Innumerable carcinoid tumourlets may develop within pulmonary lobes should there be scarring from intralo bar sequestration; these tumourlets may, in turn, be the cause of chronic lung disease. This report documents the incidental detection of multifocal carcinoid tumourlets in the lung of a 65-year-old man who had repeated episodes of lung infection, progressive dysp nea and haemoptysis; he lived at high altitude. The left lower lobe of the lung was resected surgically, during which procedure an aberrant systemic arterial supply was noticed. The patient had diffuse bronchiectasis and intralobar sequestration. The latter implies the development of abnormal lung tissue located within lobar tissue - but which does not communicate with the bronchial tree; it is supplied with arterial blood from a branch of the aorta - arising either above or below the diaphragm. There was loss of demarcation between the sequestered lung and the surrounding lower lobe lung parenchyma. The proliferation of pul monary neuroendo...

Lessons to be learned: a case study approach Carcinoid tumourlets associated with diffuse bronchiectasis and intralobar sequestration:

Objective: In view of the large number of pregnancies complicated by neural tube defect (NTD) in Khuzestan, south west Iran, this study assesses the prevalence of NTD and its ecological and social factors. Methods: This is a retrospective study, based on medical documents using an analytic assessment of NTDs in pregnant women attending the Jundi Shapur University hospitals in Ahvaz from 21 March 2002 to 20 March 2004. Results: The total number of pregnant women was 13,262 and 56 pregnancies were complicated with NTD. A large number of the infants with NTDs were female (70%) and the most common anomaly was anencephaly. The prevalence was 4.2 in 1,000 births. The percentage in primigravida was 42.6% and in multigravida 57.4%. The main risk factor for NTD was age of the mother, those between 21 and 30 being the most affected. Consanguinity is the second risk factor with 31% of couples who were close relatives. Other socio-demographic factors were also significant. Conclusion: This retrospective study confirms clinical observation that pregnancies complicated by NTD in Khuzestan are common. There are various ecological and social factors that correlate with the prevalence of NTD.

/pdf/prevalence-and-correlates-of-neural-tube-defect-in-south-77b54ebsfc.pdf

Prevalence and Correlates of Neural Tube Defect in South West Iran: Retrospective analysis

Background

Biliary atresia (BA) is a paediatric cholestatic disease characterized by a progressive fibro-inflammation of the biliary tree. Current treatment of choice is to establish good bile flow via the Kasai operation.



Aims

We aimed to identify outcome-predictive serum biomarkers in BA infant.



Methods

Thirty-three BA children recruited from 1986 to 2007 served as the baseline-study group. An additional 11 children recruited from 2008 to 2011 served as the validation group. Serum samples were collected immediately before and 6 months after the Kasai operation for the assessment of serum cytokines, including tumour necrosis factor-α (TNF-α), transforming growth factor-β (TGF-β), interferon-γ (IFN-γ), interleukin-2 (IL-2), IL-10, IL-12p40 and IL-12p70 as the candidate biomarkers.



Results

Increased serum TGF-β levels indicated a lower Knodell hepatitis activity index at Kasai operation. The serum TGF-β levels declined after the operation. Serum IL-12p40 levels before the Kasai operation were higher in the subjects with a 3-month jaundice-free status than in others (P = 0.001). A serum pre-operative IL-12p40 level of 33 pg/ml was predictive of a 3-month jaundice-free status after surgery (positive predictive value=81.0%; negative predictive value=83.3%). This biomarker was also predictive of a better outcome, in terms of 3-year survival with native liver (risk ratio [RR = 4.00]; P < 0.001), and 3-year jaundice-free survival with native liver (RR = 12.00; P < 0.001). We confirmed the predictive power of a high pre-operative IL-12p40 level on 3-month jaundice-free status in the validation group.



Conclusions

The pre-operative IL-12p40 level was a good predictive biomarker of clinical outcome in children with BA undergoing the Kasai operation.

A high serum interleukin‐12p40 level prior to Kasai surgery predict a favourable outcome in children with biliary atresia

Brain tumours are the second most c o m m o n form of cancer in children. The majority of children treated for brain tumour survive, but they can experience a number of neurological and neuropsychological sequelae. A range of tumour, treatment, and psychosocial factors cumulatively and interactively determine long-term sequelae in these children. There is a paucity of research on neuropsychological outcomes, and the determinants of these outcomes, in children treated for brain tumour with surgery-only. This is because they represent a minority of children treated for brain tumour. Research has focused on children treated with radiotherapy and chemotherapy where the effects can be devastating. The present study examined neuropsychological outcome in children treated with surgery-only to identify risk factors for poor outcome, to determine sequelae according to tumour location, and to explore the process of recovery over a one-year period.

Neuropsychological outcomes in children treated with surgery for brain tumour

This research builds on our hypothesis that white matter damage and associated neurocognitive symptoms, in children treated for cancer with cranial spinal irradiation, spans a continuum of severity that can be reliably probed using non-invasive MR technology. Quantitative volumetric assessments of MR imaging and psychological assessments were obtained in 40 long-term survivors of malignant brain tumors treated with cranial irradiation. Neurocognitive assessments included a test of intellect (Wechsler Intelligence Test for Children, Wechsler Adult Intelligence Scale), attention (Conner's Continuous Performance Test), and memory (California Verbal Learning Test). One-sample t-tests were conducted to evaluate test performance of survivors against age-adjusted scores from the test norms; these analyses revealed significant impairments in all apriori selected measures of intelligence, attention, and memory. Partial correlation analyses were performed to assess the relationships between brain tissues volumes (normal appearing white matter (NAWM), gray matter, and CSF) and neurocognitive function. Global intelligence (r = 0.32, p = 0.05) and global attentional (r = 0.49, p < 0.01) were significantly positively correlated with NAWM volumes, whereas global memory was significantly positively correlated with overall brain parenchyma (r = 0.38, p = 0.04). We conclude that quantitative assessment of MR examinations in survivors of childhood cancer treated with cranial irradiation reveal that loss of NAWM is associated with decreased intellectual and attentional deficits, whereas overall parenchyma loss, as reflected by increased CSF and decreased white matter, is associated with memory-related deficits.

Correlation of neurocognitive function and brain parenchyma volumes in children surviving cancer

Brain tumors in children and adolescents up to 18 years of age

Congenital biliary tract atresia was found in 0.58% of children dying in the first year of life. Most frequently atresia included the extrahepatic bile ducts, among them the common bile duct. Five cases (35.7%) had atresia associated with other developmental anomalies, which may suggest a genetic determination of the atresia. The most frequent cause of death of the newborns and infants with congenital biliary tract atresia was bronchopneumonia with associated biliary cirrhosis of the liver.

Biliary atresia in children

On the basis of an analysis of 2398 autopsies of infants aged up to 1 year in 194 cases congenital anomalies of the central nervous system were found (8.1%). Most cases of these anomalies were noted in the group of newborns (85%) and the most frequent anomalies were: myelomeningocele (35.6%), multiple anomalies (20.1%), congenital hydrocephalus (17%), anencephaly (14.4%) and corpus callosum malformations (3.6%). Myelomeningocele, congenital hydrocephalus, anencephaly and true microcephaly were more frequent in girls, while multiple anomalies and corpus callosum malformations were more frequent in boys.

Congenital anomalies of the central nervous system in autopsy specimens

[Developmental anomalies of the respiratory system in infants under 1 year of age in autopsy specimens from the Institute of Pathological Anatomy, Medical Academy, in Białystok 1976-1985].

Malignant neoplasms in children and adolescents up to 18 years of age

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