Chronic fibrosing pancreatitis in childhood: report of a case and literature review.

Chronic fibrosing pancreatitis in childhood is an uncommon condition of unknown etiology with a variety of clinical presentations, histopathologic features, and outcomes. The diagnosis is one of exclusion (of hereditary or secondary pancreatitis), which should include histological assessment. The histological features of this condition have been described, to our knowledge, in nine published cases. We report a case in a 13-year-old male, who presented with obstructive jaundice and subsequently had evidence of endocrine and exocrine pancreatic insufficiency, despite a surgical decompression of the pancreatic-biliary duct system.

Lipase and pepsin activity in the gastric mucosa of infants, children, and adults.

The range of activity and the location of lipase and pepsin were determined in the stomach and duodenum of infants, children, and adults. The range of lipase activity in biopsy specimens from the gastric body, in 29 subjects aged from 3 months to 26 years, was 1.8-5.3 U/mg protein (1 U is 1 mumol [3H]oleic acid released from tri-[3H]olein per minute). There were no significant differences among age groups (5-19 months, 2-4 years, 6-10 years, 11-13 years, and 15-26 years). Lipase activity was low or undetectable in the gastric antrum of all subjects. Pepsin activity in specimens from the gastric body ranged from 180 to 780 pepsin units/mg protein (using hemoglobin as substrate). The antrum had significantly lower pepsin activity (P less than 0.001) than the gastric body. As with lipase activity, there were no statistically significant differences in pepsin activity among age groups. Lipase and pepsin activity was also quantified in pinch biopsy specimens from the duodenum and duodenal bulb in 13 subjects. Contrary to lipase activity, which was almost completely absent from the duodenum or duodenal bulb, these sites contained low pepsin activity (9-78 pepsin units/mg protein). The data show that in infants and children, as previously reported in adults, gastric lipase is localized primarily in the gastric body. Tissue pepsin levels and localization, reported here for the first time, are similar to those of lipase, although, contrary to lipase, the gastric antrum has considerable pepsin activity. The identical levels of lipase and pepsin activities in infants, children, and adults indicate that the gastric phase of nutrient digestion is well developed at birth.

Intestinal lymphonodular hyperplasia of childhood: patterns of presentation.

In this retrospective analysis we searched for a constellation of signs or symptoms attributable to childhood lymphonodular hyperplasia (LNH). Of 147 children with documented LNH reviewed, 43% had lesions in the small bowel, and 57% in the large bowel. Children in this study presented with complaints of abdominal pain (58%) and bright red blood per rectum (32%). Physical examination revealed little except right lower quadrant (RLQ) abdominal tenderness and "fullness" in 35%. The pain was periumbilical, dull-cramping, rarely acute, and nonradiating. The hematochezia was most commonly streaky red in mucoid strands adhering to the stools, with no associated tenesmus. Three clinical patterns emerged: (a) Under 1 year of age most patients were male, with painless bleeding and pancolonic LNH. (b) Between 2 and 6 years, although the LNH was predominantly colonic, pain and bleeding occurred equally. (c) From 7 years old on, the main symptom was abdominal pain, but LNH distribution was nearly equal between the small bowel and the colon. To date, our long-term follow-up of the children with isolated LNH has revealed no sequelae.

Gastroesophageal reflux in infants.

Clinically significant gastroesophageal reflux occurs in one in 500 infants. Symptoms resolve in 60 to 80 percent of infants by 18 months of age. Thickened formula and position changes are often recommended. Medication and surgical intervention may be needed in refractory cases.

Colic.

Colic begins between the second and sixth week of life and usually abates by the fourth month of life. Crying may occur any time of the day but is most common after the evening feeding. Removal of cow's-milk protein from the diet eliminates colic in 30 percent of infants. Simethicone and an antispasmodic agent are sometimes helpful.

Benign elevation of serum alkaline phosphatase, transient and persistent variety.

Markedly increased serum concentration of alkaline phosphatase (AP) was discovered in seven children. Investigation showed the finding to be benign in each case. Family survey and follow-up studies revealed a familial pattern in four children and a transient, idiopathic origin in the other three. Awareness of these benign forms of hyperphosphatasemia will aid the physician in the interpretation of elevated AP.

Eosinophilic gastroenteritis.

A 4-month-old boy presented with signs of intermittent pyloric obstruction. Peripheral eosinophilia, along with radiographs showing antral-pyloric and duodenal narrowing, suggested the diagnosis of eosinophilic gastroenteritis. A brief discussion of the disease process follows.

Neuropathology, pathogenesis, and neuropsychiatric sequelae of Reye syndrome.

A clinico-pathologic correlation can be established for those survivors of Reye syndrome in the higher clinical stages who have sustained irreversible and often major neuropsychiatric deficits. The neuropathologic substrate for the clinical manifestations of mental retardation, recurrent seizures, movement disorders, sensori-motor deficits, psychobiologic maladjustment, and mediocre performance in school and on formal psychometric tests consists of multifocal infarction and astrocytosis in cerebral cortex, diencephalon, basal ganglia, and brainstem. While the mortality rate of Reye syndrome in acute stages is still significantly high (25% in our 20 cases, 52% in the tabulated cases in the higher clinical grades), the occurrence of permanent psychomotor deficits is an equally tragic socioeconomic and medical problem. Of our 15 survivors, 3 have suffered major and 1 has sustained minor brain damage. From a review of the literature, over one-third of survivors are consigned to cerebral malfunction, hence their potential for full enjoyment of life and their intellectual, emotional, and economic contributions to their families and society are sadly curtailed.

The syndrome of ring chromosome 12.

We have studied a 13 month-old girl with failure to thrive developmental delay, and dysmorphic features. At 13 months, the weight-age was 1 month, length-age was 3 months and head circumference was at the 3rd centile for 3 months. Physical findings were: Epicanthal folds, mildly cupped, apparently low-set ears, highly arched palate, short neck with low hairline, clinodactyly, and single crease of left 5th finger. The modal chromosome number was 46. Trypsin-G banding identified a ring chromosome 12; Karyotype was 46,XX,r(12)(p13q24).

Asymptomatic viral hepatitis types A and B in an adolescent population.

Sera from 95 adolescents were examined for markers of hepatitis B virus (HBV) infection and hepatitis A virus (HAV) infection. HBV markers were found in eight adolescents (8%) and evidence of previous HAV infection was found in 18 adolescents (19%); none had a history of clinically recognizable hepatitis. These findings support the growing evidence that HBV and HAV infections are diseases of the pediatric age group, and that testing of HBV vaccines when they become available for patient use will have to include a pediatric population.