Home parenteral nutrition in chronic intestinal diseases: its effect on growth and development

Nine children and adolescents with chronic intestinal diseases and growth failure were treated with home parenteral nutrition (HPN) for 7-63 months. All patients improved their growth velocities on HPN, with an average height gain of 9 cm. The mean growth velocity in six patients with Crohn's disease increased more than fivefold (from 0.67 +/- 0.4 cm/year to 3.8 +/- 1.09 cm/year), and they gained a mean of 11.4 +/- 3.4 kg/year. The three patients without Crohn's disease [intestinal pseudo-obstruction (two), severe short bowel (one)] increased growth velocity from an average of 2.9 +/- 1.1 cm to 6.1 +/- 1.34 cm/year and gained a mean of 10 +/- 2.84 kg/year. Patients without Crohn's disease grew faster on HPN than age comparable patients with Crohn's disease. Also, the two patients with Crohn's disease who were younger and had their disease for a shorter period (less than 3 years) grew better than older patients with Crohn's disease who had their disease for a mean of 8 +/- 1.3 years. All patients improved clinically, and three with Crohn's disease achieved complete remission. Five of the nine patients had psychosocial adjustment problems while on HPN. In conclusion, HPN is a relatively safe and effective means to stimulate growth in children and adolescents who cannot meet nutritional needs by the enteral route.     

Parenteral nutrition in treatment of short stature in adolescents with Crohn disease]

Growth retardation and delayed puberty occur in 20-35% of children and adolescents with Crohn's disease. Alternate day corticosteroid treatment, use of azathioprine, enteral or parenteral hyperalimentation and surgery have been advocated to reverse growth failure. Because of nonacceptance of elemental diet 7 patients with Crohn's disease and growth retardation received parenteral nutrition for 2-3 months (maximal for more than 30 months in one patient). All of them exhibited a mean weight gain of 10 kg and a mean increase of their height velocity from 2.4 to 7.1 cm/year. Main problems were bacterial infections and dislocations of the central lines. Surgery was performed in 3 adolescents immediately after parenteral nutrition. One patient showed a catch-up growth during a 30-months nocturnal home parenteral nutrition at a biological age of 21 years. Parenteral nutrition is an effective regimen to manage growth failure in children with Crohn's disease, but has to be performed for larger periods in individual cases.     

Endocrine dysfunction in children with Crohn's disease

A series of 14 children with Crohn's disease and growth retardation was screened for endocrine dysfunction. Four children presented with growth failure. All had normal cortisol responses to insulin-induced hypoglycaemia. Ten children had normal growth hormone responses in the same test, while four had abnormal responses. Gonadotrophin responsiveness to luteinizing hormone releasing hormone (LHRH) paralleled the clinical evidence of puberty, or its lack. Serum total thyroxine was normal in every patient, while serum total triiodothyronine was low in six; in these six patients serum triiodothyronine normalised with treatment. Serum folate was subnormal in 13 patients and the erythrocyte sedimentation rate was elevated in all at the time of diagnosis. Our results demonstrate a spectrum of endocrine changes seen in children with Crohn's disease, particularly prepubertal gonadotrophin responses to LHRH and a relative preservation of growth hormone and cortisol responsiveness to hypoglycaemia, with a defect in thyroxine to triiodothyronine conversion in severely ill children. Serum folate and sedimentation rate may be useful screening tests for Crohn's disease in a child presenting with failure of growth.     

Crohn's disease: the Melbourne experience

During the years 1979–1988, 63 children (38 male: 25 female) were first diagnosed as having Crohn's disease on the basis of histological findings. Twenty-nine per cent were less than 10 years old at the onset of symptoms; 40% of these cases took more than 1 year to diagnose. Most patients (59%) presented with abdominal pain and diarrhoea, but 40% presented with: a non-specific illness (14%); upper gastro-intestinal tract symptoms (14%); growth failure (6%); systemic disease (5%), or peri-anal disease (1%). There were signs of abdominal tenderness, distension, or an abdominal mass in over 50% of cases. Peri-anal Crohn's disease was present in 41% and a height less than the 10th centile was recorded at diagnosis in 45%. Children were treated with a combination of prednisolone and sulphasalazine. In 16 cases a bowel resection was performed. Surgery was indicated for localised stricture formation causing obstruction and for persistence of severe disease poorly responsive to steroids, particularly in the prepubertal child with growth failure. The extent and distribution of disease influenced outcome. Twenty-four of 39 children followed for more than 3 years are either well-controlled or disease-free at present. Offprint requests to: Spencer W. Beasley     

