[Chronic transplant nephropathy]. / Nefropatía crónica del trasplante.

In 2007 there were important scientific contributions in the field of kidney transplant and specifically in chronic transplant nephropathy (interstitial fibrosis and tubular atrophy). A new nomenclature and classification of chronic kidney disease was probably the most important contribution in this entity. Use of the C4d stain has allowed the concepts of glomerulopathy to be updated and to reveal the frequency of this entity and its impact in kidney transplant. Finally, two experimental studies provide new perspectives on the treatment of chronic kidney disease such as the use of statins or the use of pyridoxamine to block glycation end products.

[Neurological follow-up of very low birth weight newborns at the age of two years (1998-1999)]. / Seguimiento neurológico de recién nacidos menores de 1.500 gramos a los dos años de edad.

BACKGROUND: Because of improved obstetric and neonatal care, there is growing interest in the later outcome of very low birth weight newborns. OBJECTIVES: The aim of this study was to evaluate the survival rate of very low birth weight newborns and to identify disabilities at the age of 2 years. MATERIAL AND METHODS: An observational, follow-up study was performed of neonates with a birth weight of under 1,500 grams born between 1998 and 1999. The follow-up program included pediatric, maturative, neurological, psychological, ophthalmological, and audiological evaluation. Neurosensorial disabilities were classified as mild, moderate, or severe. RESULTS: One hundred thirty-six very low birth weight newborns were admitted. The survival rate was 77.9 % and 83.9 % completed the follow-up to the age of 2 years. The neurosensorial disability rate was 20.2 %; disability was severe in 9 %, moderate in 1.1 %, and mild in 10.1 %. In patients lost to follow-up, birth weight was higher, gestational age was older, and sonographic findings were more frequently normal. CONCLUSION: Survival in very low birth weight newborns has increased with improved neonatal care. The presence of sequelae was similar to that found in other follow-up studies. A substantial number of patients were lost to follow up, which influenced the disability rates.

[Idiopathic epileptic syndromes of the newborn]. / Síndromes epilépticos idiopáticos del recién nacido.

INTRODUCTION: Seizures during the neonatal period are the characteristic clinical expression of dysfunction of the nervous system. Not all the seizures seen during the neonatal period are due to epilepsy which only occurs in 10%. DEVELOPMENT: The aetiology of neonatal seizures is very varied and is mainly due to different types of aggression to the brain during this early stage of life. Epileptic syndromes are very rare during the neonatal period. In general the prognosis is very bad as occurs in infantile epileptic encephalopathy of early onset or myoclonic encephalopathy of early onset. However, the International League Against Epilepsy (ILAE) has identified and recognized some idiopathic epileptic syndromes of the neonatal period with a somewhat better prognosis. Two major groups have been established including the benign neonatal epilepsies (benign idiopathic neonatal epilepsies and benign familial neonatal seizures) and the group of status epilepticus (severe idiopathic status epilepticus). CONCLUSION: We analyze the different types of epilepsy of the newborn, form of onset, current knowledge of molecular biology, treatment and prognosis.

[The newborn infant at neurologic risk in the year 2000. Recommendations for the follow-up, incorporation of new instruments]. / Recién nacido de riesgo neurológico en el año 2000. Recomendaciones para el seguimiento, incorporación de nuevos instrumentos.

INTRODUCTION: Perinatal mortality has dropped markedly in recent years. However, there is still a considerable prevalence of neurological sequelas. Symptoms may present during the first months of life or later. Thus it is necessary to follow-up children with a clinical history of risk of neurological disorders. DEVELOPMENT: The various programmes for follow-up show slight variations in criteria of inclusion, calendar and methods of evaluation. We report the results of our follow-up of children at risk: long and short term sequelae in children of very low birth-weight and their correlation with neuroimaging findings. We review the use of some investigations (clinical examination, cranial ultrasound, CAT, magnetic resonance, EEG, visual and auditory evoked potentials and different biological, hemodynamic and nerve damage markers. CONCLUSIONS: It is necessary to: 1. Restrict the criteria for inclusion in hospital follow-up programmes; 2. Match a suitable investigative calendar to the pathology of the newborn baby; 3. Coordinate with the pediatricians of primary care areas and centres of early health care; 4. Use specific instruments for detection of mild sequelae at an earlier age; 5. Prolong follow-up at least until the age of 6-7 years old, and 6. To seek new biological markers to allow early detection of brain damage.

