Deferoxamine-induced dysplasia-like skeletal abnormalities at radiography and MRI

Background

Current thalassemia major treatment includes blood transfusion and iron chelation, which is associated with growth disturbances and radiographic changes in the long bone metaphyses.

Objective

To explore and discuss the spectrum of deferoxamine-induced bone-dysplasia-like changes in children with thalassemia major in Egypt.

Materials and methods

We studied 59 Egyptian children with thalassemia major and generalized arthralgia. All started deferoxamine treatment at 3 years of age. We conducted skeletal survey and MRI of both knees in radiographically positive children. Each child’s age, serum ferritin, age of onset and duration of therapy were compared with the radiologic findings.

Results

Twenty-two (37.3%) children had variable degrees of skeletal dysplasia-like changes similar to those described with deferoxamine intake, mostly around the knees. Mild dysplasia-like changes were seen in 4 (18%) children; moderate changes were seen in 11 (50%) children and severe changes were seen in 7 (31.8%) children. No statistically significant relationships were detected between bone changes and the children’s age, age of starting deferoxamine, duration of therapy, or serum ferritin level.

Conclusion

A wider spectrum of deferoxamine-induced bone-dysplasia-like changes was recognized despite delayed onset and small doses of therapy. These changes should be considered as a possible cause of arthropathy in children with thalassemia major, especially symptomatic children.     

MR imaging findings suggestive of posterior reversible encephalopathy syndrome in adolescents with systemic lupus erythematosus

Background

Endothelial damage, hypertension and cytotoxic medications may serve as risk factors for the posterior reversible encephalopathy syndrome (PRES) in systemic lupus erythematosus. There have been few case reports of these findings in pediatric lupus patients.

Objective

We describe clinical and neuroimaging findings in children and adolescents with lupus and a PRES diagnosis.

Materials and methods

We identified all clinically acquired brain MRIs of lupus patients at a tertiary care pediatric hospital (2002–2008). We reviewed clinical features, conventional MRI and diffusion-weighted imaging (DWI) findings of patients with gray- and white-matter changes suggestive of vasogenic edema and PRES.

Results

Six pediatric lupus patients presenting with seizures and altered mental status had MRI findings suggestive of PRES. In five children clinical and imaging changes were seen in conjunction with hypertension and active renal disease. MRI abnormalities were diffuse and involved frontal regions in five children. DWI changes reflected increased apparent diffusivity coefficient (unrestricted diffusion in all patients). Clinical and imaging changes significantly improved with antihypertensive and fluid management.

Conclusion

MRI changes suggestive of vasogenic edema and PRES may be seen in children with active lupus and hypertension. The differential diagnosis of seizures and altered mental status should include PRES in children, as it does in adults.     

A retrospective review of pituitary MRI findings in children on growth hormone therapy

Background

Patients with congenital hypopituitarism might have the classic triad of pituitary stalk interruption syndrome, which consists of: (1) an interrupted or thin pituitary stalk, (2) an absent or ectopic posterior pituitary (EPP), and (3) anterior pituitary hypoplasia or aplasia.

Objective

To examine the relationship between pituitary anatomy and the degree of hormonal dysfunction.

Materials and methods

This study involved a retrospective review of MRI findings in all children diagnosed with congenital growth hormone deficiency from 1988 to 2010 at a tertiary-level pediatric hospital.

Results

Of the 52 MRIs reviewed in 52 children, 26 children had normal pituitary anatomy and 26 had one or more elements of the classic triad. Fourteen of fifteen children with multiple pituitary hormone deficiencies had structural anomalies on MRI. Twelve of 37 children with isolated growth hormone deficiency had an abnormal MRI.

Conclusion

Children with multiple pituitary hormone deficiencies were more likely to have the classic triad than children with isolated growth hormone deficiency. A normal MRI was the most common finding in children with isolated growth hormone deficiency.     

Prevalence of radiographic semicircular canal dehiscence in very young children: an evaluation using high-resolution computed tomography of the temporal bones

Background

Previous studies suggest that semicircular canal dehiscences (SCDs) have a developmental origin.

Objective

We hypothesized that if SCDs originate during development, incidence of radiographic SCDs in young children will be higher than in adults.

Materials and methods

Thirty-four temporal bone HRCTs of children younger than 2?years and 40 temporal bone HRCTs of patients older than 18?years were reformatted and re-evaluated for presence of SCD or canal thinning. Results were compared with indications for HRCT and clinical information.

