RESUMEN
AIM: To systematically review the clinical features and outcomes of paediatric patients developing neurological complications associated with a rotavirus infection. METHODS: A systematic literature review and meta-analysis was performed, including articles published from 1984 to 2020. Neurological complications were classified into four groups: encephalitis, cerebellitis, encephalo-cerebellitis and benign convulsions with mild gastroenteritis (CwG). RESULTS: Out of 68 reports that fulfilled the research criteria, 99 cases of CwG, 39 cases of encephalitis, 18 cases of encephalo-cerebellitis and five cases of cerebellitis were collected. Ninety-five patients were from Asia. Median age was 22 (IQR 14-29) months, and the children who developed CwG were significantly younger (19, IQR 12-24 months, p < 0.0001) than the others. Status epilepticus was observed in 23% and 5% of the encephalitis and CwG groups respectively. The most frequently described neuroimaging finding were lesions of the splenium of corpus callosum. Four deaths were reported in the encephalitis group, whereas no fatal events were described in the other groups. Among the surviving children, the encephalo-cerebellitis group showed the most severe long-term outcome. All cases of CwG recovered completely. CONCLUSION: Older age at diagnosis and the development of encephalo-cerebellitis are associated with a higher risk of long-term complications.
Asunto(s)
Encefalitis , Gastroenteritis , Infecciones por Rotavirus , Rotavirus , Humanos , Niño , Adulto Joven , Adulto , Infecciones por Rotavirus/complicaciones , Infecciones por Rotavirus/diagnóstico , Gastroenteritis/complicaciones , Convulsiones/etiología , NeuroimagenRESUMEN
BACKGROUND: Juvenile spring eruption of the helices of the ears is a distinctive sun-induced condition appearing on the light-exposed skin of the ears, typically in boys and young men in early spring. OBJECTIVES: To determine clinical features and outcome of juvenile spring eruption of the ears. METHODS: We report a new outbreak in 14 Swiss-Italian children. A systematic search of the literature was also performed. RESULTS: Five outbreaks in children involved a total of 203 cases (boys, 72%), and three outbreaks in young adults involved 223 male subjects. A further 54 sporadic cases were found: 41 among children (boys, 97%) and 13 among young adult males. The typical presentation included itching and diffuse erythema of both ears starting in the evening after exposure to bright sunlight during cold weather, followed within 24-48 h by papules or blisters. No other organ system was involved. The subjects recovered spontaneously without sequelae within 1-2 weeks. In New Zealand, among 162 school-aged boys, 20 developed the condition. CONCLUSIONS: A limitation is that the analysis was based upon the scanty available literature. Juvenile spring eruption is a self-limiting and generally easy recognizable variant of polymorphic light eruption. Outbreaks tend to appear on sunny and cold spring days. Paediatricians and general practitioners might rapidly develop the skills necessary to clinically appreciate this condition.
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Brotes de Enfermedades , Eritema/etiología , Trastornos por Fotosensibilidad/epidemiología , Enfermedades Cutáneas Vesiculoampollosas/epidemiología , Luz Solar/efectos adversos , Adolescente , Factores de Edad , Niño , Diagnóstico Diferencial , Oído Externo , Eritema/diagnóstico , Femenino , Humanos , Masculino , Trastornos por Fotosensibilidad/diagnóstico , Trastornos por Fotosensibilidad/etiología , Estaciones del Año , Factores Sexuales , Enfermedades Cutáneas Vesiculoampollosas/diagnóstico , Enfermedades Cutáneas Vesiculoampollosas/etiología , Adulto JovenRESUMEN
WHAT IS KNOWN AND OBJECTIVE: Deferasirox is a new treatment of iron overload that is administered orally once-a-day, resulting in better acceptance in patients. Deferasirox-induced renal tubular dysfunction has been reported on very rare occasions. CASE SUMMARY: A 17-year-old adolescent with ß-thalassaemia on deferasirox 30 mg/kg daily presented with isolated hyperchloraemic metabolic acidosis (bicarbonate 12·9 mM, sodium 137 mM, chloride 111 mM, potassium 3·6 mM). Acidosis resolved after withdrawing deferasirox. Naranjo adverse drug reaction scale suggested that the likelihood that deferasirox was responsible for acidosis was probable. Eight cases of metabolic acidosis have been reported in patients treated with deferasirox. In most cases, acidosis was associated with further features of renal tubular dysfunction. WHAT IS NEW AND CONCLUSION: We describe herein a case of metabolic acidosis in the setting of treatment with the deferasirox. Our case and the literature indicate a potential risk of kidney toxicity on this agent.
