Atilano Lacson - Academia.edu (original) (raw)

Papers by Atilano Lacson

Archives of pediatrics & adolescent medicine, Feb 1, 2000

Journal of the Canadian Association of Gastroenterology, May 17, 2019

Background: Controversy exists about optimal methods for duodenal biopsy in diagnosis of celiac d... more Background: Controversy exists about optimal methods for duodenal biopsy in diagnosis of celiac disease (CD), in terms of both number of samples and anatomic location. The reliability of duodenal bulb biopsy has been questioned given that normal bulb architecture may mimic disease. However, multiple studies have reported patients with CD have histopathological lesions limited to proximal changes in the duodenal bulb alone. Methods: We retrospectively compared duodenal and duodenal bulb histology in a population of paediatric patients with CD and compared with a population of nonceliac controls at Stollery Children's Hospital, 2010 to 2012. Results: Fifty-seven paediatric patients diagnosed with CD and 16 nonceliac controls were included in the study. Fifty-three celiac patients (93.0%) had histopathology consistent with CD (modified Marsh score of 3A, 3B or 3C) in the duodenal bulb. The modified Marsh classification differed significantly between duodenum and duodenal bulb in nine celiac patients (15.8%). Of these, five (8.8%) had Marsh 3 in the bulb and Marsh 0 in the distal duodenum. Among controls, no patients had villous atrophy in either the distal duodenum or duodenal bulb, and all patients had a modified Marsh score of 0 at both sites. Conclusions: The results of this study reinforce that duodenal bulb samples are critically important for diagnosing CD in paediatric patients. We suggest that duodenal bulb samples be submitted in separate containers from distal duodenal samples to facilitate accurate interpretation. In contrast to prior reports, we found villous blunting and intraepithelial lymphocytosis are actually uncommon findings in paediatric patients with nonceliac gastrointestinal disorders.

Fetal and Pediatric Pathology, 2005

Complete hydatidiform mole is an abnormal conceptus characterized by hydropic villi accompanied b... more Complete hydatidiform mole is an abnormal conceptus characterized by hydropic villi accompanied by proliferating trophoblasts. Its pathogenesis is largely unknown. Endothelial nitric oxide synthase is induced by vascular endothelial growth factor and has been implicated in the pathogenesis of preeclampsia and other physiologic conditions in the placenta. C-kit is the tyrosine kinase receptor and is involved in tumor formation elsewhere in the body. Using standard immunohistochemical protocols, we studied the expression of C-kit and endothelial nitric oxide synthase in the placenta of 10 patients with complete hydatidiform mole. Cytoplasmic and nuclear staining with endothelial nitric oxide synthase was identified in the cytotrophoblast and intermediate trophoblast layers in all cases, with high staining in 7/10 and 6/8 cases, respectively. Minimal staining is identified in the syncytiotrophoblast layer. Hofbauer stromal cells were identified in 9 cases and showed low staining intensity in 7/9 cases. Cytoplasmic C-kit staining was diffuse and of low intensity. The cytotrophoblast, the syncytiotrophoblast, intermediate trophoblast, and the stromal cells had low C-kit staining intensity in 8/10, 8/10, 7/9, and 5/9 cases. These results indicate that C-kit and endothelial nitric oxide are expressed in the placentas of complete hydatidiform mole and may play a role in the pathogenesis of trophoblastic proliferation in this condition.

Pediatric Transplantation, Jun 14, 2014

Fungal respiratory infections in patients with CF are a significant concern both pre‐ and post‐lu... more Fungal respiratory infections in patients with CF are a significant concern both pre‐ and post‐lung transplantation (LTx). Fungal infection is associated with increased mortality post‐LTx, and in the past decade, the prevalence of fungal colonization in Canadian pediatric patients with CF has increased. The emergence of novel fungal pathogens is particularly challenging to the transplant community, as little is known regarding their virulence and optimal management. We present a case of a successful double‐lung transplant in a pediatric patient with CF who was infected pretransplantation with a novel yeast, Blastobotrys rhaffinosifermentans. This patient was treated successfully with aggressive antifungal therapy post‐transplantation, followed by extended fungal prophylaxis. The significance of fungal colonization and infection in children with CF pre‐ and post‐LTx is reviewed.