Nutritional support for pediatric patients with inflammatory bowel disease

Pediatric patients with ulcerative colitis and Crohn's disease often suffer from malnutrition and growth failure. This is particularly true in pubertal children. Chronic insufficient nutrient intake is most often the cause of growth failure. Both parenteral nutrition and defined enteral formulas are available to rehabilitate patients with malnutrition and growth failure. Assessment of nutritional status and growth and the use of parenteral nutrition and defined enteral formulas to reverse malnutrition, growth failure, and inflammation in pediatric patients with inflammatory bowel disease are discussed.     

Outcome of Crohn's disease diagnosed before two years of age

We describe the course of 7 patients younger than 2 years with Crohn's disease. Prolonged remission was achieved medically (5) or surgically (1), whereas one patient died of disseminated adenovirus. Three had malnutrition and growth failure. Crohn's disease very early in life does not always imply a poor prognosis; however, significant morbidity and mortality are encountered.     

Crohn's disease post-cardiac transplantation presenting with severe growth failure and delayed onset of puberty

We report a 15 yr-old girl who 10 yr post-cardiac transplantation presented with severe growth failure and delayed onset of puberty. She was found to have pan-enteric Crohn's disease and has done remarkably well on principally nutritional therapy with a significant growth spurt and the onset of menarche. The development of bowel disease whilst on immunosuppression is rare and the literature is reviewed.     

Diagnostic delay in Crohn's disease

The interval between the onset of symptoms and confirmation of the diagnosis of Crohn's disease in children is often many months or years. A number of factors contribute to this diagnostic delay, but the most significant appear to be delay in the patient seeking initial medical help and failure of the medical practitioner to consider the diagnosis at the time of the first medical contact. In this review of 55 children with Crohn's disease the intervals between onset of symptoms, first presentation and diagnosis were examined and the causes of diagnostic delay determined. The main reason a diagnosis was not made at first presentation was because of ignorance of the fact that Crohn's disease could occur in children. In older children, the combination of tiredness, lethargy and iron deficiency anaemia with vague or intermittent abdominal pain was a frequent cause of diagnostic difficulty. However, even in the absence of major gastrointestinal symptoms, the significance of growth failure and delayed puberty was usually recognised if the patient was referred to a paediatrician. Diagnostic delay could well be reduced if medical practitioners involved in the primary care of children were educated to recognise the various ways in which Crohn's disease presents in this age group. Offprint requests to: S. W. Beasley     

Inflammatory disease of the colon: ulcerative colitis and Crohn's colitis.

Ulcerative colitis differs from Crohn's colitis in several ways. In ulcerative colitis the disease is limited to the mucosa and, occasionally, the submucosa; Crohn's colitis may involve all layers of the large intestine. Ulcerative colitis almost always begins in the rectum, is diffuse, and spreads proximally. Crohn's colitis may spare the rectum and has a patchy distribution. Perianal fistulas and ulcers are rare in ulcerative colitis but are common in Crohn's colitis. Granulomas and giant cells are not found in ulcerative colitis but are seen in the majority of patients with Crohn's colitis. Colonic and extraintestinal symptoms in the two illnesses may be indistinguishable but growth failure is far more severe in Crohn's colitis and may precede intestinal symptoms by months to years. Cancer of the colon is a risk in patients with either ulcerative or Crohn's colitis but is far more common in the former. It is important to distinguish between ulcerative colitis and Crohn's colitis because response to treatment and prognosis are different. Although neither condition can be cured by medical management, patients with ulcerative colitis may respond more frequently. Unfortunately, in the pediatric age range most cases of ulcerative and Crohn's colitis may be classified as moderate to severe. Fortunately for patients with ulcerative colitis, total colectomy with ileostomy will result in cure of illness. Patients with Crohn's colitis who require surgery may obtain remission of symptoms, but the disease is likely to recur in the small intestine.     

