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Biomedical subjects

A Daneman

Publications and source records attributed to A Daneman.

At least 37 records · Page 2Linked to original sources
PubMed1999

Observer reliability in grading nephrocalcinosis on ultrasound examinations in children.

BACKGROUND: Nephrocalcinosis is often associated with a variety of hypercalcemic conditions. Diagnostic ultrasound is often used for assessing nephrocalcinosis in children, but its reliability has not been proven. OBJECTIVE: To determine the reliability of expert interpretation of sonographic films with a grading scale of severity for nephrocalcinosis. MATERIALS AND METHODS: Fifty-eight ultrasonographic films of 30 children with Williams syndrome and other conditions know to be associated with nephrocalcinosis were assessed. We used a blinded randomized design to assess intra- and interobserver reliability. RESULTS: Grades I, II, and III nephrocalcinosis were noted in 13 %, 19 %, and 27 % of the examinations, respectively. The weighted kappa coefficient was 0.80 (standard error 0.12; 95 % confidence interval 0.68-0.92) for intraobserver agreement and 0.76 (standard error 0.13; 95 % confidence interval 0.63 to 0.89) for interobserver agreement. Reliability in assessing change from one examination to the next, with independently graded films, was fair with an unweighted kappa coefficient of 0.68 (95 % confidence interval 0.38-0.96) and 0.51 (95 % confidence interval 0.21-0.80) for intra- and interobserver reliability, respectively. CONCLUSION: The severity of nephrocalcinosis can be reliably interpreted with an ultrasonography grading scale.

Child↗
PubMed1999

Malignancies in pediatric patients with ataxia telangiectasia.

BACKGROUND: Patients with ataxia telangiectasia (AT), known to have an inherent increased susceptibility to the development of cancer, may present with malignancies that are unusual for the patient's age, are often difficult to diagnose clinically and radiographically and respond poorly to conventional therapy. MATERIALS AND METHODS: We reviewed the clinical presentation and imaging studies of 12 AT patients who developed malignancies. RESULTS: Eight of the twelve patients developed non-Hodgkin's lymphoma (CNS, thorax, bone), two developed Hodgkin's disease, and two were diagnosed with gastrointestinal mucinous adenocarcinoma. CONCLUSION: The lymphomas were commonly extra nodal, and infiltrative rather than mass-like. The recognition of the tumors was often delayed due to confusion with the known infectious complications in AT patients.

Adenocarcinoma↗
PubMed1999

Unsuccessful air-enema reduction of intussusception: is a second attempt worthwhile?

Pneumatic reduction of idiopathic intussusception is successful in about 80% of cases, while 60% of the failures are reduced at surgery without resection. To determine whether delayed, repeated attempts at enema reduction of failures would reduce the need for operation in selected cases, over a 2-year period (1994-1996 inclusive), 17 infants with idiopathic intussusception underwent delayed repeat enemas 2-19 h following the first failed attempt at reduction. Clinical parameters and radiologic findings were evaluated with respect to outcome. Ten intussusceptions were successfully reduced after the second attempt in 9 and after the fourth attempt in 1. Seven children underwent a laparotomy, 5 because of failure of progressive reduction at air enema (AE). Two were taken to surgery early in the series, 1 because of perforation during a second attempt and 1 while awaiting a third reduction attempt. The 10 successful reductions all showed progressive movement of the intussusceptum on each AE; the 2 who perforated failed to show progressive reduction on their second AE. Because of these cases, the remaining 5 were referred to surgery because of failure of progressive reduction of the intussusceptum on the second attempt. At laparotomy, of the 7 unsuccessful reductions, 4 required resection and 3 had difficult manual reduction. The presence of vomiting, a mass, and/or bloody stools were not predictors of outcome. Failures had higher body temperatures (38.1 +/- 0.3 vs 37.4 +/- 0.1 degrees C, P = 0.07), heart rates (153.7 +/- 8 vs 136.9 +/- 2.1 min, P = 0.03), and longer duration of symptoms (36.8 +/- 4 vs 21.3 +/- 3.6 h; P = 0.01) than successes. Delayed repeat AEs may be safe and effective in selected cases of idiopathic intussusception, but should be considered only if significant movement of the intussusceptum is noted at each attempt. The ideal time for repeat AE reduction prior to surgery is not established, but 2-4 h appears appropriate. Pyrexia, tachycardia, and duration of symptoms greater than 36 h are relative contraindications to this course of management.

Air↗
PubMed1999

Nonocclusive small bowel infarction in familial dysautonomia syndrome.