Asunto(s)
Acidosis/sangre , Acidosis/inducido químicamente , Benzoatos/efectos adversos , Cloro/sangre , Quelantes del Hierro/efectos adversos , Triazoles/efectos adversos , Acidosis Tubular Renal/inducido químicamente , Acidosis Tubular Renal/metabolismo , Adolescente , Benzoatos/uso terapéutico , Deferasirox , Femenino , Humanos , Quelantes del Hierro/uso terapéutico , Triazoles/uso terapéutico , Talasemia beta/sangre , Talasemia beta/tratamiento farmacológicoRESUMEN
WHAT IS KNOWN AND OBJECTIVES: A problem that often affects antihypertensive drugs is the lack of formulations appropriate for childhood. Parents, therefore, crush tablets and administer the antihypertensive drug mixed with solid food or a palatable drink. Because palatability is a major modulator of adherence to prescribed medication, the palatability of crushed ß-blockers, converting enzyme inhibitors and thiazides was assessed among adult volunteers. METHODS: The palatability of crushed atenolol, bisoprolol, enalapril, lisinopril, ramipril, chlorthalidone and hydrochlorothiazide was evaluated by means of a facial hedonic scale among 20 volunteers. The calcium channel-blockers amlodipine and lercanidipine whose tastes are disliked and liked, respectively, by children were also tested. A concealed random allocation procedure was used. RESULTS: The palatability scores assigned to chlorthalidone, hydrochlorothiazide and lisinopril were superior (P < 0·002) to those assigned to atenolol, bisoprolol, enalapril and ramipril. As with children, the palatability score of lercanidipine was superior to that of amlodipine (P < 0·002). The scores assigned to the various agents were similar in women and in men and were age-independent. WHAT IS NEW AND CONCLUSION: Pulverized atenolol, bisoprolol, enalapril and ramipril are poor tasting. From the perspective of palatability, pulverized chlorthalidone, hydrochlorothiazide and lisinopril are preferable.
Asunto(s)
Antagonistas Adrenérgicos beta/administración & dosificación , Inhibidores de la Enzima Convertidora de Angiotensina/administración & dosificación , Antihipertensivos/administración & dosificación , Gusto , Tiazidas/administración & dosificación , Adolescente , Antagonistas Adrenérgicos beta/química , Adulto , Inhibidores de la Enzima Convertidora de Angiotensina/química , Antihipertensivos/química , Bloqueadores de los Canales de Calcio/administración & dosificación , Niño , Preescolar , Femenino , Humanos , Masculino , Cumplimiento de la Medicación , Persona de Mediana Edad , Tiazidas/química , Adulto JovenRESUMEN
Arterial hypertension (AH), either primary or secondary, is an important issue in childhood for its short- and long-term cardiovascular morbidity. Renal diseases are the most frequent causes of AH in children, but essential hypertension can also be detected early in life. It is important for blood pressure (BP) to be checked regularly (at least once every 5 years) in healthy children and adolescents and every year in those belonging to at-risk categories (family history of AH, low birth weight, obesity, etc). In children, AH is defined as BP recorded in three non-consecutive measurements with an appropriate device and cuff size > or = 95th centile for age, gender and height. Ambulatory BP monitoring is a valuable diagnostic tool and once AH is confirmed, a specific primary cause should always be ruled out (renovascular, cardiac, vascular, endocrine, pharmacologic, other). In case of border-line or significant AH (between 90th and 99th centile) a non-pharmacological treatment should be considered, whereas severe hypertension (>99th centile for height and age) will require pharmacological treatment (Diuretics, Angiotensin Converting Enzyme Inhibitors, Angiotensin Receptor Blockers, beta - and Calcium blockers).