Pitfall in diagnosis of Crohn's disease in a cystic fibrosis patient

Cystic fibrosis (CF) and Crohn's disease may both present as failure to thrive and recurrent intestinal obstruction. Proper treatment and adequate nutrition may reverse these manifestations and improve the patient's quality of life. We describe a girl with CF who, despite appropriate management, failed to grow and had several episodes of bowel obstruction. After the additional diagnosis of Crohn's disease was reached, the patient improved on antiinflammatory and nutritional therapy. This patient illustrates the pitfall in the diagnosis of Crohn's disease in a CF patient due to the clinical overlap between the two conditions. We suggest that therapeutic failure in a chronic disease justifies additional diagnostic efforts resulting in a completion of diagnosis and significant changes in management.     

CROHN'S DISEASE OF THE PREPUCE IN A 12-YEAR-OLD BOY: A Case Report and Review of the Literature

We report a case of Crohn's disease with involvement of the foreskin in a 12-year-old boy. One year previously, on the basis of clinical features (diarrhea with blood, perianal fissures) and histologic examination, a diagnosis of Crohn's disease was made. Subsequently, he developed phimosis and balanitis and underwent circumcision. Sections submitted from the foreskin revealed noncaseating granulomatous inflammation consistent with Crohn's disease. Crohn's disease with involvement of the genitalia is unusual. Only 26 cases including our case have been reported in the scientific literature. We have analyzed these cases with emphasis on gender, age, clinical features, duration of Crohn's disease, and probable mode of spread to the genitalia. Careful examination of sections from genital lesions, including those submitted from the foreskin, is essential to detect small isolated granulomas that may then lead to the diagnosis of inflammatory bowel disease.     

Surgery for Crohn's disease in 28 children with the presence of acid-fast bacilli

Obstruction, abscesses, fistulae, treatment failure, and growth retardation are not always absolute indications for surgery in Crohn's disease. At the Children's Hospital of Eastern Ontario we have advocated earlier surgery since 1974. Forty-eight cases are reviewed, 28 surgical (17 ileocecocolic, 2 ileocolic with normal rectum, 3 colorectal, 1 jejunal, 5 perineal). Ileocecocolic resection with primary anastomosis produced remission for at least 3.2 years. Remission in colectomy with primary ileocolic anastomosis was at least 1 year. Ileostomy and colorectal resection produced remissions for at least 1.66 years. After jejunal resection remission was 1.58 years. Significant height and weight gains in over 80% of cases encourage earlier surgery. Adequate medical treatment has not often influenced the disease process. Surgery does not remove useful, medically restorable to normal bowel. Most postoperative cases required no medications and none jave required steroids. Permanent cure of anorectal disease alone was not achieved. Surgical mortality was 0%. The average hospital stay was 11.6 days. Postoperative fistulae occurred in 1 case and closed spontaneously. Mycobacterium tuberculosis was found in 2 resected specimens and in 1 human tubercle bacilli were cultured after 5 months. Both cases healed spontaneously, and both were otherwise undistinguishable from the other cases of Crohn's disease. Both were white, native-born Canadians. On examination of all the other resected specimens in our series, no acid-fast bacilli were seen. Offprint requests to: S. Mercer     

青少年炎症性肠病伴生长激素缺乏4例并文献复习

炎症性肠病（IBD）为慢性复发性非特异性肠道炎症性病变,10%~56%的克罗恩病患儿和10%左右的溃疡性结肠炎患儿存在生长迟缓。该研究报道4例伴生长激素缺乏的青少年IBD（均为克罗恩病）,其中男3例、女1例,确诊年龄11.0~13.9岁,确诊时病程11~85个月。4例患儿的病灶分别为：单纯小肠、单纯结肠、小肠及上消化道、小肠及结肠均累及,克罗恩病活动指数27.5~45分。4例患儿的年龄别身高Z评分（HAZ）均 < -2,生长激素刺激试验均提示生长激素缺乏症。2例患儿接受了重组人生长激素联合英夫利西单抗治疗,1例仅接受英夫利西单抗治疗,另1例接受重组人生长激素联合巯嘌呤的治疗。所有患儿经治疗后HAZ评分均有改善。     

