Acute pancreatitis in a patient with partial lipodystrophy and membranoproliferative glomerulonephritis
Mehmet Bülbül1,,
Özlem Erdo
an1,
Gülay Demircin1,
Buket Altunta
2,
Leyla Memi
2 and
Ay
e Öner1
1 Dr Sami Ulus Children's Hospital and
2 Gazi University Medical School, Ankara, Turkey
Keywords: acute pancreatitis; membranoproliferative glomerulonephritis; partial lipodystrophy
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Introduction
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Partial lipodystrophy (PLD) is a rare, inherited condition, characterized by progressive loss of subcutaneous fat from the face, upper limb and trunk [1,2]. The PLD locus has been mapped to chromosome 1q with no evidence of genetic heterogeneity [3]. The onset is usually in early childhood, is often acute in nature, and sometimes follows a viral infection such as measles and varicella. The association of PLD with C3 nephritic factor (C3NeF), hypocomplementaemia, and membranoproliferative glomerulonephritis (MPGN) has been known for a long time. Hirsutism, hyperpigmentation, genital enlargement, hepatomegaly, cardiomegaly, disturbance of central nervous system function, hypermetabolism, hyperlipaemia, decreased glucose tolerance, and diabetes mellitus are other features that have been reported in association with PLD [49]. Acute pancreatitis related to this disorder has been reported by Smith et al. in two cases with renal involvement [10]. In this case report we present a child who developed acute pancreatitis in addition to PLD and MPGN.
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Case
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A 13-year-old girl was admitted to the hospital with symptoms of vomiting and abdominal pain lasting for 2 days. She was the daughter of non-consanguineous, healthy parents without any remarkable family history including lipodystrophy or renal disease. She had undergone surgery for acute appendicitis, and brid ileus 4 and 3 years prior to admission respectively. She had a history of recurrent abdominal pain relieved by antispasmodic drugs for a year. On physical examination she was a well-developed girl with a height of 158 cm and weight of 66 kg. However, her face revealed marked loss of adipose tissue, although the trunk and extremities seemed to have increased fat distribution (Figure 1
). She had pretibial oedema, abdominal distension, and tenderness.
Results of the laboratory tests were; haemoglobin 11.7 g/dl, white blood cell count 34 400/mm3 with neutrophilia and toxic granulation in peripheral blood smear. Blood urea nitrogen was 47.1 mg/dl, creatinine 3.28 mg/dl, albumin 2.9 g/dl, sodium 125 mEq/1, potassium 5.9 mEq/l, and serum amylase 1257 U/l (normal, 0220 U/l). Proteinuria (1.5 g/m2/day) and microscopic haematuria were detected in urinalysis. Third component of complement (C3) was 18.6 mg/dl (normal 50140 mg/dl); C4, 11 mg/dl (normal, 1540 mg/dl). Serological tests for Salmonella, Brucella, and mumps were negative, and no pathological micro- organisms were grown from throat, urine, or blood cultures. Abdominal ultrasonography demonstrated pseudocysts in the pancreas. The patient was treated acutely with peritoneal dialysis, antibiotics, somatostatin, nasogastric drainage, and intravenous fluid replacement therapies. Her clinical condition improved and her weight was reduced to 62.7 kg without any oedema. Percutaneous renal needle biopsy was performed on the 15th day of admission. Histological examination revealed MPGN with crescentic glomerulonephritis and tubulointerstitial involvement. Capillary and mesangial staining with immunoglobulin (Ig)M and C3 was shown on immunofluorescence microscopy.
The patient was given methylprednisolone pulse therapy (30 mg/kg) for 3 days, followed by oral prednisolone (45 mg/day) and she was started on a chronic ambulatory peritoneal dialysis (CAPD) program since her renal functions continued to deteriorate. Percutaneous drainage of the pancreatic cysts was planned. However, the patient died from infection before this procedure could be undertaken.
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Discussion
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The association between PLD and renal disease was first reported by Gellis et al. in 1958 [1]. In 1973 Peters et al. [4] emphasized the relationship of PLD with hypocomplementaemia, MPGN, and C3NeF, which is an IgG autoantibody that causes continuous activation of alternative complement pathway by blocking factor H, thus leading to uncontrolled function of C3 convertase (C3bBb). Recent studies showed that complement components found to be expressed both by the adipose tissue and renal cells were responsible for the injury caused by activation of complement cascade. Alper et al. [6] reported that adipsin, a major protein product of adipocytes, is identical to factor D, and adipocytes also produce C3 and factor B. Dysregulation of alternative complement pathways causes destruction of complement components in adipose tissue where they are produced. Lipodystrophy and tissue destruction are more significant in tissues where factor D expression is increased [1115]. In a similar way renal cells were also shown to express some of the complement components like C3, C4, and probably factor D. Therefore activation of complement pathway also leads to renal injury [1]. The occurrence of pancreatitis with MPGN, and PLD in this patient suggests three possible mechanisms: (i) uraemia, that was probably present before admission might cause pancreatitis, since uraemia is one of the potential factors generally held responsible for the development of pancreatitis; (ii) infections that developed because of dysfunction of immune defence mechanisms due to activation of complement cascade might have led to pancreatitis; (iii) the immunological mechanism that caused damage in renal and adipose tissues might also lead to injury in the pancreatic tissue in the same way.
With this case report, we wished to point to the development of pancreatitis in a patient with MPGN and PLD. Our observation may make a new contribution to clarify the etiopathogenesis of this rare association.
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Notes
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Correspondence and offprint requests to: Mehmet Bülbül MD, 73. Sokak, 86-5, Emek, Ankara, Turkey. 
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Received for publication: 19. 1.00
Revision received 6. 4.01.