Asunto(s)
Hipertensión/diagnóstico , Hipertensión/tratamiento farmacológico , Niño , Femenino , Cardiopatías/etiología , Humanos , Hipertensión/complicaciones , Hipertensión/etiología , Enfermedades Renales/complicaciones , MasculinoRESUMEN
Fifty years after the first report by Gasser and Gautier, hemolytic, uremic syndrome is rather rare but severe childhood disease. A recent survey demonstrates that more than 90% of the cases occurring in Switzerland are caused either by Escherichia coli that produces shigatoxin or by Streptococcus pneumoniae.
Asunto(s)
Infecciones por Escherichia coli/complicaciones , Síndrome Hemolítico-Urémico/etiología , Síndrome Hemolítico-Urémico/patología , Infecciones Estreptocócicas/complicaciones , Femenino , Humanos , Lactante , SuizaRESUMEN
Pediatricians currently have improved understanding of how to best manage childhood hypertension. The goal of antihypertensive drug therapy in children with secondary hypertension is currently to reduce the blood pressure below the 90th centile. Most authors currently favor therapy with a blocker of the renin-angiotensin system (a converting enzyme inhibitor or an angiotensin II antagonist) or a calcium channel blocker. In patients with kidney disease and diabetes mellitus we generally advise therapy of hypertension with a blocker of the renin-angiotensin system especially in the presence of pathological proteinuria.
Asunto(s)
Antihipertensivos/uso terapéutico , Hipertensión/tratamiento farmacológico , Niño , HumanosRESUMEN
The responsiveness of plasma aldosterone to the infusion of angiotensin II at dose rates of 2, 4, and 10 ng/kg X min was assessed in 11 normal subjects and 13 patients with mild essential hypertension before and after 4 weeks of treatment with the sympatholytic agent debrisoquine. Debrisoquine treatment caused a significant (P less than 0.01) decrease in circulating norepinephrine (-45%), but did not modify plasma levels of angiotensin II, renin, aldosterone, or epinephrine or the metabolism of sodium or potassium. In normal subjects, debrisoquine caused a significant shift to the left (P less than 0.05) of the correlation relating plasma aldosterone to plasma angiotensin II levels. In patients with essential hypertension, the aldosterone-angiotensin II interrelationship was not modified. These findings suggest that the sympathetic nervous system exerts an inhibitory influence on aldosterone responsiveness to angiotensin II in normal man, and that this physiological interaction is impaired in patients with essential hypertension.
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Aldosterona/sangre , Angiotensina II/farmacología , Hipertensión/sangre , Norepinefrina/antagonistas & inhibidores , Adulto , Anciano , Presión Sanguínea/efectos de los fármacos , Debrisoquina/farmacología , Femenino , Humanos , Infusiones Parenterales , Masculino , Persona de Mediana EdadRESUMEN
The influence of agents that inhibit sympathetic nerve activity on cardiovascular responsiveness as related to major pressor factors has been unclear. Therefore, these components were evaluated in 11 normal subjects and 13 patients with mild essential hypertension before and after 4 weeks of sympathetic neuron blockade with the agent debrisoquine. In these normal and mildly hypertensive subjects, sympathetic neuron blockade caused approximately similar decreases in urinary and supine or upright plasma norepinephrine (NE) levels (average changes in the two groups, -41% and -45%, respectively; p less than 0.05 to less than 0.005), the chronotropic dose of isoproterenol (-45% and -38%), and the NE pressor dose (-47% and -51%, p less than 0.01), while the relationship between NE-induced changes in blood pressure and concomitant plasma NE concentrations was displaced to the left (p less than 0.01). Supine heart rate was also lowered (-10% and -8%, p less than 0.05). Compared to the orthostatic variations during placebo conditions, mild postural decreases in blood pressure were apparent in both the normal and hypertensive groups (-8% and -7.5%). However, supine blood pressure was unchanged following debrisoquine treatment. Other parameters were also not consistently changed, such as total blood volume, exchangeable body sodium, urinary electrolytes, plasma epinephrine, renin, and angiotensin II (AII) levels, the pressor dose of infused AII, and the relationship between AII-induced changes in blood pressure and plasma AII measured before and during AII infusion. These findings demonstrate that the reduction in sympathetic outflow during sympathetic neuron blockade may elicit a hyperresponsiveness of alpha- and beta-adrenergic receptors that is equal in normal subjects and patients with mild essential hypertension.