Current diagnosis, management and morbidity in paediatric inflammatory bowel disease

In the 1970s several reports highlighted the long delay in diagnosis often experienced by children with Crohn's disease. In recent years this disorder has attracted much publicity, and many believe that the incidence has increased substantially. The aim of this investigation was to determine whether heightened awareness had shortened the interval to diagnosis, improved clinical management and reduced morbidity. A retrospective study was therefore carried out on 112 children with inflammatory bowel disease (64 Crohn's disease, 41 ulcerative colitis, 7 indeterminate colitis) referred to a paediatric gastroenterology department in the UK between 1994 and 1998. In Crohn's disease the median interval to diagnosis was 47 wk (maximum 7 y). In those without diarrhoea this was longer (66 vs 28 wk; p = 0.005). In ulcerative colitis the median interval was 20 wk (maximum 3 y). Even in severe colitis the median interval was 5.5 wk (range 3-9 wk) and 4 required urgent colectomy soon after referral. Many with unrecognized Crohn's disease had undergone inappropriate treatments, such as growth hormone or psychiatric therapy. Nineteen (17%) had undergone endoscopic investigations in adult units prior to referral. Malnutrition was equally common in Crohn's disease and ulcerative colitis (11%). Short stature was present in 19% with Crohn's disease, and 5% with ulcerative colitis, and was severe in 8% with Crohn's disease. There was a significant correlation between symptom duration and the degree of growth impairment present (r(s) = -0.4; p = 0.004). Conclusion: This study suggests that late diagnosis and inappropriate investigation and management are still significant problems.     

Influence of preoperative parenteral alimentation on postoperative growth in adolescent Crohn's disease

The postoperative growth rates achieved in eight early pubertal adolescent males with Crohn's disease undergoing surgery for obstructive complications is reported as a function of the use of 1 month's preoperative central venous alimentation. All patients had ileal strictures with ileocolitis, bone ages less than 13, Tanner stages 1 or 2, and growth velocities below the 3rd percentile for age. During a 3-year follow-up, those receiving preoperative venous alimentation demonstrated greater oral caloric intake (significant for 1 year) and greater postoperative growth velocity (significant for 2 years) in comparison to those patients who did not receive preoperative venous alimentation. There was no significant difference between the two groups of patients in postoperative weight gain, sexual development, and 3-year disease control.     

Presenting features of inflammatory bowel disease in Great Britain and Ireland.

BACKGROUND: Reports from individual referral centres suggest that a significant proportion of children with inflammatory bowel disease (IBD) present after prolonged delays and with impaired growth. AIMS: To prospectively document the presenting features, delay in presentation, disease localisation, and growth in newly diagnosed cases of IBD. METHODS: For 13 months, between June 1998 and June 1999, 3247 paediatricians, adult gastroenterologists, and surgeons across the UK and Ireland were prospectively surveyed each month and asked to report every newly diagnosed case of childhood IBD. RESULTS: A total of 739 new IBD cases aged less than 16 years were identified. Only one quarter of Crohn's disease (CD) cases presented with the "classic triad" of diarrhoea, weight loss, and abdominal pain; nearly half did not report diarrhoea. The median delay from onset of symptoms to diagnosis was 5 months (mean 11 months), with one fifth having symptoms of more than one year. Delays were most common in CD and in younger children. Short stature was noted only in those with CD and not with ulcerative colitis. One fifth of CD cases had disease activity in the jejunum and this group had significantly reduced stature. Ileo-colonic involvement was documented in most CD cases, with only a small minority having isolated ileal or isolated colonic disease. Pan-colitis was reported in most UC cases, with very few having only an isolated proctitis. CONCLUSIONS: Many children are diagnosed after prolonged delays and have growth failure. Improved knowledge of the presenting features of IBD, and earlier investigation of suspected cases, may help reduce the delays noted.     

