Atypical abdominal paediatric lymphangiomatosis: diagnosis aided by diffusion-weighted MRI

We report a 4-year-old child with a mesenteric mass, which on ultrasound, CT and conventional MRI appeared solid, raising lymphoma as a possible diagnosis. Diffusion weighted MRI (DW-MRI), however, suggested a low-cellularity lesion, making lymphoma less likely. Biopsy confirmed lymphangioma. DW-MRI may be a useful adjunct to conventional imaging, even in the abdomen.     

Chemical gastropathy: a distinct histopathologic entity in children

BACKGROUND: Chemical gastropathy, also known as chemical or reactive gastritis, is a well-described histopathologic entity in adults. It is characterized by presence of foveolar hyperplasia, vascular congestion, lamina propria edema, and prominent smooth muscle fibers in the absence of inflammatory cells in the gastric antral mucosa. There are no data in children on this condition. METHODS: All children showing features of chemical gastropathy in antral biopsy specimens were identified from pathology database from 1997 to 2000. Antral biopsy specimens were reviewed to assess the diagnosis of chemical gastropathy using standard diagnostic criteria. Charts of the children diagnosed with chemical gastropathy were reviewed for clinical course, endoscopic findings, and risk factors. RESULTS: Twenty-one children (12 male, 9 female) with chemical gastropathy were identified. Common presenting symptoms were epigastric pain and vomiting. Eleven children were taking multiple medications, including nonsteroidal anti-inflammatory drugs. Endoscopy revealed esophagitis in 12, antral erythema in 7, and thick bile in the stomach in 7 children. Antral histology revealed foveolar hyperplasia in 19, congestion in 20, lamina propria edema and smooth muscle fibers in 16, and absence of inflammation in 19 patients. Acid suppression was the treatment in all patients. Mean follow-up duration was 11 months in 17 children. Symptoms resolved completely in 11 and partially in 6 patients. CONCLUSIONS: As in adults, chemical gastropathy occurs in children. The factors associated with chemical gastropathy in this survey were gastroesophageal reflux disease and intake of multiple medications, including nonsteroidal anti-inflammatory drugs.     

Lymphangiography and bone scan in the study of lymphangiomatosis

Lymphangiomatosis is a rare congenital systemic lymphatic malformation involving multiple bones and other sites. A 12-year old boy with lymphangiomatosis was studied with lymphangiography and bone scan. Lymphangiogram demonstrated irregular pooling of contrast medium in both the mediastinal tumor and bones. Bone scan also revealed increased activity in areas which were not demonstrated radiographically.     

Ischio-vertebral dysplasia: a distinct entity

Background. Kyphoscoliosis is a complication of some bone dysplasias, including cleidocranial dysplasia. Objectives. We report a distinct disorder with defective ossification of the ischial rami, severe kyphoscoliosis and normal clavicles. Early recognition of this syndrome allows prevention of complications. Materials and methods. All patient cases (aged 1 day to 33 years) were selected according to the above criteria, with special attention to radiological findings, family history and follow-up (5–30 years). Results. In all eight patients, we observed the following: (a) Severe thoracic scoliosis of early onset and rapid progression, leading to rotatory dislocation. Spinal cord compression occurred in four cases with respiratory problems related to chest deformity. (b) Bilateral and symmetrical incomplete ossification of the ischial rami. (c) Peculiar facies with retrognathia. (d) Normal clavicles. Three patients were from the same family (grandmother, mother and daughter). Conclusion. Ischio-vertebral dysplasia seems to represent a true entity, with radiological and genetic findings that make it distinct from cleidocranial dysostosis. The association of kyphoscoliosis and these pelvic abnormalities is specific for this condition. Neurological and respiratory complications can be avoided if the condition is recognised early and early treatment is instituted. Received: 19 September 1997 Accepted: 9 March 1998     

Post-streptococcal reactive arthritis in children: a distinct entity from acute rheumatic fever

There is a debate whether post-streptococcal reactive arthritis (PSRA) is a separate entity or a condition on the spectrum of acute rheumatic fever (ARF). We believe that PSRA is a distinct entity and in this paper we review the substantial differences between PSRA and ARF. We show how the demographic, clinical, genetic and treatment characteristics of PSRA differ from ARF. We review diagnostic criteria and regression formulas that attempt to classify patients with PSRA as opposed to ARF. The important implication of these findings may relate to the issue of prophylactic antibiotics after PSRA. However, future trials will be necessary to conclusively answer that question.     