American journal of medical genetics, 2004

We describe an infant with multiple congenital anomalies including cleft palate and micrognathia,... more We describe an infant with multiple congenital anomalies including cleft palate and micrognathia, Mö bius sequence, developmental delay, myopathy, hydronephrosis, and bilateral clubfeet. These features are consistent with Carey-Fineman-Ziter (CFZ) syndrome (MIM 254940), which has been previously reported in six children (including two sibling pairs). Cranial magnetic resonance imaging (MRI) revealed an unusually small pons, a finding not previously described in CFZ syndrome.

Fetal And Pediatric Pathology, 2002

A case is presented of a female newborn infant delivered with an Apgar Score of 1, who could not ... more A case is presented of a female newborn infant delivered with an Apgar Score of 1, who could not be resuscitated. There was a high arched palate, bell-shaped chest, contractures of writes inflexion, ankles and knees in extension, and intrauterine fractures. Clinical discussion led to a diagnosis of arthrogryposis secondary to fetal akinesia syndrome caused by nemaline myopathy. Pathology and pathologic discussion confirmed this diagnosis.

Pediatric Cardiology, Mar 28, 2012

Neurology, Sep 1, 1983

We report clinical and pathologic findings in a 16-year-old boy whose disease began in infancy wi... more We report clinical and pathologic findings in a 16-year-old boy whose disease began in infancy with maculopapular skin lesions, followed by cyclic nodular cutaneous eruptions, intermittent enlargement of liver and spleen, episodic abdominal pain, and sporadic unexplained fever. Subsequently, various ophthalmologic disturbances, along with a multitude of neurologic signs and symptoms, dominated the clinical picture. The CNS bore the brunt of pathologic changes, characterized by widespread leptomeningeal fibrosis, ventricular enlargement, and multiple brain infarcts. Striking intimal thickening led to narrowing or occlusion of almost all the medium-sized and small extraparenchymal arteries.

Fetal and Pediatric Pathology, 2004

u Background: CXC-chemokines bearing the glutamic acid-leucine-arginine (ELR) motif (ELR þ CXC ch... more u Background: CXC-chemokines bearing the glutamic acid-leucine-arginine (ELR) motif (ELR þ CXC chemokines) are potent neutrophil chemoattractants and hence may play a role in mucosal injury seen with intestinal ischemia-reperfusion (I=R). Methods: Serum concentrations of ELR þ CXC chemokines (keratinocyte-derived chemokine(KC)=CXC ligand (CXCL) 1, macrophage in£ammatory protein (MIP)-2=CXCL 2=3, lipopolysaccharide-induced CXC chemokine (LIX)=CXCL5, and lungkine=CXCL15) were measured in a murine intestinal I=R model. F|fteen 4-week-old wild-type mice were studied in three subgroups: sham, ischemia (superior mesenteric artery [SMA] clamping for 60 min) and ischemia-reperfusion (SMA clamping for 60 min followed by reperfusion for 90 min). Results: Concentrations of KC=CXCL1 and MIP-2=CXCL2=3 in sham-treated animals (145 AE 123 and 107 AE 55 pg=mL, respectively) and the ischemia subgroup (646 AE 413 and 226 AE 129 pg=mL) were similar, but concentrations were signi¢cantly higher with reperfusion (6398 AE 2297, p<.001 and 874 AE 790 pg=mL, p ¼.04). LIX=CXCL5 and lungkine=CXCL15 concentrations did not change signi¢cantly with ischemia or following I=R. KC=CXCL1 and MIP-2=CXCL2=3 concentrations correlated positively with the severity of mucosal injury and with each other, whereas a negative relationship was observed between LIX=CXCL5 concentrations and microscopic injury scores. Conclusions: Development of mucosal injury in intestinal I=R is associated with increased serum concentrations of KC=CXCL1 and MIP-2=CXCL2=3, but not with those of LIX=CXCL5 and lungkine=CXCL15.

