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Biomedical subjects

H Aiges

Publications and source records attributed to H Aiges.

At least 19 recordsLinked to original sources
PubMed1995

Highly destructive perianal disease in children with Crohn's disease.

The perianal complications of Crohn's disease (CD) seen in children and adolescents include skin tags, anal fissures, fistulae, and abscesses. While these lesions are often chronic and variably responsive to medical therapy, only rarely are they severely destructive. In this report, we characterize the frequency, severity, and clinical course of a highly destructive form of perianal disease (HDPD) that we have noted in a number of children and adolescents with Crohn's disease. A database containing records from 350 children with inflammatory bowel disease was reviewed to identify all children with CD treated between 1970 and 1993. For each, the occurrence or absence of significant perianal pathology, including fistula, abscess, and HDPD, was determined. Pertinent clinical details were recorded for all patients. In addition, the clinical characteristics of those children with HDPD were compiled, and the courses of those with HDPD characterized. A search of the database identified 230 children and adolescents with CD followed for a total of 1,518 patient years. Sixty-seven of these patients (29% of the CD population) had significant perianal pathology. This included 6 with HDPD, 8 with complicated fistulae [rectourethroperineal (1), rectovaginal (1), rectolabial (2), and multiple communicating perineal (4)], and 53 with simple perianal fistulae or abscesses. All six with HDPD had deeply destructive perineal ulcerations, marked undermining of the perineal and perirectal tissues, and copious exudate, and often there was a deeply cleaved or fileted perineum on separating the buttocks. Two children with HDPD had fecal incontinence.

Abscess↗
PubMed1993

Growth failure in pediatric inflammatory bowel disease.

To assess whether children with inflammatory bowel disease (IBD) develop permanent impairment of linear growth, we analyzed records from 48 young adults who had IBD during childhood or early adolescence (Tanner I-III; 11.8 +/- 2.4 years old at diagnosis). All were fully grown (Tanner V; 21.1 +/- 3.0 years) at last examination. Adult heights were predicted from data obtained at or shortly after the diagnosis of IBD by three methods: height for age percentile, the Bailey-Pinneau (BP), and Roche-Wainer-Thissen (RWT) methods. Predicted adult heights were then compared with the actual ultimate height of each subject. Permanent growth failure occurred in 19-35% of subjects, depending upon the method used to assess growth. Overall, 31% (15 of 48) of the subjects had deficits of adult height identified by two or more methods, including 14 of 38 (37%) of those with Crohn's disease but only one of 10 with ulcerative colitis. Age at diagnosis of IBD, age at last examination, age at cessation of linear growth, and site of IBD did not differ between impaired and normal growth groups. Duration of corticosteroid use was longer (p < 0.05) in growth-impaired subjects. In addition, although 60% of all subjects had periods of poor growth that put them in height-for-age percentiles two or more major growth channels below previous percentiles, only 19% remained at these levels upon achieving their final adult heights. Permanent impairment of linear growth leading to clinically meaningful deficits of ultimate adult height is common in patients with IBD in childhood or early adolescence. New therapeutic approaches are needed to address this problem.

Adolescent↗
PubMed1990

Long-term 6-mercaptopurine treatment in adolescents with Crohn's disease.

Although 6-mercaptopurine is often used to treat adolescents with intractable Crohn's disease, its long-term efficacy has not yet been studied in this population. This study shows data derived from 36 adolescents (mean age +/- SD, 16.5 +/- 3.3 years; 27 males, 9 females) treated at least 6 months with 6-mercaptopurine (1.5 mg.kg-1.day-1, maximum of 75 mg/day). Sites of Crohn's disease at the start of 6-mercaptopurine therapy included 17 ileocolic, 9 pancolic, 7 small bowel, and 3 partial colon. All had received corticosteroids, sulfasalazine, antibiotics, and nutritional support for 5.0 +/- 3.0 years before administering 6-mercaptopurine, but intractable symptoms persisted. Disease activity lessened during the first year of 6-mercaptopurine, reflected by a higher Lloyd-Still disease activity score (pre, 64 +/- 9 vs. 6-mercaptopurine, 72 +/- 11; P less than 0.0001). General activity, physical examination, nutrition, and laboratory subscores all improved (P less than 0.004). Lessened disease activity occurred despite concomitant decrease in duration of prednisone use (pre, 9.5 +/- 4.2 vs. 6-mercaptopurine, 6.6 +/- 4.9 months/year; P less than 0.001) and cumulative annual prednisone exposure (pre, 3672 +/- 2106 vs. 6-mercaptopurine, 1964 +/- 1460 mg; P less than 0.0007). The frequency of perianal fistulae and abscesses also decreased (P less than 0.01) during treatment. Annual rates of hospitalization decreased in 44% of subjects during 6-mercaptopurine treatment, while increasing in only 22%. Follow-up beyond 1 year of 6-mercaptopurine treatment showed continued remission in 23 of 30 subjects. No serious complications were seen. 6-mercaptopurine is an effective long-term therapy for adolescents with intractable Crohn's disease. While inducing remission, it also has a significant steroid-sparing effect which may be of particular benefit to this population.

