591 ated primitive postures are note worthy. David et al.

found dystonic manifestations in 2 out of 50 cases reported in the literature [3]. We reviewed 78 cases of SNE with onset before the age of two and found 8 cases with extrapyramidal manifestations [1, 3-5, 7, 9, 10]. The patient of De Vivo et al. was the youngest a 4-month-old infant with hypotonia, spasticity and choreoathetoid movements which progressed until death at 7 months [5]. In the other seven cases the extrapyramidal manifestation began after the age of 8 months. Extrapyramidal symptoms are common in some metabolic disturbances, including pyruvate carboxylase deficiency, but are rarely mentioned as a symptom of SNE. Elevated blood lactate and pyruvate are usually found, especially during the acute episodes, but normal biochemical findings do not exclude the diagnosis of SNE [2, 8, 9, 10]. The CT scan findings are particularly suggestive of SNE. Hall and Gardner-Medwin first reported four cases with symmetrical low density areas in the lenticular nuclei, as in our observation [6]. On the other hand bilateral areas of low density of the basal ganglia are reported in other diseases [2]. The histological findings of necrosis, spongiosis, capillary proliferation with partial neuronal conservation affecting the basal ganglia and the brainstem but sparing the mammillary bodies confirm the diagnosis of SNE [1, 4, 8]. 7. McAndless DW, Hodgkin WE (1977) Subacute necrotizing encephalomyelopathy (Leigh disease). Pediatrics 60:935937 8. McBurney M, Leigh D, McIlwein H (1980) Erythrocyte transketolase activity in suspected cases of Leigh's disease. Arch Dis Child 55 : 789-794 9. Montpetit VJA, Andermann F, Carpenter S, Fawcett JS, Zborowska-Slius D, Giberson HR (1971) Subacute necrotizing encephalomyelopathy, a review and a study of two families. Brain 94 : 1-30 10. Pincus JH (1972) Subacute necrotizing encephalomyelopathy (Leigh's disease): a consideration of clinical features and etiology. Dev Med Child Neurol 14: 87-101

Received July 21, 1983 / Accepted June 17, 1985

Eur J Pediatr (1986) 144 : 591-593

Urinary microscopy in the diagnosis of haematuria in Sch6nlein-Henoch purpura

A. Martini ~, A. Ravelli 1, and G. Beluffi 2
1Pediatric Department of the University and 2Pediatric Radiodiagnosis Section, IRCCS "Policlinico S. Matteo", Pavia, Italy

References
1. Adams RD, Lyon G (1982) Neurology of hereditary metabolic diseases of child. McGraw-Hill Book Company, Washington New York London, pp 73-82 2. Campistol J, Fernandez-Alvarez E, Cusi V (1984) CT scan appearance in subacute necrotizing encephalomyelopathy. Dev Med Child Neurol 26 : 519-522 3. David RB, Gomez MR, Okazaki H (1970) Necrotizing encephalomyelopathy (Leigh). Dev Med Child Neurol 12:436445 4. Dayan AD, Ockenden BG, Crome L (1970) Necrotizing encephalomyelopathy of Leigh. Neuropathological findings in 8 cases. Arch Dis Child 45 : 3945 5. De Vivo DC, Haymond MW, Obert KA, Nelson JS, Pagliara AS (1979) Defective activation of the pyruvate dehydrogenase complex in subacute necrotizing encephalomyelopathy (Leigh disease). Ann Neurol 6 : 483494 6. Hall K, Gardner-Medwin D (1978) CT scan appearances in Leigh's disease. Neuroradiology 16 : 48-50

Abstract. A 5.5-year-old boy with Sch6nlein-Henoch purpura presented flank pain, macroscopic haematuria and voiding of blood clots. Radiologic examination showed a stenosing ureteritis. Treatment with prednisone was accompanied by resolution of the urologic manifestations. Ureteritis is a potentially serious complication of Sch6nlein-Henoch purpura and its incidence is probably underestimated. Less than half of the reported patients have had symptoms suggesting a urologic complication. Microscopic examination of the urinary sediment can be helpful for the precocious diagnosis of ureteritis in the Sch6nlein-Henoch purpura. Key words: Sch6nlein-Henoch purpura Macroscopic haematuria - Stenosing ureteritis - Urinary microscopy

renal involvement, usually mild, is frequent and has been reported in 2 0 % 100% of the cases depending on the authors and the criteria for renal involvement [6]. In contrast involvement of the urinary tract appears to be very unusual, although the true incidence and spectrum of this complication remains to be established. We report here the occurrence of acute ureteritis in a patient with SHP and suggest that microscopic examination of the urinary sediment can be helpful for the precocious diagnosis of urologic complication in SHP.

