Gastric Perforation in Non-Hodgkin'S Lymphoma

Oral steroids are an important component in the treatment of leukemia and lymphoma in children. Until recently it was widely accepted that treatment with oral steroids carried the risk of gastrointestinal complications, in particular gastric irritation, gastrointestinal ulceration, and hemorrhage. Though spontaneous intestinal perforation has been reported, to our knowledge, spontaneous perforation of the stomach in a child with lymphoma taking oral steroids has not previously been described. Furthermore, this report would appear to contradict recent reports claiming that steroid-induced ulcers do not arise unless the patient is also concurrently receiving nonsteroidal anti-inflammatory drugs.^1,2,3     

Traumatic prenatal sigmoid perforation due to amniocentesis

A variety of fetal injuries, including those inflicted to the gastrointestinal tract by amniocentesis, have been reported before. This brief report describes the first documented case of sigmoid perforation owing to the common procedure of amniocentesis that manifested as abdominal distention at birth. A potential link between this complication and a recent increased incidence of “intrauterine spontaneous perforation” of the gastrointestinal tract has been mentioned. Practicing radiologists are encouraged to inquire directly about the history of amniocentesis in unexplained cases of intrauterine intestinal perforation. Received: 7 September 2000 Accepted: 14 January 2001     

Idiopathic colonic perforation in the neonate

We describe a premature infant in whom spontaneous perforation of the colon was initially detected on routine abdominal films. There was no clinical evidence of necrotizing enterocolitis, peritonitis, or bowel obstruction. Surgical and pathologic findings confirmed the diagnosis of idiopathic bowel perforation. Since spontaneous gastrointestinal perforation in the neonate is often difficult to diagnose clinically, radiographic evaluation may allow earlier diagnosis and prompt surgical treatment of this life-threatening condition.     

Spontaneous intestinal perforation in a full-term infant: association with infection

The term spontaneous intestinal perforation suggests a perforation in the gastrointestinal tract of a newborn of no demonstrable cause. Only a few cases have been described in full-term newborns. The aetiology and pathogenesis of the disease are unknown although multiple theories have been proposed. Some authors suggest ischemia as the most likely cause. Conditions associated with fetal or neonatal hypoxia are important antecedents for this emerging distinct entity. We present a case of a spontaneous, intestinal perforation in a full-term neonate with urinary tract infection. There was no clinical evidence of necrotizing enterocolitis or bowel obstruction. Radiological images revealed a pneumoperitoneum. An emergency explorative laparotomy was performed. A localized linear perforation was identified in the transverse colon. Pathological examination of the resected specimens failed to reveal any etiology for the perforation. The neonate recovered rapidly, with no gastrointestinal complications. In our case none of the factors which have previously been associated with intestinal perforation could be implicated. We suggest that focal intestinal perforation is possibly the result of infection. Further studies, including careful recording of cases and close histopathological examination of resected specimens, are required in order to provide more information and improve our understanding of the aetiology of this rare occurance.     

严重胃肠道受累的幼年皮肌炎4例病例系列报告

目的 探讨幼年皮肌炎(JDM)合并严重胃肠道受累患儿的临床特点、诊断与治疗。方法 回顾性分析4例合并严重胃肠道受累及肠穿孔JDM患儿的临床资料及诊治经过,并行文献复习。结果 4例患儿(P1～P4),男1例,女3例,起病年龄1岁8个月至5岁,发现胃肠道症状于JDM确诊后4～10个月,胃肠道受累的首发症状均为腹痛。P1先后十二指肠及横结肠穿孔,P2十二指肠穿孔合并肝动脉破裂,P3肠穿孔部位不详,P4肠壁增厚;4例均为抗NXP2抗体强阳性,均使用大剂量甲基泼尼松龙、环磷酰胺、IVIG冲击治疗,P1行穿孔造瘘术后治疗2年完全缓解,在修补造瘘口后猝死;P2术后死于感染及十二指肠瘘;P3死于弥漫腹壁出血;P4自体干细胞移植术后完全缓解。检索PubMed数据库共检索到12例JDM合并胃肠道穿孔患儿,发生在JDM确诊后2个月至9年,穿孔时首发症状为腹痛,可伴有呕吐及发热;十二指肠穿孔8例,结肠穿孔3例,空肠穿孔1例,胃幽门部穿孔1例,食管颈部穿孔1例,1例不详;其中5例患儿多部位或多次穿孔。11例行外科手术;9例好转,3例死亡,其中1例死于反复肠穿孔,1例死于术后ARDS,1例死因不详。结论 难治性JDM长期不缓解可合并消化道穿孔,其消化道首发症状为腹痛;常见肠穿孔部位为十二指肠腹膜后;发生严重胃肠道受累的JDM常见肌炎特异性抗体为抗NXP2抗体;肠穿孔一旦发生病死率高,尤其是十二指肠穿孔并无有效的术式;对于难治性JDM自体干细胞移植可能是改善预后的有效措施。     