Results

SCDs were detected in 27.3% of children younger than 2?years of age (superior, 13.8%; posterior, 20%) and in 3% of adults (P? <?0.004). Of children with one radiographic dehiscence, 55.6% had multiple and 44% had bilateral SCDs on HRCT. No lateral canal SCDs were present. Thinning of bone overlying the semicircular canals was found in 44% of children younger than 2?years and 2.5% of adults (P?<?0.0001).

Conclusion

SCDs are more common on HRCTs of very young children. This supports the hypothesis that SCDs originate from discontinuation of bone deposition/maturation. However, SCDs on imaging do not necessarily correlate with canal dehiscence syndrome and should therefore be interpreted carefully.     

Iodine deficiency status amongst school children in Pauri,Uttarakhand

Objective

To assess the iodine deficiency status amongst school age children in district Pauri, Uttarakhand.

Methods

2067 children (age of 6–12 years) were included. Clinical examination of thyroid gland of each child was conducted. On-the-spot urine and salt samples were collected from children.

Results

Total Goitre Rate was found to be 16.8% and median Urinary Iodine Concentration level was 115 μg/L. Only 40.4% of salt samples had e 15 ppm of iodine.

Conclusion

There is a mild degree of iodine deficiency in school age children in district Pauri. There is a need of strengthening the National Iodine Deficiency Disorder Control Program.     

Optimizing bone surveys performed for suspected non-accidental trauma with attention to maximizing diagnostic yield while minimizing radiation exposure: utility of pelvic and lateral radiographs

Background

Skeletal surveys for non-accidental trauma (NAT) include lateral spinal and pelvic views, which have a significant radiation dose.

Objective

To determine whether pelvic and lateral spinal radiographs should routinely be performed during initial bone surveys for suspected NAT.

Materials and methods

The radiology database was queried for the period May 2005 to May 2011 using CPT codes for skeletal surveys for suspected NAT. Studies performed for skeletal dysplasia and follow-up surveys were excluded. Initial skeletal surveys were reviewed to identify fractures present, including those identified only on lateral spinal and/or pelvic radiographs. Clinical information and MR imaging was reviewed for the single patient with vertebral compression deformities.

Results

Of the 530 children, 223 (42.1%) had rib and extremity fractures suspicious for NAT. No fractures were identified solely on pelvic radiographs. Only one child (<0.2%) had vertebral compression deformities identified on a lateral spinal radiograph. This infant had rib and extremity fractures and was clinically paraplegic. MR imaging confirmed the vertebral body fractures.

Conclusion

Since no fractures were identified solely on pelvic radiographs and on lateral spinal radiographs in children without evidence of NAT, nor in nearly all with evidence of NAT, inclusion of these views in the initial evaluation of children for suspected NAT may not be warranted.     

Rib head protrusion into the central canal in type 1 neurofibromatosis

Background

Intraspinal rib head dislocation is an important but under-recognized consequence of dystrophic scoliosis in patients with neurofibromatosis 1 (NF1).

Objective

To present clinical and imaging findings of intraspinal rib head dislocation in NF1.

Materials and methods

We retrospectively reviewed clinical presentation, imaging, operative reports and post-operative courses in four NF1 patients with intraspinal rib head dislocation and dystrophic scoliosis. We also reviewed 17 cases from the English literature.

Results

In each of our four cases of intraspinal rib head dislocation, a single rib head was dislocated on the convex apex of the curve, most often in the mid- to lower thoracic region. Cord compression occurred in half of these patients. Analysis of the literature yielded similar findings. Only three cases in the literature demonstrates the MRI appearance of this entity; most employ CT. All of our cases include both MRI and CT; we review the subtle findings on MRI.

Conclusion

Although intraspinal rib head dislocation is readily apparent on CT, sometimes MRI is the only cross-sectional imaging performed. It is essential that radiologists become familiar with this entity, as subtle findings have significant implications for surgical management.     

Alpha 1 antitrypsin deficiency in children with chronic liver disease in North India

Objective

We attempted to determine the role of alpha-1-antitrypsin (AAT) deficient variants as an etiologic factor for chronic liver disease in North Indian children.

Design

This study investigated 1700 children (682 retrospectively and 1018 prospectively) (840 CLD, 410 neonatal cholestasis and 450 without liver disease) for AAT deficiency.

Setting

Tertiary referral center, All India Institute of Medical Sciences, New Delhi.

