Sudan black poisoning resulted in methemoglobinemia in a baby with congenital chyloperitoneum.

Treatment of congenital chyloperitoneum is a challenge. Conservative methods may be ineffective. Preoperative visualization of the site of lymphatic leakage is crucial, but radiological imaging is technically complicated and may not provide sufficient information, especially in small patients. To ease the detection of lymphatic leakage during surgery, preoperative feeding with fat-rich formula with Sudan Black has been recommended. However, administration of Sudan Black may result in life-threatening methemoglobinemia and liver damage without any advantage of revealing leakage during surgery. We recommend preoperative feeding with pure fat-rich formula.

Maternal Pyelonephritis as a Potential Cause of Perinatal Periventricular Venous Infarction in Term-Born Children.

Introduction: The study was designed to assess the prevalence of pregnancy and delivery associated risk factors in children suffering from neonatal or presumed periventricular venous infarction. Methods: Antenatal records and pregnancy outcome data were retrospectively assessed in children with presumed periventricular venous infarction (n = 43, born ≥36 gestational weeks) or neonatal periventricular venous infarction (n = 86, born <36 gestational weeks) and compared to a matched control group (n = 2168, ≥36 gestational weeks) from a prospective study. Results: Children with presumed periventricular venous infarction had significantly more maternal bacterial infections compared to the control group (47% vs 20%, respectively, P < .001), whereas no difference was found compared to the neonatal periventricular venous infarction group (49%, P = .80). Mothers with bacterial infection in the presumed periventricular venous infarction group had significantly more often pyelonephritis compared to the control group (50% vs 3.4%, respectively, P < .001). Conclusions: Our data show an increased risk for developing periventricular venous infarction in the case of maternal bacterial infections, especially between gestational weeks 21 and 31.

Evaluation of correlation of articular cartilage staining for DDR2 and proteoglycans with histological tissue damage and the results of radiographic assessment in patients with early stages of knee osteoarthritis.

OBJECTIVE: To determine, if staining of articular cartilage for proteoglycans (natural element of healthy and functioning cartilage) and discoidin domain receptor 2 (DDR2) (a protein associated with articular cartilage degradation) is correlated with histological tissue damage or radiographic assessment score in patients with early stages of knee osteoarthritis (OA). METHOD: 40 patients, with early stage OA were enrolled, from whom the biopsies for histological and immunohistochemical studies were obtained from edge of the femoral condyle during the arthroscopy. Semi-quantitative computer based analysis was used to evaluate the proportion of staining in histological sections. RESULTS: No correlation was shown between the proportion of tissue stained for DDR2 and histological score or the results of radiographic assessment of tibiofemoral (TF) joint. There was a negative correlation between the proportion of tissue stained for DDR2 and radiographic grade of patellofemoral (PF) OA (Spearman r=-0.34; 95% CI -0.60 to -0.02; P=0.03). No correlation was shown between the proportion of tissue stained for proteoglycans and histological score or the results of radiographic assessment of TF and PF joints. A negative correlation was found between proportion of tissue stained for DDR2 and proteoglycans. Spearman r=-0.43; 95% CI=-0.66 to -0.12; P=0.006. CONCLUSION: Production of DDR2 in articular cartilage could be related to early stages of OA, as it is significantly correlated to decrease of staining for cartilage proteoglycans. The role of production of DDR2 in cartilage may be decreased in stages, where higher grades of OA are detected on the radiographs.

The value of cartilage biomarkers in progressive knee osteoarthritis: cross-sectional and 6-year follow-up study in middle-aged subjects.

