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Sudden unexpected death in infancy (SUDI) includes sudden infant death syndrome (SIDS). Co-sleeping is regarded as a major risk factor for SIDS. Under normal circumstances, nucleated red blood cells (nRBCs) are absent from the peripheral blood and their release can occur in cases with a probable hypoxic mode of death. The aim of our study was to assess the significance of the release of nRBCs in SIDS occurring during co-sleeping and the association with hemorrhages in the dura and the lungs. 35 cases were retrospectively assigned to one of the following categories: (I) 9 SUDI (of various causes) with no co-sleeping; (II) 16 SIDS while co-sleeping; (III) cause of death in hypoxic circumstances (3 hangings, 2 cardiac malformations, 1 meningitis 1 intoxication with diazepam); (IV) 3 SIDS in the cot. nRBCs were present in 5/9 cases of Category I (mean: 0.5%); 10/16 cases of Category II (mean: 1.87%); 7/7 cases of Category III (mean: 3.8%) and 0/3 cases of Category IV (mean: 0). ANOVA one-way test showed a significance of 0.003 amongst the 4 groups. The presence of diffuse intra-alveolar hemorrhage was associated with a higher release of nRBCs (mean: 3.1%) than focal hemorrhage (mean 0.6%). nRBCs were associated with focal hemorrhages in the falx and tentorium (mean: 2.3 vs. 0.9% when no hemorrhages were seen). The high mean of nRBCs seen in the co-sleeping SIDS cases suggests a higher exposure to hypoxia in the co-sleeping group which may have led to the release of nRBCs. More cases need to be analyzed to confirm this hypothesis.

Pertussis carries a high risk of mortality in very young infants. The mechanism of refractory cardio-respiratory failure is complex and not clearly delineated. We aimed to examine the clinico-pathological features and suggest how they may be related to outcome, by multi-center review of clinical records and post-mortem findings of 10 patients with fulminant pertussis (FP). All cases were less than 8 weeks of age, and required ventilation for worsening respiratory symptoms and inotropic support for severe hemodynamic compromise. All died or underwent extra corporeal membrane oxygenation (ECMO) within 1 week. All had increased leukocyte counts (from 54 to 132 x 10(9)/L) with prominent neutrophilia in 9/10. The post-mortem demonstrated necrotizing bronchitis and bronchiolitis with extensive areas of necrosis of the alveolar epithelium. Hyaline membranes were present in those cases with viral co-infection. Pulmonary blood vessels were filled with leukocytes without well-organized thrombi. Immunodepletion of the thymus, spleen, and lymph nodes was a common feature. Other organisms were isolated as follows; 2/10 cases Para influenza type 3, 2/10 Moraxella catarrhalis, 1/10 each with respiratory syncytial virus (RSV), a coliform organism, methicillin-resistant Staphylococcus aureus (MRSA), Haemophilus influenzae, Stenotrophomonas maltophilia, methicillin-sensitive Staphylococcus aureus (MSSA), and candida tropicalis. We postulate that severe hypoxemia and intractable cardiac failure may be due to the effects of pertussis toxin, necrotizing bronchiolitis, extensive damage to the alveolar epithelium, tenacious airway secretions, and possibly leukostasis with activation of the immunological cascade, all contributing to increased pulmonary vascular resistance. Cellular apoptosis appeared to underlay much of these changes. The secondary immuno-compromise may facilitate co-infection.