[Acute disseminated encephalomyelitis in childhood. Report of 10 cases]. / Encefalomielitis aguda diseminada en la infancia: presentación de 10 casos.

INTRODUCTION: Acute disseminated encephalomyelitis (ADEM) is a postinfectious encephalitis that is usually preceded by an infectious disease or vaccination. The clinical presentation has a wide spectrum and complementary exams are none specific, except magnetic resonance imaging (MRI) findings showing multifocal white-matter lesions similar to those seen en multiple sclerosis. PATIENTS AND METHODS: We report 10 children with the diagnosis of ADEM. We describe the clinical course and response to treatment. RESULTS: The prodroms were fever in all cases except one. The most common neurological symptoms were consciousness impairment, headache and seizures. The cerebrospinal fluid examination was abnormal in 9 patients with positive serologic test to enterovirus in one of them. MRI showed hyperintense multifocal subcortical white-matter lesions on T2-mediated images. Treatment with steroids was given to 5 patients, steroids and immunoglobulins to one patient and symptomatic treatment to the rest. From the last group one patient relapsed and then received corticosteroid treatment. The follow up revealed a complete recovery in 6/7 patients that received steroids. Three patients have sequelae and of these, 2 received only symptomatic treatment. CONCLUSIONS: The diagnosis of ADEM is based on clinical and radiologic features, once other entities have been excluded. At the moment of suspicious of ADEM a brain-spinal chord MRI should be done, seeing that TAC brings not much information at the beginning. The treatment with steroids seems to be the most effective and the prognosis good, specially in cases that respond rapidly to it.

[Usefulness of calcium acetate as a chelating of phosphorus in patients in hemodialysis with secondary hyperthyroidism]. / Utilidad del acetato cálcico como quelante del fósforo en pacientes en hemodiálisis con hiperparatiroidismo secundario.

In this study, we prospectively evaluated the efficacy of calcium acetate in patients with chronic renal insufficiency on hemodialysis programme with secondary hyperparathyroidism and hyperphosphatemia, which are difficult to control by means of the usual finders (calcium carbonate and aluminium hydroxide) and who were treated with pulses of calcitriol. We studied 10 patients. The inclusion criteria were: a serum phosphorus higher than 6.5 mg/dl, a serum PTHi higher than 250 pg/ml and a serum calcium higher than 9.5. The former therapy was stopped at the time of the patient was included in the study. Calcium acetate was initially introduced with doses between 2.5-4 g/day according to previous calcium and phosphate values. Also, all patients were initially treated with intermittent subcutaneous bolus of Calcitriol were modified and adjusted according to serum concentrations of calcium, phosphorus and PTHi. The concentration of calcium in the dialyzed was of 1.25 mmol/l. Fortnightly total calcium, phosphate and alkaline phosphatase serum determinations and monthly aluminium and PTHi serum determinations were carried out. During the 6 months treatment, a decrease was observed in serum concentrations of phosphate (p < 0.01), aluminum (p < 0.02) and PTHi (p < 0.001) with no changes in the values of calcium (p = ns) nor alkaline phosphatase (p = ns). The incidence of hypercalcemia was low during the follow-up period (11% of all biochemical serum determinations) and was easily controlled. We can conclude that calcium acetate is a sure and effective finder of phosphorus with a very good tolerance. Administered together with pulses of calcitriol, and the use of a low calcium concentration in the dialysate, it does not increase the risk of hypercalcemia.

[Application of the trigemino-facial reflex in acquired peripheral facial paralysis in childhood]. / Aplicación del reflejo trigémino-facial en la parálisis facial periférica adquirida en la infancia.

The AA report the results of the blink reflex and facial nerve stimulation in 16 children with acquired facial paralysis. Generally these verifications permitted the localization of the most impaired nerve segment and thereafter to give a recovery prognosis.