American Journal of Clinical Pathology, May 1, 1979

Peliosis of the spleen is a rare condition that accompanies peliosis hepatis. Two cases are prese... more Peliosis of the spleen is a rare condition that accompanies peliosis hepatis. Two cases are presented, and a detailed morphologic description is given. The lesions are confined to the red pulp and appear to arise by progressive distention of splenic sinuses, with eventual thrombosis and organization. Peliotic involvement of the spleen is probably more common than realized and can have serious clinical implications.

Journal of stroke and cerebrovascular diseases, Jul 1, 2016

Microscopic polyangiitis (MPA) is an ANCA-associated vasculitis (AAV; ANCA denotes antineutrophil... more Microscopic polyangiitis (MPA) is an ANCA-associated vasculitis (AAV; ANCA denotes antineutrophil cytoplasmic antibody) that causes necrotizing inflammation of small blood vessels. Renal and pulmonary manifestations are common whereas central nervous system (CNS) involvement, and in particular spinal disease, is rare. We reviewed a case of MPA presenting with spinal intradural hemorrhage and intracerebral hemorrhage. We also summarized all reported cases of AAV with spinal cord involvement in the literature (database search included MEDLINE, Embase, Scopus, and Proquest with no date or language restriction). We reviewed 20 cases of AAV with spinal cord involvement (12 granulomatosis with polyangiitis [GPA], 4 eosinophilic granulomatosis with polyangiitis, 2 MPA, and 2 cases diagnosed as AAV only) and reported demographic information, clinical features, methods of diagnosis, treatment, and patient outcome. Although CNS involvement has been associated with a poor prognosis, 14 of 18 cases that reported outcome data achieved remission during follow-up. Death occurred in 3 patients diagnosed with GPA and in 1 patient with MPA. Our patient with MPA deteriorated rapidly despite use of prednisone and died. AAV can present with brain and spinal cord involvement, even in the absence of systemic disease. CNS disease may be responsive to immunosuppressive therapy (e.g., steroids and cyclophosphamide) in several of the cases reviewed.

Journal of Pediatric Gastroenterology and Nutrition, Apr 28, 2023

The prevalence of pediatric serological negative celiac disease (SNCD) is poorly described, with ... more The prevalence of pediatric serological negative celiac disease (SNCD) is poorly described, with few recognized clinical predictors beyond immunoglobulin A (IgA) deficiency or reduced gluten intake. The purpose of this retrospective review was to describe the prevalence of SNCD at the Stollery Children’s Hospital and identify clinical features to help in recognition of these cases. Patients with a positive biopsy and negative serology (SNCD) were compared to those with positive biopsy and serology. SNCD diagnosis required clinical correlation and either confirmatory genetics or follow up endoscopy on a gluten-free diet. Of the 424 patients who met celiac disease (CD) criteria, 4.7% (n = 20) fulfilled our criteria for SNCD. There was a significant difference in the rates of IgA deficiency between the 2 groups, but no other clinical features were found that allowed for ready identification of SNCD patients.

American journal of medical genetics, Jan 9, 2003

Pediatric Nephrology, Dec 1, 1994

The case of a 6-year-old Inuit female with the epidemic form of hemolytic uremic syndrome (HUS) w... more The case of a 6-year-old Inuit female with the epidemic form of hemolytic uremic syndrome (HUS) with myocardial involvement and probable cardiac tamponade is presented. This case illustrates the multisystemic nature of the syndrome, and to our knowledge, cardiac tamponate as a probable terminal event in HUS has not been reported previously.

