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.

[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.

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.

[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.

[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.

[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.

[Proposed protocol for the study of cerebrovascular disease in childhood]. / Propuesta de protocolo de estudio de las enfermedades cerebrovasculares de la infancia.

AIM: The etiology of cerebrovascular disease in the paediatric population, remains unknown in up to 40% of the cases ("idiopathic"), but recent advances could improve this percentage. We devised a comprehensive study protocol for such investigation aimed at the identification of potentially modifiable risk factors for paediatric stroke. PATIENTS AND METHODS: From the 141 patients initially registered in our data base for stroke population (from January 1984 until December 1995), we invited all the patients with idiopathic cerebrovascular disease to complete the study protocol. New cases appeared from January 1996 until July 1999 were also included. RESULTS: A total of 68 cases were identified. We found an etiology in 38% and in 76% of the cases we found at least one risk factor for stroke. Mild hyperhomocysteinemia was the most frequent risk factor identified (36% of patients versus 5% of controls), one of them an infant with fatal haemorrhagic infarct with classic homocystinuria. 31% of the patients had thrombotic risk factors (protein S, protein C, antithrombine III deficiency, factor V Leiden, etc). 17.6% had unspecific febrile illness at the time of the cerebral infarction and 11.6% had minor head injuries before the stroke. CONCLUSIONS: The use of the protocol improves the identification of potentially modifiable risk factors for stroke in childhood and may serve as a practical guideline for clinicians. The stroke protocol is as important as management strategies for acute stroke or for recurrence prevention, currently under consideration in the adult population.

[Non-traumatic cerebral hemorrhage in childhood: etiology, clinical manifestations and management]. / Hemorragia cerebral no traumática en la infancia: etiología, manifestaciones clínicas y manejo.

OBJECTIVE: Non-traumatic intracranial hemorrhage is a rare occurrence in children, with the most frequent etiology being vascular malformation. Our aim was to review the etiology and management of spontaneous cerebral hemorrhage in children. PATIENTS AND METHODS: We have reviewed the patients admitted to our intensive care unit (ICU) with spontaneous intracranial hemorrhage from 1980 to 1998. The etiology, symptoms and their clinical management were analyzed. RESULTS: Forty patients with non-traumatic intracranial hemorrhage were admitted during this time period. The mean age was 6.8 years and the male/female ratio was 2.4/1. This condition presented with a sudden onset in 83%. The most common form of presentation was headache, vomiting and altered consciousness. The most frequent etiology was vascular malformation (32.2%). Initial management was symptomatic and addressed the prevention or treatment of intracranial hypertension and seizures. Mortality was 38% and there were sequelae in 35%. CONCLUSIONS: Management of critical non-traumatic intracranial hemorrhage consists in preventing or treating cerebral hypertension. The monitoring of intracranial pressure, jugular oxygen saturation and transcranial Doppler allows an indirect measurement of cerebral blood flow. Prompt excision of the hematoma improves the outcome. In vascular malformations, endovascular embolization or radiosurgery are possible.

[Embryonic pathology caused by maternal phenylketonuria. A cause of underdiagnosed mental retardation. A report of 8 cases]. / Embriopatía por fenilcetonuria materna. Una causa de retardo mental poco diagnosticada. Revisión de ocho observaciones.

OBJECTIVE: Maternal phenylketonuria (MPKU) is characterized by intrauterine growth retardation, microcephaly, congenital malformations (mainly cardiac defects), dysmorphic facial features and mental retardation. There are women of child-bearing age that do not know that they are affected by phenylketonuria (PKU) and their pregnancies could result in damage to the fetus expressed as different neurological and congenital abnormalities. PATIENTS AND METHODS: We report 8 patients from 4 families. The first family had two offspring with intrauterine growth retardation, microcephaly and psychomotor retardation. The second family consisted of a daughter with mental retardation (without further data), a second baby which died during the first day of life, and a third child which died at 7 months of age with cardiac defects, microcephaly and dysmorphic features. Another child had intrauterine growth retardation, microcephaly psychomotor retardation, dysmorphic features and cardiac defects (coarctation of the aorta and subaortic stenosis). The third family had a son with microcephaly and mental retardation. The fourth family had a boy that died at 3 weeks of age with microcephaly, dysmorphic facial features, congenital heart disease (mitral atresia and septal defects) and Meckel diverticulum and a girl 5 years of age with intrauterine growth retardation, microcephaly and mental retardation. In all cases the mothers were unaware that they were affected by PKU and had mild intellectual defects. Two of them had PKU phenotypes. CONCLUSIONS: The offspring of PKU mothers untreated during pregnancy are affected by characteristic embriopathies related to the level of phenylalanine during pregnancy. In Spain, massive routine newborn screening was introduced around 1980-1985 and at present there are women of child-bearing age and are unaware that they are affected by the disease and that their pregnancies may result in fetal damage, as we demonstrate in these 8 patients. When faced with women with mental handicap or with antecedents of offspring with mental retardation, cardiac defects, microcephaly or intrauterine growth retardation, the determination of maternal phenylalanine concentrations is recommended. These teratogenic pathologies tend to disappear, but for the moment it is necessary to prevent this teratogenicity. The diagnosis is easy, avoids complementary exams, may help family studies and allows the implementation of dietary restriction during the subsequent pregnancy that would prevent fetal damage.

[Moyamoya disease. A cause of vascular occlusion in childhood]. / Enfermedad de Moya-Moya. Una causa de oclusión vascular en la infancia.

OBJECTIVE: The objective of this study was to review the cases of Moya-Moya diagnosed in our center. PATIENTS AND METHODS: We reviewed the Moya-Moya cases diagnosed from 1979 to 1997. We evaluated the following elements: age of clinical onset, sex, clinical features, complementary examinations, neuroimage, treatment and follow-up. RESULTS: Six patients were diagnosed. The first case appeared in 1979 and the last in 1997. These included four boys and 2 girls, with ages between 5 months and 14 years. The initial clinical feature in all six cases was acute hemiparesis, noting that in one case this was preceded by homolateral seizures. Neuroimaging revealed ischaemic infarction areas in brain CT or MNR. The diagnosis was based on angiography, where in four cases there appeared bilateral occlusion of the internal carotid, or of the anterior or middle cerebral arteries and in the other two there was a unilateral occlusion of the interior carotid and middle cerebral arteries. Regarding etiology, in four patients the dysfunction was due to either fibromuscular dysplasia of the carotid, neurofibromatosis, cranial trauma or to Down's syndrome. In the other two cases no other primary cause was found. CONCLUSIONS: Acute stroke is an infrequent disease of pediatric age patients, however it is necessary to do a thorough angiography study to rule out the Moya-Moya like vascular anomalies.

[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.

[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.

[Myopathy and polyneuropathy caused by colchicine]. / Miopatía y polineuropatía por colquicina.

A forty-six year old man with chronic renal failure and a toxic chronic liver disease developed progressive muscle weakness after a long trial with colchicine. Physical exam revealed muscle weakness and proximal muscle atrophy with hyporeflexia. Serum levels of creatine kinase were high and signs of myopathy and axonal and demyelinating polyneuropathy was evidenced in electrophysiological studies. Muscle biopsy disclosed a vacuolar myopathy, disruption of myofibers and dilatation of sarcoplasmic reticulum. The clinical pictures was attributed to a toxic myopathy and polyneuropathy due to colchicine, thus this treatment was discontinued. Four week later, the patient was symptom free, the levels of seric creatine kinase were normal and a new muscle biopsy was normal, with disappearance of previous histological findings.