Servicio de Neurología del Hospital Sant Joan de Déu / Neurology Department of the Hospital Sant Joan de Déu

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Ambulatory blood pressure monitoring in kidney transplant patients: RETENAL study.

Hypertension is common following renal transplantation, affecting up to 80% of transplant recipients. It is generally accepted that hypertension is associated with poor graft survival and reduced life expectancy, contributing to increased cardiovascular risk factors and mortality rates. The aim of the study was to compare the blood pressure (BP) control in kidney transplant patients through the use of ambulatory BP monitoring (ABMP) versus office BP measurements (oBP). A multicenter, cross-sectional, observational study was conducted in 30 nephrology/kidney transplant units. Eligible patients included hypertensive cadaveric kidney transplant recipients aged <70 years, with a functioning kidney for at least 1 year and with an estimated glomerular filtration ≥30 mL/min/1.73 m(2) and a serum creatinine < 2.5 mg/dL. Recorded data included demographic characteristics, oBP, and ABPM and labroatory investigations. The 868 patients showed a mean recipient age of was 53.2 ± 11.6 years and mean follow-up after transplantation, 5.5 ± 2.8 years. Mean systolic and diastolic oBP were 140.2 ± 18 and 80.4 ± 10 mm Hg, respectively. Seventy-six percent of patients had oBP higher than or equal to 130/80 mm Hg. Mean 24 hour ABPM were 131.5 ± 14 and 77.4 ± 8.7 mm Hg for systolic and diastolic BP, respectively. Using the ABPM, we observed that 36.5% of subjects were controlled (mean 24-hour BP < 130/85 mm Hg). The two methods (oBP and ABPM) showed significant agreement. After ABPM, 65% of patients diagnosed as true controlled hypertension were considered to have white-coat RH. In clinical practice ABPM may help for better adjustment of drugs for adequate BP control.

Palatal tremor in childhood: clinical and therapeutic considerations.

Palatal tremor (PT) is a rhythmic movement of the soft palate that often causes an ear click. PT can be symptomatic (SPT) or essential (EPT). The symptomatic form usually occurs in adults and the essential form mainly occurs in children. Several different treatments for EPT in children appear in the literature with variable reported efficacy. This report details four paediatric patients with EPT (three males, one female; mean age 6y 4mo [SD 6mo]; age at onset 6-7y) treated with piracetam (2-oxo-1-pyrrolidine acetamide). Piracetam was used to treat EPT because of its antimyoclonic properties. All children showed a good response to doses of 100 to 300mg/kg/day. EPT relapsed on withdrawal of piracetam and remitted on reintroduction. Piracetam's effect on EPT was sustained. It is concluded that piracetam is an effective drug for the treatment of EPT in children.

[Periventricular leukomalacia: its retrospective diagnosis in children with spastic diplegia]. / Leucomalacia periventricular: diagnóstico retrospectivo en niños con diplejía espástica.

Typical images of residual periventricular leukomalacia (PVL) have been searched for in a review of the CT scans of 135 children with spastic diplegia. Among the 135 children studied, 90 (66.6%) of them had had a CT scan with 51 (56.6%) showing typical images of residual PVL. Only 6 (11.7%) of these 51 children were identified in the early stages by cranial ultrasound and 11 (21.5%) of the 51 were diagnosed by CT scan. The remaining 34 (66.6%) were diagnosed retrospectively during our study and none of them were identified as PVL in the first examination by CT scan. In addition to CT scan examination, 3 children were explored by MRI that confirmed the diagnosis of PVL. The results of our study indicate that images in CT scans reported as slight ventricular dilation, normotensive hydrocephalus, hydrocephalus with irregular limits, subcortical atrophy or periventricular heterotopia could corresponds to images of residual PVL in the appropriate context. We emphasize the importance of CT scan examination for the retrospective diagnosis of PVL in elder children in which cranial ultrasound examination had not been performed during the neonatal period.

[Intracranial arachnoid cysts in children. Review of 34 cases]. / Quistes aracnoideos intracraneales en el niño. Revisión de 34 observaciones.

