Biomedical subjects
Publications and source records attributed to M G Bianchetti.
A 2-month-old infant with hereditary hyperekplexia, umbilical and bilateral inguinal hernias and history of poor feeding was noted to have severe normotensive metabolic alkalosis: sodium 132 mmol/L, potassium 3.4 mmol/L, chloride 77 mmol/L, pH 7.55, carbon dioxide tension 56.3 mmHg and bicarbonate 48.0 mmol/L. After parenteral rehydration and treatment with clonazepam, laboratory parameters normalized.
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.
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.
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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.
AIM: Efforts are currently made to detect vesicoureteric reflux (VUR) early after urinary infections in order to limit secondary renal damage. This study investigated the extent to which recommendations for the detection of VUR are put into practice, and their influence on the age at diagnosis. METHODS: The age at diagnosis of VUR after urinary tract infections was analysed in 126 patients (48M, 78F) referred to a tertiary centre in Milan between 1976 and 1999. RESULTS: The median age at diagnosis was 34 mo in subjects born before and 8 mo (p < 0.001) in those born after 1988. The difference was statistically significant in female but not in male subjects. The figures from Milan were compared with those for 102 patients (35M, 65F) born between 1946 and 1970, treated in Melbourne and reported in 1976. In Melbourne the median age at diagnosis was 1-2 y for boys and 5-6 y for girls; in Milan, the corresponding figures were <1 y and 1-2 y. The difference between Melbourne and Milan was statistically significant for both genders. CONCLUSION: In Milan VUR is now detected earlier than in the past. This trend is more marked in females than in males, but reflux is still detected earlier in boys.
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Vesicoureteric reflux is subcategorized into primary and secondary. Secondary vesicoureteric reflux results from increased bladder pressure duo to anatomic outlet obstruction or neuropathic disturbances. Primary vesicoureteric reflux was felt to result from a congenitally short mucosal tunnel length but this concept has been thrown into question. Recent studies suggest an association between lower urinary tract dysfunction and primary vesicoureteric reflux. Primary vesicoureteric reflux is often associated with kidney damage. It has been traditionally assumed that in children with primary vesicoureteric reflux kidney damage results from reflux of infected urine into the renal tissue. While there is unarguable proof that kidney damage can be acquired by the reflux of infected urine, the extent of reflux nephropathy explained by this mechanism has been overemphasized. Recent observations indicate that there are two categories of primary reflux disorder: a mild reflux associated with an acquired renal scarring secondary to infections which affects most females and a proportion of males; and a prenatal high-grade vesicoureteric reflux with a congenital nephropathy characterized by generalized hypodysplastic features which almost exclusively affects boys. Treatment options of primary vesicoureteric reflux range from surgical ureteric reimplantation to antimicrobial prophylaxis. Findings from comparative trials of prophylactic antibiotics and surgical management of children with high-grade vesicoureteric reflux do not show difference in renal growth and acquisition of new scars or renal function for 10 years. The factors accounting for the outcome in the mentioned studies are that most damage occurs at a very early stage and that severely damaged kidneys will either remain stable or progress to end-stage kidney disease, despite all efforts to cure the reflux.
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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.
UNLABELLED: The purpose of this analysis was to investigate biochemical disturbances at presentation and initial fluid resuscitation before surgery in infantile pyloric stenosis. The charts of 139 consecutive infants (113 boys and 26 girls) between 7 d and 20 wk of age with hypertrophic pyloric stenosis were reviewed. The infants were treated at the Department of Pediatric Surgery, University of Bern, Switzerland, in the period between 1987 and 1997. A trend towards hypokalaemia (13 of the 139 patients), hypochloraemia (39 patients) and especially metabolic alkalosis (98 patients) was frequently noted on admission. In 84 patients, data on fluid management and on circulating sodium, potassium, chloride and the acid-base balance immediately before surgery were also available. In these patients a significant correlation was found between the parenteral chloride dose given for fluid repair (y = 0.310 x; r = 0.54; p < 0.001) and the changes in plasma bicarbonate. The equation indicates that a chloride dose of 10 mmol/kg body weight is required to reduce plasma bicarbonate on average by 3 mmol/. CONCLUSION: Since assessment of the fluid volume stated by physical examination and history is inaccurate in infants with vomiting, the severity of metabolic alkalosis helps to define the amount of fluid required for repair.
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.
UNLABELLED: Blocking the formation of angiotensin II with converting enzyme inhibitors is an established intervention for kidney disease. The advent of antagonists of the angiotensin II receptor has increased the options for inhibiting the renin-angiotensin-aldosterone system. In adults, angiotensin II antagonists have antihypertensive and antiproteinuric effects similar to those of converting enzyme inhibitors and an adverse effect profile similar to that of placebo. In children, no information is available on angiotensin II antagonists. A total of 20 children (aged 4 to 17 years) with chronic kidney disease received the angiotensin II antagonist irbesartan given once daily. They had hypertension (n = 11), overt proteinuria (n = 3), or both (n = 6). At last follow-up, 2 to 17 months after starting irbesartan (median dosage: 3.3 mg/kg body weight daily), arterial pressure was significantly reduced: the systolic value by 16 [6-22] and the diastolic value by 11 [4-22] mmHg (median and interquartile range). In nine patients with proteinuria, the urinary albumin/creatinine ratio significantly decreased by 145 [105-209] mg/mmol. The frequency of reported adverse events was similar before and with irbesartan. CONCLUSION: In children with chronic kidney disease the effects of the angiotensin II antagonist irbesartan on arterial pressure and proteinuria mimic those observed with the converting enzyme inhibitors. The effectiveness of a single daily dose and the paucity of side-effects suggest that angiotensin II antagonists expand therapeutic options for inhibiting the renin-angiotensin-aldosterone system in children.
UNLABELLED: Chronic recurrent multifocal osteomyelitis is a rare chronic inflammatory musculoskeletal process observed in children and young adults. Recently, the acronym SAPHO syndrome (for synovitis, acne, pustulosis, hyperostosis, osteitis) was coined to emphasise the association between osteo-articular inflammations and different skin abnormalities which are aseptic and filled with neutrophils. In adults, chronic recurrent multifocal osteomyelitis is now a classical manifestation of SAPHO syndrome. Chronic skin disorders were seen in eight of ten children on follow-up at the University Children's Hospitals in Bern and Zurich and in 61 of 260 paediatric cases reported in the literature. The different skin lesions were palmoplantar pustulosis (n = 40), non-palmoplantar pustulosis (n = 6), psoriasis vulgaris (n = 16) or severe acne (n = 4). More rarely Sweet syndrome (n = 2) or pyoderma gangrenosum (n = 1) were reported. CONCLUSION: The synovitis, acne, pustulosis, hyperostosis, osteitis syndrome is pertinent even in paediatrics since skin involvement is frequent.