To determine the possible diagnostic and prognostic value of cartilage biomarkers in early-stage progressive and nonprogressive knee osteoarthritis (OA) in a population-based cohort of middle-aged subjects with chronic knee pain. Design tibiofemoral (TF) and patellofemoral (PF) radiographs were graded in 128 subjects (mean age at baseline, 45 ± 6.2 years) in 2002, 2005, and 2008. Cartilage degradation was assessed by urinary C-telopeptide fragments of type II collagen (uCTx-II), synthesis by serum type II A procollagen N-terminal propeptide (sPIIANP), and articular tissue turnover in general by cartilage oligomeric matrix protein (sCOMP). Several diagnostic associations were found between all studied biomarkers and progressive osteophytosis. COMP and CTx-II had a predictive value for subsequent progressive osteophytosis in multiple knee compartments and in case of CTx-II-also for progressive JSN. Over the first 3 years (2002-2005), significant associations were observed between COMP and progressive osteophytosis, whereas 3 years later (2005-2008) between CTx-II and progressive JSN. Thus, the associations between cartilage markers (COMP, CTx-II) and progression of radiographic OA features--osteophytes and JSN--were different between 2002-2005 and 2005-2008. Logistic regression revealed that for every unit increase in COMP level, there was 33 % higher risk for TF osteophyte progression. During early-stage OA, the presence and progression of osteophytosis is accompanied by increased level of cartilage biomarkers. This is the first study to demonstrate biochemical differences over the course of knee OA, illustrating a phasic nonpersistent character of OA with periods of progression and stabilization.

The prevalence and progression of radiographic knee osteoarthritis over 6 years in a population-based cohort of middle-aged subjects.

Details of the development of early knee osteoarthritis (OA) are largely unknown. The prevalence and progression of radiographic knee OA over 6 years in middle-aged subjects with chronic knee pain is investigated. In a prospective population-based study, tibiofemoral (TF) and patellofemoral (PF) radiographs were graded in 128 subjects (mean age 45 ± 6.2 years) for the presence of osteophytes and joint space narrowing (JSN). Radiographic progression was defined as: (i) the presence of osteophytes and/or JSN in subjects with no previous OA or (ii) an increase in the grade and/or number of already existing osteophytes and/or JSN. Altogether 56% (72/128) of subjects had knee OA, the majority of them was diagnosed with OA grade 1. In 57% of cases, radiographic OA was based on the presence of osteophytes alone versus 13% on JSN. More than 1/3 of subjects had isolated PF joint involvement. Knee OA progression rate over 6 years was 56% (71/128). During 6 years, a non-linear course of radiographic OA progression with intermittent periods of progression and stabilization was observed. Individual course of OA revealed distinct subsets of radiographic progression. Osteophytosis is an important early radiographic sign of OA and its progression. Isolated PF joint involvement is a frequent expression of knee OA. In middle-aged subjects, the progression rate of knee OA over 6 years was 56%. A non-linear course of radiographic OA progression was observed. Several radiographic subsets refer to the heterogeneity of the OA process.

Predictive value of clinical and radiological findings in inflicted traumatic brain injury.

AIMS: The aim of this study is to evaluate the value of early radiological investigations in predicting the long-term neurodevelopmental outcome of infants with inflicted traumatic brain injury (ITBI). METHODS: Clinical and radiological investigations of 24 infants with ITBI were performed during the acute phase of injury (1-3 days), and during the early (4 days up to 3 months) and late (>9 months) postinjury phases. The clinical outcome in survivors (n = 22) was based on the Rankin Disability Scale and the Glasgow Outcome Score. RESULTS: Five out of 24 infants (21%) had a poor neurodevelopmental outcome (death and severe disability), 17 infants (71%) had different developmental problems and 2 infants were normal at the mean age of 62 (54-70) (95% CI) months. A low initial Glasgow Coma Scale score of 8 or below [p < 0.05, OR 13.0 (1.3-133.3)], the development of brain oedema [p < 0.005, OR 13.0 (1.6-773)], focal changes in the basal ganglia during the acute phase [p < 0.01, OR 45 (2.1-937.3)], the development of new intracerebral focal changes early postinjury [p < 0.05, OR 24.1(1.0-559.1)], a decrease in white matter [p < 0.01, OR 33 (1.37-793.4)] and the development of severe atrophy before 3 months postinjury [p < 0.05, OR 24 (11.0-559.1)] were significantly correlated with a poor neurodevelopmental outcome. CONCLUSIONS: Early clinical and radiological findings in ITBI are of prognostic value for neurodevelopmental outcome.

