[Abdominal symptoms necessitating surgical intervention as the initial presentation of Henoch-Schönlein purpura in children - case reports]. / Objawy brzuszne wymagajace interwencji chirurgicznej inicjujace zespól Schönleina-Henocha u dzieci - opis przypadków.

Henoch-Schönlein purpura (HSP) is the most common pediatric autoimmune vasculitis. Gastrointestinal symptoms of HSP including abdominal pain, diarrhea, and vomiting may precede skin changes by several days. We present diagnostic challenges in two adolescents with HSP and severe abdominal symptoms necessitating surgical intervention before the development of skin changes. CASE REPORT 1: A 15-year old boy with 7 day history of abdominal pain, and bloody vomiting (1-2 x per day) without diarrhea. A suspicion of acute appendicitis was raised and the boy was operated on the 7th day since the initial symptoms. The appendix showed some reactive inflammation and was removed during laparotomy which also revealed enlarged mesenteric lymph nodes and a modest amount of fluid in the pelvic cavity. During the first day after the surgery, skin changes typical for HSP developed on lower limbs and buttocks. CASE REPORT 2: A 12-year old girl with 7 day history of abdominal pain, without diarrhea or vomiting. On the day of admission hemorrhagic rash appeared on lower limbs. Laparotomy was performed on 14th day after onset of abdominal pain - large amounts of serous, blood-stained fluid, massive dilation of small intestine with ecchymoses in mucous membrane, segmental infiltration and stiffening of intestinal wall were found. Normal appendix was removed. CONCLUSIONS: Severe abdominal symptoms may precede skin changes in children with HSP, resulting in diagnostic and therapeutic challenges. When considering laparotomy in children with an atypical "acute abdomen" presentation, other manifestations of HSP should be sought.

Clinical remission of Henoch-Schönlein purpura nephritis after a monotherapeutic tonsillectomy.

We herein describe an adult male patient who presented with tonsillitis, purpura, hematuria, and proteinuria. The serological analyses revealed elevated serum antistreptolysin, and the throat culture yielded Lancefield group A ß-hemolytic streptococci. A renal biopsy revealed mild mesangial proliferation associated with granular mesangial depositions of IgA and C3, consistent with Henoch-Schönlein purpura nephritis (HSPN). Initially, the patient was treated with dipyridamole, which was followed by limited improvements in the proteinuria and hematuria. Nine months later, the tonsillitis relapsed due to GAS infection and deteriorated urinary abnormalities were noticed, which finally disappeared after monotherapeutic tonsillectomy. Although the impact of tonsillectomy on the treatment of HSPN remains to be characterized, our observations suggest that tonsillectomy in the present patient played a pivotal role in facilitating the recovery of the renal injuries. Because the arbitrary application of a tonsillectomy appears to be accompanied by ethical concerns, the evaluation of the clinical benefits of this procedure should be addressed more directly in the future.

Successful therapy with tonsillectomy for severe ISKDC grade VI Henoch-Schönlein purpura nephritis and persistent nephrotic syndrome.

Henoch-Schönlein purpura (HSP) is a systemic disorder characterized by leukocytoclastic vasculitis involving the capillaries and the deposition of IgA immune complexes. Renal involvement is the principal cause of morbidity and mortality in children with HSP. We report here a 13-year-old girl with Henoch-Schönlein purpura nephritis (HSPN) of International Study of Kidney Disease in Children (ISKDC) grade VI and persistent nephrotic syndrome despite receiving conventional therapy, such as prednisolone, methylprednisolone and urokinase pulse therapy and plasmapheresis (PP). The patient was treated with tonsillectomy, which subsequently decreased proteinuria, induced the disappearance of microscopic hematuria, and improved renal pathological findings. A regimen of methylprednisolone and urokinase pulse therapy plus PP with tonsillectomy may be an effective and useful therapy for some children with severe HSPN children of ISKDC grade VI and persistent nephrotic syndrome.

Ciprofloxacin-induced hemorrhagic vasculitis.

