Information on stock identification and spatial stock structure provide a basis for understanding fish population dynamics and improving fisheries management. In this study, otolith shape analysis was used to study the stock structure of blue whiting (Micromesistius poutassou) in the northeast Atlantic using 1693 samples from mature fish collected between 37°N and 75°N and 20°W and 25°E. The results indicated two stocks located north and south of ICES Divisions VIa and VIb (54°5N to 60°5N, 4°W to 11°W). The central area corresponds to the spawning area west of Scotland. Sampling year effects and misclassification in the linear discriminant analysis suggested exchanges between the northern and southern stocks. The results corroborate previous studies indicating a structuring of the blue whiting stock into two stocks, with some degree of mixing in the central overlap area.

There is increasing demand for information on predator–prey interactions in the ocean as a result of legislative commitments aimed at achieving sustainable exploitation. However, comprehensive data sets are lacking for many fish species and this has hampered development of multispecies fisheries models and the formulation of effective food-web indicators. This work describes a new compilation of stomach content data for five pelagic fish species (herring, blue whiting, mackerel, albacore and bluefin tuna) sampled across the northeast Atlantic and submitted to the PANGAEA open-access data portal (www.pangaea.de). We provide detailed descriptions of sample origin and of the corresponding database structures. We describe the main results in terms of diet composition and predator–prey relationships. The feeding preferences of small pelagic fish (herring, blue whiting, mackerel) were sampled over a very broad geographic area within the North Atlantic basin, from Greenland in the west, to the Lofoten Islands in the east and from the Bay of Biscay northwards to the Arctic. This analysis revealed significant differences in the prey items selected in different parts of the region at different times of year. Tunas (albacore and bluefin) were sampled in the Bay of Biscay and Celtic Sea. Dominant prey items for these species varied by location, year and season. This data compilation exercise represents one of the largest and most wide-ranging ever attempted for pelagic fish in the North Atlantic. The earliest data included in the database were collected in 1864, whereas the most recent were collected in 2012. Data sets are available at doi:10.1594/PANGAEA.820041 and doi:10.1594/PANGAEA.826992.

Evidence from morphometric, meristic, oceanographic, genetic and otolith microstructure studies suggest complexity in the structure of the blue whiting (Micromesistius poutassou) population in the Northeast Atlantic. However the boundaries between stock components and the degree to which they overlap on the spawning grounds are uncertain. Blue whiting are therefore currently assessed and managed as a single stock. This study uses otolith shape analysis to provide further insight into the stock structure of blue whiting in the NE Atlantic at a critical period of their life history: spawning. Otolith shape analysis is useful for stock discrimination as it can identify groups of fish which may have been spatially or temporally discrete at some stage in their life history. In this study, blue whiting were sampled in 2003 and 2010, from the northern and southern extremes of the spawning ground and from around the Porcupine Bank and Rockall Trough. Spatial variation in otolith shape was examined in an attempt to elucidate boundaries between stock components. Cluster analysis of the otolith shape data revealed two distinct morphotypes; although some overlap did occur, fish of morphotype I occupied a more northerly distribution than fish of morphotype II. These findings are consistent with previous observations from otolith microstructure and oceanographic modelling, and support the hypothesis of northern and southern components in the blue whiting population which may overlap to varying degrees in the centre of the spawning distribution.

Report of the Benchmark Workshop on Pelagic Stocks (WKPELA 2012), 13–17 February 2012 Copenhagen, Denmark

