Biomedical subjects
Publications and source records attributed to M P Lavocat.
Botulism was suspected in a 17-day-old breastfed infant who developed over 2 days progressive muscular weakness and hypoventilation. The patient also presented with pupil dilation and light unresponsiveness. The electroencephalogram was normal. Full recovery was obtained after 85 days of artificial ventilation. Diagnosis was confirmed by the presence of the botulin toxin B in the patient serum. The source of the infection was not identified.
Antenatal Bartter Syndrome (ABS) is a rare autosomic recessive tubulopathy characterized by idiopathic hydramnios, fetal polyuria and elevated levels of amniotic chloride. It is related to mutations affecting several transporters in the loop of Henle e.g. the Na-K-2Cl cotransporter, the chloride channel CLC-NKB and the potassium channel ROMK. We report two cases of ABS in siblings born to consanguineous parents (first cousins). The first pregnancy showed hydramnios of unknown etiology at week 23. Two amnio drains were performed at weeks 26 and 27. The baby was born in week 29 and developed polyuria with hyponatremia, hypokalemia and hyperaldosteronism. After eliminating diabetes insipidus and adrenal insufficiency, ABS was diagnosed. The baby was treated with 0.5 mg/kg/d indomethacine, which controlled the polyuria and the hydroelectrolytic disorder. The second pregnancy showed idiopathic hydramnios at week 24. The elevated amniotic chloride level (above 112 mmole/l) led to the antenatal diagnosis of ABS. The mother was treated with 1 mg/kg/d indomethacine until week 31 in order to stabilize the hydramnios. Two amnio drains at weeks 31 and 33 allowed the pregnancy to be prolonged until week 34. A genetic study of the family showed homozygosity of the NKCC2 gene marker suggesting its implication in the disease.
UNLABELLED: Chickenpox has a high risk of invasive group A streptococcal disease and necroziting fasciitis. CASE REPORT: A five-year-old girl, during chickenpox treated with ibuprofen, developed sepsis and edematous and necrotic lesions of the pelvis and the abdominal wall. The child improved with surgical treatment and adjunction of clindamycin to the antibiotic therapy. CONCLUSION: We review the optimal medical and surgical treatment of necrotizing fasciitis and discuss the role of chickenpox and non steroidal antiinflammatory agents in this disease.
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UNLABELLED: Volvulus with or without malrotation are infrequent in the extremely premature newborn. CASE REPORTS: Intestinal volvulus in seven premature newborns are reported with abdominal distention, bright and tense skin without visible bowel loops and spiraled bowel loops on the abdominal X-ray. Intestinal resection was avoided due to early diagnosis. We identified abdominal wall massages as a risk factor, because no new cases have occurred since interdiction of these practices. CONCLUSIONS: Symptoms and radiologic findings are relatively specific for excluding the diagnosis of necrotizing enterocolitis in premature newborns. Abdominal nursing could be the incriminating factor.
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The diagnosis of fetomaternal alloimmune thrombocytopenia (FMAT) was made in a newborn with thrombocytopenia and intracranial hemorrhage. The first child of the family was severely affected with neurodevelopmental sequelae secondary to intracranial hemorrhage. According to the maternal HPA phenotype, close to 100% of subsequent pregnancies could be expected to be affected as the homozygous state was observed in both platelet systems. Another infant was born after a poorly followed pregnancy and was affected as was his elder brother. Prednisolone was given during another pregnancy. A thrombocytic newborn without intracranial hemorrhage was delivered by prudent cesarian section. The infant received platelet transfusion (maternal platelets). We present case histories of FMAT, and stress the conditions for prenatal diagnosis and management.
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At present, 99Tcm-dimercaptosuccinic acid (DMSA) renal scintigraphy is the most sensitive examination for the detection of parenchymal damage during acute pyelonephritis (APN) in children. This prospective study had three aims: (1) to evaluate the medium-term evolution of the scintigraphic abnormalities, to find a prognostic criterion of scintigraphic evolution; (2) to assess the correlation between the severity of early or late scintigraphic damage and selected clinical factors; and (3) to compare the permanent scintigraphic renal scars with intravenous urography (IVU) 2 years after the acute infection. Seventy-four children (mean age 32 months), presenting with a first clinical episode of pyelonephritis and an initial scintigraphic abnormality, were included in the study. Patients with a history of urinary tract infection (UTI), uropathy other than vesico-ureteral reflux (VUR) and a relapse of acute pyelonephritis were excluded. All children underwent control scintigraphy (mean 9 months after APN) and 43 had an IVU (mean 26 months after APN). Fifty-seven children (77%) still have scintigraphic abnormalities of varying severity (7 atrophic kidneys). Initial relative DMSA uptake of less than 45% results in a worse scintigraphic prognosis. The age of the child has no bearing on the severity of the initial renal involvement or on the evolution of the scintigraphic abnormalities. The rapid introduction of antibiotics (< 12 h) significantly improves the scintigraphic prognosis (P < 0.01). The presence of reflux (n = 39) leads to more serious initial damage, but we did not find any effect on later evolution in this study, in which all reflux was low grade in nature. Among the 43 children who had an IVU, 5 showed typical urographic and scintigraphic renal scars in the corresponding region and 38 showed a normal IVU with 28 cases of scintigraphic abnormalities. A DMSA scan is more sensitive than IVU for the detection of renal scarring after a first episode of APN.
