Gastrointestinal manifestations in Henoch-Schönlein purpura: a review of 261 patients

Aim: Henoch‐Schönlein purpura is an IgA‐mediated autoimmune vasculitis of children. It often presents with symptoms including purpuric rash, abdominal pain, renal involvement or arthritis. Abdominal pain is a frequent symptom in children with HSP and raises the suspicion of intussusception or perforation. We sought to evaluate abdominal pain via stool occult blood and image studies. Methods: A retrospective study of 261 patients diagnosed with Henoch‐Schönlein purpura from December 1991 to December 2001 was conducted. Image studies, including abdominal echo, abdominal CT and panendoscopy, were performed for patients who suffered from abdominal pain. Results: Of the 261 patients, 151 (58%) had abdominal pain, and 46 (17.6%) suffered either overt gastrointestinal bleeding or had positive stool occult blood. Seven patients had gross bloody stools. One acute intussusception and one bowel perforation were noted. One patient suffered from hypovolemic shock due to massive gastrointestinal bleeding. When stool occult blood was 3+ or 4+, the incidence of a positive image finding was high. Conclusion: We found that stool occult blood and image studies may be necessary regarding severe gastrointestinal involvement. Ultrasonography is an important tool when intussusception or bowel perforation is suspected. Monitoring the vital signs is important, especially in patients with massive gastrointestinal bleeding.     

Duodenojejunitis: is it idiopathic or is it Henoch-Schönlein purpura without the purpura?

BACKGROUND: Henoch-Sch?nlein purpura is a small-vessel vasculitic disease that most often affects the skin. Gastrointestinal manifestations have been well described, including duodenojejunal inflammation (DJI). METHODS: Four children with DJI and clinical features of HSP are described, in whom the rash was either not present or appeared atypically late in the illness. RESULTS: The characteristic rash did not develop in three children, and it developed much later in one. The patients (three boys and one girl) were aged between 7 and 9 years (mean, 7.5 years). Growth characteristics were normal. In all patients, pain occurred acutely with colicky abdominal pain in the spring or fall of the year, and all stools were positive for occult blood. No infectious cause was identified. Upper gastrointestinal endoscopic examinations demonstrated significant visual and histologic duodenitis in a pattern consistent with previous reports in children with known HSP. Factor XIII activity was absent. Immunoglobulin A levels were increased in three of four children. All children made a prompt recovery with the administration of intravenous glucocorticoids. In one child, the characteristic rash of HSP developed 18 weeks after the initial examination. CONCLUSION: Duodenojejunal inflammation may be the primary manifestation of HSP, even in the absence of the characteristic rash.     

A Case of Henoch-Sch?nlein Purpura with P369S Mutation in MEFV Gene

Background

Henoch-Schönlein purpura (HSP) is the most common vasculitis of childhood. HSP can affect multiple organs presenting with a characteristic rash in most of the patients. Familial Mediterranean Fever (FMF) is an inherited inflammatory disease common in mediterranean populations. HSP is the most common vasculitis seen in children with FMF.

Case Presentation

A 16 year old boy was referred with history of abdominal pain lasting for 20 days. He was hospitalized and had appendectomy. Due to the persistence of his abdominal pain after surgery he was admitted to our hospital. His physical examination showed palpable purpuric rashes symmetrically distributed on lower extremities. Abdominal examination revealed periumbilical tenderness. Laboratory tests showed elevated erythrocyte sedimentation rate, Creactive protein and fibrinogen. Urinalysis revealed microscopic hematuria and severe proteinuria. The fecal occult blood testing was positive. Based on these clinic findings, the patient was diagnosed as HSP with renal, gastrointestinal tract and skin involvement. We performed DNA analysis in our patient because he had diagnosis of vasculitis with severe symptoms and found that he was carrying heterozygote P369S mutation.

Conclusion

Our case is noteworthy as it indicates that it may be important not to overlook presence of FMF mutations in patients with a diagnosis of severe vasculitis.     