Cerebroretinal microangiopathy with calcifications and cysts (CRMCC) is a rare multisystem disorder characterized by extensive intracranial calcifications and cysts, leukoencephalopathy, and retinal vascular abnormalities. Additional features include poor growth, skeletal and hematological abnormalities, and recurrent gastrointestinal bleedings. Autosomal-recessive inheritance has been postulated. The pathogenesis of CRMCC is unknown, but its phenotype has key similarities with Revesz syndrome, which is caused by mutations in TINF2, a gene encoding a member of the telomere protecting shelterin complex. After a whole-exome sequencing approach in four unrelated individuals with CRMCC, we observed four recessively inherited compound heterozygous mutations in CTC1, which encodes the CTS telomere maintenance complex component 1. Sanger sequencing revealed seven more compound heterozygous mutations in eight more unrelated affected individuals. Two individuals who displayed late-onset cerebral findings, a normal fundus appearance, and no systemic findings did not have CTC1 mutations, implying that systemic findings are an important indication for CTC1 sequencing. Of the 11 mutations identified, four were missense, one was nonsense, two resulted in in-frame amino acid deletions, and four were short frameshift-creating deletions. All but two affected individuals were compound heterozygous for a missense mutation and a frameshift or nonsense mutation. No individuals with two frameshift or nonsense mutations were identified, which implies that severe disturbance of CTC1 function from both alleles might not be compatible with survival. Our preliminary functional experiments did not show evidence of severely affected telomere integrity in the affected individuals. Therefore, determining the underlying pathomechanisms associated with deficient CTC1 function will require further studies.

Characterization of suitable habitat for settlement of juvenile flatfish is important for the management of nursery areas. Food availability is one important determinant of habitat quality that can affect the condition and growth, and thus survival, of flatfish. Spatial and temporal variation in diet has been widely studied for several species of flatfish. However, levels of intraspecific variation in diet at small spatial scales are relatively unknown, with most studies focusing only on large scale variability. This study investigates how diet, growth and condition of juvenile plaice, Pleuronectes platessa, varies over two spatial scales (10s of kilometres and 100s of metres). Juvenile plaice were collected from three beaches and from three replicate hauls on each beach using a beach seine in September 2007 and 2008. Gut content analyses of 108 juvenile plaice within the size-range of 70–90 mm were carried out. Diet composition in plaice guts differed among beaches and hauls suggesting that food abundance and availability differed at both spatial scales. A significant positive correlation was observed between a morphological condition index and the prey diversity in the gut. This suggests that fish which specialize on a limited number of prey items (perhaps due to a greater abundance of certain prey) may do better than fish which feed on a wide range of prey types. Significant differences in condition were observed between hauls and between beaches, while recent and total otolith growth varied between beaches but not between hauls. The results highlight the importance of considering small scale variation when attempting to link habitat quality to feeding, growth and condition of juvenile flatfish.

Ileal dysgenesis is an uncommon condition of unknown etiology occurring in the distal ileum in the region of the vitelline duct. The CT appearance of this lesion, although not previously described to our knowledge, is characteristic. We report a patient with ileal dysgenesis who had an abdominal CT scan to evaluate chronic iron deficiency anemia and protein-losing enteropathy. Recognition of this lesion by pediatric radiologists is important; so that surgical treatment, which is simple and effective, can be initiated quickly.

Serum amylase and lipase frequently rise during bouts of acute pancreatitis, and measurement of these enzymes provides important diagnostic information. We report a pediatric patient with persistent elevations of serum lipase resulting from macrolipasemia, a complex of lipase with IgG, rather than pancreatitis. (J Pediatr 2002;141:129-31).

X-linked liver glycogenosis (XLG) is probably the most frequent glycogen-storage disease. XLG can be divided into two subtypes: XLG I, with a deficiency in phosphorylase kinase (PHK) activity in peripheral blood cells and liver; and XLG II, with normal in vitro PHK activity in peripheral blood cells and with variable activity in liver. Both types of XLG are caused by mutations in the same gene, PHKA2, that encodes the regulatory alpha subunit of PHK. To facilitate mutation analysis in PHKA2, we determined its genomic structure. The gene consists of 33 exons, spanning >/=65 kb. By SSCP analysis of the different PHKA2 exons, we identified five new XLG I mutations, one new XLG II mutation, and one mutation present in both a patient with XLG I and a patient with XLG II, bringing the total to 19 XLG I and 12 XLG II mutations. Most XLG I mutations probably lead to truncation or disruption of the PHKA2 protein. In contrast, all XLG II mutations are missense mutations or small in-frame deletions and insertions. These results suggest that the biochemical differences between XLG I and XLG II might be due to the different nature of the disease-causing mutations in PHKA2. XLG I mutations may lead to absence of the alpha subunit, which causes an unstable PHK holoenzyme and deficient enzyme activity, whereas XLG II mutations may lead to in vivo deregulation of PHK, which might be difficult to demonstrate in vitro.