Vehicle-associated closed trauma-induced stroke in a 27-day-old girl.

Birth trauma, but not postnatal trauma, has been recognized as a cause of cerebral infarction in newborns. We report a case of cerebral infarction in a 27-day-old girl after a car accident. During the car accident, the child was properly restrained to the child's safety seat. The patient was admitted to the hospital for observation because of pronounced irritability. There were no focal neurological symptoms on admission. Twenty-eight hours after the accident, the child developed focal tonic-clonic seizures and mild right-sided hemiparesis. The seizures were successfully treated with phenobarbital at a dose of 30 mg per day. Computed tomography and magnetic resonance imagining performed on the second and third days after the accident, respectively, showed subdural hemorrhage in the occipital regions and cerebral ischemia in the left parieto-occipital region. Control imaging 10 days later showed signs of reperfusion. Persistent child irritability after head trauma is one of the indicating factors for performing an emergency computed tomography scan of the head.

Association between ultrasonographic findings and bone/cartilage biomarkers in patients with early-stage knee osteoarthritis.

Little is known regarding the association between ultrasonographic (US) findings and biomarkers of bone and cartilage in individuals with knee osteoarthritis (OA). We investigated (1) US findings in early-stage knee OA and (2) the association between US findings and bone/cartilage biomarkers. A population cohort aged 35-55 years (n = 106) with early-stage knee OA was investigated. US examination was performed according to European League against Rheumatism (EULAR) guidelines using a 7.5-MHz probe. Biomarkers of bone resorption (CTx-I) and formation (PINP), cartilage resorption (U-CTx-II) and synthesis (S-PIIANP), and general bone and cartilage biomarkers (OC, COMP) were assessed. The most prevalent US findings were tendon calcification, synovial thickening, and suprapatellar effusion. In women, the presence of tendon calcification and Baker's cysts could predict 36% of the variability in U-CTx-II levels. The presence of osteophytes and tendon calcification predicted up to 38% of the variability of PIIANP concentration. Defects in subchondral bone, meniscal changes, and effusion predicted up to 29% of the variability in COMP levels. Tendon calcification was related to cartilage synthesis (based on PIIANP levels) in men and to cartilage degradation (based on U-CTx-II concentrations) in women. US signs of synovitis were reflected metabolically by markers of joint tissue metabolism. Tendon calcification, synovial thickening, and effusion were common US findings in early-stage knee OA. US-detectable findings were substantially responsible for the variability in bone and cartilage biomarkers, associations reflective of the active metabolism of soft tissues in early-stage OA.

Changes in cerebral and visceral blood flow velocities in asphyxiated term neonates with hypoxic-ischemic encephalopathy.

OBJECTIVE: The purpose of this study was to evaluate changes in the Doppler blood flow velocity (BFV) in the cerebral and visceral arteries in asphyxiated term neonates. METHODS: The BFV was measured in 47 asphyxiated and 37 healthy term neonates in the anterior cerebral artery, middle cerebral artery, basilar artery, internal carotid artery, celiac artery (CA), superior mesenteric artery (SMA), and renal artery (RA) up to the age of 60 to 149 days. RESULTS: At the age of 12 to 120 hours after asphyxia, the mean BFV had increased, and the resistive index (RI) had decreased (P < .05) in all cerebral arteries in neonates with severe hypoxic-ischemic encephalopathy (HIE) compared with the control group. In neonates with severe HIE, the mean BFV in the RA had significantly decreased at the age of 3 to 240 hours, and the RI had increased at the age of 24 to 240 hours, normalizing by the age of 21 to 59 days compared with the control group (P < .05). In the SMA, a decreased mean BFV was found in neonates with severe HIE compared with those with mild to moderate HIE only at the age of 24 to 36 hours. In neonates with mild to moderate HIE, the mean BFV had increased in the SMA and CA compared with the control group at the age of 2 to 11.9 hours. CONCLUSIONS: A severe alteration of the cerebral and visceral BFV takes place during the first days after asphyxia in neonates with different severities of HIE.