Post-streptococcal reactive arthritis in children: a distinct entity from acute rheumatic fever

Inflammatory myositis is reported in 4-16% of adult systemic lupus erythematosus (SLE) patients. The aim of this study was to determine the prevalence of myositis in a cohort of pediatric SLE patients in the southeastern United States. A retrospective chart review was performed of 55 SLE patients evaluated by Pediatric Rheumatologists in Alabama since January 1, 2008. Patients were defined as having myositis if they satisfied one of the following categories: 1) Proximal muscle weakness on exam with lower extremity muscle edema on MRI; 2) Proximal muscle weakness with elevation in CK, AST, aldolase, or LDH muscle enzymes; or 3) Patient reported weakness or muscle pain and an elevated CK. Inflammatory myositis was present as a feature of SLE in 31% (n = 17) with a 95% confidence interval of 19-45%, statistically different from the reported rates of 4-16% (p < 0.0001). Myositis was positively associated with the presence of anti-ribonucleoprotein antibodies (p = 0.009). Negative associations with myositis were the presence of anti-double stranded DNA antibodies (p = 0.02) and hematologic disorders (p = 0.02). Thus, in the state of Alabama, pediatric SLE myositis is present at a statistically higher rate than previously published values of adult SLE myositis, possibly reflecting geographic (genetic or environmental) and/or age-of-onset related influence(s).     

Recurrent abdominal pain in children in developing countries frequently has an organic basis

A total of 1,602 children with recurrent abdominal pain (RAP) were admitted to the Department of Paediatric Surgery at Safdarjang Hospital, New Delhi, India during the period August 1974 – December 1990. The children included in the study had had three or more episodes of pain persisting for more than 3 weeks. Major causes were worm infestation, abdominal tuberculosis, giardiasis, amoebic colitis, and urinary tract lesions (67.1%). Organic lesions were identified in 89.5% of patients, and only in 18 cases was RAP attributed to psychogenic factors.     

Mesenteric lymphadenopathy in children examined by US for chronic and/or recurrent abdominal pain

Background Children with recurrent abdominal pain often undergo US to confirm or exclude organic disease.Objective To assess the prevalence of mesenteric lymphadenopathy on US in these children.Materials and methods We prospectively studied 189 children with recurrent abdominal pain with US of the abdomen, using graded compression. The results were compared with 73 children in a control group. The children in both groups were divided into three age groups. The size, number, morphology and location of mesenteric lymph nodes were noted, as well as additional findings. Pediatricians followed the patients from 3 months to 1 year, and a repeat US study was done in 30 children.Results Mesenteric lymphadenopathy was present in 116 of 189 children (61.4%), with the greatest prevalence in boys in the younger age groups. The location of the nodes was mainly in the right lower quadrant. In the control group, 7 of 73 children had mesenteric lymphadenopathy, a significantly lower prevalence than in the study group (P<0.001). Additional findings, apart from lymphadenopathy, were present in 27 (14.2%) of the 189 children in the study group, and in 5 (6.8%) of the 73 children in the control group.Conclusion Mesenteric lymphadenopathy is a common, and often the only abnormal, finding on US in children with recurrent abdominal pain.The Chaim Sheba Medical Center, Tel Hashomer, is affiliated with the Sackler Faculty of Medicine, Tel Aviv University     

Paediatric haematohidrosis: an overview of a rare but clinically distinct condition

Haematohidrosis is a rare and dramatic condition in which bleeding occurs spontaneously from intact skin. We report the case of a nine‐year‐old boy with a typical clinical presentation. The case highlights how challenging it can be for medical professionals to recognise and evaluate rare conditions. A review of the literature was performed, showing that haematohidrosis is mainly a paediatric condition. Our case together with findings from the review indicates that treatment with Beta blockers may be effective for treatment of haematohidrosis in children. Conclusion: Paediatric haematohidrosis is a rare, but clinically distinct condition. Treatment with Beta blockers may be tested.     