Case report
C.Z., a 5.5-year-old boy, was admitted with a history of 3 days of pain and swelling of the ankles and wrists, 2 days of multiple petechiae on the lower limbs and i day of recurrent colicky abdominal pain. Eight days prior to hospitalisation he presented a mild sore throat with low grade fever. On physical examination the abdomen was diffusely tender, the knees and ankles were swollen, hot and tender, and a purpuric rash was present on the lower limbs and buttocks. Blood pressure was normal. Pertinent laboratory findings were as follows: white cell count 11200/mm 3, haemoglobin 13.3 g/ dl, platelet count 238000/mm 3, total serum proteins 6.9 g/dl, serum albumin 3.7 g/dl, serum creatinine 0.6 mg/dl, creatinine clearance 102 ml/min per 1.73

Introduction
Sch6nlein-Henoch purpura (SHP) is characterised by a purpuric skin rash, joint pain and abdominal symptoms;
Offprint requests to: Alberto Martini, M.D., Department of Pediatrics, University of Pavia, Policlinico S. Matteo P. le Golgi, 27100 Pavia, Italy Abbreviations: SHP = Sch6nlein-Henoch

purpura; IVP = excretory urogram

592

Fig. 1. IVP. Right kidney dilatation. Tapering of the right ureter, which is dilated with irregular smooth filling defects. Only a few small filling defects are detectable in the upper portion of the left ureter, which is normal in size

m 2. Urinalysis showed 2+ albumin and 20-30 red cells per high power field; more than 80% of the red cells in the sediment had conventional morphology; they were uniform in size and shape and not distorted. Serum immunoglobulins, C3 and C4 were normal and rheumatoid factor, hepatitis B antigen and antinuclear antibodies were absent. The antistreptolysin O titre was 100. Skin biopsy revealed vascular deposits of IgA. During the second hospital day the child had macroscopic haematuria associated with right flank pain and vomiting and voided some blood clots. A n excretory urogram (IVP) (Fig. 1) showed dilatation of the calices and pelvis of the right kidney: the ureter was dilated and presented a tapering appearance at the lower lumbar level regaining the normal size in the pelvic region. While the contours of the renal cavities appeared smooth and regular, the ureteral wall was irregular with numerous small round filling defects, especially in the mid-lumbar portion. The left cavities were normal and the ureter showed no tapering, but a few, very small filling defects were evident in the proximal portion. The bladder was normal with smooth regular contours. Radiologic findings were consistent with the presence of subepithelial haemorrhage on the right producing mild ureteral stenosis and dilatation of the renal cavities. Treatment with prednisone i mg/kg was begun and over the next few days symptoms resolved promptly. Sub-

sequent urinalyses confirmed the presence of red cells with conventional morphology; low-grade proteinuria disappeared in 3 days and microhaematuria in a week. The child was treated on discharge with alternate-day prednisone administration, which was reduced gradually and suspended. A t home the symptomatology did not recur except for two transient episodes of petechial rash on the lower limbs. Three months later a follow-up IVP was normal.

Discussion

In this patient the diagnosis of SHP was based on the characteristic clinical features and the finding of vascular deposition of IgA in the skin biopsy. During hospitalisation flank pain, macrohaematuria and voiding of blood clots suggested a urologic complication. IVP showed a stenosing ureteritis; treatment with prednisone was accompanied by resolution of the urologic manifestations. Ureteritis seems to be very rare in SHP; including ours, only 11 patients have been reported in the literature with this complication [2-5, 7, 9], which can be serious as more than half of the patients have required surgical correction of the stenotic segment of the ureter. The efficacy of corticosteroids in the treatment of SHP ureteritis is open to question. In our patient, as in others [7],

therapy was accompanied by resolution of the ureteritis but both spontaneous healing [4, 9] and progressive stenosis despite steroid treatment [5] have been reported. It should be noted, however, that in our case the ureteritis was recognised and treated shortly after the onset of the SHP symptoms. The true incidence of ureteritis in SHP is probably underestimated. Only a few of the patients presenting ureteritis had symptoms suggesting a urologic complication, five complained of flank pain with gross haematuria and voiding of clots in three of them. In most patients ureteritis has been discovered by chance during an IVP performed for other reasons (urinary tract infections, needle renal biopsy). In fact although haematuria is quite frequent in SHP, it is usually ascribed to kidney involvement, so urologic investigations are rarely performed. Since ureteritis may be a serious complication requiring surgery and since precocious treatment with corticosteroids may be helpful in preventing stenosis, early diagnosis is important. Recently it has been shown that careful examination of the urinary sediment is helpful in differentiating glomerular from non-glomerular bleeding [1, 8]. If the morphology of the red cells in the urinary sediment is distorted with variation in size and shape and fragmentation, the haematuria is probably of glomerular origin, while if the red cells are uniform in size