The authors report a case of a 14-year-old boy with familial dysautonomia (FD) in whom a small-bowel infarction developed during a dysautonomic crisis. Atypical features of the presentation included hypotension with prolonged fever and abdominal distension. The authors postulate that the bowel infarction was caused by prolonged hypoperfusion. It is recognized that the small bowel in normal subjects can adapt to periods of ischemia without irreversible injury. The authors speculate that the known abnormal systemic cardiovascular regulation in patients with FD may adversely affect splanchnic blood flow, which led to the catastrophic consequences in this case. This report draws attention to the risk of significant ischemic complications during a dysautonomic crisis, especially in the face of atypical features, and emphasizes the challenging cardiovascular management of such patients.

Adolescent↗
PubMed1999

Clinical utility of adrenal ultrasonography in the diagnosis of congenital adrenal hyperplasia.

Rapid, accurate diagnosis of congenital adrenal hyperplasia (CAH) is essential in the neonate with ambiguous genitalia, life-threatening salt loss, or both. We aimed to determine the accuracy of adrenal ultrasonography in the diagnosis of CAH in a retrospective analysis of 52 children with ambiguous genitalia or salt-losing crises. Adrenal ultrasounds were interpreted as follows: "normal" if the adrenals were normal in size (adrenal limb width <4 mm), had a smooth surface, and a central echogenic stripe with a hypoechoic rim, and "abnormal" if they were increased in size (limb width >4 mm), had a lobulated or cerebriform surface, or showed abnormal echogenicity. Group 1 consisted of 25 neonates and infants with CAH; group 2, 19 children with conditions other than CAH; and group 3, 8 with treated CAH: 7 receiving replacement therapy and 1 whose mother received glucocorticoids during pregnancy. In all children in groups 2 and 3, adrenal ultrasounds were read as normal. In group 1 adrenal ultrasonography was normal in 2 (8%) and abnormal in 23 (92%). Thus adrenal ultrasonography has a sensitivity of 92% and a specificity of 100% for diagnosing CAH. Adrenal ultrasonography is a highly sensitive and specific adjunct in the diagnosis of CAH. The presence of enlarged, lobulated adrenals with stippled echogenicity is invariably associated with CAH.

17-alpha-Hydroxyprogesterone↗
PubMed1999

A trial of growth hormone therapy in well-controlled hypophosphataemic rickets.

OBJECTIVE: Conventional therapy of hypophosphataemic rickets (HR) with oral phosphate and calcitriol does not always result in normal linear growth. Recombinant human growth hormone (rhGH) offers theoretical advantages as an adjunctive therapy. We aimed to determine the effects of adjunctive rhGH therapy in children with well-controlled HR. PATIENTS: In this report, 5 prepubertal children (aged 3.5-10.9 years) with well-controlled HR on conventional therapy were given adjunctive standard dose rhGH therapy for one year. DESIGN AND MEASUREMENTS: Height, growth velocity, metabolic markers of calcium and phosphate metabolism, body composition, bone mineral density, wrist and knee X-rays, and renal sonography were assessed at regular intervals. Height and growth velocities were also calculated 12 months after ceasing rhGH therapy. RESULTS: After 12 months therapy with rhGH, no significant biochemical or radiological benefits were observed. A significant increase in height SD score was observed (P = 0.023), but this was not associated with any increase in the growth velocity SD score and appears to have been due to catch-up growth caused by conventional therapy alone. When rhGH therapy was ceased, no significant decreases in mean height SD or growth velocity SD scores were observed. CONCLUSIONS: In well-controlled hypophosphataemic rickets patients receiving conventional therapy, adjunctive therapy with standard dose rhGH offers no benefits in linear growth or rachitic disease markers.

Body Composition↗
PubMed1998

Non-operative management of traumatic pancreatic pseudocysts associated with pancreatic duct laceration in children.

Objective. To assess the successful non-operative management in traumatic pancreatic pseudocysts (TPP) associated with duct laceration in children. Surgical therapy (cystogastrostomy or distal pancreatectomy with splenic salvage) has been classically considered the treatment of choice for those pseudocysts. Materials and methods. This report presents the clinical and imaging findings in two children with TPP and pancreatic duct disruption observed either on endoscopic retrograde cholangiopancreatography or injection via catheter drainage. Results. Both children responded to long-term cyst drainage. Conclusion. Although the experience is limited, the authors suggest that pancreatic injury associated with duct laceration can respond to non-operative management.

Adolescent↗
PubMed1998

Periventricular band of increased echogenicity: edema or calcification?