Pathologic childhood aerophagy: an under-diagnosed entity

Three children with pathologic childhood aerophagy are described. This entity is characterized by progressive abdominal distension during the day, non-distended abdomen in the morning, and visible air swallowing. The condition is usually self-limited, and treatment is symptomatic and by reassurance. Early recognition and diagnosis of this condition might help avoid unnecessary and expensive diagnostic investigations.     

Autosomal recessive hyperimmunoglobulin E syndrome: a distinct disease entity

OBJECTIVE: The autosomal-dominant form of the hyperimmunoglobulin E syndrome (AD-HIES) has been described as a multisystem disorder including immune, skeletal, and dental abnormalities. Variants of AD-HIES are known but not well defined. METHODS: We evaluated 13 human immunodeficiency virus-seronegative patients from six consanguineous families with an autosomal-recessive form of hyperimmunoglobulin E syndrome (AR-HIES) and 68 of their relatives. RESULTS: Persons affected with AR-HIES presented with the classical immunologic findings of hyperimmunoglobulin E syndrome, including recurrent staphylococcal infections of the skin and respiratory tract, eczema, elevated serum immunoglobulin E, and hypereosinophilia. In addition, severe recurrent fungal and viral infections with molluscum contagiosum, herpes zoster, and herpes simplex were noted. Autoimmunity was seen in two patients. Central nervous system sequelae, including hemiplegia, ischemic infarction, and subarachnoid hemorrhages, were common and contributed to high mortality. Notably, patients with AR-HIES did not have skeletal or dental abnormalities and did not develop pneumatoceles, as seen in AD-HIES. In lymphocyte proliferation assays, patients' cells responded poorly to mitogens and failed to proliferate in response to antigens, despite the presence of normal numbers of lymphocyte subpopulations. CONCLUSION: The autosomal-recessive form of hyperimmunoglobulin E syndrome is a primary immunodeficiency with elevated immunoglobulin E, eosinophilia, vasculitis, autoimmunity, central nervous system symptoms, and high mortality. AR-HIES lacks several of the key findings of AD-HIES and therefore represents a different, previously unrecognized disease entity.     

Urinary enzyme excretion patterns in children

The need for a sensitive marker of renal parenchymal damage in children remains extant. The detection of renal scarring is presently dependant upon imaging modalities with the implicit problems of exposure to radiological investigation. We describe and evaluate urinary enzymology as a technique that may identify injury to the renal parenchyma from a variety of patho-physiological conditions.     

Laparoscopic gastrostomy in children

During a 30-month period, 28 children aged 6 months-15 years underwent fashioning of a laparoscopic gastrostomy. Indications for operation included: feeding difficulties and failure to thrive in neurologically impaired children (13); chronic renal failure (9); and others (6). There were 17 conventional tube and 11 button gastrostomies. Twelve children had insertion of a gastrostomy alone; the others underwent a concomitant laparoscopic Nissen fundoplication (NFP). The average operation time for gastrostomy alone was 65 min (range 35–104) and for gastrostomy plus NFP 155 min (range 130–246). There were no specific laparoscopic complications. Two patients who required large volumes of eternal drugs and peritoneal dialysis from the 1st post-operative day developed minor external leaks from their stomas. It appears that laparoscopy provides for safe and precise positioning of any standard balloon or button gastrostomy. It is a particularly attractive technique for use in patients already undergoing a laparoscopic fundoplication and those in whom other minimally invasive techniques are contraindicated or fail.     

General lymphangiomatosis in a child

The lymphographic findings in a 4 year old girl with general lymphangiomatosis are presented. The number of iliac and lumbar lymphatics had increased markedly and the lymph nodes were enlarged with very loose structure and with contrast medium distributed in scattered droplets and filiform channels.