The Journal of Pediatrics, Feb 1, 1998

Langerhans cell histiocytosis may be seen with goiter and histiocytic infiltration of the thyroid... more Langerhans cell histiocytosis may be seen with goiter and histiocytic infiltration of the thyroid. We report a 2 1 ⁄2-year-old boy who had goiter and primary hypothyroidism develop, later had pulmonary disease, and died of neurologic involvement. Autopsy lesions suggested a transitional dendritic cell precursor of the epidermal Langerhans cell. Of the reported cases of Langerhans cell histiocytosis with goiter in children and adolescents, 82% were male when the relative incidence of Langerhans cell histiocytosis is two males to one female.

Acta Paediatrica, Dec 1, 1991

Dissection of the aorta is very rare in children, but classically occurs in the presence of Marfa... more Dissection of the aorta is very rare in children, but classically occurs in the presence of Marfan syndrome or other connective tissue disorder. We present a case of spontaneous dissection in a 12-year-old boy whose half brother has an idiopathic dilated aorta and whose mother has also required surgery for dissection of a dilated aorta. No features of connective tissue disorder were present in any family member.

Cancer, May 15, 1988

Two cases of hormonally active, metastasizing malignant mixed germ cell-sex cord-stromal tumors a... more Two cases of hormonally active, metastasizing malignant mixed germ cell-sex cord-stromal tumors are described in otherwise normal prepubertal girls. Isosexual precocity was noted 5 months and 1 month before surgery. One child died 1 year after presentation, and the other was alive, with no apparent tumor, 1 year after diagnosis. These two cases represent the first recorded instance of a unique tumor in which metastases of several cell types were encountered.

Animals, Jun 25, 2022

This article is an open access article distributed under the terms and conditions of the Creative... more This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY

Background Alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV) is a rare,... more Background Alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV) is a rare, fatal, congenital lung disorder involving abnormal development of the capillary vascular system around the alveoli of the lungs, which clinically presents as persistent pulmonary hypertension of the newborn (PPHN) refractory to treatment. It has been linked to the gene FOXF1 on chromosome 16q24.1-q24.2. Histopathological examination by lung biopsy is the gold standard for diagnosis. Materials and methods We present four cases of ACD/MPV who were referred for ECMO support with a diagnosis of PPHN with no apparent congenital anomalies. Results All the newborns had an overwhelming course, with PPHN and hypoxemia refractory to treatment. The diagnosis of ACD/MPV was established by ante-mortem lung biopsy in all cases. Intensive care treatment was withdrawn post diagnosis, with none of the four surviving. Conclusions Early lung biopsy for a histological diagnosis allows expensive and ineffective treatment to be avoided. Lung biopsy can be performed with low risk and highdiagnostic yield for alveolar capillary dysplasia.

Pediatric and Developmental Pathology, Mar 1, 2002

Macrophagic myofasciitis (MMF) is a rare, seemingly emerging entity among adult patients in Franc... more Macrophagic myofasciitis (MMF) is a rare, seemingly emerging entity among adult patients in France. We encountered two children with the first two cases of MMF in North America. A 5-year-old male with chronic intestinal pseudo-obstruction required nighttime parenteral nutrition. Abnormal pupillary reflexes and urinary retention suggested a diffuse dysautonomia, which prompted a neurological diagnostic work-up. A 3-yearold child had developmental delay and hypotonia. Both children received age-appropriate immunizations. Quadriceps muscle biopsy from each child showed the typical patchy, cohesive centripetal infiltration of ␣-1antitrypsin ϩ , ␣-1-antichymotrypsin ϩ , CD68 ϩ , PAS ϩ , CD1a Ϫ , S-100 Ϫ , factor XIII Ϫ granular macrophages with adjacent myofiber atrophy, dilated blood vessels, and mild endomysial and perimysial fibrosis. No myonecrosis was observed and no discrete granulomas were seen. A single aluminum peak was demonstrated on energy dispersive X-ray microanalysis. The etiology of the clinical symptoms in these cases and in cases reported as MMF remains intriguing. Despite numerous stains to demonstrate organisms, most infectious causes leading to macrophage activation were ruled out. These cases are being reported to increase awareness of this condition and to encourage a systematic epidemiologic and clinicopathologic study in North America.