OBJECTIVE:To identify complications amenable to prevention in adults with glycogen storage disease (GSD) types Ia, Ib, and III and to determine the effect of the disease on social factors. DESIGN:Case series and clinical review. SETTING:Referral medical centers in the United States and Canada. PATIENTS:All patients with GSD-Ia (37 patients), GSD-Ib (5 patients), and GSD-III (9 patients) who were 18 years of age or older. MEASUREMENTS:Ultrasound or radiographic studies identified liver adenomas, nephrocalcinosis, or kidney stones. Radiographic studies identified osteopenia. Reports of the clinical examination, serum chemistry results, and social data were obtained. RESULTS:For patients with GSD-Ia, problems included short stature (90%), hepatomegaly (100%), hepatic adenomas (75%), anemia (81%), proteinuria or microalbuminuria (67%), kidney calcifications (65%), osteopenia or fractures or both (27%), increased alkaline phosphatase (61%) and gamma-glutamyltransferase (93%) activities, and increased serum cholesterol (76%) and triglyceride (100%) levels. Hyperuricemia was frequent (89%). Patients with GSD-Ib had severe recurrent bacterial infections and gingivitis. In patients with GSD-III, 67% (6 of 9) had increased creatinine kinase activity. Four of these patients had myopathy and cardiomyopathy. CONCLUSIONS:For GSD-Ia, hyperuricemia and pyelonephritis should be treated to prevent nephrocalcinosis and additional renal damage. For GSD-Ib, granulocyte-colony-stimulating factor may prevent bacterial infections. For GSD-III, more data are required to determine whether the myopathy and cardiomyopathy can be prevented. Most of the patients with GSD-I and GSD-III had 12 or more years of education and were either currently in school or employed.

DiagnosisSeptember 1, 1994Gliadin antibody test had moderate sensitivity for celiac disease in adultsJames P. Keating, MDJames P. Keating, MDSt. Louis Children's Hospital, St. Louis, Missouri, USA (J.P.K.)Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/ACPJC-1994-121-2-051 SectionsAboutFull Text ToolsAdd to favoritesDownload CitationsTrack Citations ShareFacebookTwitterLinkedInRedditEmail Source CitationBodé S, Gudmand-Høyer E. Evaluation of the gliadin antibody test for diagnosing coeliac disease. Scand J Gastroenterol. 1994 Feb;29:148-52.References1 Knudtzon J, Fluge G, Aksnes L. Routine measurements of gluten antibodies in children of short stature. J Pediatr Gastroenterol Nutr. 1991;12:190-4. Google Scholar2 Cacciari G, Salardi S, Volta U, et al. Prevalence and characteristics of coeliac disease in type 1 diabetes mellitus. Acta Paediatr Scand. 1987;76:671-2. Google Scholar3 Corazza G, Valentini RA, Frisoni M, et al. Gliadin immune reactivity is associated with overt and latent enteropathy in relatives of celiac patients. Gastroenterology. 1992;103:1517-22. Google Scholar Author, Article, and Disclosure InformationAffiliations: St. Louis Children's Hospital, St. Louis, Missouri, USA (J.P.K.) PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails September 1, 1994Volume 121, Issue 2Page: 51KeywordsAlcoholicsAtrophyBiopsyCeliac diseaseCellsChildrenChronic diarrheaChronic pancreatitisCirrhosisCrohn's diseaseDietEnzyme linked immunosorbent assayGastroenteritisGastroenterology and hepatologyIrritable bowel syndromeMorbidityPopulation statisticsSpecificityUlcerative colitis ePublished: 9 March 2020 Issue Published: September 1, 1994 Copyright & PermissionsCopyright © 1994 by American College of Physicians. All Rights Reserved.Loading ...