Asunto(s)
Debrisoquina/uso terapéutico , Hipertensión/tratamiento farmacológico , Isoquinolinas/uso terapéutico , Receptores Adrenérgicos/efectos de los fármacos , Sistema Nervioso Simpático/fisiología , Adulto , Anciano , Angiotensina II/metabolismo , Presión Sanguínea/efectos de los fármacos , Femenino , Frecuencia Cardíaca/efectos de los fármacos , Humanos , Masculino , Persona de Mediana Edad , Norepinefrina/metabolismo , Receptores Adrenérgicos/fisiología , Renina/sangreRESUMEN
Considering the documented, potentially undesirable influence of various thiazide-type or loop diuretics on serum lipoproteins, we prospectively investigated in 69 men (mean age +/- SEM, 32 +/- 1 years) the metabolic effects of the new diuretic-antihypertensive compound indapamide. Compared to placebo, indapamide (2.5 mg/day) given for 6 to 8 weeks lowered (p less than 0.02 to less than 0.001) blood pressure (supine values from 148/98 +/- 3/2 to 137/93 +/- 3/2) in 29 men with mild to moderate essential hypertension, but not in 40 healthy men. In both groups, significant (p less than 0.05 to less than 0.001) decreases in body weight (-0.8 kg) and plasma potassium (-0.6 mmol/L), and increases in plasma uric acid (+20%), renin activity (+200%), and aldosterone documented good compliance. There were no significant changes in total cholesterol (in all subjects, from 208 +/- 6 to 213 +/- 6 mg/dl), low- or very low-density lipoprotein (VLDL) cholesterol (127 +/- 6 to 129 +/- 6 and 21 +/- 1 to 21 +/- 2 respectively), high-density lipoprotein cholesterol (50 +/- 1 to 51 +/- 1 mg/dl), total triglycerides (Tg) (108 +/- 5 to 112 +/- 6 mg/dl), VLDL-Tg, apoproteins A1 and A2, plasma glucose, epinephrine, norepinephrine, sodium, calcium, magnesium, and creatinine; apoprotein B (84 +/- 2 to 88 +/- 3 mg/dl) and plasma insulin after glucose loading dose tended to be increased minimally. The absence of distinct lipoprotein alterations after short-term indapamide treatment may be of clinical and epidemiological interest.
Asunto(s)
Antihipertensivos/farmacología , Diuréticos/farmacología , Indapamida/farmacología , Lipoproteínas/sangre , Adulto , Apoproteínas/sangre , Análisis Químico de la Sangre , Peso Corporal/efectos de los fármacos , HDL-Colesterol/sangre , Evaluación de Medicamentos , Humanos , Masculino , Estudios Prospectivos , Triglicéridos/sangreRESUMEN
Little attention has been paid to interactions between circulating levels of calcium, PTH, and 1,25-dihydroxycholecalciferol [1,25(OH)2D] and bone mineral density in primary renal magnesium deficiency. Plasma and urinary electrolytes, and circulating levels of calciotropic hormones were studied in 13 untreated patients with primary renal tubular hypokalemic alkalosis with hypocalciuria and magnesium deficiency. The blood ionized calcium concentration was significantly lower in patients than in controls. Despite this fact, PTH and 1,25-(OH)2D levels were similar in both groups of subjects. The negative linear relationships between PTH and ionized calcium, which significantly differed between Gitelman patients and healthy subjects in terms of intercept; the negative relationship between ionized calcium and 1,25-(OH)2D, which was comparable in both groups; and the positive relationship between 1,25-(OH)2D and PTH, which was identical in both groups, point both to a blunted secretion of PTH induced by magnesium depletion and to the lack of interference of the latter with the activation of 1 alpha-hydroxylase by PTH. The similar bone mineral density at the lumbar spine by dual energy x-ray absorptiometry in 11 patients and 11 healthy subjects argues against chronically sustained negative calcium balance.