This paper illustrates the cases of two infants with an identical appearance of a periventricular band (PVB) of increased echogenicity on cranial sonography, shown on CT to be due to edema in one and calcification in the other. Such a PVB due to edema has not been reported previously, and its similarity to calcification on sonography is emphasized.

Brain Edema↗
PubMed1998

Colo-colic intussusception associated with pneumatosis cystoides intestinalis.

This paper describes pneumatosis cystoides intestinalis in association with colo-colic intussusception in a young teenager. The intussusception was easily reduced at barium enema. The recognition of the characteristic filling defects in the barium column facilitates a correct diagnosis. This association has only been reported previously in six adults.

Adolescent↗
PubMed1998

Asymmetric medullary nephrocalcinosis in two children.

Medullary nephrocalcinosis (MNC) is usually a bilateral process with symmetric involvement of both kidneys. Asymmetric medullary nephrocalcinosis has been previously reported in the literature, but has not been well illustrated or explained. We report the sonographic findings in two pediatric patients with hypercalcemia. In both patients an unrelated unilateral renal abnormality, (renal vein thrombosis in one and obstructive hydronephrosis in the other) prevented the development of MNC in the affected kidney, probably by decreasing the glomerular filtration rate and/or altering the renal tubular function.

Child↗
PubMed1998

Patterns of recurrence of intussusception in children: a 17-year review.

PURPOSE: Patterns of recurrence of intussusception (INT) were reviewed to determine whether changes in management have affected the rate and patterns of recurrence as well as long-term outcome in children with multiple (i. e., 2 or more) recurrences. MATERIALS AND METHODS: Review was done of 763 children with 876 intussusceptions, including (1) recurrence rate, (2) patterns of recurrence (number of and interval between recurrences), (3) reducibility, (4) pathologic lead points (PLP), (5) operative findings and (6) long-term follow-up in those with multiple recurrences. RESULTS: Above features (1)-(6) were the same in those managed with barium enema (1979-1985) and those managed with air enema (1985-1996). Overall recurrence rate was 9 %; 11 % with barium enema and 8 % with air enema. Sixty-nine patients had 113 recurrences: 47/69 (68 %) and 1 recurrence and 22/69 (32 %) had multiple recurrences. Multiple recurrences presented as isolated episodes or in clusters up to 8 years. Reducibility was 100 % for initial INT and 95 % for recurrent episodes; there were no perforations. Surgery, in 4 with irreducible recurrence, revealed no PLP. PLP were present in 5 (8 %): 2 (4 %) with 1 recurrence and 3 (14 %) with multiple recurrences. No pattern of recurrence was predictive for PLP. Long-term follow-up (up to 15 years) available in 11 with multiple recurrences revealed a favourable outcome. CONCLUSIONS: Rates and patterns of recurrence did not change with altered management. Because of the high reduction rate of recurrences, lack of perforation and favourable long-term follow-up, we recommend radiological reduction for recurrent INT. Multiple recurrences are not a contraindication. A careful search for PLP is mandatory. Surgery should be reserved for irreducible recurrences or for demonstrated PLP.

Adolescent↗
PubMed1998

The value of sonography, CT and air enema for detection of complicated Meckel diverticulum in children with nonspecific clinical presentation.

BACKGROUND: Complicated Meckel diverticulum (MD) in children does not always present with painless rectal bleeding and its presentation can then produce a difficult diagnostic dilemma. In this clinical setting, sonography (US), CT or even air enema may be the first modality chosen to evaluate these children rather than the radionuclide Meckel scan (RNMS). PURPOSE: To assess the value of US, CT and air enema for detection of complicated MD. MATERIALS AND METHODS: Review of clinical, imaging, surgical and pathological findings in 64 children (55 males, 9 females) aged 4 days -14 years (mean = 3.7 years) with MD seen during an 8-year period, 1990-1997. RESULTS: (a) In 33 patients with rectal bleeding, MD was detected on RNMS in 32. Ten of these 32 had other imaging studies, all of which were negative. (b) The other 31 patients, with varied clinical presentations, did not undergo RNMS. In these 31 and the 1 with a negative RNMS, 14 (44 %) had imaging features highly suggestive for the diagnosis of MD on US in all 14, on CT in 1, and on air enema in 3. The radiological spectrum of the inflamed, hemorrhagic MD is illustrated. CONCLUSION: The inflamed, hemorrhagic and the inverted, intussuscepted MD have a spectrum of features recognizable on US, CT and air enema. Some of these appearances are specific, others are not. Knowledge of and recognition of these features will facilitate detection of complicated MD in larger numbers of children presenting with symptoms other than the classic history of painless rectal bleeding and also in those with normal RNMS.