593 and shape and not distorted, as in our cases, the haematuria is probably not glomerular. We suggest that in patients with SHP and haematuria careful examination of the urinary sediment should be carried out: the presence of normal red cell morphology should be interpreted as suggestive of involvement of the urinary and IVP and/or sonography should be performed. 2. Grotte G, Gierup J, Olsen L (1977) Pyeloureteric obstruction causing anuria in Henoch-Sch6nlein syndrome. Z Kinderchir 20:185-190 3. Hayat P, Sonsino E, Bonpand Y, StoraCastaing N, Weisgerber G (1978) St6nose ischemique de l'uret~re au cours d'un purpura rhumat6ide. Nouv Presse Med 7: 3913-3920 4. Junquera JM, Rodriguez-Vigil E, RoSa M, Miguel MA, Galbe M, Gonzalez-Abascal R, Lorenzo J (1978) Estenosis bilateral "alternante" en el curso de un Sch6nleinHenoch. Acta Urol Esp 2 : 89-90 5. Kher KK, Sheth KJ, Makker SP (1983) Stenosing ureteritis in Henoch-Sch6nlein purpura. J Urol 129 : 1040-1042 6. Meadow R (1979) Sch6nlein-Henoch syndrome. Arch Dis Child 54: 822-824 7. Mougenot P, Mitrofanoff P, Bouissou F, Dor6 F, Faur6 C (1978) Stenosing ureteritis during Henoch-SchSnlein purpura in children. Ann Pediatr 21 : 215-222 8. Rizzoni G, Braggion F, Zacchello G (1983) Evaklation of glomerular and nonglomerular hematuria by phase-contrast microscopy. J Pediatr 103 : 370-374 9. Thompson JS, McAlister WH (1975) Subepithelial hemorrhage in the renal pelvis and ureter simulating pyeloureteritis cistica. Pediatr Radiol 3 : 156-157

References
1. Fairly KF, Birch DF (1982) Hematuria: a simple method for identifying glomerular bleeding. Kidney Int 21 : 105-108

Received March 21, 1985 / Accepted July 11, 1985

Case report
Eur J Pediatr (1986) 144 : 593-596

Infantile multisyslem inflammatory disease: another case of a new syndrome

F. Lampert
Kinderpoliklinik, University of Giessen, Feulgenstrage 12, D-6300 Giegen, Federal Republic of Germany

Abstract. A 4-year-old girl with neonatal onset of chronic diffuse urticarial rash, head enlargement, protruding eye balls, bilateral arthritis of the knees, growth and mental retardation, and signs in blood and cerebrospinal fluid of chronic inflammation is presented and compared to two similar cases reported by us previously. Including this new patient there are now 14 documented cases with this specific inflammatory syndrome whose aetiology remains unknown. In the present case, however, elevated antibody titres against I.ric.Borrelia antigen were found in the serum. Key words: Infantile inflammatory disease - Neonatal onset

since 1973 [6, 5, 1, 7, 2, 4] and an editor's proposal [3] that a new syndrome has emerged encourages me to describe another patient with features similar to those in two patients who were observed by us 11 years ago in Munich with an "infantile chronic relapsing inflammation of the brain, skin and joints".

Case report
T.S. was admitted directly after delivery (birth date 03/18/81, gestation period 37 weeks, birth weight 2150 g, birth length 47 cm, head circumference 31 cm) to the Children's Hospital of the University of Giessen because of maculopapular skin rash, hepatosplenomegaly, anaemia and fever accompanied by recurrent infections (enteritis, bronchitis, rhinitis, cystopyelitis). One older brother of the patient is healthy and does not show any abnormalities. The father of the patient

Introduction
The description of two patients [9] identical to 11 cases reported in the literature

acquired a peculiar "rheumatic disease" with vasculitis (and uveitis?) in March 1979, 1 year prior to conception of the patient during a temporary stay in Argentina and was treated for several years. During the first hospital stay an Xray of the patient's chest revealed hypertrophy of the heart. A n E C G gave pathological results with a pronounced defect of depolarization. A n E E G was normal. Chromosome analysis from peripheral blood lymphocytes was normal (46,XX). The amount of C-reactive protein was elevated. No circulating immune complexes were detected in the blood. Bone marrow aspiration showed increased granulopoesis (82.5%), decreased erythropoesis (1.5%) and normal lymphocytes (16%). The further course was characterized by retarded growth and development, head enlargement with a wide open fontanel (cerebral atrophy could be demonstrated by CT scanning), protruding eye balls, conjunctivitis, blepharitis, massive enlargement of cervical, axillary and inguinal lymph nodes, bilateral arthritis of the knees and an itching maculo-papular rash which was aggravated in sun and heat. The recent appearance of the patient aged 41/2 years including a knee Xray is shown in Figs. l a and b. Laboratory data from this and two previously described cases are summarized in Table 1. Treatment consisted of low dose pred-