Incidence and prevalence of inflammatory bowel disease in children in South-Western Sweden

Hildebrand H, Brydolf M, Holmquist L, Krantz I, Kristiansson B. Incidence and prevalence of inflammatory bowel disease in children in South-Western Sweden Acta PEdiatr 1994;83:640–5. Stockholm. ISSN 0803–5253
The incidence and prevalence of inflammatory bowel disease were estimated in all children less than 16 years of age living in the city of Göteborg and in three counties in South-Western Sweden, from 1983 to 1987. One hundred and thirty-two patients were classified according to set criteria into one of four diagnostic categories: ulcerative colitis, Crohn's disease, probable Crohn's disease and indeterminate colitis. The crude incidence of inflammatory bowel disease was 5.3 per 100 000 children per year and the prevalence 21.5 per 100000 children. This study lends support to the hypothesis that Crohn's disease has increased among Swedish children. Crohn's disease now appears to be at least as common as ulcerative colitis. Thirty-five of 55 patients first classified as indeterminate colitis or probable Crohn's disease later fulfilled the criteria of ulcerative colitis or Crohn's disease during a mean follow-up period of 4.6 years. This study emphasizes the importance, in epidemiological studies of inflammatory bowel disease, of inciuding those cases where a definite diagnosis of ulcerative colitis or Crohn's disease cannot be established initially and of re-evaluating the initial diagnosis regularly.     

Growth as a parameter of inflammation in Crohn's disease, using roentgen stereophotogrammetric analysis

Growth rate was measured in 12 children with Crohn's disease and growth retardation, using roentgen stereophotogrammetric analysis. The method was chosen as it provides an opportunity to record growth rate accurately during short periods of time. Growth was followed at variable intervals of 4–12 weeks depending on the clinical situation. Growth rate was shown to correlate with symptoms, laboratory tests and nitrogen balance. These findings indicate that growth, when it is affected, fluctuates in a similar way as albumin, acute phase proteins and symptoms, all assumed to reflect the disease activity. Short-term growth measurements appear to be helpful in monitoring the disease progress or response to therapy in children with Crohn's disease, particularly when growth retardation is the only manifestation of the disease.     

Status of elemental diets in the therapy of Crohn disease in childhood

Since the seventieth low molecular weight formulas, "elemental diets", are applied in acute Crohn's disease in addition to drug therapy. In small bowel involvement, therapeutic efficiency in active disease is as good as salazosulfapyridine combined with corticosteroids. Physiological changes under elemental diet have been reported: decrease of gastric and pancreatic secretion, changes of bacterial bowel flora and in patients with Crohn's disease decreased fecal bile acid excretion and decreased intestinal losses of lymphocytes were described. Further, the absence of allergens in the formula and the quick and complete resorption are discussed to be important to clinical improvement in Crohn's disease. Indications for elemental diet are acute small bowel disease, intestinal obstruction, malnourishment and growth retardation. Further studies are needed to examine if elemental diets are effective in gastrointestinal fistulas and extraintestinal symptoms in Crohn's disease.     

Tumor necrosis factor-alpha is not elevated in children with inflammatory bowel disease

Chronic undernutrition and high-dose daily corticosteroid therapy are well-accepted causes of growth failure in children with inflammatory bowel disease. Occasionally, children are seen with minimal gastrointestinal symptoms but in whom severe anorexia and profound growth impairment are evident. Recent observations that elevated serum levels of tumor necrosis factor-alpha (TNF) in cachexia associated with a number of disease states have suggested a similar possible role in inflammatory bowel disease. Accordingly, we determined TNF levels in 45 children and adolescents with inflammatory bowel disease (18 ulcerative colitis, 27 Crohn's disease) at varying times during their clinical course and compared them to values obtained from a group of 25 children with functional bowel disease. No differences were noted in serum TNF levels between the children with inflammatory bowel disease and the control population. Values were generally within the range of the lower limit of detection of the assay. In the children with inflammatory bowel disease, there was no significant correlation between TNF levels and disease activity or growth parameters. Our observations suggest that elevated TNF levels are not associated with inflammatory bowel disease in children.