Peptic ulcer disease in children and adolescents. A ten-year experience

We report a retrospective review of peptic ulcer disease (PUD) in children seen at Children's Hospital of Michigan, over 10 years (1971-1980). Of the 61 included, 31 had primary ulcers and 30 had stress ulcers. The diagnosis was made by either barium meal or endoscopy, at surgery, or at autopsy. Stress ulcers were commoner in children less than 4 years of age and were precipitated by various conditions leading to shock. The majority of these patients required surgery for perforation or bleeding. Primary ulcers were more common in children older than 4 years. The most common presenting symptoms in this group were abdominal pain and gastrointestinal bleeding. The majority of patients with primary PUD received medical therapy in the form of antacids and/or cimetidine. In one-third of these patients, surgery was necessary for complications such as intractable pain, perforation, or massive recurrent hemorrhage.     

Post-radiotherapy apparent diffusion coefficient (ADC) in children and young adults with high-grade gliomas and diffuse intrinsic pontine gliomas

Objectives: Diffusion-weighted magnetic resonance imaging (DW-MRI) offers potential to monitor response and predict survival in high-grade gliomas (HGG) and diffuse intrinsic pontine gliomas (DIPG). We hypothesized that post-radiotherapy DW-MRI may provide prognostic imaging biomarkers in children and young adults with these tumors. Methods: Patients aged ≤21?years diagnosed between 2005 and 2012 were eligible. The tumor median apparent diffusion coefficient (ADC) and its 5th percentile (C5-ADC) were determined at the first post-radiotherapy scan and at the time of radiological progression. DW-MRI parameters were correlated with survival endpoints, temozolomide use and pseudoprogression, when it occurred. Results: Out of 40 patients (20 HGG, 20 DIPG), 23 had evaluable DW-MRI post-radiotherapy and 25 at radiological progression. There were 6 episodes of pseudoprogression. Hazard ratios (95%CI) for progression-free survival were 0.998 (0.993–1.003) for median ADC and 1.003 (0.996–1.010) for C5-ADC. Hazard ratios (95%CI) for overall survival were 1.0009 (0.996–1.006) for median ADC and 0.998 (0.992–1.004) for C5-ADC. Post-radiotherapy median and C5-ADC values were not significantly different between patients treated with radiotherapy alone versus radiotherapy/temozolomide. The median and C5-ADC values were not significantly different at the time of pseudoprogression compared to those at tumor progression. Conclusions: Post-radiotherapy median ADC and C5-ADC were not prognostic, nor able to differentiate radiosensitization with temozolomide or occurrence of pseudoprogression in this cohort of HGG and DIPG patients. Further exploration of alternative DW parameters, study timepoints or data modeling may contribute to the development of prognostic/predictive imaging biomarkers for children and young adults with HGG or DIPG.     

Intracranial metastasis of Wilms' tumor involving the tectal plate without pulmonary involvement. Case report.

Intracranial metastasis without pulmonary involvement of Wilms' tumor is very rare, and most previously reported metastatic sites have been in the cerebral parenchyma. We experienced a rare case of metastasis of Wilms' tumor in the tectal plate without pulmonary involvement. A 3-month-old boy was admitted to our hospital due to hydrocephalus. After insertion of a ventriculoperitoneal shunt, there were no neurological deficits. Five months after the operation, a mass 5 cm in diameter extending from the tectal plate into the third ventricle was found. A mass 10 cm in diameter within the retroperitoneal space, which occupied the right kidney, was also found. Both tumors were resected and diagnosed histologically as Wilms' tumor. Despite every effort including chemotherapy and radiotherapy, the patient died at 14 months old. The majority of children with hydrocephalus showing thickening of the tectal plate would most likely have tectal glioma; however, this unusual case report reminds us that metastasis of Wilms' tumor can occur in the tectal plate causing hydrocephalus.     

Management of status dystonicus in children. Cases report and review

Status dystonicus (SD) is a medical emergency weighed by a relevant morbidity and mortality. It mainly affects patients with primary or secondary dystonia and is often triggered by events such as fever, infections, exposure medications or their abrupt cessation. We report on three patients presenting with SD. Two of them were affected by a static encephalopathy and the other one by a neurodegenerative disorder such as megalencephalic leukoencephalopathy with subcortical cysts (MLC). To our knowledge this is the first patient affected by MLC presenting with SD. All our patients underwent continuous infusion of midazolam, in association with pimozide and trihexyphenidyl, which led to complete resolution of muscular spasms in two patients. In the other one a complete cessation of dystonic spasms was obtained after intrathecal baclofen. From a therapeutic point of view there are no evidence-based management guidelines in SD. The approach is empiric and based on very limited anecdotal reports. On the basis of our observations and an extensive review of the literature we delineated a possible therapeutic strategy of SD in children.     