Ciprofloxacin-induced hemorrhagic vasculitis is a rare side effect. It has only been described in 10 case reports since 1989. However, recently we were confronted with two cases within 1 month. In one patient the vasculitis resolved after termination of the ciprofloxacin therapy; in the other patient the ciprofloxacin-induced hemorrhagic vasculitis was superimposed on a severe forefoot infection, leading to progressive gangrene and a below-knee amputation. Ciprofloxacin is among the standard treatments for infected ischemic ulcers; in the rare case of ciprofloxacin-induced hemorrhagic vasculitis, it might be interpreted as progression of infection, instead of a complication of the treatment, thus leading to faulty diagnosis and treatment. The intention of this case report is to raise awareness for anyone prescribing ciprofloxacin as treatment for infected diabetic and ischemic ulcers.

Updated Oxford classification and the international study of kidney disease in children classification: application in predicting outcome of Henoch-Schönlein purpura nephritis.

BACKGROUND: Henoch-Schönlein purpura nephritis (HSPN) shares many similarities with IgA nephropathy. We aimed to analyze the predictive value of the International Study of Kidney Disease in Children (ISKDC) classification and the updated Oxford classification for IgA nephropathy in HSPN patients. METHODS: Data of 275 HSPN patients (aged≥14 years) were retrieved, and all of them underwent a renal biopsy. We re-classified the biopsies according to the ISKDC classification and the updated Oxford classification to analyze their correlations with clinical features and renal outcomes. The renal endpoints were defined as ≥30% reduction in baseline estimated glomerular filtration rate (eGFR) in 2 years, doubling of serum creatinine (Scr) or end stage renal disease. RESULTS: During follow-up period of 56(30,86) months, 30(10.9%) patients reached renal endpoints. Segmental sclerosis was the only pathological feature independently associated with renal endpoints (HR 4.086, 95%CI 1.111-15.026, P = 0.034). Tubular atrophy/ interstitial fibrosis was associated with eGFR and Scr levels, and its correlation with renal endpoints was found by univariate analysis. Endocapillary hypercellularity was associated with 24 h urine protein and is of prognostic value in univariate analysis. Mesangial hypercellularity was not associated with clinical features or renal endpoints. Crescents were associated with 24 h urine protein, Scr and eGFR levels, but both ISKDC and updated Oxford classifications of crescents were not associated with renal endpoints by multivariate analysis. CONCLUSIONS: The updated Oxford classification can help in disease management and renal outcome prediction of HSPN.

Renal transplantation in adults with Henoch-Schonlein purpura: long-term outcome.

BACKGROUND: Little information is available about the long-term outcome of renal transplantation in adults with Henoch-Schonlein purpura (HSP). METHODS: We compared the outcomes of 17 patients with HSP who received 19 renal transplants with those of 38 controls matched for time of transplantation, age, gender and source of donor. The mean post-transplant follow-up was 109 +/- 99 months for HSP patients and 110 +/- 78 months for controls. RESULTS: The actuarial 15-year patient survival was 80% in HSP patients and 82% in controls, and the death-censored graft survival was 64% in HSP patients and in controls. The risks of acute rejection, chronic graft dysfunction, arterial hypertension and infection were not different between the two groups. In eight grafts (42%) recurrence of HSP nephritis was found (0.05/patient/year). In spite of therapy, one patient died and four eventually restarted dialysis respectively 10, 32, 35 and 143 months after renal transplant. Seventy-one percent of grafts transplanted in patients with necrotizing/crescentic glomerulonephritis of the native kidney had HSP recurrence in comparison to 12% of recurrences in patients with mesangial nephritis (P = 0.05) CONCLUSIONS: Long-term patient and allograft survival of HSP patients was good. However, 42% of HSP patients, particularly those with necrotizing/crescentic glomerulonephritis of the native kidneys, developed a recurrence of HSP nephritis that eventually caused the loss of the graft function in half of them.

Tonsillectomy in the treatment of pediatric Henoch-Schönlein nephritis.