Patients

Of 1250 liver disease patients, 98 (7.8%) were suspected to be AAT deficient on the basis of screening tests (low serum AAT levels and/or absent/faint alpha-1-globulin band on serum agarose electrophoresis and/or diastase resistant PAS positive granules on liver biopsy).

Main outcome measures

AAT deficient Z or S allele in suspected patients.

Results

Z or S allele was not observed on phenotyping (1700 subjects), or with PCR-RFLP, SSCP and sequencing done in 50 of 98 suspected AAT deficient patients. A novel mutation G-to-A at position 333 in exon V was found in two siblings having positive immunohistochemistry for AAT on liver biopsy, both of whom had significant liver disease with portal hypertension.

Conclusion

In conclusion, AAT deficiency as an etiologic factor for chronic liver disease in childhood appeared to be uncommon in North India     

Co-morbidities in hospitalized children with severe acute malnutrition

Objective

To study the co morbidities in hospitalized children with severe acute malnourishment.

Methods

104 severe acute malnourished children were included.

Results

54% had diarrhea and 27.8% had acute respiratory tract infections. Tuberculosis was diagnosed in 22% of cases (60.8% cases in children 6–12 mo old). Malaria and Measles were diagnosed in 3.8% each, and HIV infection was seen in 2.9% cases. Signs of vitamin B and vitamin A deficiency were seen in 14.4% and 5.8% cases, respectively. Malaria and HIV were not found to be major co morbid conditions.

Conclusions

Timely identification and treatment of various co-morbidities is likely to break undernutrition-disease cycle, and to decrease mortality and improve outcome.     

Persistent diarrhea: Risk factors and outcome

Objective

To identify risk factors associated with Persistent diarrhea (PD) and deaths due to PD.

Methods

This prospective case control study included 60 children with PD (cases) and 60 children (controls) with acute diarrhoea (AD). Detailed history, examination and appropriate investigations were done for all children. Crude Odds ratio was calculated for each risk factor by univariate analysis and adjusted odds ratio was calculated by multivariate logistic regression.

Results

Prior antibiotic use, steroid use, anemia, vitamin A deficiency, malnutrition, LRI, UTI, oral candidiasis, and hyponatremia, were statistically significant risk factors by univariate analysis. Prior antibiotic use, vitamin A deficiency, malnutrition and LRI were independently associated with PD by multivariate logistic regression analysis. The risk factors for mortality were stool frequency more than 10 times per day, severe malnutrition, oral candidiasis, hypoalbuminemia and HIV positivity.

Conclusions

The presence of these risk factors should alert the clinician to take appropriate measures, to decrease the mortality.     

Fractures in infants and toddlers with rickets

Background

Rickets affects young infants and toddlers. However, there is a paucity of literature regarding the types of fractures that occur in rachitic patients.

Objective

To evaluate the age of patients at which radiographically evident rickets occurs, and to characterize the age incidence and fractures that are observed in infants and toddlers with radiographically evident rickets.

Materials and methods

A retrospective study of children younger than 24 months was performed. Clinical data and radiographs were reviewed. Radiographs obtained within 1 month of the diagnosis were evaluated for the presence or absence of osteopenia, presence or absence of fraying–cupping, and presence and characterization of fractures.

Results

After exclusion criteria were applied, 45 children were included in the study. Children with rickets evident by radiograph were in the age range of 2–24 months. Fractures were present in 17.5% of the study group, exclusively in mobile infants and toddlers. Fracture types included transverse long bone fractures, anterior and anterior-lateral rib fractures, and metaphyseal fractures. All fractures occurred exclusively in patients with severe, overtly evident rickets.

Conclusion

Fractures occur in older infants and toddlers with overt rickets and can be seen by radiograph. Fractures do not resemble high-risk non-accidental trauma fractures.     

Melamine-induced urinary calculi in infants—sonographic manifestations and outcomes 1 year after exposure

Background

In 2008, the melamine-tainted-milk incident started with reports of increased incidence of urolithiasis in infants in China. Affected children were screened for urolithiasis.

Objective

The purpose of this study was to analyze sonographic characterization of infant melamine-induced urolithiasis.

Materials and methods

Transabdominal US examination was done in 603 infants with melamine-induced calculi. The imaging characteristics of calculi and hydronephrosis were analyzed. Follow-up US imaging was performed.