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Síndrome de Bartter/metabolismo , Calcio/metabolismo , Hormonas/metabolismo , Adolescente , Adulto , Síndrome de Bartter/clasificación , Densidad Ósea , Calcitriol/sangre , Calcio/sangre , Niño , Femenino , Humanos , Riñón/metabolismo , Vértebras Lumbares/metabolismo , Magnesio/sangre , Magnesio/orina , Masculino , Hormona Paratiroidea/sangre , SíndromeRESUMEN
To elucidate the mechanisms involved in calcium-mediated blood pressure (BP) control, plasma norepinephrine (NE), epinephrine, renin activity, and angiotensin II (AII) levels and the cardiovascular pressor responsiveness to NE and AII were assessed before and during acute mild hypercalcemia or short-term calcium (Ca) inhibition with nifedipine in 20 normal and five borderline hypertensive subjects. In normal subjects, systolic BP and plasma NE and epinephrine concentrations were increased significantly (p less than 0.05) during an acute rise in serum Ca of 3.1 mg/dl (intermediate rate Ca infusion, 0.05 mg/kg/min for 3 hours), but not following an increase of 1 mg/dl (low rate Ca infusion, 0.034 mg/kg/min for 2 hours). In the borderline hypertensive group, low-rate Ca infusion elevating serum Ca by 1 mg/dl was associated with a slight increase in systolic BP (p less than 0.05) and plasma catecholamines. In both groups, the pressor responses to infused NE and AII, and plasma renin and AII levels, were unchanged during mild to moderate hypercalcemia. Nifedipine given for 2 weeks (average dose, 48 mg/d) reduced BP significantly (p less than 0.05) in the borderline hypertensive subjects only and NE pressor responses in both groups (p less than 0.025), but had no significant effect on plasma catecholamines, renin, or AII levels. These findings suggest that the adrenergic BP control mechanism may be more dependent on clinical variations in calcium metabolism than the angiotensin BP regulatory mechanism. Acute hypercalcemia may increase BP at least in part by causing an increase in adrenergic activity without an equivalent decrease in cardiovascular reactivity. Calcium inhibition with nifedipine may modify noradrenergic BP control by lowering the NE pressor reactivity without causing an equivalent increase in adrenergic activity.
Asunto(s)
Presión Sanguínea , Calcio/fisiología , Hipertensión/fisiopatología , Adulto , Aldosterona/sangre , Angiotensina II/sangre , Angiotensina II/farmacología , Epinefrina/sangre , Femenino , Frecuencia Cardíaca , Humanos , Masculino , Nifedipino/farmacología , Norepinefrina/sangre , Norepinefrina/farmacología , Renina/sangreRESUMEN
A prospective evaluation was started in 1976 to study the influence of diuretics alone or combined with beta-blockers on serum lipoproteins in normal or hypertensive subjects. Compared to placebo conditions, 4 or 6-weeks' monotherapy with various diuretics significantly (p less than 0.05) increased the beta-lipoprotein fraction (furosemide, 80 mg/day or chlorthalidone, 100 mg/day; n = 16) or low-density lipoprotein-cholesterol (LDL-C) (chlorthalidone, 100 mg/day, n = 27 men; tienilic acid, 250 mg/day, n = 16 men, clopamide, 5 mg/day, n = 17 men; or muzolimine, 20 to 40 mg/day, n = 13 men or post-menopausal women). No increase in LDL-C was noted in 43 men (32 normal, 11 with mild hypertension) treated with indapamide, 2.5 mg/day. Serum high-density lipoprotein-cholesterol and apoproteins A1, A2 and B were not consistently changed by any of these agents. In women, chlorthalidone (100 mg/day) significantly increased LDL-C in the (100 mg/day) significantly increased LDL-C in the post-menopausal (n = 18) but not in the pre-menopausal (n = 22) state. Increases in LDL-C caused by chlorthalidone monotherapy were prevented or reversed by the addition of a beta-blocker, usually propranolol or atenolol (n = 18); increases in LDL-C during clopamide monotherapy were reversed after the addition of the beta-blocker pindolol (10 mg/day, n = 17). In all studies, variations in beta-lipoprotein or LDL-C levels could not be explained by changes in blood volume, serum glucose or insulin and did not correlate with alterations in blood pressure, plasma potassium, renin, aldosterone, adrenaline or noradrenaline. These observations indicate that various diuretics may increase serum LDL-C in men or post-menopausal women. Pre-menopausal women may often be protected from this side-effect. Long-term studies are now needed to clarify the pathogenic and prognostic relevance of lipoprotein changes induced by diuretics. In the meantime, it is of clinical interest that indapamide had no significant effect on serum lipoproteins and that certain beta-blockers appear to prevent or reverse increases in LDL-C during diuretic treatment in men and post-menopausal women.