The exact pathophysiology of HSN remains to be elucidated. Hence, a therapeutic strategy that enables curative treatments for all the various grades of HSN patients has yet to be established. We report our experience performing tonsillectomy combined with steroid therapy for 16 pediatric proteinuric Henoch-Schönlein nephritis (HSN) patients. All patients exhibited hematuria and proteinuria in their first HSN attack with the mean age of onset 7.7 years (range 4.75 - 13.9 years). Nine patients were diagnosed with clinically severe HSN presenting with massive proteinuria (> 1 g/m(2)/day). Renal biopsy findings performed in 6 patients were Grade II (3), Grade III (2) and Grade IV (1) according to the International Study of Kidney Diseases in childhood classification. Tonsillectomy was performed after 1-4 cycles of methylprednisolone pulses during oral prednisolone (0.5 - 1.5 mg/kg/day) therapy. In 2 patients, oral cyclophosphamide therapy was added before the tonsillectomy. The interval between the onset of HSN and tonsillectomy was 97.4 +/- 24.5 days (range 27 424 days). In all patients, proteinuria had disappeared by 6 months after the tonsillectomy and the urine findings had normalized. The interval between therapy initiation and complete remission was 9.6 +/- 2.0 months (range 2 - 26 months). Over follow-up periods of 4.9 +/- 0.6 years (range 2.2 - 9.3 years), no recurrences of Henoch-Schonlein purpura or HSN were observed. There was a significant correlation between early tonsillectomy performance and decreased time until normalization of the urine findings, indicating that the tonsils may have pivotal roles in the initiation and progression of HSN. Their elimination might promote the reversal of nephritis. Although this study is retrospective, we suggested that tonsillectomy at an early stage of HSN may be beneficial by shortening the period of illness and contributing to clinical recovery. Randomized controlled trials will be needed to confirm this supposition.

Meningococcal septicaemia complications involving skin and underlying deeper tissues--management considerations and outcome.

OBJECTIVE: To describe surgical experience with purpura fulminans related to meningococcaemia in a single institution, and to suggest a management protocol. METHODS: A retrospective review was done of patients admitted to the intensive care unit at Red Cross War Memorial Children's Hospital in Cape Town with the clinical diagnosis of purpura fulminans. RESULTS: During a 28-year period (1977-2005) 112 children (average age 3.4 years) were treated for meningococcaemia with purpura fulminans. Overall mortality was 10.7%. Local treatment consisted of measures to improve circulation, infection control and healing of necrotic tissue. Demarcation of necrotic areas was evident at 5.5 days and the average area of skin necrosis was 14% total body surface area (range 2-85%). The lower limbs were predominantly affected. Purpura fulminans resolved in 35 children (31.2%) without skin necrosis. Skin grafting was required in 77 children (68.8%). Factors associated with a poor outcome for peripheral extremity salvage were progressive irreversible skin changes, early disappearance of distal pulses, tense cold swollen extremities and intense pain on passive movement of the affected extremity. Amputations were performed proximal to the area of necrosis, on average 27 days after injury. CONCLUSIONS: Meningococcaemia is a disease with potentially devastating consequences. Early surgical consultation is essential. Skin- and soft-tissue-releasing incisions should be considered early to reduce the incidence of extremity necrosis. Small necrotic areas usually separate spontaneously with secondary healing or can be excised and sutured. Larger necrotic areas should be excised only after demarcation has been established, and can be covered with delayed skin grafting. Amputation should be conservative but may require revision.

[Treatment of Henoch-Schönlein purpura nephritis in children with tonsillectomy].

Henoch-Schönlein purpura nephritis (HSPN) is an important complication of Henoch-Sch6nlein purpura (HSP). Pathological findings in the kidney are similar to those for IgA nephritis, which is characterized by the deposition of IgA immune complex in the glomerular mesangium. Since a tonsillectomy is useful for IgA nephritis, this procedure holds great promise for the treatment HSPN. In the present study, we assessed the effectiveness of a tonsillectomy in children with HSPN whose conditions could not be controlled by medication, including predonisone and cyclophosphamide. Seven patients (four boys and three girls) with histologically well-defined HSPN who had undergone a tonsillectomy between 1998 and 2000 and who had been followed for more than 6 postoperative months were retrospectively examined. The age of the patients ranged between 3 and 13 years (average, 7.6 +/- 3.2 years) at the time of operation. Postoperative changes in urinary data were assessed according in the severity of the pathological findings in the kidney and the patient's clinical condition. The severity of the pathological findings in the kidney was determined based on the classification of the International Study of Kidney Disease (ISKDC) and the Index of Glomeruler Lesion (IGL). All patients examined in the present study had an ISKDC classification of over grade II. One patient had a grade IV classification, 3 had a grade III classification, and 3 had a grade II classification. The patients were placed in one of five clinical groups: (1) nephritic-nephrotic syndrome, (2) acute nephritic syndrome, (3) nephritic syndrome, (4) over 1 g/day of proteinuria without hypoalbuminemia or oedema, or 5) below 1 g/day of proteinuria with or without hematuria. One patient was classified in group 1, 1 was group classified in 2, 2 were classified in group 3, 2 were classified in group 4 and 1 was classified in group 5. The mean observation period was 74 +/- 6.4 months. The hematuria and proteinuria resolved in all patients, regardless of their preoperative pathological or clinical severity, within 12 postoperative months. During the observation period, no relapse was observed. Moreover, all medication, including steroid use, was stopped within the observation period. Therefore, a tonsillectomy was considered to be effective for the treatment of children with HSPN whose conditions cannot be controlled using medication.

