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.

[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.

Pattern of primary vasculitis with peripheral ischemic manifestations: report of a case series and role of vascular surgery.

AIM OF THE WORK: The aim of the present work was to study the role of vascular surgery in the management of primary vasculitis patients with peripheral ischemic manifestations. PATIENTS AND METHODS: Ten primary vasculitis patients with peripheral ischemic manifestations were studied and reviewed for the diagnosis, clinical manifestations, investigations, treatment options and role of vascular surgery. The Birmingham Vasculitis Activity Score (BVAS) was recorded. RESULTS: Giant cell arteritis was present in one patient; granulomatosis with polyangiitis in 5, essential cryoglobulinemic vasculitis in 3 and 1 (child) had Henoch-Schönlein purpura. They showed the following peripheral vascular manifestations: intermittent claudications, Raynauds, deep venous thrombosis and thrombophelebitis in 10% each; digital ulceration and trophic changes in 20% while acrocyanosis and dry gangrene were present in 30%. Renal involvement was present in 60% of patients. The mean BVAS was 11.5 ± 6.57 at initial presentation. The disease activity remarkably improved over the disease course in all patients to be at their last visit (2.6 ± 2.22) (p=0.002). Regarding the vascular surgery role in their management, in addition to their medical treatment, 40% required an additional surgical intervention. Two had a minor amputation of the toes; one performed thoracoscopic cervical sympathectomy and another needed tibial angioplasty. CONCLUSION: Primary vasculitis patients presenting with peripheral ischemic manifestations require surgical attention. Their management is essentially medical and individualized to the diagnosis and presenting symptoms. Endovascular treatment may offer a safe and less invasive approach in high surgical risk patients. Sympathectomy is of high therapeutic potential in those with severe pain and trophic changes.

[Treatment of thoracic and abdominal cavity perforation complicated by Henoch-Schonlein purpura nephritis in a patient with high-voltage electric burn].

A 55-year-old male patient suffered from severe high-voltage electric burn with an area of 20%TBSA full-thickness injury. The injury involved the distal end of left upper limb, right trunk, and whole abdominal wall. Fracture of the 7th-10th ribs was found in the right side of chest, with perforation of abdominal cavity, and bilateral pleural effusion was found. Part of the small intestine was necrotic and exposed. At the early stage, xeno-acellular dermal matrix was grafted after debridement of abdominal wound; peritoneal lavage was performed; negative pressure drainage was performed in orificium fistula of intestine for promoting the adhesion between perforated intestine and abdominal scar. Two orificium fistulas formed after closure of abdominal granulation wound by autologous skin grafting. Eschar of chest wall and denatured ribs were retained. The risk of infection of thoracic cavity was decreased by promoting the adhesion between lung tissue and chest wall. During the treatment, the patient was diagnosed with Henoch-Schonlein purpura nephritis by renal biopsy, with the symptoms of purpura in the lower limbs, heavy proteinuria, severe hypoalbuminemia, edema, etc. After control of kidney damage by immunosuppressive treatment instead of glucocorticoid, alleviation of the levels of proteinuria and blood albumin, free latissimus dorsi myocutaneous flap was excised to repair chest wall, and free skin graft was excised to repair intestinal fistula. After all the wounds were successfully covered, the patient was treated with glucocorticoid combined with immunosuppressants for more than 1 year. The patient was followed up for 3 years, and his renal function was completely recovered with satisfactory clinical outcome.

Long-term outcome of renal transplantation patients with Henoch-Schonlein purpura.

BACKGROUND AND OBJECTIVES: Although Henoch-Schönlein purpura (HSP) is the most common form of renal vasculitis in childhood, progression to ESRD is rare, and there are few data on outcomes of renal transplantation in patients with HSP. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: This is a matched retrospective cohort study of renal allografts using the United Network of Organ Sharing database (1987 to 2005). Of the 189,211 primary renal allografts, there were 339 with a diagnosis of HSP. The primary end point was allograft survival. RESULTS: Compared with the remainder of the database, the HSP population was younger (25 years versus 46 years), and had a higher proportion of women (47% versus 40%), live donors (50% versus 35%), and Caucasians (77% versus 60%). Controlling for age, gender, donor source, ethnicity, and year of transplantation, death-censored graft survival for patients with HSP was 80.0% at 5 years and 58.8% at 10 years compared with 79.0% at 5 years and 55.4% at 10 years in the non-HSP population. Among patients with reported causes of graft loss, failure from recurrent disease occurred in 13.6% of patients with HSP, compared with 6.6% in the non-HSP population. When analyzing allograft survival in recipients with HSP compared with those with IgA nephropathy, there was no difference in 10-year allograft survival (58.4% and 59.3%, respectively). CONCLUSIONS: These data indicate that although there is an increased risk of graft failure attributable to recurrent disease in patients with HSP, a diagnosis of HSP has little effect on overall renal allograft survival.

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.

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.

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.

Púrpura de Henoch-Schõnlein com acometimento incomum de face / Unusual face involvement in Henoch-Schõnlein purpura

A Púrpura de Henoch-Shõnlein é uma vasculite de causa idiopática, que se caracteriza pelo depósito predominante de IgA na parede dos pequenos vasos, envolvendo tipicamente pele, intestino, articulações e glomérulo renal. O acometimento cutâneo ocorre principalmente em membros inferiores e região glútea, sendo raramente encontrado em face e membros superiores. Relatamos o caso de uma paciente do sexo feminino, com seis anos de idade, que apresentava lesões púrpuricas em região auricular, periorbital, malar, mentoniana e membros superiores, além de artralgia e volvo intestinal. Após tratamento cirúrgico e pulsoterapia com glicocorticoide, houve regressão dos sintomas, sem maiores complicações.

