Severe shoulder dystocia leading to neonatal injury: a case control study.

BACKGROUND AND OBJECTIVES: Severe shoulder dystocia (SD) is associated with neonatal brachial plexus injuries and skeletal fractures, with the former being the commonest cause for litigation related to birth trauma. The aim of this case-control study was to evaluate risk factors for birth injuries in cases presenting with SD. METHODS: Between January 2000 and December 2006, 22 babies who sustained brachial nerve paralysis or skeletal fractures following severe SD and requiring admission to Special Care Baby Unit (SCBU) were identified. The control group (n = 22) comprised the next infant delivered who was deemed to have SD but did not suffer significant birth injuries. Antenatal, labour and postnatal data were collected and compared between the two groups. RESULTS: The study and control groups had similar median maternal age (28 vs. 26.5 years), gestational age at delivery (40 vs. 40 weeks) and estimated blood loss (300 vs. 225 ml) (both P > 0.05, Mann-Whitney test). Median 1 min Apgar scores (5.5 vs. 7), maternal BMI (31.34 vs. 27.19 kg/m(2)) and duration of second stages (53.8 vs. 49.2 min) were also statistically similar in both groups (P > 0.05). However, compared to controls, brachial nerve injuries and skeletal fractures were more likely to occur in mothers with gestational diabetes (5/22 vs. 1/22) or who had previous big babies (4/22 vs. 1/22) (both P < 0.05, Fisher's exact test). Babies who had birth injuries were also more likely to have greater median birth weights (4.3 vs. 3.8 kg) and postnatal anthropometric measurements such as head circumference (35 vs. 34 cm) and ponderal indices (81.9 vs. 74.3 kg/m(3)) compared to controls. CONCLUSION: In babies with SD, brachial nerve injuries and skeletal fractures are more likely to occur in those with greater birthweights but also larger length to weight ratios. In these babies, assessment of abdominal circumference and biacromial length by magnetic resonance imaging (MRI) may help predict the likelihood of severe SD, especially in mothers with identifiable risk factors. However, further research in larger controlled trials are still needed to determine their predictive value.

Tratamiento del síndrome nefrótico corticorresistente / Treatment of corticosteroid-resistant nephrotic syndrome

El síndrome nefrótico corticorresistente representa el 5-10 por ciento del total de síndromes nefróticos en el niño. En realidad, bajo este mismo concepto se agrupan entidades tan heterogéneas como son la glomerulosclerosis segmentaria y focal, la esclerosis mesangial difusa, la glomerulonefritis membranosa, la glomerulonefritis mesangiocapilar e incluso el síndrome nefrótico por lesiones mínimas. Aunque se trata de un grupo dispar de entidades, presentan como característica común la resistencia, en mayor o menor grado, al tratamiento con corticoides. Muchos de los estudios en la bibliografia los presentan como un grupo común y emplean la misma pauta de tratamiento. El presente trabajo aborda el tratamiento de todas estas entidades en conjunto, pero incidiendo en particular en el tratamiento de la glomerulosclerosis segmentaria y focal (AU)

Towards guidelines for dialysis in children with end-stage renal disease.

There are few data describing the current practices of treatment selection for children with end-stage renal disease (ESRD). In an effort to establish a consensus among Spanish pediatric nephrologists for inclusion and exclusion criteria for renal replacement therapy in children with ESRD, in 1995 we surveyed members of the Spanish Pediatric Nephrology Association. Although only 43% of members responded, pediatric nephrologists and bioethicists studied the results and compiled a list of ten guidelines for treatment of children with ESRD. The proposed guidelines are meant to be a starting point for further discussion. An emphasis on flexibility, individual case assessment, and consideration of the best interests of the patient must remain central to any treatment plan. Decision making should ideally be shared by parents, professionals, the child, when appropriate, and ethics committees, as necessary.

Nosocomial Legionnaire's disease in a children's hospital.