A ten-year review of neonatal sepsis and comparison with the previous fifty-year experience

Records have been kept prospectively in our institution since 1928 of all positive blood cultures taken from neonates. Using a modification of objective Centers for Disease Control criteria to define sepsis, we reviewed the records of all neonates with positive blood cultures for the years 1979 to 1988 inclusive and found 270 cases of sepsis. The sepsis rate for infants less than or equal to 30 days of age was 2.7 cases/1000 live births, with a mortality rate from sepsis of 15.9%. There was an increase in sepsis due to commensal species (CS) over the period (P less than 0.007). The number of infants in the nursery who developed sepsis when more than 30 days of age also increased (P less than 0.002), as did the rate of sepsis from CS in this group (P less than 0.001). Isolation of CS from the blood with fulfillment of the modified Centers for Disease Control criteria was associated with a 13.7% mortality rate, whereas isolation of CS without fulfillment was associated with a 4% rate (P less than 0.01).     

Primary omental torsion in children: ten-year experience

Primary omental torsion (POT), is a rare cause of acute abdomen commonly affecting obese male adults, whereas it is extremely rare in children. In this retrospective study, we present our experience regarding the management of five children with POT and discuss the diagnostic and therapeutic implications of this entity. We retrospectively reviewed the medical records of children diagnosed for POT, from January 1996 to July 2006 at our department. Among 2,734 children operated for acute appendicitis, five patients were diagnosed with POT (ratio 1:587 or 0.18%). Clinical presentation, laboratory findings, diagnostic imaging results as well as surgical and histological findings were reviewed. There were four boys and one girl, M/F ratio 4:1, with a mean age of 9.5 years (range 7.2-10.3). All subjects were obese and their weight percentages were over 85% for their age group. On admission the clinical symptoms and laboratory findings were similar to those of acute appendicitis. They were submitted to laparotomy and the surgical findings were; free serosanguineous fluid in the peritoneal cavity, normal appendix and an ischemic twisted mass of the omentum at the right side of the abdomen. The mass and the appendix were excised and the postoperative course was uneventful. The histological examination of the specimens revealed hemorrhagic ischemic necrosis of the omentum and normal appendix. POT is very rare in children. In the pediatric age group the clinical presentation and the laboratory findings are similar to those of acute appendicitis and it is extremely difficult to be diagnosed preoperatively. Obesity seems to be an important predisposing high-risk factor. Excision of the twisted omentum is the treatment of choice.     

Miliary tuberculosis in children. A clinical review

Eighty cases of miliary tuberculosis admitted to our hospital between January 1981 and December 1984 were reviewed. The age of the patients ranged from 3 months to 12 years, with an average of 2 years 2 months (26.5 months). Nine cases (11.25%) died during hospitalization due to the severe condition at the time of admission. Only 8 patients (10%) were in good nutritional condition. Seventy-two patients (90%) had been visiting the primary health care clinic for several times since 2-3 months but were never diagnosed as suffering from tuberculosis. Fever or recurrent fever were found in 78 cases (97.5%), anorexia in 65 cases (81.3%), chronic and/or recurrent cough in 72 cases (90%) and malaise in 43 (53.8%). Forty-one (51.3%) denied the presence of a close contact with source of infection. Hepatomegaly was found in 44 cases (55%), 19 (23.8%) of which were associated with splenomegaly. Choroidal tubercle was found in 4 cases; 1 case with coxitis, 1 with brain tuberculoma, 1 with ascites, 1 with endobronchitis and 1 with hepatitis. Forty-three (53.8%) were tuberculin negatives, 24 of which become positives after treatment. Fourteen cases had BCG scar. History of measles was found in 21 cases. Children with longterm and recurrent fever, anorexia, decrease of body weight and recurrent cough should be suspected of having TB thus enabling to get an early diagnosis.     

Hormonal findings in obese children. A review

Serum thyroid hormones in childhood obesity are not altered but caloric intake affects monoiodination of T4 to T3 and rT3. Plasma cortisol and urinary free cortisol concentrations are normal. Increase in cortisol production and secretion rate is reflected in increased values of urinary 17-hydroxycorticosteroids. Elevated urinary 17-ketosteroids are caused by increased androgen synthesis accounting for the increased height velocity in obese preadolescents and for the accelerated skeletal maturation. In both sexes earlier onset of puberty is noticed without remarkable alterations in gonadal steroids. Whether altered prolactin concentrations reflect neuroendocrine abnormalities remains unclear. Impairment of growth hormone release in face of normal or high somatomedins is not of clinical significance. Basal and stimulated insulin concentrations are high. Insulin resistance exists because glucose tolerance is simultaneously impaired. This is due to reduction in insulin receptor numbers and post-receptor defects in insulin action. Weight loss is effective in normalizing the above mentioned hormonal defects.     

Acute phenolphthalein ingestion in children. A retrospective review.

The Patient Management Exchange for this edition of the Journal features a research study on phenolphthalein ingestion done at the Pittsburgh Poison Center. The purpose of selecting this article was twofold. First, the material presented about the management of phenolphthalein ingestion in children is valuable information for health care practitioners working in ambulatory settings. Second, this work is an excellent illustration of how research can be incorporated into one's clinical practice. This study also demonstrates that research does not always involve a long and cumbersome process. Instead, research can evolve from the need of health care professionals to answer a simple question that is raised about a particular aspect of their patient practice.     