Results

Comet-tail sign was seen behind the calculus of <4 mm. Calculi of ≥4 mm were found in 299 inpatients with clear posterior border and with or without light shadowing. Solitary and multiple stones had similar incidence. Incidence of calculi in the inferior renal calyx was the highest (55.2%) in inpatients. Calculus size in inpatients age 2–3 years was smaller than that of children younger than 2 years old (P?<?0.05). Inpatients age 2–3 years had the highest incidence rate (48.0%) of hydronephrosis.

Conclusion

Calculi of <4 mm manifested as hyperechoic foci near the renal papillae, while calculi of ≥4 mm usually manifested as echogenic foci with visible inferior edge in the renal calyx. Hydronephrosis was a common imaging finding in inpatients ages 2–3 years.     

A prospective study of radiographic manifestations in Hutchinson-Gilford progeria syndrome

Background

Progeria is a rare segmental premature aging disease with significant skeletal abnormalities. Defining the full scope of radiologic abnormalities requires examination of a large proportion of the world’s progeria population (estimated at 1 in 4 million). There has been no comprehensive prospective study describing the skeletal abnormalities associated with progeria.

Objective

To define characteristic radiographic features of this syndrome.

Materials and methods

Thirty-nine children with classic progeria, ages 2–17?years, from 29 countries were studied at a single site. Comprehensive radiographic imaging studies were performed.

Results

Sample included 23 girls and 16 boys—the largest number of patients with progeria evaluated prospectively to date. Eight new and two little known progeria-associated radiologic findings were identified (frequencies of 3–36%). Additionally, 23 commonly reported findings were evaluated. Of these, 2 were not encountered and 21 were present and ranked according to their frequency. Nine abnormalities were associated with increasing patient age (P?=?0.02–0.0001).

Conclusion

This study considerably expands the radiographic morphological spectrum of progeria. A better understanding of the radiologic abnormalities associated with progeria and improved understanding of the biology of progerin (the molecule responsible for this disease), will improve our ability to treat the spectrum of bony abnormalities.     

Atresia of the bilateral pulmonary veins: a rare and dismal anomaly identified on cardiac CT

Background

Imaging findings of bilateral pulmonary vein atresia have not been described.

Objective

To describe cardiac CT findings and clinical outcomes of bilateral pulmonary vein atresia.

Materials and methods

Three newborns with bilateral pulmonary vein atresia were encountered at our institution during a period of 8 years. We evaluated prenatal echocardiographic findings, clinical presentations, postnatal echocardiographic findings, chest radiographic findings, cardiac CT findings and clinical outcomes.

Results

All newborns presented immediately after birth with severe cyanosis, respiratory distress and acidosis that were unresponsive to medical management. Prenatal and postnatal echocardiographic studies and chest radiography were misleading, inconclusive or nonspecific in making the diagnosis in these children; however cardiac CT clearly demonstrated atresia of the bilateral pulmonary veins with multiple small mediastinal collateral veins and pulmonary edema. Surgical treatments were not feasible for this anomaly. Their clinical outcomes were universally dismal and all infants died within 3 days.

Conclusion

Cardiac CT provides an accurate diagnosis of bilateral pulmonary vein atresia and leads to prompt treatment decision in these children.     

Three-dimensional skull models as a problem-solving tool in suspected child abuse

Background

The value of 3-D skull models in evaluation of young children with suspected child abuse is not known.

Objective

The purpose of this study was to assess the value of 3-D skull models as a problem-solving tool in children younger than 2 years.

Materials and methods

We performed a retrospective study on 73 children (ages 0–24 months) seen by a child protection team (CPT) who were undergoing head CT between August 2007 and July 2009.

Results

Of the 73 children, volume-rendered 3-D models were obtained in 26 (35.6%). Three-dimensional models changed initial CT interpretation in nine instances (34.6%). Findings thought to be fractures were confirmed as normal variants in four children. Depressed fractures were correctly shown to be ping-pong fractures in two cases. In one case, an uncertain finding was confirmed as a fracture, and an additional contralateral fracture was identified in one child. A fracture seen on skull radiographs but not seen on axial CT images was identified on the 3-D model in one case. Changes in interpretation led to modification in management in five children.

Conclusion

Use of 3-D skull models can be a problem-solving tool when there is discordance among the CT reading, subsequent radiographic investigations and clinical evaluation.     

Reverse vertical transmission of hepatitis-B from transfusion-infected children to biological mothers

Background

Perinatal and horizontal are the common modes of transmission of hepatitis-B virus in children.

Case characteristics

Two mother-child pairs with children having received multiple blood transfusions in past.