BACKGROUND: Only a few cases of nosocomial Legionella sp. infection have been reported in children. We report the clinical and epidemiologic data of five nosocomial legionellosis cases that occurred in the Pediatric Nephrology Service between August, 1994, and December, 1998, and the control measures adopted. METHODS: The Hospital Materno-Infantil Vall d'Hebron, Barcelona, is a 407-bed tertiary care hospital. The pediatric kidney transplant unit has three isolated beds in the same ward within the Pediatric Nephrology Service. Diagnostic workup to establish Legionella pneumophila infection included culture, fluorescent antibody and serologic studies. Macrorestriction analysis of genomic DNA was used as epidemiologic markers of the isolated strains. RESULTS: In May, 1996, a case of L. pneumophila serogroup 6 pneumonia was identified in a 19-year-old youth who had received a kidney transplant 16 days earlier. Retrospective and prospective analysis of legionellosis cases diagnosed at our center up to August, 1994, yielded four additional cases. Four patients had had a kidney transplant and were receiving immunosuppressive therapy, and the fifth had been diagnosed with systemic lupus erythematosus with renal involvement. L. pneumophila serogroup 6 was isolated in bronchial secretions in four cases; in the fifth patient the diagnosis was made by serology. L. pneumophila serogroup 6 was isolated from potable water of the hospital. Molecular epidemiologic methods revealed the identity of the environmental and clinical isolates. Showering was implicated as the most feasible means of exposure to contaminated water. CONCLUSIONS: Nosocomial legionellosis, albeit rare in children, should be considered in the differential diagnosis of pneumonias, particularly in immunosuppressed children, because the fatality rate may be high without early diagnosis and treatment.

Autosomal recessive Alport syndrome: linkage analysis and clinical features in two families.

BACKGROUND: Genetic heterogeneity is a well-known feature of Alport syndrome (AS). Most families with AS show an X-linked dominant pattern of inheritance but about 15% of families show an autosomal inheritance of the disease. Autosomal recessive AS may account for 10% of the total number of cases and is caused by mutations in the COL4A3 and COL4A4 genes. The clinical spectrum of this rare disorder has not been well clarified. METHODS: We present two families with AS. Two affected members of these families have entered end-stage renal disease (ESRD) in their 30s, and the other three are older than 15 years and have normal serum creatinine. Four of the five patients have deafness but none have ocular abnormalities. Two have been transplanted and have not suffered from anti-GBM antibody nephritis. Men and women are equally affected. We have performed linkage analysis for chromosome 2 with the following markers: D2S279, COL4A3/4 DNTR, COL4A4 RFLP Hae III. RESULTS: We demonstrate that both families, one of them consanguineous, are linked to the COL4A3/4 locus. CONCLUSIONS: We can conclude that the only significant difference between the X-linked and the autosomal recessive forms of AS lies in the fact that in the latter females are as affected as males; thus the idea that autosomal recessive AS causes ESRD during childhood must be discarded. Other clinical features such as age of deafness or the presence of post-transplant anti-GBM antibody nephritis show no differences between the entities. Thus an accurate familial study is mandatory in patients with AS, as the identification of the different patterns of inheritance may cause a great difference in genetic counselling. Linkage analysis is the only effective molecular diagnosis that can be performed nowadays.

Long-term effects of cyclosporine A in Alport's syndrome.

BACKGROUND: In 1991, our initial results of cyclosporine A (CsA) administration in eight patients with Alport's syndrome were published. A significant decrease in or disappearance of proteinuria and apparently good tolerance to CsA were observed in all patients. METHODS: CsA administration has been maintained in these eight patients with the aim of obtaining further information about the clinical course of the disease. The ages of these eight patients currently range from 15 to 27 years, and the mean duration of treatment is from 7 to 10 years (x = 8.4 years). RESULTS: Renal function has remained stable, with no evaluable changes in serum creatinine levels compared with pre-CsA treatment values. Proteinuria in all patients has either remained negative or are values far lower than pretreatment levels. A second renal biopsy was performed in all patients after five years of CsA administration. No aggravation of the lesion present at the first biopsy or lesions typical of cyclosporine intoxication was observed. CONCLUSIONS: After a mean duration of 8.4 years and with no deterioration in renal function, we found possible beneficial effects of the continued treatment of CsA in patients with Alport's syndrome who present evidence of progression to renal insufficiency.