严重胃肠道受累的幼年皮肌炎4例病例系列报告

目的 探讨幼年皮肌炎(JDM)合并严重胃肠道受累患儿的临床特点、诊断与治疗。方法 回顾性分析4例合并严重胃肠道受累及肠穿孔JDM患儿的临床资料及诊治经过,并行文献复习。结果 4例患儿(P1～P4),男1例,女3例,起病年龄1岁8个月至5岁,发现胃肠道症状于JDM确诊后4～10个月,胃肠道受累的首发症状均为腹痛。P1先后十二指肠及横结肠穿孔,P2十二指肠穿孔合并肝动脉破裂,P3肠穿孔部位不详,P4肠壁增厚;4例均为抗NXP2抗体强阳性,均使用大剂量甲基泼尼松龙、环磷酰胺、IVIG冲击治疗,P1行穿孔造瘘术后治疗2年完全缓解,在修补造瘘口后猝死;P2术后死于感染及十二指肠瘘;P3死于弥漫腹壁出血;P4自体干细胞移植术后完全缓解。检索PubMed数据库共检索到12例JDM合并胃肠道穿孔患儿,发生在JDM确诊后2个月至9年,穿孔时首发症状为腹痛,可伴有呕吐及发热;十二指肠穿孔8例,结肠穿孔3例,空肠穿孔1例,胃幽门部穿孔1例,食管颈部穿孔1例,1例不详;其中5例患儿多部位或多次穿孔。11例行外科手术;9例好转,3例死亡,其中1例死于反复肠穿孔,1例死于术后ARDS,1例死因不详。结论 难治性JDM长期不缓解可合并消化道穿孔,其消化道首发症状为腹痛;常见肠穿孔部位为十二指肠腹膜后;发生严重胃肠道受累的JDM常见肌炎特异性抗体为抗NXP2抗体;肠穿孔一旦发生病死率高,尤其是十二指肠穿孔并无有效的术式;对于难治性JDM自体干细胞移植可能是改善预后的有效措施。     

Meconium‐related ileus in extremely low‐birthweight neonates: Etiological considerations from histology and radiology

Background: A nationwide survey on neonatal surgery conducted by the Japanese Society of Pediatric Surgeons has demonstrated that the mortality of neonatal intestinal perforation has risen over the past 15 years. The incidence of intestinal perforation in extremely low‐birthweight (ELBW) neonates has been increasing as more ELBW neonates survive and as the live‐birth rate of ELBW has increased. In contrast to necrotizing enterocolitis (NEC) and focal intestinal perforation (FIP), the pathogenesis of meconium‐related ileus, defined as functional bowel obstruction characterized by delayed meconium excretion and microcolon, remains unclarified. Methods: The histology of 13 ELBW neonates with intestinal perforation secondary to meconium‐related ileus was reviewed, and the radiology of 33 cases of meconium‐related ileus diagnosed on contrast enema was reviewed. Specimens obtained from 16 ELBW neonates without gastrointestinal disease served as age‐matched controls for histological assessment. Results: The size of the ganglion cell nucleus in meconium‐related ileus and in control subjects was 47.3 ± 22.0 µm² and 37.8 ± 11.6 µm², respectively, which was not significantly different. In all cases of meconium‐related ileus, contrast enema demonstrated a microcolon or small‐sized colon, with a gradual caliber change in the ileum and filling defects due to meconium in the ileum or colon, showing not‐identical locations of caliber changes and filling defects. Conclusion: Morphological immaturity of ganglia was not suggested to be the pathogenesis of meconium‐related ileus. Impaction of inspissated meconium is not the cause of obstruction, but the result of excessive water absorption in the hypoperistaltic bowel before birth, although the underlying mechanism responsible for the fetal hypoperistalsis remains unclear.     