Observation

Both the mothers developed acute hepatitis-B infection whereas children were demonstrated to be having chronic infection with hepatitis-B.

Outcome

One mother cleared her hepatitis-B in fection whereas it persisted in the other. Both children required anti-viral treatment.

Message

Hepatitis-B virus may rarely get transmitted from infected children to their mothers causing acute infection.     

Restriktive Diäten

Background

An increasing number of parents offer their children an alternative diet that differs from current scientific recommendations. This may be attributed to ecological, ethical, philosophical or religious reasons. In addition, there is a heterogeneous group of children needing to comply with food restrictions due to a specific medical condition.

Objectives

This review discusses possible adverse effects of restrictive diets and gives recommendations to prevent deficiencies.

Material and methods

The prevalence and characteristics of restrictive diets were evaluated together with an analysis of possible deficiencies and current expert recommendations.

Results

The most common alternative diets are whole food, lacto-ovo vegetarian, vegan, and macrobiotic diets. The risk of developing deficiency symptoms rises proportionally to the extent of dietary restriction. A deficiency of vitamin B₁₂, vitamin D, calcium, iodine, and animal proteins are often observed. Particularly infants of vegan mothers have a high risk of severe vitamin B₁₂ deficiency leading to permanent neurological impairment. Consumption of home-made infant milk preparations may also lead to a high risk of deficiency. Restrictive diets due to medical conditions require an evidence-based diagnostic work-up. Especially in the area of food allergies or intolerance, diets are often initiated without adequate diagnostic criteria, unnecessarily leading to deficiencies and quality of life impairment.

Conclusion

Pediatricians must recognize the risks of restrictive diets and advise parents to be cautious. A detailed consultation about potential consequences and reasonable supplementation should, if possible, be carried out together with an experienced dietician.     

Effect of Glucocorticoids on Serum Lipid Profile and Endothelial Function and Arterial Wall Mechanics

Objective

To determine the effect of glucocorticoids on lipid profile, endothelial function and arterial wall mechanics in children.

Methods

Thirty patients who had received glucocorticoids for 4 to 8 wk were compared with 30 age and sex matched healthy controls. Baseline evaluation included weight, height, body mass index (BMI), blood pressure (BP), lipid profile and Ultrasonographic evaluation of brachial artery for endothelial dependant as well as endothelial independent vasodilatation and evaluation of common carotid artery for intima media thickness (IMT) and arterial wall mechanics. All of these parameters were evaluated two more times- after 4 wk of steroid therapy and 2 wk after stopping the drug.

Results

Patients were found to have significant increase in BMI; systolic and diastolic BP; total and LDL cholesterol and carotid IMT and also a decrease in cross sectional compliance (CSC) after 4 wk of steroid therapy (oral prednisolone). However, all these parameters returned towards baseline, 2 wk after stopping the drug. No endothelial dysfunction was observed in these patients.

Conclusions

Four to eight wk of glucocorticoids use in children leads to reversible changes in BMI, systolic and diastolic BP, total and LDL cholesterol, carotid IMT and CSC.     

Treatment of Iron Deficiency Anemia in Children: A Comparative Study of Ferrous Ascorbate and Colloidal Iron

Objective

To compare the efficacy of ferrous ascorbate and colloidal iron in the treatment of iron deficiency anemia in children.

Methods

Eighty one children, aged 6 mo to 12 y, were screened for iron deficiency anemia (IDA) and those diagnosed with IDA were randomized to receive ferrous ascorbate or colloidal iron for a period of 12 wk, such that each child received elemental iron 3 mg/kg body weight/d. Increase in hemoglobin (Hb) level was the primary outcome measure. Assessment was performed at baseline, wk 4, wk 8 and wk 12.

Results

Of 81 children screened, 73 were included in the study. The mean rise in Hb at the end of the 12 wk was significantly higher in ferrous ascorbate group than the colloidal iron group [3.59?±?1.67 g/dl vs. 2.43?±?1.73 g/dl; P?<?0.01]. Significantly higher proportion of children receiving ferrous ascorbate (64.86 % vs. 31.03 %; P?<?0.01) became non-anemic in comparison to colloidal iron.

Conclusions

Ferrous ascorbate provides a significantly higher rise in hemoglobin levels in comparison to colloidal iron. The study supports the use of ferrous ascorbate in the pediatric age group, providing evidence for its role as an efficient oral iron supplement in the treatment of iron deficiency anemia.