Clinical, radiological, and therapeutical aspects of 34 cases of childhood intracranial arachnoid cysts are reviewed. Two thirds (23) were located supratentorially, the remaining 11 had an infratentorial localization. Of the first group 15 were situated at the sylvian region, 6 were hemispherical and 2 suprasellar. Clinical manifestations were present before 6 months of age in 68% of all the cases. Macrocephaly and focal seizures were the most common clinical findings. Skull X-Ray examination was helpful only when cysts were located at sylvian region. CT Brain Scan was positive in all cases (32) in which it was performed, dynamic studies with Metrizamide were necessary in 6 cases and arteriography in 4 cases to definitively prove the diagnosis. Surgical approach (removal of cysts or cyst/ventriculo-peritoneal shunting) was undertaken in 20 cases, with unequal results. Authors comment on possible etiopathogenic factors, indications for use of dynamic studies with Metrizamide and rational guide for surgical therapy on these patients.

[Multicystic encephalomalacia in twin pregnancies]. / Encefalomalacia multiquística en gestaciones gemelares.

Multicystic encephalomalacia (ME) is a rare entity in the pediatric age. In ME brain tissue in substituted by cavities of variable size. ME has different etiologies being asphyxia and circulatory alterations the most important factors. In monozygotic twins there is an increased incidence of structural anomalies than dizygotic twins. We present four twin patients with ME. Three of them had a prenatal dead sibling. The fourth pair of twins had a twin-twin transfusion. We want to stress the utility of brain echography in early diagnosis of ME.

[Alternating hemiplegia in infancy: clinical features, clinical course and treatment based on three cases]. / Hemiplejía alternante en la infancia: forma de presentación, evolución y tratamiento en tres observaciones.

Alternating hemiplegia is an infrequent form of complicated migraine. Clinical course has similarities with seizure disorders and correct diagnosis may be difficult. We report three patients whose onset in early childhood was with general impairment, transient hemiplegia, ocular movements and vasomotor symptoms. Clinical course of alternating hemiplegia is characterized by progressive neurologic deterioration. Intermittent motor impairment is alternating in side and later during the episodic attacks headache is present. Laboratory, electrophysiologic and neuroradiologic procedures are not demonstrative. In this report we show the findings in three patients in relation to the symptoms they presented, the utility of paraclinical investigations and their response to flunarizine treatment.

[Phenylalanine metabolites in hyperphenylalaninemic children]. / Metabolitos de fenilalanina en niños hiperfenilalaninémicos.

Phenylalanine and its organic acid derivatives, phenylacetate, mandelic, o-hydroxy-phenylacetate, phenyllactate and phenylpyruvate are increased due to the enzymatic block in the normal pathway of phenylalanine metabolism in phenylketonuric and hyperphenylalaninemic patients. These organic acids are neurotoxic and whether they are responsible for behavior and learning problems in hyperphenylalaninemic children remains to be seen. The purpose of the present study was to evaluate: 1) variations of the organic acid derivatives of phenylalanine during dietary treatment and 2) the usefulness of its determination to further adjust the phenylketonuric diet. Twenty-eight children, ages 4 months to 16 years, were studied. On the same day, the level of phenylalanine in the plasma and phenylalanine metabolites in freshly collected urine samples were tested. Phenylalanine metabolites were detected in some patients with blood phenylalanine levels below 242 microM/L and they rose as levels of blood phenylalanine increased with the phenylpyruvic acid being the highest when blood phenylalanine levels were greater than 424 microM/L. These compounds are usually not detected in normal urine. However, we have or learning problems, thus we do not make further adjustments in the PKU diet on the basis of phenylalanine metabolites.

[Magnetic resonance++ with gadolinium in Sturge Weber syndrome]. / Resonancia magnética con gadolinium en el síndrome de Sturge Weber.

We report four patients with Sturge Weber syndrome in which the pial angioma was visualized better with Gd-DOTA enhanced MR imaging. MR alone demonstrated thickened cortex with diminished convolutions, abnormal white matter and prominent subependymal veins. However, Gd-DOTA enhanced MR imaging improved the visualization of the angioma, localizing and demonstrating the extent of the malformation in Sturge Weber syndrome. We believe that this is the procedure of choice to recognize the extent of the intracranial involvement and which would help to select those patients that would benefit from surgical treatment.