Arterial stiffness, carotid artery intima-media thickness and plasma myeloperoxidase level in children with type 1 diabetes.

OBJECTIVE: The present study investigated the functional-structural changes of the arteries along with a new biochemical marker of atherosclerosis, plasma myeloperoxidase level, in children with type 1 diabetes (T1DM). METHODS: We studied 30 children with T1DM, aged 4.7-18.6 years (mean T1DM duration 5.4+/-3.4 years, mean HbA(1c) 9.8%) and 30 healthy subjects, matched by sex, age and body mass index. Fasting blood samples were obtained for myeloperoxidase (MPO). Ultrasonography and pulse wave analysis were used to measure carotid intima-media thickness (IMT), augmentation index corrected to a heart rate of 75 (AIx@75) and pulse wave velocity (PWV). RESULTS: Children with diabetes had significantly higher plasma MPO levels (p=0.006), increased AIx@75 (p=0.02), IMT (p=0.005) and IMT standard deviation scores (IMT SDS) (p=0.02), compared to the control group. IMT SDS correlated positively with HbA(1c) (r=0.39, p=0.05). PWV, adjusted for age and mean arterial blood pressure, correlated with diabetes duration (r=0.49, p=0.02). CONCLUSIONS: Children with T1DM have substantially elevated plasma levels of myeloperoxidase as well as atherosclerosis-related structural and functional changes of the arterial wall.

Low cerebral blood flow velocity and head circumference in infants with severe hypoxic ischemic encephalopathy and poor outcome.

AIMS: To evaluate long-term changes in cerebral blood flow velocity (CBFV) and head circumference in asphyxiated infants. METHODS: CBFV was measured in 83 asphyxiated and 115 healthy term infants in anterior and middle cerebral, basilar and internal carotid artery (ICA) up to the age of 60-149 days. The psychomotor development and head circumference was followed for 18 months. RESULTS. Mean CBFV was increased (p < 0.05) during the first days after asphyxia in infants with severe hypoxic-ischemic encephalopathy (HIE) (n = 25) compared to control group or infants with mild to moderate HIE (n = 58) with maximum values found at the age of 36-71.9 h: in ICA (mean [95% CI]) 31.2 (25.5-36.6) cm/s in severe HIE infants compared to 13.0 (12.2-13.9) cm/s in controls. Decreased (p < 0.0001) mean CBFV developed in severe HIE infants by the age of 21-59 days: in ICA 14.1 (11.5-16.8) cm/s compared to 22.9 (21.4-24.4) cm/s in controls. Infants with severe HIE had similar mean height but lower head circumferences compared to controls (p < 0.05) at the age of 21-59 days. CONCLUSION: The high mean CBFV found in infants with severe HIE during the first days after asphyxia is temporary and low CBFV and head circumference develops by the age of 21-59 days.

Developmental changes in cerebral and visceral blood flow velocity in healthy neonates and infants.

OBJECTIVE: The purpose of this study was to evaluate the changes in Doppler blood flow velocity (BFV) in cerebral and visceral arteries during infancy. METHODS: The BFV was measured in 37 healthy term neonates in the anterior cerebral artery (ACA), middle cerebral artery (MCA), basilar artery, internal carotid artery (ICA), celiac artery (CA), superior mesenteric artery (SMA), and renal artery (RA). RESULTS: The mean BFV increased and the resistive index decreased (P < .05) in all cerebral arteries, SMA, and CA by the age of 12 to 23.9 hours and in the RA by the age of 24 to 35.9 hours compared with 2 to 11.9 hours. A further significant increase (P < .05) of the mean BFV occurred in all arteries except the ICA and CA by the age of 72 to 120 hours compared with 12 to 23.9 hours. By the age of 21 to 59 days, the mean BVF doubled in all investigated arteries compared with 2 to 11.9 hours, with a further significant increase (P < .05) by the age of 150 to 240 days in cerebral and renal arteries. There was no correlation between the mean blood pressure (BP) and mean BFV in the ACA and MCA. However, there was a positive correlation (r > or = 0.5; P < .05) between the BP and BFV in the RA and SMA at the age of 12 to 23.9 hours. CONCLUSIONS: A significant increase in the cerebral and visceral BFV occurs normally throughout infancy, with the visceral BFV affected by BP changes during the first day of life.