以腹型为首发表现的儿童过敏性紫癜临床分析

目的了解以腹部症状为首发表现的儿童过敏性紫癜(HSP)临床特征,并探讨胃镜和全腹计算机断层扫描(CT)对其早期诊断价值。方法选取2017-09-01—2019-08-31就诊于中国医科大学附属盛京医院小儿消化内科以腹痛等消化道表现为首发症状的腹型HSP住院患儿169例为观察组,以皮疹为首发症状142例为对照组,对临床资料进行回顾性分析。结果两组HSP患儿性别、年龄与好发季节差异无统计学意义(P>0.05);观察组有前驱感染史和(或)病原学阳性者、ASO升高发生率低于对照组(P<0.05);观察组患儿中有33例(19.53%)无皮疹;两组间消化道出血发生率差异无统计学意义(P>0.05);观察组白细胞计数、C反应蛋白、血小板计数、D-二聚体水平较对照组升高(P<0.05),而白蛋白、IgA、补体C3水平较低(P<0.05);但两组间降钙素原水平、过敏原特异性IgE阳性率和总IgE水平差异均无统计学意义(P>0.05);两组间尿蛋白和红细胞升高率差异无统计学意义(P>0.05);观察组便潜血阳性率较高(P<0.05);腹腔三维超声及全腹CT对腹型HSP阳性检出率差异有统计学意义(P<0.05),而胃镜和全腹CT阳性检出率差异无统计学意义(P>0.05);两组对激素敏感度、紫癜肾炎发生率差异无统计学意义(P>0.05),但观察组复发率更高(P<0.05)。结论部分以腹痛为首发表现的HSP患儿可不出现皮疹,其白细胞计数、C反应蛋白、血小板计数、D-二聚体水平、便潜血阳性率、低白蛋白血症发生率较以皮疹为首发表现的HSP患儿升高。全腹CT和胃镜对腹型HSP的早期诊断有重要价值。以腹痛和皮疹为首发表现的HSP患儿激素敏感度、紫癜肾炎发生率差异无统计学意义,但以腹痛为首发表现的HSP患儿更易复发。     

SARS-CoV-2-associated Henoch–Schönlein purpura in a 13-year-old girl

In the context of the current coronavirus disease 2019 (COVID-19) pandemic, cutaneous lesions are being described. Here, we report on a 13-year-old girl with SARS-CoV-2-associated Henoch–Schönlein purpura and Epstein–Barr virus (EBV) infection. She presented without any respiratory symptoms, only a purpuric skin rash, abdominal pain, low-grade fever, and pharyngitis. Virology tests by polymerase chain reaction (PCR) were positive for SARS-CoV-2 and EBV. The potential association of Henoch–Schönlein purpura and SARS-CoV-2 should be kept in mind in order to reduce the spread of the virus, particularly in children with few respiratory symptoms.     

Intra-abdominal manifestations of Henoch-Schönlein Purpura

Gastrointestinal involvement occurs in approximately two thirds of children with Henoch-Schönlein Purpura (HSP) and usually is manifested by abdominal pain. Abdominal symptoms precede the typical purpuric rash of HSP in 14–36%; the symptoms may mimic an acute surgical abdomen and result in unnecessary laparotomy. Major complications of abdominal involvement develop in 4.6% (range 1.3–13.6%), of which intussusception is by far the most common. The intussusceptum is confined to the small bowel in 58%; its frequent inaccessibility to demonstration by contrast enema means that ultrasonography is the investigation of choice. Ultrasonography complements serial clinical assessment, clarifies the nature of the gastrointestinal involvement and reduces the likelihood of unnecessary surgery. Bowel ischaemia and infarction, intestinal perforation, fistula formation, late ileal stricture, acute appendicitis, massive upper gastrointestinal haemorrhage, pancreatitis, hydrops of the gallbladder and pseudomembranous colitis are seen infrequently. Earlier diagnosis and prompt treatment of intra-abdominal complications has reduced the mortality from 40% to almost zero.     

Haemorrhagic bullous lesions in a 3-year-old girl with Henoch-Schölein purpura

Henoch–Schönlein purpura (HSP) is the most common vasculitis in children and is characterized by cutaneous purpura, arthritis, abdominal pain and nephritis. We report a case involving a 3‐year‐old girl with HSP who displayed rapidly evolving haemorrhagic bullae from primary purpuric lesions during systemic corticosteroid therapy. Conclusion: The bullae disappeared within 7 days of systemic corticosteroid therapy, although some scarring of the skin occurred. Also, bullae should not be considered as a poor prognostic factor of renal outcome in HSP.     

Acute appendicitis occurring immediately post-renal transplant

A 10-yr-old boy who presented with vomiting and abdominal pain 12 days after an uneventful renal transplant had no identifiable transplant-related cause for these symptoms. Four days later a perforated appendix was diagnosed. His case illustrates that common abdominal pathologies may present differently in immunosuppressed, transplanted patients, causing diagnostic delay.     

