme sous gadolinium et embolisation à la colle d'une fistule artérioveineuse post-biopsique chez un insuffisant rénal.

Percutaneous renal biopsy is frequently used in diagnosis of renal dysfunction. Nevertheless, complications, mostly vascular, may occur. Treatment must be minimally invasive with nephron preservation, especially in kidney failure. We present an interesting therapeutic approach with highly selective catheterization under gadolinium and glue embolization, leading to total cure of iatrogenic fistula without renal deterioration.

Reversible severe vascular rejection after plasmapheresis.

Vascular rejection in a transplanted child is frequently associated with bad prognosis on the short and long term. All therapeutic modalities available nowadays as monoclonal antibodies, intravenous cyclosporine or plasma exchange have given conflicting results. We report the case of a boy who was transplanted from his mother at the age of three. His primary disease was a cortico-resistant rapidly progressive glomerulonephritis; the immediate postoperative period was satisfactory but he developed a severe vascular rejection on day 5 post-transplantation, while on triple immunosuppression therapy associating antithymocyte globuline, prednisolone and azathioprine. Acute vascular rejection was corticoresistant but responded well to plasma exchange. In our patient, we believe that plasmapheresis was efficaceous on the reversibility of vascular rejection and moreover that he had not responded to corticotherapy even a week post methylprednisolone in the absence of any change in immunosuppression during plasmapheresis. No randomized study was realized until now to prove the efficacy of plasmapheresis in children with vascular rejection. The sporadic cases reported on the reversibility of such rejection after plasmapheresis, the innocuity of this therapy, if well performed in children, encourage us to consider this treatment modality in resistant vascular rejection.

Renal biopsy in children in a developing country in 61 consecutive cases.

Percutaneous renal biopsy in children is a safe procedure. It is used for definitive diagnosis, prognosis and evaluation of the response to therapy. Repeated percutaneous biopsies are currently performed especially in allograft kidneys and in native kidneys as well. No major complications were noted in our series. The use of the new automated technique with small disposable needles in a well sedated child and experienced operator minimizes the complication rate. The important clinical benefit of the percutaneous renal biopsy overcomes the minimal risk of the procedure. Mesangiocapillary glomerulonephritis and focal segmental glomerulosclerosis are the predominant histological findings in our series. More data from other centers are necessary to evaluate the real incidence of the different entities of renal diseases in our country.

Dilemma of oxalosis in end stage renal failure: isolated kidney allograft or hemodialysis.

We report the case of a 10-year-old girl who received a cadaveric kidney transplant for oxalosis after a period of 12 months on hemodialysis. The donor was a 6-year-old child. Cold ischemia was four hours. Diuresis occurred immediately in the operating room. Mean daily diuresis was maintained at 8 liters: first by i.v. perfusion, then by nocturnal continuous nasogastric hydration. In addition to the usual immunosuppressive drugs, she received pyridoxine, sodium citrate, phosphate, hydrochlorothiazide and magnesium. Daily hemodialysis was performed from Day 1 to Day 9 and four additional sessions every other day. The postoperative course was satisfactory. Oxaluria was elevated initially at 1074 mg/24 h (normal < 50 mg/24 h). One year later, mean daily diuresis is still 8 liters, renal function is normal and oxaluria is at 296 mg/24 h. Repeated graft sonography showed no nephrocalcinosis, but mild oxalate deposits are noted on renal biopsy. Isolated renal transplantation was successful in our patient. It allowed us to stop hemodialysis and to avoid extra-renal accumulation of oxalate. Despite this success, we are convinced that long term prognosis is uncertain and liver transplantation should be realized to correct definitely the biochemical defect.

[Kidney transplantation in the child. Experiences of l'Hôtel-Dieu de France]. / La transplantation rénale chez l'enfant. Expérience de l'Hôtel-Dieu de France.

