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Biomedical subjects

L Garel

Publications and source records attributed to L Garel.

At least 37 records · Page 2Linked to original sources

Alagille's syndrome associated with cystic renal disease.

Although renal abnormalities have been described in children with Alagille's syndrome, cystic kidney disease has not often been documented, and then usually only at necropsy. Three children with Alagille's syndrome are described, in two of whom a unilateral multicystic dysplastic kidney was detected by prenatal ultrasound; in the other, a solitary cortical cyst was found later in childhood. All have normal renal function, growth, and liver synthetic function but continue to have clinical and biochemical signs of cholestasis. These cases show that unilateral cystic kidney disease with or without renal dysplasia may be associated with Alagille's syndrome, that the clinical course is not necessarily unfavourable, and that Alagille's syndrome should be included in the differential diagnosis of cystic kidney disorders associated with cholestatic liver disease. Patients with Alagille's syndrome should be evaluated by renal ultrasound.

Adolescent↗

Fetal congenital diaphragmatic hernia: accuracy of sonography in the diagnosis and prediction of the outcome after birth.

OBJECTIVE: This study evaluated the diagnostic accuracy of prenatal sonography in congenital diaphragmatic hernia (CDH) and assessed sonographic predictors of postnatal outcome. MATERIALS AND METHODS: Sonograms and medical records of 43 fetuses with CDH were retrospectively reviewed. Sonographic features were correlated to clinical evolution and surgical and pathologic findings. RESULTS: CDH was diagnosed prenatally with sonography in 40 cases. Intrathoracic stomach and liver were routinely identified. Ipsilateral lung tissue could not be differentiated from herniated content. Contralateral lung was identified in all cases except two. The overall survival rate was 43% after excluding terminated pregnancies. Besides associated malformations and chromosomal anomalies, the only statistically significant predictor of survival was the quantification of the contralateral lung area at the level of an axial four-chamber view: The survival rate was 86% when the contralateral lung area was equal to or greater than one half the area of the hemithorax. CONCLUSION: Sonography is highly accurate for prenatal diagnosis od CDH. Sonography also assists the prognostication of postnatal outcome in isolated CDH by allowing quantification of the contralateral lung area on a four-chamber view.

Female↗

Renovascular hypertension in children: curability predicted with negative intrarenal Doppler US results.

PURPOSE: To assess whether intrarenal Doppler ultrasound (US) enables prediction of the outcome of renovascular hypertension in a pediatric population. MATERIALS AND METHODS: A prospective study of 29 children with renin-mediated hypertension was performed. In these patients, intrarenal Doppler US was performed before angiography. Doppler US and angiographic findings were compared with patient outcome with respect to treatment required and blood pressure (BP) status after therapy. Cure was defined as a normal BP without medication. RESULTS: Intrarenal Doppler US was positive in 15 patients (52%), group 1, and negative in 14 patients (48%), group 2. In 10 patients (67%) in group 1, the severity of the vascular lesion precluded permanent cure. Cure was achieved in all patients in group 2, except for patients with neurofibromatosis. Thus, there was a statistically significant association (P < .05) between cure and a negative Doppler US result. CONCLUSION: In children with renovascular hypertension, a negative Doppler US result enables the prediction that a cure is more likely to be achieved with endovascular therapy or surgery.

Angioplasty, Balloon↗

Transient renal artery stenosis in infants: myth or reality?

We describe two infants with bilateral renal artery stenoses and severe hypertension. Adequate control of blood pressure was achieved by medical management. Endoluminal balloon dilatation of the renal arteries had been deferred because of their small size. On follow-up it was noticed that blood pressure had become progressively easier to control, with lesser amounts of antihypertensive medication. In fact, medication could be completely discontinued and blood pressure remained normal. On repeat arteriogram, bilateral renal artery stenoses had almost resolved in both patients. The etiology of the stenoses was not established in these patients. However, regardless of etiology, the transient nature of hypertension and renal artery stenoses in these two cases demonstrates that patient medical management of hypertension in infants can be a valid therapeutic option and occasionally obviate the need for unnecessary risky procedures.

Antihypertensive Agents↗

A tiny renal renin-secreting tumor.

A small 5 mm renal renin-secreting tumor is described in a child. Suggestions are made to circumvent the difficulties in investigating such cases. It is stressed that the diagnosis is probably often missed especially in the case of very small tumors.

Adenocarcinoma↗

Testicular torsion in infants and children: diagnosis with Doppler sonography.

The accuracy of Doppler sonography in the evaluation of testicular torsion was tested in 65 boys (aged 0-18 years) with acute scrotal pain or swelling. All patients underwent pulsed Doppler sonography of both testes, followed by scintigraphy (n = 16) and/or surgery (n = 34) and a close clinical follow-up for 4-8 months (n = 31). Color Doppler sonography was performed in 29 patients. The testicular artery was deemed patent if Doppler shifts from branches within the parenchyma could be found. Surgery revealed 19 cases of testicular torsion, 17 of which were diagnosed with Doppler sonography. There were six technical failures, in which no signals could be found on either side. In four boys, no color signals were obtained in either testis but subsequent examination with a mechanical sector scanner and pulsed Doppler sonography yielded arterial signals. Doppler sonography was successful in 59 of 65 boys (91%) and yielded a sensitivity of 89% and specificity of 100%. Pulsed Doppler sonography with mechanical sector scanners was more sensitive than color Doppler sonography. Intermittent torsion was missed both with Doppler sonography and scintigraphy.