Course of Henoch-Schönlein nephritis after renal transplantation. Report on ten patients and review of the literature.

The frequency and the risk factors for clinical recurrence of Henoch-Schönlein nephritis following renal transplantation (TP) remain largely unknown. We report on 14 transplants performed at our center in 10 patients, detail the evolution of 2 of them with clinical recurrence, and review 64 other transplants reported in the literature. In our series, all patients are currently alive. Seven grafts are well-functioning 22-295 (mean, 97) months after TP without any sign of clinical recurrence. Five grafts were lost from rejection. Clinical recurrence occurred in 2 patients who were on cyclosporine/azathioprine/prednisone therapy. Pooling our series with that of Hasegawa et al., the actuarial risk for renal recurrence and for graft loss due to recurrence was 35 and 11% at 5 years after TP, respectively. In our series, duration of original disease was 2 and 28 months in the 2 patients with recurrence versus 31-144 months in the others without recurrence. In the literature, this duration was < or = 36 months in all 7 patients with recurrence. Recurrence occurred despite a > 12-month delay between disappearance of purpura and TP in our 2 patients and in 3 of 6 previously reported recurrences. We conclude that Henoch-Schönlein purpura nephritis frequently recurs after TP. Recurrence (1) seems to be associated with a shorter duration of the original disease, (2) can occur despite a delay of more than 1 year (as commonly advised) between disappearance of purpura and TP, and (3) is not prevented by a triple immunosuppressive regimen that includes cyclosporine.

Surgical evaluation of Henoch-Schönlein purpura. Experience with 110 children.

Henoch-Schönlein purpura is a disorder of unknown origin that is probably related to an autoimmune phenomenon. This report concerns 110 children (mean age, 6.2 years; range, 6 months to 14 years) with Henoch-Schönlein purpura. Seventy-two (65%) had abdominal pain associated with nausea and vomiting, bloody stool, and upper gastrointestinal bleeding. Sixty patients with abdominal pain were evaluated and treated conservatively. However, 12 patients underwent laparotomy. Six underwent unnecessary appendectomy for wrongly diagnosed appendicitis. Bowel resection was performed in one patient for an obstructive ileal lesion. Six additional patients had intussusception; surgery was required in three, while barium enema reduction was successfully accomplished in three others. Massive gastric hemorrhage required ligation, vagotomy, and pyloroplasty in two instances. One child with severe scrotal pain, hemorrhage, and swelling underwent unnecessary scrotal exploration. Four additional patients with similar symptoms avoided operation after a testicular scintiscan demonstrated good blood flow. A high index of suspicion and early diagnosis of Henoch-Schönlein purpura based on clinical, roentgenographic, and laboratory findings may avoid unnecessary operations in most cases. However, life-threatening complications (hemorrhage, obstruction, and intussusception) may occur and require operative intervention. All of the patients survived.

Necrotizing group A streptococcal infections associated with streptococcal toxic shock syndrome.

BACKGROUND: Group A streptococci (GAS) cause a variety of life-threatening infectious complications, including necrotizing fasciitis (NF), purpura fulminans (PF), and streptococcal toxic shock syndrome (strepTSS), in which bacteremia is associated with shock and organ failure. METHODS: We reviewed our experience in the management of patients with necrotizing GAS infections from 1991 to 1995. RESULTS: Eight adult patients (6 NF, 2 PF) were identified. Patients presented with fever, leukocytosis, and severe pain, and rapidly developed shock and organ dysfunction. The diagnosis of strepTSS was confirmed in 6 cases. A total of 54 surgical procedures were required, including widespread debridements and amputations. Two patients died (25%). CONCLUSIONS: Recognition of the need for aggressive diagnosis and surgical treatment of this most rapidly progressive surgical infection is necessary for successful management.