A retrospective study was conducted on all kidney transplantations performed between January 1993 and June 1996 in our multidisciplinary pediatric department. Thirteen children with a mean age of 6 years (3.5-12) were transplanted during the study period after an average waiting time of 2 years on dialysis for cadaveric transplants and 3.5 months for living related ones. No urologic complication was noted; however, intra-abdominal approach was decided to be performed only in children less than 9 kg. This decision was taken after the occurrence of one intestinal intussusception and two ileal obstructions. During these 3.5 years, five rejection episodes were treated: three were steroid responsive, two were resistant but responded respectively to plasmapheresis and to OKT3. The actuarial survival of the grafts and recipients were 100% for an average mean time of follow-up of 18 months. The latest serum creatinine and creatinine clearance averages were respectively 66 mumol/l and 105 ml/min/1.73 m2. In seven transplanted children for more than 18 months growth was satisfactory. Eleven children returned to school. Kidney transplantation is the optimal treatment for children and infants with chronic renal failure; however, this technique needs a highly experienced team and a permanent close follow-up. Some children with end stage renal disease can directly have preemptive transplantation which is becoming our recent choice to avoid the hemodialysis strain.

Pediatric renal biopsy: ambulatory versus overnight hospitalization.

Renal biopsy may be safely performed on selected pediatric patients in an ambulatory care setting. In order to verify the safety of this new procedure, we performed 51 renal biopsies on an ambulatory care basis over a five-year period (from December 1993 to December 1998): twenty-eight biopsies on transplanted kidneys and twenty-three of native kidneys. During the same period, 56 renal biopsies were performed during a regular hospitalization of a minimum of 24 hours. We compared the two groups: Ambulatory procedure group and overnight hospitalization group. There were no significant differences between the two groups in mean age, adequacy of renal samples and complications such as post-biopsy pain or hematuria. After this encouraging experience, we are proposing the ambulatory procedure to all pediatric patients in our unit. Parents will be informed that if macroscopic hematuria is noted in the immediate post-biopsy period, hospitalization might be necessary.

[Multicenter study of children with terminal renal failure in Lebanon]. / Etude multicentrique des enfants en insuffisance rénale terminale au Liban.

A retrospective study was conducted in June 1997 concerning all Lebanese children with end stage renal failure. This study was able to recognize 20 children and infants (age less than 15) dialysed regularly in 8 hemodialysis centers. The other 23 centers are not following children. Thirteen children are dialysed on bicarbonate and seven on acetate. Children receive erythropoietin occasionally; 30% of them are polytransfused, 60% of them suffer from anemia. Four children have never received immunization against hepatitis B and three are seropositive for hepatitis C. None of these children has regular school attendance and psychological support for the child and his family do not exist. We conclude that the situation of children on hemodialysis in Lebanon is alarming. Solutions are possible and available; they need to be undertaken urgently.

Bartter syndrome in a neonate: early treatment with indomethacin.

The neonatal form of Bartter syndrome is characterized by intrauterine onset of polyuria leading to severe polyhydramnios. We report a patient with the early onset of the syndrome and a similar history in a previous sibling who died in early neonatal life. The patient is a female product of 33 weeks of gestation complicated by severe polyhydramnios. Her birth weight was 2,100 g. Polyuria led to severe dehydration on the 3rd day of life. Laboratory studies showed hypokalemia, hyponatremia, and elevated plasma levels of renin and aldosterone. Hypercalciuria was associated with echographic evidence of nephrocalcinosis. Indomethacin therapy resulted in a significant reduction in urine volume and correction of biochemical abnormalities. Growth and development are satisfactory after 4 years of indomethacin therapy, but nephrocalcinosis remains unchanged.

Kidney transplantation after a severe form of pseudotumor cerebri.

Pseudotumor cerebri is a syndrome characterized by intracranial hypertension (intracranial pressure >200 mmH2O) and a normal ventricular system. The diagnosis should be made as early as possible to prevent impairment of vision. Several diseases have been reported in association with pseudotumor cerebri in pediatric patients, and have been occasionally also noted with chronic renal failure, heart and renal transplantation. We report a 7-year-old boy who complained of severe headaches and visual impairment 2 years after hemodialysis for renal hypoplasia. Pseudotumor cerebri was suspected and, despite treatment with corticosteroids, acetazolamide, and lumboperitoneal diversion, visual impairment worsened. Bilateral optic nerve sheath decompression (ONSD) was performed without success and the child completely lost his vision within 2 weeks. He was successfully transplanted 2 months later. Two years post transplantation, the blind child has a normal renal function and school performance. Pseudotumor cerebri must be rapidly suspected in a child with renal failure suffering from headaches and papilledema. Visual loss may progress rapidly and ONSD seems to be the best surgical treatment when medical treatment fails. In this patient renal transplantation was well tolerated, with no deterioration in the neurological status over 2 years of follow-up.