Tertiary hyperparathyroidism after renal transplantation.

Tertiary hyperparathyroidism is considered as an autonomous proliferation state of the parathyroid glands with biological hyperfunction resistant to calcium/vitamin suppressor therapy. This phenomenon is thought to be secondary to monoclonal inactivation of tumoral growth suppression factor located on chromosome 11. Three patients, 13, 15, and 22 years of age, with chronic renal insufficiency of long evolution who presented with tertiary hyperparathyroidism following renal transplantation are described. The three patients underwent subtotal parathyroidectomy with consequent normalization of biochemical parameters of phospho-calcium metabolism in the first few weeks post surgery. Pathologic study showed adenoma in the affected glands with hyperplasia of the rest. We believe that in patients with long-term renal insufficiency an aggressive treatment, either medical or surgical, of secondary hyperparathyroidism which is continued after renal transplantation may be useful in preventing the development of tertiary hyperparathyroidism.

[Berger's disease in children: its form of presentation, pathological anatomy and evolution in 22 cases]. / Enfermedad de Berger en el niño: forma de presentación, anatomía patológica y evolución en 22 casos.

We have reviewed 22 cases of Berger's disease in children (glomerular nephritis with mesangial IgA deposits), all of which were diagnosed by renal biopsy between 1976 and the present time. We describe the clinical and pathological findings in these patients. In addition, we put special emphasis on the evolution of the disease in relationship to some of the parameters that have been reported in the literature as being related to a bad prognosis of glomerular function such as, massive proteinuria at the onset of the disease, histological classification, presence of deposits of IgM or fibrinogen derivatives and glomerular sclerosis. All of the patients started with hematuria, 21 of which had gross hematuria (95%). Fourteen patients (63%) showed proteinuria (2 of which also had a temporary nephrotic syndrome). Five children showed some transient decrease in glomerular filtration rate and another patient rapidly developed renal failure and then end stage renal disease. We were able to follow 15 children for 3 years: 8 (53%) still showed outbreaks of gross hematuria, 5 (33%) only had microhematuria and 2 (14%) showed no signs of hematuria. Four children (27%) still had proteinuria. The glomerular filtration rate was still normal in all but two children (one with rapid evolution to end stage renal disease and another with a glomerular filtration decrease of 20%). Ten children were followed for 6 to 13 years. After 6 years, 2 (20%) still showed outbreaks of gross hematuria, 1 (10%) still had proteinuria.(ABSTRACT TRUNCATED AT 250 WORDS)

Renal calcifications in patients with autosomal recessive polycystic kidney disease: prevalence and cause.

OBJECTIVE: We investigated the prevalence of renal calcifications in children with autosomal recessive polycystic kidney disease and studied the metabolic changes that could cause this complication. SUBJECTS AND METHODS: Nine patients with known autosomal recessive polycystic kidney disease were examined with sonography and CT and screened for biochemical evidence of metabolic causes of nephrocalcinosis. RESULTS: CT showed bilateral renal calcifications in seven of the nine patients. The two patients without renal calcifications were less than 1 year old. Four patients had only a few calcifications and three patients had many. The severity of the renal calcifications correlated with the degree of kidney failure. All patients with kidney failure were found to have urine acidification defects. Hypocitraturia was present in all patients. CONCLUSION: Our results show that renal calcifications are common in older children with autosomal recessive polycystic kidney disease. Hypocitraturia and the urine acidification defect resulting from kidney failure are the leading factors in the pathogenesis of the calcifications.

[Renal transplantation in children: punction-aspiration biopsy and renogram with 99mTc-DTPA]. / Trasplante renal en niños: biopsia por punción-aspiración y renograma con 99mTc-DTPA.