Eosinophilic enteritis due to cow's milk allergy: Possible cause of anastomosis failure following repair of focal intestinal perforation

Reports of cow's milk allergy (CMA) after neonatal gastrointestinal surgery have recently increased. In recent years it has been suggested that the development of CMA after gastrointestinal surgery in newborn infants is due to an immune function. In addition, the development of CMA might be synergistically exacerbated by congenital abnormalities of the intestinal mucosa, general conditional changes and local damage to the intestine by invasive surgery, and poor pre‐ or post‐surgical nutrition. CMA manifests as a variety of symptoms, such as mild vomiting and bloody stool, decreased activity, poor oral intake, and ileus. CMA may also rarely cause gastrointestinal perforation. Here, we report the case of a newborn infant who developed CMA following repair of focal small intestinal perforation, in which eosinophilic enteritis was suspected to be a possible cause of anastomosis leakage.     

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??Objective To compare the etiologic factors??clinical features and prognosis in newborns with gastrointestinal perforation. Methods A retrospective study of the clinical data from 80 cases with diagnosis of gastrointestinal perforation collected from January 2004 to December 2015 was performed. Based on whether they were full-term??the cases were divided into two groups??then the clinical features??etiologic factors and prognosis were compared. Results Among the 80 neonates with gastrointestinal perforation??there were 62 preterm infants and 18 full-term infants. The main causes of gastrointestinal perforation for the two groups was necrotizing enterocolitis??with the most frequent clinical symptom of abdominal distension.cases. Compared with full-term infants??the incidence of preterm shock??dyspnea??DIC??and poor reaction was significantly higher in preterm infants??P??0.05??.The mean time period of perforation in preterm infants group was 9??1.75??20?? d??while it was 4.5??1??7.75?? d in full-term infants group.There were 62 cases that received surgical treatment??8 cases of which were gastric perforation and 54 cases were intestinal perforation. The exact site of perforation of the 18 cases who refused surgical treatment was not clear. Of all the cases??32 infants died with the overall mortality rate of 40%. For the preterm infants??the mortality was 41.9% ??26 cases????while it was 33.3% ??6 cases?? in the full-term infants group. Conclusion Neonatal gastrointestinal perforation is one of the serious diseases in neonatal period??which has a very high mortality rate. Early diagnosis??active treatment and timely surgical intervention can significantly improve survival rates and improve the prognosis.     

Anaplastic large cell lymphoma of the esophagus in a pediatric patient

We report a 3-year-old boy who initially presented with abdominal pain and was subsequently found to have an esophageal perforation. The child did not respond to conservative management, and subsequent lymphadenopathy led to a lymph node biopsy demonstrating an anaplastic lymphoma kinase (ALK)+ anaplastic large cell lymphoma. Esophageal perforation and thickening is most commonly seen in children with a history of esophageal intervention or foreign body/caustic ingestion. Esophageal involvement in children with non-Hodgkin lymphoma (NHL) has not, to our knowledge, been reported in the literature. This case illustrates an unusual presentation of pediatric NHL.     

Pemphigus foliaceus

Pemphigus foliaceus is a skin disease in which antibodies against the cell surface of keratinocytes destroy the adhesion between epidermal cells, thereby producing blisters. It is a rare disease in childhood, and treatment guidelines for juvenile pemphigus foliaceus are lacking. An 8 year old boy with pemphigus foliaceus is described. He did not respond to topical steroids, and the condition flared up when high dose oral steroids were tapered. The lesions resolved completely in four weeks on dapsone, which was maintained for nine months with no major adverse effects, except for a moderate increase of the methaemoglobin concentration at the outset of treatment. There has been no evidence of disease reactivation in more than nine months of follow up since dapsone withdrawal.

     

Basiliximab monotherapy following B-cell lymphoma after pediatric liver transplantation and anti-CD20 therapy

The chimeric, monoclonal antibody basiliximab inhibits the proliferation and clonal expansion of activated T cells. To date basiliximab has been used only in combination with other immunosuppressive agents for rejection prophylaxis after solid organ transplantation. An infant underwent liver transplantion (LTx) at the age of 5 months because of biliary atresia. The primary immunosuppression consisted of cyclosporine and prednisolone. As a result of a steroid resistant rejection episode on day 26 post-LTx we had to switch the initial immunosuppressive regiment to tacrolimus and steroids. Because of severe cholestasis and assumed impaired enteral resorption we were forced to administer an unusually high dosage (2 mg/kg/day) of tacrolimus. Four months after LTx an intestinal B-cell lymphoma was diagnosed when the patient suffered from a small bowel perforation. After stopping the immunosuppressive medication we started treatment with the anti-CD20 monoclonal antibody rituximab for B-cell depletion. During the 12 wk no B cells were detectable in the peripheral blood by flow cytometry. In this setting we started a monotherapy with repetitive doses of basiliximab for immunosuppression. During the following course there was no further rejection and no recurrence of the tumor. From this experience we conclude that monotherapy with basiliximab after LTx and anti-CD20 treatment for B-cell lymphoma is efficient and safe.     