Diffuse intrinsic pontine tumors: a study of primitive neuroectodermal tumors versus the more common diffuse intrinsic pontine gliomas

Object The diagnosis of diffuse pontine tumors has largely been made on the basis of MRI since the early 1990s. In cases of tumors considered "typical," as a rule, no biopsy specimen has been obtained, and the tumors have been considered diffuse intrinsic pontine gliomas (DIPGs). There have been sporadic reports that primitive neuroectodermal tumors (PNETs) of the pons may not be distinguishable from the DIPGs by radiological imaging. This study presents 2 cases of diffuse pontine PNETs with molecular evidence that these are indeed PNETs, distinct from DIPGs, thus supporting biopsy of diffuse pontine tumors as a standard of care. Methods Biopsy specimens were obtained from 7 diffuse pontine tumors and snap frozen. Two of these 7 tumors were identified on the basis of pathological examination as PNETs. All 7 of the diffuse pontine tumors were analyzed for gene expression using the Affymetrix HG-U133 Plus 2.0 GeneChip microarray. Gene expression was compared with that of supratentorial PNETs, medulloblastomas, and low- and high-grade gliomas outside the brainstem. Results Unsupervised hierarchical clustering analysis of gene expression demonstrated that pontine PNETs are most closely related to PNETs of the supratentorial region and not with gliomas. They do not cluster with the 5 DIPGs in the study. Thirty-eight genes, including GATA3, are uniquely differentially expressed in pontine PNETs compared with other types of pediatric brain tumors, including DIPGs and other PNETs at a false discovery rate statistical significance of less than 0.05. Conclusions The cluster and individual gene expression analyses indicate that pontine PNETs are intrinsically different from DIPGs. The 2 pontine PNET cases cluster with supratentorial PNETs, rather than with DIPGs, suggesting that these tumors should be treated with a PNET regimen, not with DIPG therapy. Since diagnosis by imaging is not reliable and the biology of the tumors is disparate, a biopsy should be performed to enable accurate diagnosis and direct potentially more effective treatments.     

Post-dural puncture headaches in children. A literature review

Post-dural puncture headache (PDPH) is a well recognised complication of spinal and epidural anaesthesia. It can also occur after diagnostic or therapeutic lumbar puncture. Few cases have been reported in children. We reviewed the literature regarding definition, aetiology, incidence, risk factors, prevention and treatment, in order to provide some recommendations. Significant factors include age, gender, needle diameter, needle tip design, orientation of the tip during puncture, previous PDPH, history of migraine and repeated attempts to achieve puncture. There is no evidence for the use of increased fluids or bed rest to prevent PDPH. Once the diagnosis is made, conservative therapy is recommended for 48 h. Persistent PDPH can be treated in several ways; an epidural blood patch is one of the most effective methods. CONCLUSION: occurrence of post-dural puncture headache after lumbar puncture in children is rare. There are conflicting data about risk factors, prevention and treatment.     

Acute pediatric digoxin ingestion. A ten-year experience

A retrospective study conducted in three major pediatric teaching hospitals revealed only 41 cases of acute digoxin ingestion with well-documented serum concentrations. All patients who were symptomatic at presentation (27%) had digoxin concentrations greater than 2 ng/mL (2.6 nmol/L). Only one patient had a transient elevation of the serum potassium concentration. Electrocardiographic (ECG) abnormalities (bradycardia, 1 degree or 2 degrees atrioventricular block, and ST depression) were present in 11 patients. Seven of the 11 patients had ECG abnormalities delayed more than five hours after ingestion. None of these ECG abnormalities were life-threatening. Serum digoxin concentrations ranged from 0.2 to 11.6 ng/mL (0.3 to 14.9 nmol/L). Serum half-lives were rapid (approximately three hours) in an initial phase and longer (approximately 20 hours) in a second phase. Our findings were as follows: acute pediatric digoxin ingestions are not common and are usually not severe; signs and symptoms on presentation predict a digoxin concentration greater than 2 ng/mL (2.6 nmol/L); a correlation between serum potassium and digoxin concentrations was not observed; non-life-threatening bradycardia and conduction disturbances were noted; and a serum digoxin concentration greater than 2 ng/mL (2.6 nmol/L) in the absence of signs or symptoms or ECG abnormalities soon after ingestion does not accurately predict their occurrence later in the course.     

Scorpion sting in children. A review of 51 cases

Scorpion sting in children is a hazardous and potentially lethal condition. Fifty-one infants and children were admitted to the Pediatric Departments at the Hadassah-Hebrew University Hospitals in Jerusalem, during a 5-year period, following scorpion sting. Fifteen (29.4%) had severe systemic signs of envenomation and two (3.9%) died. Analysis of our data showed that patients with severe toxicity were brought to the hospital after a significantly longer time lapse than were the patients with mild-to-moderate symptoms. The current management of children with scorpion envenomation consists of administration of specific antivenom and close surveillance in an intensive care unit, where vital signs and continuous cardiac monitoring enable early initiation of therapy for life-threatening complications, such as cardiac and respiratory failure, convulsions, or hypertension.