Asunto(s)
Antagonistas Adrenérgicos beta/farmacología , Colesterol/sangre , Diuréticos/farmacología , Hipertensión/sangre , Indapamida/farmacología , Lipoproteínas/sangre , Triglicéridos/sangre , Antagonistas Adrenérgicos beta/administración & dosificación , Adulto , Apolipoproteínas/sangre , Apolipoproteínas B , HDL-Colesterol , LDL-Colesterol , Diuréticos/administración & dosificación , Quimioterapia Combinada , Femenino , Humanos , Indapamida/administración & dosificación , Lipoproteínas HDL/sangre , Lipoproteínas LDL/sangre , Masculino , Menopausia , Estudios ProspectivosRESUMEN
In adults the calcium antagonist amlodipine given once a day has proved to be an attractive addition to the antihypertensive armamentarium. The present report describes our experience in 43 paediatric outpatients (26 boys and 17 girls, aged between 1.1 and 19, median 9.8 years) with chronic kidney diseases. The patients were given amlodipine for 16 weeks as part of their antihypertensive treatment. Before amlodipine arterial pressure was 150 (142-163)/90 (84-95) mm Hg (median and interquartile range). Six patients withdrew from amlodipine because of oedema, flushing or headache. In the remaining patients amlodipine 7.7 (6.9-9.4) mg/m(2) body surface area once a day significantly decreased arterial pressure by 17 (13-22)/10 (7-13) mm Hg. The efficacy of amlodipine was more pronounced in girls than in boys. No changes in heart rate, body weight and circulating haemoglobin, sodium, potassium and creatinine were noted. In none of the patients circulating potassium, sodium or creatinine changed by more than 0.5 mmol/l, 5 mmol/l respectively 20%. In 11 patients concomitantly treated with cyclosporine the dosage and the trough-level of this agent were stable throughout the trial. In conclusion the present experience in paediatric outpatients with chronic kidney diseases supports the view that amlodipine is an effective and rather well tolerated antihypertensive drug when given once a day.
Asunto(s)
Amlodipino/farmacocinética , Antihipertensivos/uso terapéutico , Enfermedades Renales/tratamiento farmacológico , Adolescente , Adulto , Factores de Edad , Amlodipino/administración & dosificación , Amlodipino/efectos adversos , Antihipertensivos/efectos adversos , Presión Sanguínea/efectos de los fármacos , Niño , Protección a la Infancia , Preescolar , Enfermedad Crónica , Femenino , Frecuencia Cardíaca/efectos de los fármacos , Humanos , Hipertensión Renal/tratamiento farmacológico , Lactante , Glomérulos Renales , Modelos Lineales , Masculino , Estudios Prospectivos , Encuestas y Cuestionarios , Equivalencia Terapéutica , Resultado del Tratamiento , Población BlancaRESUMEN
In adults, the term specific pulmonary renal syndrome describes disorders with pulmonary and glomerular manifestations and includes Wegener's granulomatosis, Goodpasture disease, and systemic lupus erythematosus. Nonspecific pulmonary renal syndrome refers to either pulmonary disease complicating glomerular disease, or glomerular diseases following pulmonary disease. Since little is known regarding pulmonary renal syndrome in childhood, we reviewed the charts of 21 pediatric patients with pulmonary renal syndromes treated by the Department of Pediatrics, University of Bern between 1991 and 1998; we also reviewed the pediatric literature that deals with specific pulmonary renal syndromes. Specific pulmonary renal syndrome was noted in 3 children with systemic vasculitis (Wegener granulomatosis, N = 2; microscopic polyangiitis, N = 1) and 2 with systemic lupus erythematosus. Nonspecific pulmonary renal syndrome was observed in 12 patients with pulmonary edema (N = 9), pulmonary thromboembolism (N = 2), and pulmonary infection (N = 1) complicating the course of a glomerular disease, and in 4 children with a pulmonary disease followed by a glomerular disease. Review of the literature disclosed 52 cases of specific pulmonary renal syndrome other than systemic lupus erythematosus: Wegener granulomatosis (N = 28), Goodpasture disease (N = 13), and Henoch-Schönlein purpura (N = 11). In addition, hemolytic uremic syndrome complicated pneumococcal pneumonia in 32 cases. We conclude that pulmonary renal syndromes need to be looked for in childhood. Apart from Wegener granulomatosis, Goodpasture disease, and systemic lupus erythematosus, Henoch-Schönlein purpura and hemolytic-uremic syndrome occasionally have both pulmonary and renal features.