Adolescent↗
PubMed1998

Neonatal adrenal congestion: a sonographic-pathologic correlation.

We reviewed the clinical, sonographic and pathologic findings in eight neonates in whom diffuse enlargement and abnormal echogenicity of the adrenal glands was documented sonographically. Four of the patients suffered from perinatal asphyxia and two others required mechanical ventilation for other reasons. Six patients died and one suffers from severe development delay, cerebral palsy and failure to thrive. Sonographically, the glands were enlarged, their surface was smooth and there was loss of the central echogenic stripe. Diffuse sinusoidal congestion was found histologically in all five in whom autopsies were performed. These sonographic findings represent part of the spectrum of adrenal changes in neonatal asphyxia and other causes of perinatal stress, and may be associated with poor outcome because of other sequelae of asphyxia.

Adrenal Glands↗
PubMed1998

Congenital thymic cysts in children--mostly misdiagnosed.

BACKGROUND/PURPOSE: Thymic cysts are rare lesions of the neck and mediastinum that are difficult to diagnose. Often considered inconsequential, these lesions can frequently be symptomatic. In this report the authors contrast their experience with that of the literature. METHODS: From 1984 through 1997, the authors encountered 14 patients with this lesion. All cysts were completely excised. Patients that had an acquired cyst of the thymus were excluded from this series. RESULTS: Of the 14 patients ranging in age from 2 weeks to 16 years, seven patients had cervical masses, five had mediastinal masses, and two children had both sites involved. Seven children were symptomatic with wheezing and upper respiratory infection, with cough and fever being the most common clinical features. Investigations included chest radiograph, contrast esophagram, sonography (US) and computerized tomography (CT). Displacement of vital mediastinal or neck structures was observed in eight patients. Only two patients received correct diagnosis before surgery. Successful and complete excision of all cysts was achieved. The cysts were benign and ranged in size from 2 to 22 cm in diameter. CONCLUSION: Often forgotten, thymic cysts are rare benign lesions that should be considered in the differential diagnosis of cervical and mediastinal masses in children.

Adolescent↗
PubMed1997

Sonographic appearances of inverted Meckel diverticulum with intussusception.

This study illustrates the sonographic findings of inverted Meckel diverticulum acting as a lead point of an intussusception in five patients. In four patients, the inverted diverticulum was seen as a segment of blind-ending, thick-walled bowel projecting for a variable distance from the apex of the intussusceptum. The larger diverticula had a characteristic bulbous shape. The central serosal surface of the inverted diverticulum was filled with fluid in one patient, with fluid and fat in another, and with echogenic fat only in the other two. The presence of fat was confirmed by CT in one patient. The features illustrated in these four patients appear to be specific. In the fifth patient, the sonogram revealed a nonspecific echogenic mass at the apex of the intussusceptum. Recognition of these features on sonography may obviate the need for further investigation.

Child↗
PubMed1997

Abdominal calcification in cystic fibrosis with meconium ileus: radiologic-pathologic correlation.

BACKGROUND: There is confusion in the radiological literature as to the site of abdominal calcification in cystic fibrosis (CF) with meconium ileus (MI) in neonates. PURPOSE: To correlate the site of radiographic abdominal calcification with histologic and operative findings. MATERIALS AND METHODS: A review of clinical, radiographic, surgical and histologic data in 58 neonates with CF and MI. RESULTS: Abdominal calcification was identified in 15 (26 %) neonates: on an abdominal radiograph in 8 (13 %), at laparotomy in 3 and histologically in 10 (37 %) of the 27 resected specimens. The radiographic pattern of calcification varied from small specks in three cases to small, better-defined areas in two. In the other three patients, the calcification was more extensive and curvilinear. Histologically, calcification was found to be intramural in ten resected specimens, of which two also had intraluminal and one serosal calcification. The more extensive, curvilinear calcification identified radiographically correlated with histologically proven dystrophic intramural calcification. The less marked flecks or discrete areas of radiographic calcification may represent intramural, serosal or intraluminal calcification. CONCLUSION: Intramural calcification is common microscopically in CF with MI. Extensive radiographic calcification in these patients is more likely to represent intramural rather than serosal or intraluminal calcification.

Calcinosis↗
PubMed1997

An unusual position of the gallbladder following nephrectomy for large neoplasms.

The cases of two children are presented in whom the gallbladder was found to lie in an unusual position in the ipsilateral renal fossa following nephrectomy for large neoplasms. This postoperative visceral position must be recognized, as the appearance on cross-sectional imaging can mimic postoperative fluid collections at the resection site or tumor recurrence.

Adolescent↗