BACKGROUND: Renal transplantation in children raises numerous diagnostic problems. The renography obtained with diethyltriaminopentaacetic acid marked with (99mTc metastable technetium (99mTc-DTPA) was compared with fine needle aspiration biopsy (FNAB) in search of a better interpretation of post-transplant crisis: rejection, acute tubular necrosis, toxicity by cyclosporin A and infection. METHODS: Sixteen acute post-transplant episodes were studied in 13 children submitted to renal transplantation. The post-transplant time was 6 days to 2.5 years. The basal renography and FNAB were carried out following the initial clinical manifestations and over a period of less than one week. The vascular and renographic phases were evaluated by interpreting the renography and were compared to previous renographies. The diagnosis of the aspiration biopsy was expressed as: normal, acute tubular necrosis, total necrosis, toxicity by cyclosporin A, viral infection and rejection. RESULTS: Results agreed in 14 out of 16 cases: 1 normal, 3 acute tubular necrosis by renography and cellular necrosis by cytology, 9 rejections and 1 infection (increase in renal transit time). In the 2 cases with total necrosis of 100% followed by loss of renal allograft, the renographic diagnosis was severe vascular rejection with a negative prognosis. CONCLUSIONS: In this series FNAB confirmed the renography as useful in the early diagnosis of complications which may appear in children undergoing renal transplantation.

Effect of cyclosporin A on proteinuria in patients with Alport's syndrome.

Eight patients with Alport's syndrome and massive proteinuria (129 +/- 60.57 mg/m2 per hour) were treated with cyclosporin A (CyA) for 8 months. The average dose of CyA administered to all patients was 4.21 +/- 0.26 mg/kg per day and blood CyA levels of 63.4 +/- 4.1 ng/ml were attained. In five patients, proteinuria abated during the 3rd week of treatment. In the remaining three, all of whom had low creatinine clearance (82.0, 46.0 and 43.2 ml/min per 1.73 m2 respectively), proteinuria persisted but at levels lower than before treatment: 32.5 +/- 15.9 mg/m2 per hour versus 183.3 +/- 29.7 mg/m2 per hour. No permanent decrease in creatinine clearance was observed in any of these patients throughout treatment. In those patients in whom proteinuria abated, it reappeared 2 weeks after discontinuation of CyA treatment. We observed no significant increases in angiotensin II plasma levels in our patients during CyA administration. Although we have shown that CyA will reduce massive proteinuria in patients with Alport's syndrome, we cannot yet recommend its use as a therapeutic measure.

Growth disparity in monozygotic twins discordant for chronic renal disease.

Growth data in seven pairs of monozygotic twins only one twin affected by renal disease are given. The data suggest that renal disease not only retards normal growth and sexual maturation but also affects growth potential. Therefore evaluation of the effect of renal disease and its treatment on growth should include estimation of the genetic potential for growth.

Renal arterial hypertension in pediatric patients.

Three hundred twenty-three children with hypertension due to different kidney disorders were analysed. Acute transient hypertension was the most frequent form of renal hypertension and was usually due to acute post-infectious glomerulonephritis. Chronic hypertension was frequently associated with renal insufficiency. In glomerular disorders the appearance of hypertension was usually accompanied by renal failure.

Long-term treatment with captopril in pediatric patients with severe hypertension and chronic renal failure.

Captopril was given to 42 hypertensive patients aged 1 to 17 years who were treated by regular haemodialysis because of terminal renal failure. Initially all patients were on antihypertensive treatment without sufficient control of blood pressure. Under captopril all patients presented a significant decrease of both systolic and diastolic blood pressures. The mean decrease of systolic blood pressure was from 162 to 114 and that of diastolic blood pressure from 106 to 86 mmHg. Plasma renin activity rose significantly in all cases whereas plasma aldosterone dropped and reached normal levels for age. The maximum effective dosage was 3 mg/kg/day. Total duration of treatment with captopril ranged from 1 1/2 to 6 1/12 (mean 3 2/12) years. At last observation the dosage of captopril varied between 0.3 and 3 mg/kg/day.

Long-term treatment with captopril in paediatric patients with severe hypertension and chronic renal failure.

Forty two children with end stage renal failure and hypertension on chronic haemodialysis have been treated with captopril for from 18 to 78 months. Satisfactory control has been obtained in doses of 0.3 to 3.0 mg/kg given every 24 or 48 hours. Tolerance was good. The results of the present study suggest that captopril is a suitable drug for long-term use in paediatric patients.