Asunto(s)
Enfermedades Renales/patología , Enfermedades Pulmonares/patología , Adolescente , Enfermedad por Anticuerpos Antimembrana Basal Glomerular/patología , Niño , Femenino , Síndrome Hemolítico-Urémico/patología , Humanos , Vasculitis por IgA/patología , Enfermedades Renales/complicaciones , Enfermedades Pulmonares/complicaciones , Lupus Eritematoso Sistémico/patología , Masculino , Pronóstico , Síndrome , Vasculitis/patologíaRESUMEN
Since published data on the course and prognosis of encephalitis in Central Europe is limited, we retrospectively evaluated 104 children with either acute strict sense encephalitis (n = 80) or acute cerebellar ataxia (n = 24) treated at the Department of Pediatrics, University of Bern, Switzerland, between 1980 and 1991. Of the 80 patients with strict sense encephalitis, four (5%) died acutely and 28 (36%) of 78 followed up had sequelae - eight patients with severe, six with moderate and 14 with mild sequelae. Young age and seizures were shown to correlate with poor outcome. Among the 24 patients with acute cerebellar ataxia, there was no fatal outcome and none developed severe residua, but six had mild and one had moderate sequelae. Initial cerebrospinal fluid white cell count was significantly higher in these children with sequelae compared with those without any sequelae after acute cerebellar ataxia.
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Encefalitis , Enfermedad Aguda , Ataxia Cerebelosa/diagnóstico , Encefalitis/epidemiología , Encefalitis/etiología , Encefalitis/terapia , Femenino , Humanos , Masculino , Estudios Retrospectivos , Índice de Severidad de la Enfermedad , Suiza , Resultado del TratamientoRESUMEN
Among the different forms of hereditary renal tubulopathies associated with hypokalemia, metabolic alkalosis and normotension, two main types of disorders have been identified: Gitelman disease, which appears to be a homogeneous post-Henle's loop disorder, and Bartter syndrome, a heterogeneous Henle loop disorder. A specific gene has been found responsible for Gitelman disease, encoding the thiazide-sensitive Na-Cl cotransporter (TSC) of the distal convoluted tubule. From a phenotypic point of view the characteristic findings of this disease are hypocalciuria, hypomagnesemia and tetanic crises appearing during childhood or later. Many subjects are asymptomatic. At least three different genes have been shown to be responsible for Bartter syndrome, characterized by mutations in the proteins encoding respectively the bumetanide-sensitive Na-K-2Cl cotransporter, the inwardly-rectifying renal potassium channel and a renal chloride channel, all protein transports located in the ascending limb of Henle's loop. Mutations in the first two transport proteins have been demonstrated in patients with the hypercalciuric forms of Bartter syndrome associated with nephrocalcinosis (respectively Bartter syndrome type I and II), who were often born after pregnancies complicated by polyhydramnios and premature delivery. Mutations in the gene encoding a renal chloride channel were recently recognized in patients with a Henle tubular defect not associated with nephrocalcinosis (Bartter syndrome type III). Most of the latter group of patients were normo-hypercalciuric and presented dehydration and life-threatening hypotension in the first year of life. However, these three genes do not explain all the patients with Bartter syndrome which unlike Gitelman disease, appears to be a very heterogeneous disorder. Clearance studies, especially if done during furosemide and/or hydrochlorothiazide administration, have been helpful in identifying the site of tubular involvement. Considering both phenotypic and genotypic data, we propose a clinical-pathophysiological and molecular approach to diagnose the different tubulopathies associated with hypokalemic metabolic alkalosis.