Acute infectious purpura fulminans: a 15-year retrospective review of 28 consecutive cases.

Acute infectious purpura fulminans (AIPF) is a rare syndrome of hemorrhagic infarction of the skin, extremity loss, and intravascular thrombosis. It progresses rapidly and is accompanied by disseminated intravascular coagulation and vascular collapse. The victims often succumb to the disease. Our objective was to investigate the clinical manifestations, outline the clinical course, and delineate factors related to mortality among the patients with AIPF. Patients diagnosed with AIPF over a 15-year period were reviewed retrospectively for patient history, comorbid conditions, progression of clinical course, and medical and surgical management. The vast majority of the patients were under the age of 7; however, the disease process can be seen in adults. The overall mortality rate was 43 per cent. The major predisposing factors were history of recent upper respiratory infection, recent surgery or childbirth, young age, and absence of a spleen. The most common clinical manifestations were skin discoloration, disseminated intravascular coagulation, fever, and septic shock. The most common bacteria cultures were Neisseria meningitidis, Hemophilus influenzae, and Streptococcus pneumoniae. There appears to be a higher mortality in patients who did not undergo a surgical intervention. Compartment syndrome needs to be evaluated early on in the presentation. Rapid diagnosis, intensive care unit management, and prompt surgical consultation and debridement may decrease the mortality.

Free-tissue transfer for limb salvage in Purpura fulminans.

A series of 13 patients is described to demonstrate the experience of the authors with free-tissue transfer for limb salvage in patients with purpura fulminans. A total of seven free-flap procedures were performed, with a loss of flap in one patient. The flaps were used for lower-extremity salvage in six patients and for upper-extremity salvage in one. Purpura fulminans is a devastating illness caused by endotoxin-producing bacteria such as meningococcus and pneumococcus. Clotting derangements and systemic vasculitis often lead to widespread tissue necrosis in the extremities. Local tissue is usually not available to cover vital structures in these complex wounds. In these situations, free-tissue transfer is necessary to achieve limb salvage. Microsurgical reconstruction in patients with purpura fulminans is a formidable challenge. Because of high platelet counts and systemic vasculitis, successful microvascular anastomosis is difficult. Abnormally high platelet counts persist well into the subacute and chronic phases of the illness. Pretreatment with antiplatelet agents before microvascular surgery may be beneficial. The systemic nature of the vascular injury does not permit microvascular anastomosis to be performed outside the "zone of injury." Extensive vascular exposure, even at a great distance from the wound, does not reveal a disease-free vessel. The friable intima is difficult to manage with a standard end-to-side anastomosis, but conversion to end-to-end anastomosis may salvage free-tissue transfers in cases in which intimal damage is too severe to sustain a patent anastomosis. Patients often have peripheral neuropathies caused by the underlying disease; however, this resolves with time and is not a contraindication to limb salvage.

Massive infectious soft-tissue injury: diagnosis and management of necrotizing fasciitis and purpura fulminans.

LEARNING OBJECTIVES: After studying the article, the participant should be able to: 1. Describe the most common bacteriology of necrotizing fasciitis and purpura fulminans. 2. Describe the "finger test" in the diagnosis of necrotizing fasciitis. 3. Discuss the three presentation patterns of necrotizing fasciitis. 4. Discuss the pathophysiology of acute infectious purpura fulminans. 5. Discuss the treatment strategies for necrotizing fasciitis and purpura fulminans, including the use of artificial skin substitutes. Necrotizing fasciitis and purpura fulminans are two destructive processes that involve skin and soft tissues. The plastic and reconstructive surgeon may frequently be called on for assistance in the diagnosis, treatment, and/or reconstruction of patients with these conditions. Understanding the natural history and unique characteristics of these processes is essential for effective surgical management and favorable patient outcome. A comprehensive review of the literature pertaining to these two conditions is presented, outlining the different pathophysiologies, the patterns of presentation, and the treatment strategies necessary for successful management of these massive infectious soft-tissue diseases.