BACKGROUND AND AIMS Confocal laser endomicroscopy (CLE) is a recent development that enables surface and subsurface imaging of living cells in vivo at 1000 x magnification. The aims of the present study were to define confocal features of celiac disease (CD) and to evaluate the usefulness of the CLE in the diagnosis of CD in children in comparison to histology. PATIENTS AND METHODS Nine patients (8 girls) with a median age of 8.35 years (range 2-12.66 years) and a median weight of 28.3 kg (range 11-71 kg) were suspected with CD and 10 matched controls underwent oesophagogastroduodenoscopy using the confocal laser endomicroscope (EC3870CILK; Pentax, Tokyo, Japan). Histologic sections were compared with the confocal images of the same site by 2 experienced paediatric histopathologists and endoscopists, all of whom were blinded to the diagnosis. RESULTS The median procedure time was 17 minutes (range 8-25 minutes). Confocal features of CD were defined and a score was developed. A total of 1384 confocal images were collected from 9 patients and 10 controls. Five images from each patient and control were selected and compared with the biopsy specimen of the same site. The sensitivity, specificity, and positive predictive value for the confocal images in comparison to the histology were 100%, 80%, and 81%. The kappa inter-observer agreement between the 2 endoscopists was 0.769 (P = 0.018) and between the 2 histopathologists was 0.571 (P = 0.05). CONCLUSIONS Confocal endomicroscopy offers the prospect of diagnosis of CD during ongoing endoscopy. It also enables targeting biopsies to abnormal mucosa and thereby increasing the diagnostic yield, especially when villous atrophy is patchy in the duodenum.

To evaluate the feasibility and utility of confocal laser endomicroscopy (CLE) in the description of normal gastrointestinal (GI) mucosa and in the diagnosis of GI disorders in children, in comparison to histology.Forty-four patients (19 female) median age 10.9 years (range 0.7-16.6 years) with suspected or known GI pathology underwent esophago-gastro-duodenoscopy (OGD) (n = 36) and/or ileocolonoscopy (IC) (n = 31) with CLE using sodium fluorescein and acriflavine as contrast agents. Histological sections were compared with same site confocal images by two experienced pediatric and GI histopathologists and endoscopists, respectively.Duodenum and ileum were intubated in all but one patient undergoing OGD and IC. The median procedure time was 16.4 min (range 7-25 min) for OGD and 27.9 min (range 15-45 min) for IC. A total of 4798 confocal images were compared with 153 biopsies from the upper GI tract from 36 procedures, and 4661 confocal images were compared with 188 biopsies from the ileocolon from 31 procedures. Confocal images were comparable to conventional histology both in normal and in pathological conditions such as esophagitis, Helicobacter pylori gastritis, celiac disease, inflammatory bowel disease, colonic heterotopia, and graft versus host disease.CLE offers the prospect of targeting biopsies to abnormal mucosa, thereby increasing diagnostic yield, reducing the number of biopsies, decreasing the burden on the histopathological services, and reducing costs.

We describe a case of a 15-year-old male presenting with an unusual right-sided paratesticular mass in whom the diagnosis of an ovarian-like paratesticular müllerian-type papillary serous tumor was made. The tumor, also referred to as serous papillary cystadenoma by some authors, depicted papillae with fibro-vascular cores lined by a bland pseudostratified ciliated epithelium. The immunohistochemistry showed the tumor to have diffuse reactivity against BerEp4, epithelial membrane antigen, and progesterone receptor; focal reactivity against cytokeratins AE1/AE3, cytokeratin 7 (CK7), Ca125, estrogen, and carcinoembryonic antigen; and negative reactivity for calretinin, Wilms tumor 1 (WT1), and CK20. Surgical management involved resection of the tumor followed by regular follow-up. Paratesticular müllerian-type papillary serous tumor is an infrequent lesion that has seldom been described in the pediatric-age patient. To our knowledge, whereas only 2 cases of serous papillary borderline malignancy have been previously described in this age group, this is the 1st time a serous papillary tumor is reported in a child. The pediatric surgeon, the clinicians, and the pathologist should be aware of this entity, as a conservative management would be preferable.

Three cases of cranial vault defects in fetuses are described. All 3 cases underwent postmortem examination, and in 2 of them postmortem magnetic resonance imaging was also performed. The phenotypical differences between the cases illustrate a spectrum of cranial vault defects, associated anomalies, and pathogenic mechanisms involved in the etiology of this rare malformation. All 3 cases were male, and the cranial vault defects corresponded to exencephaly with acrania/acalvaria caused by amniotic band syndrome, acalvaria, and acrania presenting in a chorangiopagus parasiticus twin.

This case outlines the potential complexity of autosomal recessive polycystic kidney disease (ARPKD). It highlights the challenges involved in managing this condition, some of the complications faced and areas of uncertainty in the decision making process. With a paucity of published paediatric cases on this subject, this should add to the pool of information currently available.

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