Acutely and retrospectively diagnosed perinatal stroke: a population-based study.

BACKGROUND AND PURPOSE: There are not very many epidemiological studies on perinatal stroke, and many authors suggest that this may be an underdiagnosed condition. The aim of the study was to estimate the incidence of perinatal arterial ischemic and hemorrhagic stroke in Estonia, to study the first clinical signs and to identify possible differences in predisposing factors and outcome between acutely and retrospectively diagnosed cases of perinatal stroke. METHODS: A retro- and prospective study of acutely (within the first month) and retrospectively diagnosed ischemic and hemorrhagic cases of perinatal stroke was conducted in a children population born in the eastern and southern regions of Estonia during the years 1994 to 2003. Patients were identified from a pilot study, hospital records, and an inquiry of child neurologists and general practitioners. The diagnosis was confirmed in 38 (12 were diagnosed acutely and 26 retrospectively) cases by neuroradiology (MRI or CT). RESULTS: The incidence rate of perinatal stroke in Estonia is 63 per 100,000 live births. Main clinical findings in the neonatal period were seizures, abnormalities of muscular tone, and disturbed level of alertness. Previously identified risk factors occurred in 32% of cases. Children with early diagnosis had more often adverse events during pregnancy and delivery (P<0.05) and developed more severe stage of hemiparesis compared with children with late diagnosis (P<0.05). CONCLUSIONS: The incidence rate of 63 per 100,000 live birth is higher than previously reported. Detailed analysis of the first signs of perinatal stroke may improve the early diagnostics of perinatal stroke.

Inflicted traumatic brain injury (ITBI) or shaken baby syndrome (SBS) in Estonia.

BACKGROUND: Inflicted traumatic brain injury (ITBI) or shaken baby syndrome (SBS) is recognized as a major cause of disability and death in the paediatric population. AIM: To find out the incidence of ITBI in Estonia. METHODS: 26 cases of ITBI were recognized: four children died, 22 survived. RESULTS: Of 26 children, 20 (77%) were boys and six (23%) were girls. Median age at admission to hospital was 3.9 mo, and the boys were younger than the girls. CONCLUSION: The overall incidence of ITBI was 28.7 per 100,000 infants. In the prospective group the incidence was 40.5 per 100,000, and in retrospective group 13.5 per 100,000. ITBI is not rare but not always a recognized form of child abuse. Healthcare professionals should be more aware of this condition.

Bone tumorigenesis induced by alpha-particle radiation: mapping of genetic loci influencing predisposition in mice.

The present study was carried out to determine the extent to which genetic factors modify the incidence of radiation-induced bone tumorigenesis in mice, and to map putative susceptibility genes. We conducted a genome-wide linkage analysis in a cohort of 47 interstrain backcrossed mice. After the mice were injected with the bone-seeking alpha-particle-emitting radionuclide (227)Th, 21 of the mice developed osteosarcomas. Two loci, one on chromosome 7 close to D7Mit145 and a second on chromosome 14 (D14Mit125), exhibited suggestive linkage to osteosarcoma predisposition, with LOD scores of 1.37 and 1.05, respectively. The LOD score increased considerably when interaction between these two loci was taken into account (LOD = 3.48). Nine of 12 mice inheriting a susceptibility allele at both loci developed osteosarcomas after (227)Th injection, compared to only four osteosarcomas in 18 animals that did not inherit either of the susceptibility alleles. Variance component analysis revealed that these genetic factors determine approximately one-fifth of the total incidence of osteosarcomas. This study demonstrates the presence of a genetic component that modulates predisposition to radiation-induced osteosarcoma.