Abscess↗
PubMed1989

Human ontogeny of the bile duct to portal space ratio.

Paucity of interlobular bile ducts is a common feature of cholestatic liver disease in premature infants. Whereas a bile duct to portal space ratio of 0.9 to 1.8 is cited by Alagille as the norm for children, there are no data regarding the normal bile duct to portal space ratio in preterm infants. In this study, by examining liver tissue obtained from autopsied fetuses and infants, we have determined that the bile duct to portal space ratio increases as a function of postconceptional age. After 38 weeks postconception, a ratio equal to or greater than 0.9 was seen in eight of nine subjects. Therefore, in evaluating premature infants for duct paucity, liver biopsies should be obtained only after 38 weeks. Prior to 38 weeks postconception, a bile duct to portal space ratio less than 0.9 may be physiologic.

Bile Ducts, Intrahepatic↗
PubMed1989

Home nocturnal supplemental nasogastric feedings in growth-retarded adolescents with Crohn's disease.

We hypothesized that supplemental nutritional support delivered at home by nocturnal nasogastric feedings would result in accelerated growth in growth-retarded adolescents with Crohn's disease. Eight Tanner stage I adolescents with Crohn's disease, mean age 14 yr 5 mo, had a mean weight gain of 0.38 kg and height gain of 1.4 cm for the year before initiation of nasogastric feedings. All had been either asymptomatic or had only minimal symptoms in the year before the study, but were ingesting only 55%-80% of their daily required caloric intake. The subjects were taught to pass by themselves a nasogastric feeding tube, through which 1000-1500 ml of commercial, nonelemental isocaloric formula was infused during sleep to supplement their usual dietary intake. After 12 mo of nocturnal feedings, the subjects had a mean weight gain of 11.75 kg and a mean height gain of 6.98 cm. Six control subjects, matched for age and degree of growth and sexual retardation at the beginning of the study period, but who had refused the nasogastric feedings, had no change in weight and height during the same period of observation. We conclude that home nocturnal nasogastric feedings can achieve dramatic improvement in weight gain and linear growth in motivated adolescents with Crohn's disease and growth retardation.

Adolescent↗
PubMed1989

Does proctosigmoiditis in inflammatory bowel disease presage the imminent onset of symptoms?

This study was undertaken to determine if asymptomatic children and adolescents with inflammatory bowel disease and moderate to severe anorectosigmoid inflammation might remain symptom-free for at least 12 months without specific intrarectal therapy. We prospectively studied 13 asymptomatic patients 6-21 years of age (four with Crohn's disease and nine with nonspecific colitis) with previously documented anorectosigmoid inflammation. Of these 13, four had moderate to severe anorectosigmoid inflammation both endoscopically and histologically. These four patients (two with Crohn's disease and two with nonspecific colitis) were entered into the second phase of the study. Three were receiving sulfasalazine, and one received methylprednisolone, 4 mg/day, and 6-mercaptopurine, 50 mg/day. None received intrarectal therapy. Clinical evaluation revealed that all four remained asymptomatic for 12 months despite the continued presence of moderate to severe anorectosigmoid inflammation. These results indicate that in children and adolescents with inflammatory bowel disease, the presence of inflammation of the anorectosigmoid does not necessarily correlate with or presage the onset of symptoms of proctosigmoiditis. Therefore, active inflammation of the anorectosigmoid is not the sole prerequisite for intrarectal therapy. The clinician should be guided by the symptoms of the patient, not by the presence or absence of active anorectosigmoid inflammation.

Adolescent↗
PubMed1989

Amyloidosis in children with inflammatory bowel disease.

Reactive systemic or secondary amyloidosis occurs in 1-29% of adults with Crohn's disease, but only sporadic cases of amyloidosis have been recognized in children with inflammatory bowel disease. We therefore have studied operative specimens (ileal, ileocolonic, and colonic) from 46 children (30 with Crohn's disease and 16 with ulcerative colitis) to determine the frequency of amyloid deposits. Sections of bowel, skin, and lymph nodes (n = 940) were stained by Congo red and examined by light microscopy and by polarized light. Amyloid deposits were found in only one of 46 subjects, an 18-year-old girl who had had Crohn's disease for 6 years. Intestinal amyloid deposits, present 16 months before the clinical diagnosis of amyloidosis, were patchy and seen predominantly in the intestinal mucosa. We conclude that amyloidosis is rare in children requiring surgery for Crohn's disease and ulcerative colitis. Examination of Congo red-stained sections can detect even subclinical amyloidosis. The amyloid deposits in our patient, which were both patchy and consistently mucosal, suggest that multiple endoscopic biopsy samples, not necessarily containing submucosa, are sufficient for diagnosis.

Adolescent↗
PubMed1985

Linear growth following surgery in children and adolescents with Crohn's disease: relationship to pubertal status.