Asunto(s)
Alcalosis/fisiopatología , Síndrome de Bartter/fisiopatología , Hipopotasemia/fisiopatología , Presión Sanguínea/fisiología , Niño , Preescolar , Genotipo , Humanos , Lactante , Recién Nacido , Pruebas de Función Renal , Túbulos Renales/fisiopatología , FenotipoRESUMEN
AIMS: Studies dealing with the increased tendency to stone formation noted in cystic fibrosis, focus on enteric hyperoxaluria. It is well recognized, however, that low urine volume, hypocitraturia and perhaps even hypercalciuria are further risk factors for stone formation. METHODS: Nineteen patients with cystic fibrosis (14 boys and 5 girls, aged 10-23, median 15 years) underwent a standard protocol for metabolic evaluation of the lithogenic tendency. In 10 patients, the study was repeated after treatment with recombinant human growth hormone 43 microgram/kg body weight daily for 12 months. RESULTS: The metabolic evaluation disclosed low urine output in 12, hyperoxaluria in 8 and hypocitraturia in 9 of the 19 cystic fibrosis patients. The mentioned parameters were not influenced by treatment with recombinant human growth hormone. CONCLUSION: The report indicates that in cystic fibrosis low urine volume, hypocitraturia and hyperoxaluria act in concert and contribute to the likelihood of stone formation. This tendency is not modified by treatment with recombinant human growth hormone.
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Fibrosis Quística/complicaciones , Cálculos Renales/etiología , Adolescente , Adulto , Oxalato de Calcio/orina , Distribución de Chi-Cuadrado , Niño , Citratos/orina , Femenino , Glicolatos/orina , Hormona de Crecimiento Humana/administración & dosificación , Humanos , Masculino , Proteínas Recombinantes/administración & dosificación , Factores de Riesgo , Estadísticas no Paramétricas , Micción/fisiologíaRESUMEN
The recognized existence of a circadian pattern in extracellular magnesium balance might mirror either an inherent rhythm in the homeostasis of this ion or dietary factors. Since in vitro insulin enhances cellular magnesium uptake, the circadian rhythm in extracellular magnesium metabolism might be modulated at least in part by carbohydrate intake. To assess this hypothesis, the effects of oral glucose loading on plasma total and ionized magnesium were investigated in lean healthy humans with a negative family history for essential hypertension and diabetes mellitus. Plasma total and ionized magnesium was similar before glucose loading and 30, 60, 90, 180, and 210 min thereafter. It is therefore concluded that in healthy humans the circadian pattern of extracellular magnesium is not modulated by the metabolic and hormonal mechanisms that adjust the concentration of glucose.
Asunto(s)
Glucemia/análisis , Glucosa/farmacología , Magnesio/sangre , Administración Oral , Adulto , Glucemia/metabolismo , Calcio/sangre , Ritmo Circadiano , Estudios de Cohortes , Femenino , Glucosa/administración & dosificación , Humanos , Insulina/sangre , Magnesio/metabolismo , Masculino , Persona de Mediana Edad , Potasio/sangre , Factores de TiempoRESUMEN
Rhabdomyolysis occurred in two apparently healthy boys aged 9.5 and 5.5 years after general anesthesia with suxamethonium. Mild hyperkalemia and renal failure were observed in the first patient, who was subsequently diagnosed with Becker dystrophy. In the second patient, the clinical presentation was not classic for malignant hyperthermia and a muscle biopsy failed to disclose any pathological finding. A review of the literature revealed 66 pediatric cases (56 boys and 10 girls) of anesthesia-associated rhabdomyolysis. Forty-nine (74%) cases were caused by an underlying, mostly unrecognized congenital muscle disease, and 14 (21%) cases were caused by malignant hyperthermia susceptibility. Hyperkalemia (23 patients), cardiac arrhythmias (38 patients), renal failure (4 patients), and death (11 patients) were the most serious complications of anesthesia-associated rhabdomyolysis. The neuromuscular blocking agent suxamethonium had been used in at least 43 of the patients reported in the literature.