Adolescent↗

Duplex Doppler sonographic examinations of the testis in prepubertal boys.

This retrospective study of 143 pediatric patients with unilateral acute scrotal disease was done to assess the value of duplex Doppler sonographic examination prior to puberty (110 patients) in comparison to a pubertal group (33 patients) in a pediatric hospital, where the examinations are done by staff radiologists and radiology residents of varying degrees of expertise and experience with Doppler technique. All patients seen during an 18 month period were included. The unaffected side was examined in most patients and served as control. The normal Doppler shift in the center of the prepubertal testis was found to be 0.2 to 0.5 kHz, when using a 5 MHz duplex Doppler probe. With puberty, the Doppler shift increased to 0.5 to 1 kHz. Of 18 patients (10 prepubertal) with testicular torsion, five (three prepubertal) had false-positive Doppler shift. In four of these five cases, faulty placement of the Doppler sample volume cursor was probably the cause. Using a multi-way frequency analysis, puberty was found to have no significant influence on results of Doppler signal (chi square = 0.1346; P = 0.7137). Duplex Doppler sonographic examination is as useful to rule out testicular torsion in prepubertal boys as it is after puberty. Meticulous technique is essential. The opposite side should be examined first and serves as control for the affected one. Results showing no flow in the center of the diseased testis with positive flow in the unaffected one should lead to further clinical action (scintigraphy or surgery).

Adolescent↗

Diaphragm of the gallbladder: a case report.

Gallbladder anomalies are rare and normally affect its shape, size, and position. We report on a 3-year-old boy with a gallbladder divided transversally by a windsock-type diaphragm, isolating the fundus from the rest of the biliary tree. Bilobed and multiseptated gallbladder have been described before, but this is the first isolated case of a congenital hourglass gallbladder.

Child, Preschool↗

Stenosis of the renal artery: assessment of slowed systole in the downstream circulation with Doppler sonography.

Slowing and dampening of systole in the arterial network distal to stenosis is a well-known Doppler sign of severe arterial stenosis. To determine whether this sign is present in boys and girls with such stenosis, intrarenal Doppler curves (acceleration index [AI] and resistive index [RI]) were compared with findings on renal arteriograms in 20 boys and girls; the AI was also measured in 10 boys and girls without renal disease. Statistical analysis of AI and RI measurements was performed. Eleven of 32 renal arteries were normal. The normal AI was 4.0-7.0; in arteries with greater than 75% stenosis, the AI was 0.7-1.7. In five arteries studied after angioplasty, the AI had changed from 0.7-1.5 to 4.0-5.6 at the first posttreatment examination (performed 28 hours to 1 week after angioplasty), and it remained normal during the 3-year follow-up period. In kidneys with stenotic arteries, the RIs were lower (0.43-0.54) than in healthy subjects (0.56-0.63). Regression and correlation coefficients of AI and RI measurements were statistically significant, and discrimination between normal arteries and those with greater than 75% stenosis was excellent.

Child↗

Intussusception in children: reliability of US in diagnosis--a prospective study.

To assess the diagnostic value of ultrasound (US) in clinically suspected acute intestinal intussusception in children, the authors prospectively compared US and enema studies in 83 episodes. None of the cases negative at US proved to be intussusception at enema study (negative predictive value = 100%). The sensitivity of US was 100%, and the specificity was 88%. Most of the sonograms were initially obtained and examined by residents. Several unsuspected abnormalities were also found with US. The authors conclude that a few months of training in US appears to be sufficient for high-accuracy investigation of clinically suspected intussusception, enabling selection of those patients in need of an enema.

Barium Sulfate↗

Antenatal diagnosis of ovarian cysts: natural history and therapeutic implications.

A retrospective study from 1980 to 1990 shows 29 ovarian cysts in 27 patients diagnosed by prenatal ultrasound performed between 28 and 38 weeks of gestation. Ten patients underwent surgery, 17 patients were observed with serial ultrasound. Delayed good quality sonograms after spontaneous resolution of the cyst in a selected group of 7 patients showed restoration of a normal ovarian anatomy. The size of the cyst and/or its sonographic characteristics are the 2 main factors for deciding a conservative or a surgical management.

Female↗

Surgical indications in antenatally diagnosed ovarian cysts.

The antenatal diagnosis of ovarian cysts poses a therapeutic dilemma because the natural history of these lesions is not well known. A retrospective review from 1980 to 1989 showed 29 ovarian cysts in 27 patients diagnosed by prenatal ultrasonography performed between 28 and 36 weeks of gestation. Nineteen cysts were initially observed. Eleven cysts resolved (diameter less than 2 cm), three are decreasing, three were lost to follow-up, and two underwent resection. Eight patients underwent surgical exploration immediately following birth. The diagnosis of benign ovarian cyst was confirmed histologically in all cases. A review of the literature showed an additional 230 cases of antenatally diagnosed ovarian cysts. Simple cysts of the ovary tend to resolve spontaneously and, therefore, may be treated conservatively. Serial ultrasonography allows accurate diagnosis and long-term assessment of ovarian cysts in the neonate and may prevent unnecessary oophorectomy. Patients with cysts larger than 4 cm may be candidates for percutaneous aspiration, or should undergo removal of the cyst because of a significant risk of torsion. Complex cystic masses, symptomatic ovarian cysts, and cysts that do not resolve should be removed.

Female↗

Normal portal venous diameter in children.

A study was conducted on 156 children, in whom the portal veins were measured using standard real time ultrasonography. Knowledge of the normal dimension may be important in patients with liver disease.

Adolescent↗