Henoch-Schõnlein Purpura is an idiopathic vasculitis characterized by deposits of immunoglobulin, mainly IgA, on the walls of small vessels, typically involving the skin, gut, joints, and renal glomeruli. Cutaneous involvement affects specially the lower limbs and buttocks, and it is seldom found on the face and upper limbs. We report the case of a 6-year old girl with purpuric lesions over the auricular, periorbital, malar, and mentonian regions and the upper limbs, arthralgia, and intestinal torsion. After surgical treatment and pulse therapy with glucocorticoids, her symptoms subsided without further complications.

Efficacy of early dental and ENT therapy in preventing nephropathy in pediatric Henoch-Schönlein purpura.

In a previous study, we demonstrated the benefit of tonsillectomy for early recovery from Henoch-Schönlein purpura (HSP) nephritis (HSPN), suggesting the pathological role of tonsils in HSP (Inoue et al., Clin Nephrol 67:298-305, 2007). In this study, we evaluated the efficacy of extensive eradication of infectious foci directly connected to the tonsils, including those involved in oral as well as ear, nose, and throat (ENT) diseases, in reducing the nephropathy in HSP. For this purpose, we examined the focal points of infection in 40 newly diagnosed HSP patients. After these focal points of infection had been identified, they were extensively eradicated; when the clinical course was intractable, we also considered tonsillectomy. After administering such therapy to HSP patients, we prospectively followed them up for 0.6 to 8 years. The identified focal infections included dental caries in 28 (70%), apical periodontitis in 21 (53%), rhinosinusitis in 19 (48%), tonsillitis in five (13%), and otitis media in four (10%) of the 40 patients. Seventeen patients (43%) had more than two simultaneous infectious foci, whereas, in five (13%), no infectious focus was found. In 32 patients, antimicrobial treatment with concurrent dental and/or ENT therapy resulted in a complete cure without development of HSPN or recurrent attacks. In eight patients, we performed tonsillectomy-adenotonsillectomy to treat their clinical symptoms, including aggravated purpura and recurrent attacks of HSP or HSPN. All patients were completely cured. The overall incidence of HSPN was only three out of the 40 patients (8%). Oral and ENT diseases were found with high percentages in HSP patients. Early and extensive treatment for these lesions and tonsillectomy-adenotonsillectomy for intractable cases may prevent the complication of HSPN, contributing to the early curing of HSP.

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.

Purpura fulminans: a case series managed at a regional burn center.

Purpura fulminans (PF) is a protein C deficiency disease process with a high case fatality rate; however, overall incidence of the disease remains relatively very low. The similarity between skin necrosis secondary to PF and full-thickness skin burns provides the rationale for treating PF case in a burn center. In this case series we reviewed our experiences in managing PF and their associated favorable outcomes. Retrospective chart review of five PF cases managed between September 2004 and August 2006 at our Burn Center with 100% survival. Management of cases following the standard care of the Burn Center for a full-thickness burn included antibiotics, fluid resuscitation, surgical debridement with skin grafting, and activated protein C (Drotrecogin alfa) replacement. Two patients required amputations of extremities and all had surgical debridement. One required hemodialysis and two needed both hemodialysis and positive-pressure mechanical ventilator. No patient experienced any bleeding complications during or after surgery while receiving activated protein C. Early diagnosis and treatment at a burn center may reduce mortality and morbidity and loss of extremities in PF cases.

The use of artificial dermis (Integra) and topical negative pressure to achieve limb salvage following soft-tissue loss caused by meningococcal septicaemia.

Meningococcal sepsis leading to purpura fulminans leaves survivors with extensive areas of skin and soft-tissue necrosis. Soft-tissue loss in the limbs may result in large areas of exposed bone, leaving a choice between free tissue transfer and amputation. We present a case of meningococcal sepsis where the entire medial and lateral surfaces of the tibiae were exposed with loss of anterior muscle compartments on each side. Faced with the possibility of bilateral above-knee amputation, these were instead covered using the dermal replacement Integra (Integra LifeSciences Corp.), in conjunction with an antimicrobial dressing, topical negative pressure dressing and subsequent skin grafting. This management decision achieved rapid wound closure avoiding amputation. Additional secondary reconstruction with microvascular free flaps was performed to preserve joint function.

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.

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.

Efficacy of tonsillectomy plus methylprednisolone pulse therapy for a child with Henoch-Schoenlein purpura nephritis.

Henoch-Schoenlein purpura (HSP) is a systemic disorder characterized by a leukocytoplastic vasculitis involving small vessels with the deposition of immunoglobulin A (IgA) immune complexes. Renal involvement is the principal cause of morbidity and mortality in children with HSP. We report here an 11-year-old boy with Henoch-Schoenlein purpura nephritis (HSPN) accompanied by recurrent purpura and persistent nephropathy despite conventional therapy such as prednisolone, methylprednisolone pulse therapy and immunosuppressive agent (Mizoribine). The patient was treated with tonsillectomy plus methylprednisolone pulse therapy. This treatment decreased proteinuria, induced disappearance of microscopic hematuria, and improved renal pathological findings. Tonsillectomy plus methylprednisolone pulse is effective and useful therapy for some children with recurrent purpura and persistent nephropathy.