Studies of the effect of surgery on growth failure in adolescents with Crohn's disease have revealed conflicting data. To better determine the role of surgery for growth delay, growth data from 26 patients with Crohn's disease with intestinal resections and/or ileostomies were reviewed, 3 of whom had surgery twice. Operations were performed on 14 Tanner Stage I, 1 Tanner II, 1 Tanner III, and 13 Tanner IV or V patients. In the prepubertal group, 13 of the 14 had growth impairment, only one of whom had surgery primarily for that growth failure. One year after operation, 11 of 13 Tanner I growth failure patients experienced an increase in height velocity of 5.38 +/- 1.18 cm/yr (mean +/- SE;P less than 0.01); 9/11 achieved normal height velocity for Tanner I. Two attained their preillness height percentiles at one year follow-up, while 5 patients attained their preillness height percentiles 2.5 to 10 years following surgery. Of the four who failed to achieve normal height velocity, 3 had early recurrence of active disease. The Tanner Stage II and III patients both had growth failure, and both had a growth spurt following surgery. Of those who were Tanner Stage IV or V at the time of surgery, 5 of 13 had growth failure. Following surgery, none had an increase in height velocity. These data suggest that when patients with Crohn's disease and growth failure are prepubertal and surgery is performed primarily because of failure of medical therapy and/or other complications, a postoperative growth spurt may be expected within one year.

Adolescent↗
PubMed1985

Cholescintigraphic abnormality in a case of Kawasaki syndrome.

A 26-month-old girl with Kawasaki syndrome (mucocutaneous lymph node syndrome) is presented. Liver function studies were abnormal and sonographic examination revealed hydrops of the gallbladder. The Tc-99m DISIDA cholescintigraphy demonstrated both early and delayed nonvisualization of the gallbladder. A photopenic area was noted in and below the gallbladder fossa and there was medial and upward displacement of the common bile duct. It appears that Kawasaki syndrome may result in nonvisualization of the gallbladder by cholescintigraphy. Accordingly, this diagnosis should be added to the list of conditions associated with nonvisualization of the gallbladder by biliary scintigraphy.

Child, Preschool↗
PubMed1985

Sulfasalazine and renal tubular function: lack of an effect.

Sulfasalazine (SASP) is frequently used in the treatment of chronic inflammatory bowel disease (IBD), particularly colitis. Because the drug poses a theoretical risk for renal tubular damage, 26 patients, 8-18 years of age, with Crohn's ileocolitis were studied. Thirteen children were receiving SASP while 13 served as disease controls. Renal tubular function was assessed by measurement of urinary beta 2-microglobulin and n-acetylglucosaminidase activity. No abnormalities were found on routine measurement of renal function. Similarly, urinary beta 2-microglobulin and n-acetylglucosaminidase activities were within normal limits for patients receiving SASP, as well as for disease controls. Although there is a theoretical risk for renal tubular damage from the prolonged use of SASP, this study would suggest that IBD patients receiving the drug are at no greater risk for renal injury than their counterparts not receiving the medication.

Adolescent↗
PubMed1984

Perianal disease in children and adolescents with Crohn's disease.

The clinical and pathological features of perianal disease in children and adolescents with Crohn's disease have been delineated. Of the 149 patients (mean age 12.1 yr, range 0.75-21 yr, 68% male), 73 (49%) had perianal disease including 51 with fissures and tags, 10 with fistulas, and 12 with abscesses. Ileocolitis was seen in 68% of all patients and in 75% of patients with perianal disease (p greater than 0.10). Rectal inflammation was present in 94% of patients with fistulas or abscesses, or both, versus 63% of all patients without perianal disease (p less than 0.025). Anorectal granulomata were found in 47% of fistula/abscess patients versus 9% of non-perianal disease patients (p less than 0.05). A variety of medical regimens failed to consistently affect these lesions favorably. Four of 10 patients with fistulas healed after 1-5 yr of intermittent discharge. Nine of 12 patients with abscesses required surgical drainage, and 50% subsequently developed chronic fistulas. Granulomata were equally distributed between those patients who healed and those who developed chronic fistulas. Although an important cause of morbidity in childhood Crohn's disease, perianal disease is not progressively destructive in the majority of patients.

Abscess↗
PubMed1983

Urethroperineal-rectal fistula in Crohn's disease.

A 19-year-old white male with Crohn's disease, who complained of passing urine per rectum and having retrograde ejaculations, was noted to have a urethroperineal-rectal fistula. The fistulous communication remained patent despite pharmacologic therapy, a diversion ileostomy, and a total proctocolectomy. A fistulectomy and definitive urethral repair finally resulted in resolution of the problem.

Adult↗
PubMed1982

Progressive esophageal dysfunction in chronic granulomatous disease.

Chronic granulomatous disease of childhood (CGD), a hereditary disorder of neutrophil function, affects the gastrointestinal tract in a variety of ways. Esophageal involvement has only rarely been reported. An 11-year-old boy with CGD and progressive esophageal dysmotility is described. Repeated radiographic, endoscopic, and motility studies revealed a markedly atonic esophagus with varying function of the lower esophageal sphincter. Pharmacologic therapy and esophageal dilatations were unsuccessful in establishing adequate esophageal function. A feeding gastrostomy was required for nutritional support.

Child↗