OBJECTIVE: Accurate diagnosis of pyelonephritis using clinical and laboratory parameters is often difficult, especially in children. The main aims of this prospective study were to compare the value of different imaging techniques [renal sonography, cortical scintigraphy with technetium-99m dimercaptosuccinic acid (99mTc DMSA) and computed tomography (CT)] in detecting renal involvement in acute urinary tract infections and to determine the sensitivity of DMSA scans for permanent renal scars 6 months later. MATERIALS AND METHODS: Between February 1992 and January 1993, 55 children admitted to our pediatric unit with febrile symptomatic urinary tract infections were eligible for analysis. Ultrasonography (US), DMSA scanning and micturating cystourethrography were performed in every case. Only 18 children underwent CT. A second DMSA scan was performed in 48 children a mean of 7.5 months after the first. RESULTS: US abnormalities were found in 25 children (45 %). The first DMSA scan showed a parenchymal aspect suggestive of pyelonephritis in 51 patients (93 %). Among the 18 patients studied by CT, 14 had abnormalities. Normal US findings did not rule out renal parenchymal involvement. Scintigraphy appeared to be more sensitive than CT for renal involvement. The frequency and degree of initial renal parenchymal damage seemed to correlate with vesicoureteral reflux, but the most severe initial parenchymal defects were not associated with marked clinical or laboratory manifestations. Repeat DMSA scans, performed on 45 kidneys with abnormalities at the first examination, showed resolution in 19, improvement in 16, persistence in 8 and deterioration in 2. The prevalence of vesicoureteral reflux was not higher in patients with renal scarring on the second DMSA scan than in patients whose scans showed an improvement. CONCLUSION: DMSA scans should be considered as a reference in the detection and follow-up of renal scarring associated with acute urinary tract infection as this technique is more sensitive than US and CT, the latter being unsuitable because it entails radiation exposure and sedation of patients.
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Small children have often been reported to have poor outcome after kidney transplantation (KT). Recent reports from North America have shown that the use of living-related donors improves patient and graft survival. We report the experience in one centre of primary cadaveric KT using sequential immunosuppression in nine children aged 8-30 months and weighing 5.4-9.8 kg; donors were 0.7-12.3 years old. Four patients had pre-emptive KT and the other five were on peritoneal dialysis; the mean +/- SD waiting time was 2.0 +/- 2.4 months. Perioperative care has been published previously. The surgical approach was intraperitoneal if the aorta and vena cava were used (n = 7) and extraperitoneal for common iliac vessels anastomosis (n = 2); the duration of surgery was 3.5 +/- 0.9 h and the time for vascular anastomosis was 32 +/- 6 min. The recipients received ATG, azathioprine, prednisone and delayed administration of cyclosporin A. The patients were followed for 12-98 (median 41) months and showed good graft function (inulin clearance 63-100 ml/min/1.73 m2); only one child with recurrent haemolytic uraemic syndrome lost his graft three months post-transplantation and died after he had received a second graft. None of the recipients required post-transplant dialysis; arterial hypertension involved four children and was related to graft artery stenosis in two. Growth improved by 0.24 +/- 0.48 SD score of height per year.(ABSTRACT TRUNCATED AT 250 WORDS)
Urinary calcium/creatinine ratio (U Ca/creat) was studied in infants, term and preterm newborn babies. In 31 1.5-24 months old healthy infants, the median U Ca/creat was 0.16 mmol/mmol (range 0.013-1.17) and was similar to the value obtained in children older than 4 years. In 55 healthy full-term newborns studied in the first week of life, the median U Ca/creat was 0.12 mmol/mmol. However the range of values was extremely wide (0.0006-4.75), suggesting that the U Ca/creat ratio is of little interest to screen for hypercalciuria during the neonatal period. In 31 premature newborns, the median U Ca/creat was 1.08 mmol/mmol, a value significantly higher than in the two other groups (P < 0.001); as in the term newborns there was a very wide range of values (0.057-6.83). However after excluding the premature babies with elevated serum 25 OHD level, this difference was not statistically significant.
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The authors report on a case of tuberous sclerosis diagnosed in the neonatal period on the basis of intracardiac tumor, rib anomalies and cerebral calcifications. At 4.5 months of age the infant presented acute abdominal pains which led to the discovery (ultrasound identification confirmed by CT scan) of a giant ectasia of the whole abdominal aorta. The infant died 2 days later from the rupture of this aortic aneurysm.
Acute mediastinitis is uncommon. When it occurs, it usually follows an esophageal perforation or thoracic surgery. We report on a case of a 2-year-old girl with non traumatic mediastinitis secondary to a pharyngitis, due to Staphylococcus aureus. The anatomic pathways that connect the cervical region with the mediastinum explains how the infection can spread within the cervicothoracic spaces. Knowledge of this anatomy is also important in evaluating the possible causes of symptoms and signs in this area, and the possible complications of infections in these regions. The treatment associates antibiotics and drainage of the abscess by either surgical procedure or computed tomography guided selective needle aspirations.
The relationship between 24 hour urinary calcium excretion (U Ca/24 h) and urinary calcium/creatinine ratio (U Ca/creat) measured for morning and evening urine samples was studied in 56 children aged 4-15 years and hospitalized for benign conditions. Depending on the length of hospitalisation, 1 to 3 determinations of U Ca/24 h and U Ca/creat ratio were carried out for each child. Mean +/- SD U Ca/24 h was 0.05 +/- 0.058 mmol/kg. Mean U Ca/Cr, expressed in mmol/mmol, was 0.368 for total 24 h urine, 0.358 for the morning sample and 0.358 for the evening sample respectively. A good correlation was found between U Ca/24 h and 24 h U Ca/Cr ratio (r = 0.89), morning U Ca/creat (r = 0.83) and evening U Ca/creat ratio (r = 0.81) respectively. It is concluded that determination of the U Ca/Cr ratio for morning or evening urine samples is an efficient means of detecting hypercalciuria.