In Reply. —Since water intoxication has been a rare entity, it is not surprising that studies providing critical comparison of hypertonic saline vs mannitol have not been published. I agree with Nutman and Hill that mannitol is a reasonable choice, but it has not been proven to be safer or more effective. Although discussion of refinements of therapy is interesting, prevention is a more attractive topic. Interventions directed to the population at risk might include education, public service announcements, or modification of the instructions given to new mothers when they leave the hospital. Incentives to promote breast-feeding in the context of the Supplemental Food Program for Women, Infants, and Children (WIC) may be the most meaningful project. Finberg 1 has suggested financial incentives to eligible mothers in the WIC program who breast-feed. Study of that approach in an appropriately structured fashion is overdue.

In Reply. —I did believe, when the article was written, that my work could be used as a reason for supporting to encourage breast-feeding but I doubt that depriving poor infants of adequate formula by weakening the WIC program will increase breast-feeding. This summer, I admitted a 3-month-old breast-fed infant with seizures and a serum sodium level of 114 mmol/L. The grandmother had advised the mother to stop nursing for a day and to give water instead. The infant had taken an amount of water similar to the infants in our report. Caretakers should know that interrupting feeds and offering a hungry infant water can cause water intoxication. Parents and health workers should avoid all situations in which hungry infants are given water instead of breast milk or infant formula. Increasing the availability of breast milk or formula, especially to infants living in poverty, would be an important preventive step.

Despite this book's sensational subtitle,The True Story of Nurse Genene Jones and the Texas Baby Murders,Elkin's style is much more like that of Berton Roueche, the author ofEleven Blue Men,than that of a tabloid. Hand over hand, he pulls himself and the reader along a chain of tragic events occurring in a pediatric intensive care unit (PICU) and a pediatric practice in San Antonio in 1980. Injecting succinylcholine in the place of immunizations, a licensed vocational nurse (the Texas equivalent of a licensed practical nurse) converted routine visits to the office of a young pediatrician into "codes." She elbowed experienced emergency medical service personnel aside in a helicopter and gave lethal injections to an infant in front of them, then took credit for early recognition of the "deterioration" that they failed to detect. In the PICU Jones' patients had a high incidence of unexpected hemorrhage on

Munchausen syndrome by proxy has received considerable attention in the medical literature. These reports, however, usually focus on descriptions of the medical aspects of the often bizarre patterns of parent-child behaviors which characterize this syndrome. Despite the destructiveness of these behaviors, few attempts have been made to examine the syndrome from the perspective of a variant form of child maltreatment. In light of this, the authors will: (1) define and briefly summarize the history of the syndrome; (2) review the literature focusing particular attention on the definition of the syndrome, pinpointing its relationship to child abuse; (3) provide case studies of the syndrome to illuminate the interplay of medical and psychosocial aspects of the problem; and (4) suggest methods of intervention.

In a few passages of this casebook the reader knows that an experienced clinician is in charge of the emergency room. He or she warns that most infants in the emergency room have sinus tachycardia from sepsis or shock, not paroxysmal atrial tachycardia, and that use of verapamil can have tragic consequences. Then the clinician spells out a reasonable approach to the treatment of paroxysmal atrial tachycardia (page 191). Fifty pages later this sophistication is seen again in a savvy but short description of the necessity and method of funduscopic examination as a prerequisite to lumbar puncture for meningitis. These brief glimpses of wisdom, coming from a group of young (six of nine contributors are residents or fellows) pediatricians in a field that is now beginning to examine itself and to teach, are promising. Unfortunately, most of the book, which is a series of 50 case reports followed by suggested