Surgical implications of Henoch-Schönlein purpura.

Intestinal intussusception, perforation, necrosis, and massive gastrointestinal bleeding are the surgical conditions most often seen in Henoch-Schönlein purpura (HSP). We reviewed the charts of 183 patients who were diagnosed with HSP at two children's hospitals over a 17-year period. Ten patients underwent laparotomy. Intussusception was the most common surgical lesion. Sequential barium enema and upper gastrointestinal series established the diagnosis of intussusception preoperatively in five of six patients. Two patients developed jejunal perforation and segmental ileal ischemia. Although gastrointestinal bleeding occurred in 52% of patients, the bleeding was self-limiting and no blood transfusion was required. Laboratory data were not helpful in differentiating patients with and without surgical problems.

Reconstructive surgery in children after meningococcal purpura fulminans.

BACKGROUND/PURPOSE: Purpura fulminans (PF) is a serious, often life-threatening disease. As more children are surviving their disease, surgeons are presented with increasing numbers of multiple and complicated wounds as sequelae of PF. The purpose of this paper is to review the management of nine cases of PF, and present the reconstruction method in treating bilateral exposed elbow and knee joints. METHODS: All cases of pediatric patients with PF and treated by the division of plastic and reconstructive surgery between 1986 and 1998 were reviewed. RESULTS: Seven children (78%) had meningococcal PF, and one (11%) had PF after Haemophilus influenza septicemia. PF developed in one (11%) but with no growth in either blood or cerebrospinal fluid cultures. Five children (56%) required amputation procedures. Two children (22%) required knee disarticulation. Two patients (22%) had free myocutaneous flap transfers for bone coverage. One (11%) had PF involving the face. CONCLUSIONS: Meningococcal PF is a rare, often life-threatening disease generally of childhood. More children are surviving their diseases but with devastating sequelae. Successful reconstructive treatment outcome of these children requires a multidisciplinary team approach involving multiple specialties. The goal is to preserve function, maintain maximal length, and salvage limbs when possible. Flexibility and innovation are necessary in treating these multiple and complicated wounds.

Massive gastric hemorrhage: an unusual complication of Henoch-Schönlein purpura.

Two severely ill children with multisystem involvement of Henoch-Schönlein purpura developed massive upper gastrointestinal hemorrhage, requiring operative ligation of gastric stress ulcers, and vagotomy and pyloroplasty. Arteriographic embolization was also used in one of the children when she developed recurrent hemorrhage. Survival in both children emphasizes the need for aggressive management of the many complications these children may develop.

Integra Artificial Skin as a useful adjunct in the treatment of purpura fulminans.

Purpura fulminans is a devastating disorder characterized by rapidly progressing hemorrhagic necrosis of the skin, vascular collapse, and disseminated intravascular coagulation. It is most often seen in children, and it is usually preceded by meningococcemia or another infection. Most often, the disorder results in severe skin loss, but it can also result in the need for extremity amputations. In extreme cases, wound coverage after excision may be problematic because of the limited existence of donor sites and the need for amputation revisions. The case of a 21/2-year-old male requiring amputations of all four extremities due to severe purpura fulminans is presented to illustrate the use of Integra Artificial Skin (Integra Lifesciences Corp., Plainsboro, NJ) to obtain immediate wound closure. Integra Artificial Skin is a bilayered skin substitute that engrafts to a viable wound bed. In the case presented here, where the viability of the underlying tissue of the amputated stumps was questionable, the artificial skin acted as an indicator of that viability. It engrafted well onto the upper extremity stumps, which were of excellent viability, but it needed to be replaced on the lower extremity stumps, which required further debridement and amputation revisions. The use of artificial skin spared the patient the immediate use of his limited and valuable autograft sites. In conclusion, Integra Artificial Skin can be a useful adjunct in the treatment of severe purpura fulminans that includes skin and extremity necrosis.

Management of cutaneous manifestations of extensive purpura fulminans in a burn unit.

Purpura fulminans is a rare disease, which may have devastating cutaneous manifestations. It usually follows an infectious illness, and although it most commonly occurs in children, it can occur in adults. The pathogenesis may be related to a relative deficiency of protein C and/or protein S. A case of an adult male is presented to illustrate the management of the severe full-thickness skin loss and the use of surgical excision and allograft in this disease.