Pancreatitis associated with Henoch-Schonlein purpura

A 7-year-old Chinese boy presented with acute pancreatitis. The characteristic rash of Henoch-Schonlein purpura (HSP) did not develop until nine days later, together with painful scalp swelling and calf pain. Acute pancreatitis has only rarely been reported in association with HSP and never before as the sole presenting feature.     

Henoch-Schonlein syndrome in northern Indian children.

In order to evaluate clinical features and renal pathological findings of Henoch-Schonlein syndrome (HSS) in northern Indian Children, we studied 47 such cases. The mean age at onset was 8.5 yr; sex ratio (M:F) 2.6:1. The clinical features were purpuric rash (96%), abdominal pain (64%), Henoch-Schonlein nephritis (51%) and arthralgias (47%). Patients younger than 6 yr also showed urticarial rash or edema of scalp and extremities. Henoch-Schonlein nephritis (HSN) and abdominal symptoms were more common in older cases. The manifestations of HSN were asymptomatic hematuria and/or proteinuria (n = 15), acute nephritic syndrome (n = 6), and nephrotic syndrome (n = 3). The severity of clinical manifestations correlated with the renal pathologic findings. On follow up, 29% cases showed renal impairment. The prognosis was poor in patients with the acute nephritic or nephrotic syndrome and crescents in more than 50% glomeruli. Combination of clinical data and renal biopsy findings are important in assessing the long-term outcome in cases with HSN.     

Munchausen's Syndrome in Children: Bizarre Clinical and Laboratory Features

A 10- year-old girl showed macroscopic hematuria, purpuric rash, bloody stool and complained of abdominal pain and joint pain for three years. The extensive investigations including intravenous urography, cystoscopy, barium enema, upper gastrointestinal X-rays, sigmoidoscopy and renal biopsy failed to reveal the sources of bleeing for urinary anmd gastrointestinal tracts and to explain abdominal and joint pains. the major blood group of the patient was B, while that in the urine obtained by the patient without supervised conditions was A or AB. This finding indicated that macroscopic hematuria was factitious in origin and the patient stated that symptoms were fabricated by herself. The various psychological tests failed to disclose the motives for this bizarre behavior and to obtain the evidence of psychosis. This case presents the youngest and fourth case of Munchausen's syndrome in children and suggests that when bizarre clinical and laboratory findings are observed, Munchausen's syndrome should be suspected and its consideration may obviate harmful and unnecessary investigations as well as medications.     

Rare clinical presentation mode of intestinal malrotation after neonatal period: Malabsorption-like symptoms due to chronic midgut volvulus

BACKGROUND: Many different and non-specific clinic presentation modes of malrotation anomalies (MA) have been reported after neonatal period. The authors describe four children with MA presented with malabsorption-like clinical features. METHODS: Three children aged from 8 months to 7 years, with a history of long-standing diarrhea and failure to thrive attributed to malabsorption, were referred to Department of Pediatric Surgery, for evaluation of suspected MA. Another patient, a 10-year-old boy who was treated for malabsorption for 6 years, presented with acute duodenal obstruction findings. The duration of symptoms averaged 35 months, ranging 8 months to 6 years. All patients had undergone extensive evaluation and empiric trials of different formulas with no improvement in their symptoms. One underwent jejunal biopsy. RESULTS: Primary presentation complaints were chronic diarrhea and failure to thrive in three patients. Their histories revealed chronic (in one) and intermittent colicky (in two) abdominal pain, and intermittent nonbilious vomiting (in three). The remaining patient presented with acute illness, with chronic diarrhea, failure to thrive, and intermittent abdominal pain and vomiting on his history. They were below 30th percentile according to body weight and height. Laboratory studies revealed hypoproteinemia, hypoalbunemia, raised liver function tests, and anemia in all patients. The patient who presented acutely had double-bubble sign on the plain abdominal film obtained at admission. In the other three, plain films obtained during an attack of abdominal pain and/or vomiting revealed findings of partial intestinal obstruction. The diagnosis was confirmed by upper gastrointestinal series. At their laparotomy, a classical type of malrotation with circumstantial evidence of chronic volvulus was noted. All patients had normal laboratory values between postoperative 3 and 5 weeks, and they were up to 30th percentile at the end of the 6 months. CONCLUSIONS: Malrotation anomalies should be included in the differential diagnosis in a child presented with malabsorption-like clinical features.     

丙种球蛋白冲击治疗儿童腹型过敏性紫癜疗效观察

目的 探讨大剂量丙种球蛋白冲击治疗腹型过敏性紫癜（HSP）的疗效。方法 33 例腹型HSP患儿随机分为地塞米松组（15 例）和丙种球蛋白组（18 例）。地塞米松组患儿采用地塞米松和常规治疗方法进行治疗;丙种球蛋白组患儿在常规治疗基础上,给予大剂量丙种球蛋白冲击治疗。观察两组患儿临床疗效和复发率。结果 丙种球蛋白组患儿皮疹开始和完全消退时间、腹痛缓解和血便消失时间明显短于地塞米松组（P P > 0.05）;丙种球蛋白组患儿有效率（95%）明显高于地塞米松组（65%）,差异有统计学意义（P P 结论 大剂量丙种球蛋白冲击治疗腹型HSP 症状缓解快、有效率高、复发率低。     

过敏性紫癜胃镜特征与临床分析

目的探讨胃镜检查在以腹痛为首发症状的过敏性紫癜(HSP)诊断中的意义。方法对HSP患儿37例通过胃镜检测胃及十二指肠黏膜病变,病变部位取活组织检查,并分析其病变特征与临床的关系。结果病变检出率为62.2%。其中单纯皮肤紫癜检出率为31.3%;伴急性腹痛症状者检出率为100%;单纯关节炎1例和紫癜性肾炎2例均未检出病变。病损以黏膜糜烂、充血、水肿及出血为主要特征。结论HSP患儿胃镜下消化道黏膜病变检出率较高,胃镜检查对儿童HSP,特别是以腹痛为首发症状HSP的早期诊断和鉴别诊断有重要意义。     

Henoch-Schönlein purpura with hypocomplementemia in children

BACKGROUND: The clinical course and prognosis of Henoch-Schonlein purpura (HSP) associated with hypocomplementemia are not clear. METHODS: The clinical findings of 10 children with HSP and hypocomplementemia were studied. RESULTS: Purpuric rash in all patients, abdominal pain in five, and arthralgia in nine were noted. The findings in HSP were not different from others with HSP. In eight patients, infection preceded hypocomplementemia. Serum levels of CH50, C3 or C4 were depressed variously. Complement levels returned to normal within 5 weeks in all patients. Antistreptolysin-O (ASO) titer was elevated in all patients and nephritis occurred in eight patients. Six patients had generalized edema and hypertension. Macroscopic hematuria occurred in two patients and heavy proteinuria in five patients. One patient was diagnosed as having poststreptococcal acute glomerulonephritis (PSAGN) combined with HSP nephritis according to renal biopsy findings. In three of eight patients with nephritis, abnormal urinary findings continued for more than 1 year. CONCLUSIONS: Hypocomplementemia in children with HSP was transient and was not related to severity of HSP. Incidences of elevated ASO titer and nephritis were high. The nephritis resembled PSAGN during the acute stage and long-term clinical courses varied. These findings suggest PSAGN may be associated with HSP nephritis.     

Intussusception secondary to anaphylactic reaction to salmon roe (ikura)

Anaphylactic food reaction often involves gastrointestinal symptoms, such as vomiting and abdominal pain, but to date, there have been no publications documenting the association between food hypersensitivity and intussusception. Herein is reported the case of a 2‐year‐old boy with intussusception accompanied by anaphylactic food reaction. The patient without known allergies complained of severe abdominal pain following ingestion of salmon roe for the first time. Dyspnea, wheezing and generalized urticaria also developed. Subsequently, he had stools containing jelly‐like blood with mucus. At hospital arrival, physical examination identified an abdominal mass in the right lower quadrant; imaging confirmed the diagnosis of colo‐colic intussusception. This patient was successfully treated with enema and no pathological findings were identified via radiology. Laboratory results supported the presence of IgE‐mediated allergy to salmon roe in the present patient. To our knowledge, this is the first report to describe the possible association between intussusception and a hypersensitive food reaction.     

Myocarditis presenting as gastritis in children

Vomiting and abdominal pain are common pediatric complaints encountered by emergency physicians. The differential diagnosis of abdominal pain is extensive. Herein, we report 2 cases with fatal myocarditis who initially presented with abdominal pain and vomiting. Both cases were presented with abdominal pain, vomiting, and loose stools. On arrival at our emergency department, hypotension, tachycardia, and cyanotic extremities were found. Their serum troponin-I levels were elevated. The echocardiogram demonstrated poor left ventricular performance and a decreased ejection fraction. In both cases, an arrhythmia and a coma developed within hours and were shortly followed by death. The clinical presentations of acute myocarditis are variable, ranging from an initial mild discomfort to acute progressive heart failure, and at times, even death. Abdominal pain may be a manifestation of systemic disease, an extra-abdominal lesion, or myocarditis. Although myocarditis associated with abdominal pain or vomiting remains a diagnostic challenge to physicians, it should be considered in the differential diagnosis of children with gastritis and hypotension or who are refractory to rehydration therapy.     

Superior mesenteric artery syndrome: Risk factor for duodenal involvement in Henoch–Schönlein purpura

Background: The anatomical location of the third portion of the duodenum is between the superior mesenteric artery (SMA) and the abdominal aorta (AA). When the aorto‐mesenteric angle (AMA) is small and the aorto‐mesenteric distance (AMD) is short, the duodenum becomes tightly compressed between these two blood vessels. Severe compression can obstruct the duodenum, resulting in vomiting and abdominal pain. This clinical condition is termed superior mesenteric artery syndrome (SMA syndrome). The duodenum is frequently affected in Henoch–Schönlein purpura (HSP). The aim of the present study was to verify that duodenal anatomy mimicking SMA syndrome is a risk factor for duodenal involvement and abdominal pain in HSP. Methods: A prospective case–control study involving 12 HSP patients compared with 48 age‐ and sex‐matched children (controls) was conducted. Bowel abnormalities were defined as thickened wall >3 mm and paralytic ileus detected on ultrasound. AMA, AMD and obesity index (OI) were compared between the two groups on Mann–Whitney testing. The correlation between duodenal bowel abnormalities and SMA syndrome was examined using Fisher's exact test. Results: AMA, AMD and OI were significantly smaller in the HSP patients (P < 0.001, 0.003 and 0.026, respectively). All HSP patients, but only 10 controls, met the ultrasound diagnostic criteria for SMA syndrome (100% vs 20.8%, P < 0.001). Conclusions: Duodenal compression by SMA against the AA, which mimics SMA syndrome, may lead to duodenal bowel abnormalities with abdominal pain in thin children with HSP.     

Childhood cholelithiasis in a district hospital

Cholelithiasis is a rare finding in children, even though recent series show increased detection of this disease. A retrospective study was performed in children with a diagnosis of cholelithiasis between 1993 and 2005 in the Reina Sofia Hospital in Tudela (Spain). Eighteen patients with cholelithiasis and three with biliary sludge were detected. Predisposing factors for cholelithiasis were prematurity and parenteral nutrition (one patient), sepsis (two patients), obesity (one patient), and a family history of the disease (one patient). The disease was idiopathic in 11 patients. Gallstones were detected in two patients presenting with appendicular symptoms. One child with biliary sludge had received treatment with ceftriaxone as a predisposing factor. All patients were diagnosed by ultrasound. Plain abdominal X-ray detected lithiasis in 12 of the 15 patients (80 %) with cholelithiasis who underwent this procedure. The most frequent symptoms were abdominal pain (seven patients), abdominal pain and vomiting (five patients), and diarrhea (one patient). Two patients presented with appendicular symptoms. Fourteen patients underwent surgery (open cholecystectomy in two and laparoscopic cholecystectomy in 12). None of the patients required emergency surgery. Cholelithiasis in children is an unusual finding, but is not exceptional and is associated with nonspecific symptoms. Plain abdominal X-ray is useful in diagnosis but the main diagnostic technique is ultrasonography.     

Neonatal exchange transfusion with irradiated whole blood. Preliminary results

Some clinical manifestations following exchange transfusion (ET) could result from graft versus host disease secondary to the introduction of viable foreign T lymphocytes: skin rash, fever, acute and sometimes bloody diarrhea or enterocolitis. Between February 1985 and January 1989 the blood used for 31 ET was irradiated at 40 grays. We compared the manifestations occurring during the days following ET to those occurring after 44 previous ET with non irradiated blood during the period January 1981 to January 1985. From 1981 to 1985, 13 of 44 infants developed problems within 3 days following ET: an erythematous macular skin rash in 4; gastrointestinal manifestations (diarrhea, vomiting and rectal bleeding, necrotizing enterocolitis) in 7; both skin lesions and a gastrointestinal problem in 2. Since 1985, 27 infants had no problems whereas only 4 developed gastrointestinal or cutaneous manifestations: NEC in a preterm infant, abdominal distension with rectal bleeding, fever and petechial rash in 2 infected infants. These data show a dramatic decrease of complications since the irradiation of blood products has been started: 30% with non irradiated, 13% with irradiated blood.