Lymphoblastic lymphoma: Late relapse in childhood

This report describes two children with lymphoblastic lymphoma who relapsed more than 2^1/2 years from diagnosis. Relapses occurred at seven and 20 months after completion of treatment. Their therapy consisted of an intensive pulse chemotherapy program combined with radiation therapy. Initial relapse after two years' treatment has been extremely rare in patients receiving contemporary chemotherapy programs, and two-year survival without disease has been considered a cure. These cases illustrate that late relapses can occur after intensive chemotherapy and that two-year disease-free survival must not be interpreted as a complete cure.     

Eosinophilic oesophagitis: epidemiology, clinical aspects, and association to allergy

Eosinophilic oesophagitis is characterised by age-dependent symptoms mimicking gastrooesophageal reflux disease, a distinct endoscopic appearance and a histological picture with extensive infiltration of eosinophils in the oesophageal mucosa. Eosinophilic oesophagitis is more frequently seen in males, and patients often belong to the paediatric or adolescence age groups. The exact prevalence of eosinophilic oesophagitis is unknown, but it has been suggested that the United States has a higher prevalence than Europe. Several treatment algorithms have been suggested, including elemental diets, oral steroids, inhaled (swallowed) steroids, and leucotriene receptor antagonists. Detailed information on the eosinophilic inflammatory processes in the oesophageal mucosa was initially obtained from animal models, in particular with regard to the role of interleukin-5 and the chemokine eotaxin-1 in eosinophilic recruitment. Studies have suggested a cytotoxic effect of eosinophilic degranulation products on nerve fibers in the gastric/intestinal mucosa, implicating a direct effect of allergic inflammation on gastrointestinal motility. Human studies recently have emphasized the role of eotaxin-3 and identified a single nucleotide polymorphism probably related to disease susceptibility.     

Outcome of primary peritoneal drainage for perforated necrotizing enterocolitis: comparison between laparotomy and drainage.

Perforation of the gastrointestinal tract in neonates is still associated with high mortality rates. Laparotomy is usually required to treat gastrointestinal perforation, however peritoneal drainage under local anesthesia has been also described as an alternative mode of treatment. In our institute, laparotomy was the first choice for the management of gastrointestinal perforation in neonates until 1999. Because of the high mortality rates in this group of patients, our policy has since changed to the use of primary peritoneal drainage instead. The aim of this study is to compare the effectiveness of primary peritoneal drainage (PPD) and primary laparotomy (PL) procedures in the management of gastrointestinal perforation due to necrotizing enterocolitis in neonates. Between 1994 - 1998, ten babies with intestinal perforation underwent PL, whereas fifteen newborns with similar findings were treated with PPD between 1999 and 2003. Eight (80 %) of the patients died in the PL group prior to 1999. In the PPD group 8 (53.3 %) of babies required no further treatment and were discharged without any complications. Four (26.7 %) patients in this group needed laparotomy later, and three (75 %) of them survived. In conclusion, we believe that PPD is more effective than PL for the management of perforated necrotizing enterocolitis in neonates. Laparotomy can be used in particularly unresponsive cases after primary peritoneal drainage.     

The Activity of Valproic Acid in the Treatment of Refractory Hyperleukocytosis in a Child with Acute Lymphoblastic Leukemia

Hyperleukocytosis may be associated with an early morbidity and mortality due to leukostasis. Cytoreductive therapies—such as steroids, hydroxiurea, exchange transfusion, and leukapheresis—have been used for the prevention of leukostasis. Herein, the valproic acid used for the treatment of the hyperleukocytosis did not respond to known therapies in a child; lymphoblastic lymphoma developed bone marrow relapse under the treatment is discussed.     

Ki-1+ ANAPLASTIC LARGE CELL LYMPHOMA IN A CHILD WITH UNPREDICTABLE CLINICAL COURSE

Ki-1⁺ anaplastic large cell lymphoma (Ki-1⁺ ALCL) is a subtype of non-Hodgkin lymphoma (NHL) with defined histopathological characteristics but with highly variable clinical presentation and outcome. Although in most of the patients the disease behaves as an intermediate- or high-grade lymphoma, some patients present with an indolent clinical course. Factors that determine the clinical behavior of this lymphoma have not yet been identified. A case is reported of a 13-year-old girl who initially presented with Ki-1⁺ ALCL but later developed recurrent localized cutaneous disease and followed a clinical course similar to that of a low-grade lymphoma.     

Pseudohypoaldosteronism in a child with Down syndrome. Long-term management of salt loss by ion exchange resin administration

At 4 weeks of age, an infant with Down syndrome developed severe dehydration and salt loss with the typical features of pseudohypoaldosteronism (PHA). Plasma renin activity, 11-deoxycorticosterone, corticosterone and aldosterone levels were all increased severalfold over the normal range for age, thus excluding an adrenal biosynthetic defect. Clinical condition, hyponatraemia and hyperkalaemia could be rapidly normalised by the ion exchange resin Resonium A^® administered first as enema and later orally (3 g/day). At that time, no further salt supplementation was necessary. At 18 months of age, Resonium A could be completely withdrawn with neither clinical deterioration nor electrolyte abnormalities. However at 31/2 years of age, plasma renin activity and aldosterone were still markedly elevated while precursor steroids were normal and the clinical condition satisfactory. No side effects were observed with the Resonium A^® therapy.The combination of trisomy 21 and PHA is very unusual. Similarly, the successful treatment of severe renal salt loss during infancy by sodium supplementation and concomitant potassium withdrawal via an oral ion exchange resin has not yet been described and warrants further therapeutic trials.Abbreviations PHA pseudohypoaldosteronism - PRA plasma renin activity - DOC 11-deoxycorticosterone     

Randomised controlled study of oral erythromycin for treatment of gastrointestinal dysmotility in preterm infants

AIM: To evaluate the effectiveness of oral erythromycin as a prokinetic agent for the treatment of moderately severe gastrointestinal dysmotility in preterm very low birthweight infants. METHODS: A prospective, double blind, randomised, placebo controlled study in a tertiary referral centre of a university teaching hospital was conducted on 56 preterm infants (< 1500 g) consecutively admitted to the neonatal unit. The infants were randomly allocated by minimisation to receive oral erythromycin (12.5 mg/kg, every six hours for 14 days) or an equivalent volume of placebo solution (normal saline) if they received less than half the total daily fluid intake or less than 75 ml/kg/day of milk feeds by the enteral route on day 14 of life. The times taken to establish half, three quarters, and full enteral feeding after the drug treatment were compared between the two groups. Potential adverse effects of oral erythromycin and complications associated with parenteral nutrition were assessed as secondary outcomes. RESULTS: Twenty seven and 29 infants received oral erythromycin and placebo solution respectively. The times taken to establish half, three quarters, and full enteral feeding after the drug treatment were significantly shorter in the group receiving oral erythromycin than in those receiving the placebo (p < 0.05, p < 0.05 and p < 0.0001 respectively). There was also a trend suggesting that more infants with prolonged feed intolerance developed cholestatic jaundice in the placebo than in the oral erythromycin group (10 v 5 infants). None of the infants receiving oral erythromycin developed cardiac dysrhythmia, pyloric stenosis, or septicaemia caused by multiresistant organisms. CONCLUSIONS: Oral erythromycin is effective in facilitating enteral feeding in preterm very low birthweight infants with moderately severe gastrointestinal dysmotility. Treated infants can achieve full enteral feeding 10 days earlier, and this may result in a substantial saving on hyperalimentation. However, until the safety of erythromycin has been confirmed in preterm infants, this treatment modality should remain experimental. Prophylactic or routine use of this medication for treatment of mild cases of gastrointestinal dysmotility is probably not warranted at this stage.     

Treatment and prevention of necrotizing enterocolitis.

Necrotizing enterocolitis (NEC) is the most common serious, acquired gastrointestinal disorder in the newborn infant. Although many variables are associated with development of NEC, only prematurity has been consistently identified in case-controlled studies. Traditionally, the diving seal reflex has been invoked as the mechanism responsible for ischaemic injury and necrosis. Intestinal ischaemia is likely to be the final common pathway in NEC; however, it is due to the release of vasoconstricting substances, such as platelet activating factor, rather than perinatal asphyxia. Bacteria and/or bacterial toxins are likely to have a key role in the pathogenesis of NEC by fostering production of inflammatory mediators. The role of feeding practices in the pathogenesis of NEC remains controversial. Treatment of infants with NEC generally includes a regimen of bowel rest, gastric decompression, systemic antibiotics and parenteral nutrition. Infants with perforation are generally operated upon; however, there has been recent interest in primary peritoneal drainage as an alternative. Prevention of NEC still remains elusive. Avoidance of preterm birth, use of antenatal steroids and breast-milk feeding are practices that offer the greatest potential benefits. Use of any other strategy should await further trials.