1/8. colitis in chronic granulomatous disease.BACKGROUND: Involvement of the gut in chronic granulomatous disease (CGD) has been previously described and colitis highlighted. However, the nature and histopathology of the colitis are unclear and have been thought to be non-specific or similar to Crohn's disease. methods: Seven patients with CGD, suffering from gastrointestinal symptoms were prospectively studied. RESULTS: All patients had anaemia; other symptoms were failure to thrive (5/7) and diarrhoea (5/7). Most had microcytic anaemia (5/7), increased platelets (7/7), and increased erythrocyte sedimentation rate (6/6). Endoscopically there was a friable erythematous mucosa in 6/7. The histological features present in all patients consisted of a colitis with paucity of neutrophils, increased numbers of eosinophils, eosinophilic crypt abscesses, pigmented macrophages, and nuclear debris. In some granulomas were present (2/7). CONCLUSIONS: colitis is a common cause of gastrointestinal symptoms in CGD and is caused by a non-infective inflammatory process. The histology has specific features, which are distinctive from those seen in Crohn's disease.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/8. ataxia telangiectasia. A case report.The case of an eight year old girl with ataxia telangiectasia (AT) is described. She presented at seven years of age with gait problems and was found to have the neurological, dermatological and immunological features characteristic of AT along with a history of frequent sino-pulmonary infections. This report highlights the refractory nature of the disease, the difficulties in medical management, and the problems posed by late diagnosis which can compromise patient care. This is a rare inherited form of ataxia which has not been previously reported in West Indian literature.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
3/8. Vallecular cyst: a cause of failure to thrive in an infant.Congenital vallecular cyst is fairly uncommon in neonates and infants. Although benign in nature, it may cause stridor and even life-threatening airway obstruction in early infancy. A 3-month-old male baby presented with failure to thrive and respiratory distress was found to have a vallecular cyst. Marsupialization with CO(2) laser was performed.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
4/8. Infantile multisystem inflammatory disease: a specific syndrome?We report two patients with infantile onset of evanescent rash, fever, arthropathy with severe deformities, periosteal changes, chronic meningitis, hydrocephalus, convulsions, developmental delay, papilledema, unusual uveitis, and lymphadenopathy. A few patients with similar findings have been previously reported. Although some similarity exists between findings in these patients and in others with systemic juvenile rheumatoid arthritis, they appear to differ both in regard to the nature and severity of the clinical and pathologic features. We suggest that this group of patients has a separate rheumatic disorder not yet included in the standard classifications of the childhood rheumatic diseases.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
5/8. Two Prader-Willi/angelman syndrome loci present in an isodicentric marker chromosome.We found an abnormal 47,XX, mar karyotype in a patient with developmental delay, hypotonia, microcephaly, failure to thrive, and cognitive delay. When metaphases were hybridized with Prader-Willi and Angelman loci-specific probes by the FISH technique, two sites were noted at opposite positions on the marker chromosome. The alphoid satellite dna probe documented the isodicentric nature while retention of the p arms on both sides of the marker chromosome was demonstrated by beta satellite probe. The patient does not exhibit manifestations of either syndrome despite the presence of these loci in tetrasomic dose. The present investigation suggests that other marker chromosomes be reevaluated, as their clinical manifestations are quite variable.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
6/8. Functional vomiting disorders in infancy: innocent vomiting, nervous vomiting, and infant rumination syndrome.Pediatric gastroenterologists have tended to view gastroesophageal reflux (GER) as a disease in and of itself--a disease that can be diagnosed "objectively" with use of numerical data from esophageal ph monitoring and cured with pharmacologic or surgical treatment. What is often forgotten is that the data derived from esophageal ph monitoring and other techniques may identify the presence of abnormal GER but tell nothing about its pathogenesis. The usual approach to infants who feed poorly, vomit, or fail to gain weight is to identify the presence of abnormal GER, rule out underlying organic causes of vomiting, and then diagnosis primary GER disease. The baby is then treated with pharmacologic, dietary, or positional therapy and, ultimately, if these therapies fail to eradicate the symptoms attributed to GER, surgical fundoplication, which stops vomiting regardless of its causes. The pediatric literature on infant vomiting and GER is almost devoid of research into the nature and possible relationships among infant stress, vomiting, feeding difficulties, and failure to grow. Clinically, the quality of the maternal-infant relationship is frequently approached superficially, with psychosocial aspects treated as less important in infants considered to have primary organic disease amenable to medical or surgical treatment. Psychosocial factors in the pathogenesis of the infant's symptoms are often not pursued beyond assessment for possible abuse or neglect. It has been known for centuries that stress or excitement affects gastrointestinal function and symptoms. Although the field of infant psychiatry has produced a substantial literature on the nature of stresses that affect both infants and mothers, the pediatric literature on vomiting and failure to thrive seldom acknowledges the existence or importance of these contributions. In clinical practice, failure to explore psychosocial aspects that may contribute to vomiting, feeding difficulties, or failure to thrive may result in missed opportunities for less invasive, more effective therapy at best, and countertherapeutic treatment at worst. This article describes three functional vomiting disorders of infancy, their distinguishing characteristics, hypotheses regarding their pathogenesis, and principles of comprehensive management.- - - - - - - - - - ranking = 2keywords = nature (Clic here for more details about this article) |
7/8. Changes in psychopathology of parents of NOFT (non-organic failure to thrive) infants during treatment.This clinical case-study of 50 infants suffering from NOFT (non-organic failure to thrive) and their parents supports the idea that the feeding problem is intimately related to parental disorders. We find a high rate (70%) of parental psychopathology (axis I diagnosis applying DSM-III-R) at the time of referral and a significant reduction (to 37%) during treatment of the infants and their parents. After a year only 12% of the parents were diagnosed with psychiatric disorders. In contrast personality disorders (axis II diagnosis applying DSM-III-R) show more stability and can be regarded as a trait variable, whereas the psychiatric disorders are of a more reactive nature. These conclusions may be influenced somewhat by the strictly hospital based design of our pilot study (infants and parents contacted only after clinical referral) and by inclusion only of firstborn infants. Nevertheless, they point to the psychopathology of parents as a main cause for non-organic failure to thrive. Psychopathological traits such as severe attachment behavior problems and primary bonding difficulties may have been latent and only became manifest due to the task of nurturing an infant for the first time.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
8/8. A multidisciplinary approach for the treatment of children with failure to thrive.failure to thrive is a complex problem affecting long-term health and development of the child. This paper describes a multidisciplinary approach to management and treatment of children and families where there is a child who is failing to thrive. The experience described in this paper is based on many years work of an interdisciplinary nature involving a Paediatrician, Psychologist and Dietician which lead to the creation of a special clinic for these children in 1993.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |