Kayode Adelusola

Quartan malaria-associated childhood nephrotic syndrome: now a rare clinical entity in malaria endemic Nigeria

BACKGROUND The study determined (i) whether or not quartan malaria nephropathy (QMN) is still a major cause of childhood nephrotic syndrome (CNS) in Nigeria, (ii) secondary causes other than QMN and their associated glomerular pathology and (iii) renal and patient outcome. METHODS The study was a prospective non-randomized study of consecutive cases of secondary CNS. Patients with idiopathic CNS were excluded. RESULTS Twenty-four of 78 (30.8%) CNS cases were of secondary aetiology. Overall mean ages at onset of secondary CNS aetiology and CNS onset were 8.97 +/- 3.59 (1-15.3) and 9.95 +/- 3.15 (5-15.3) years, respectively. Male (14)/female (10) ratio was 1.4. Secondary causes comprised systemic lupus erythematosus (SLE, 37.5%), sickle cell anaemia (SCA, 16.7%), hepatitis B virus (HBV, 16.7%) infection, Churg-Strauss syndrome (12.6%), SLE/human immunodeficiency virus infection (4.2%), rhabdomyosarcoma (4.2%), bee stings (4.2%) and Addisons disease (4.2%). The overall cumulative complete remission (CR) rate was 88.0%. Remission was sustained in 11 of 16 (68.8%) CR patients, while one patient (6.25%) relapsed; the remaining four patients (24.95%) were yet to attain sustained remission. Median relapse-free period was 10.5 (0.75-25) months. Cumulative renal survival was 75.2% at 3 years. Three patients were lost to follow-up, while two died. Overall cumulative patient survival probability at 36 months was 90.8%. All patients were followed for a median period of 12.5 (0.11-36.0) months. CONCLUSION Overall outcome of CNS has improved significantly compared to the 1960s and 1970s when the poor outcome of QMN was the predominant glomerular lesion in Nigeria. While quartan malaria-associated nephrotic syndrome has become a rare clinical entity, SLE, SCA and HBV infection have become the major secondary aetiologies of CNS in Nigeria.

AUTOPSY DIAGNOSIS OF ENDEMIC BURKITT LYMPHOMA AS THE PRIMARY ETIOLOGY OF ACUTE RENAL FAILURE IN CHILDREN

Two cases of Burkitt lymphoma are reported who presented atypically with acute renal failure and significant proteinuria as initial features of the lymphoma. The cases underscore the need for high index of suspicion for Burkitt lymphoma in any child with rapidly enlarging kidneys and acute renal failure of obscured origin in parts of the world where Burkitt lymphoma is endemic.

Incidental carcinoma of the prostate gland presenting with initial manifestation of disseminated intravascular coagulopathy (dic) in a middle aged man: a case report

BackgroundIncidental carcinoma of the prostate gland is a common clinical problem among elderly males but this malignancy presenting initially with features of Disseminated Intravascular Coagulopathy (DIC) in the African blacks is rare. Disseminateded intravascular coagulathy is the most frequent coagulation disorder in patients with prostate cancer, However DIC as a first manifestation of prostate cancer is unusual.Case reportThis paper reports a case of a 56 year old Nigerian civil servant who presented initially with clinical features of DIC characterised by bleeding from multilple orifices but was subsequently diagnosed at autopsy to be infiltrating adenocarcinoma of the prostate.ConclusionThis rare case of DIC should be considered especially in elderly men when no other cause can be found for coagulopathy.

Hypertension, erythrocyturia and proteinuria in childhood non-Hodgkin's lymphoma.

Aim:  The objectives were to determine the prevalence and outcome of hypertension, significant microerythrocyturia and proteinuria among children with acute renal failure (ARF) due to Burkitt‐type non‐Hodgkin’s lymphoma (BNHL).

Clinicopathology of childhood-onset renal systemic lupus erythematosus

Aims:  To determine the clinicolaboratory renal manifestations; glomerular, extra‐glomerular histopathologic lesions; renal tubular dysfunction (RTD) frequency and outcome of a short‐term renal follow up in Nigerian children with systemic lupus erythematosus (SLE).

Bellini duct carcinoma of the kidney masquerading as an iliac bone tumour in an adult Nigerian

Bellini Duct Carcinoma (BDC) of the Kidney is a rare type of Renal Cell Carcinoma. It usually presents with features of local advancement or metastasis and rarely diagnosed incidentally. We present a case report of a young man who was found to have BDC of the Right Kidney following presentation with an iliac bone tumour. A 40 year old man presented to the Orthopaedic outpatient clinic on account of right sided pelvic pain and limping following a trivial fall at home. There was no antecedent history of loin pain, loin mass or haematuria. On evaluation, he was found to have a huge right iliac bone tumour invading the contiguous muscles. An incidental hypodense central ipsilateral renal mass with enlarged peri-hilar lymph nodes were found. He subsequently had right radical nephrectomy via a right sub-coastal approach and wide local excision of the Iliac bone tumour in two separate procedures. The resection margins were negative for tumour cells. Histology of the resected specimens were consistent with a metastatic right BDC of the kidney. He had a smooth post-operative recovery. One third of BDC of the kidney presents with metastasis. A high index of suspicion is required in order to diagnose BDC following such unusual presentations.

Lymphomas in Ile-Ife, Nigeria: Immunohistochemical Characterization and Detection of Epstein-Barr virus Encoded RNA.

BACKGROUND The proper histopathological characterization of malignant lymphomas requires the use of immunohistochemistry along with other molecular pathology techniques. MATERIALS AND METHODS Malignant lymphomas histologically diagnosed in our hospital were reclassified according to the WHO scheme using immunohistochemistry while in-situ hybridization was performed for the detection of Epstein-Barr virus encoded RNA. RESULTS There were 83 cases of lymphoma. The male to female ratio was 1.9:1 while the overall mean age was 41.7 years. Non-Hodgkin lymphomas (NHL) constituted about 79.5% of cases. The majority of cases (98.8%) were B-cell lymphomas. Nine subtypes of lymphomas were identified with diffuse large B-cell lymphomas (56.4% of which were of the germinal centre type) constituting the largest group (47.0%). Intermediate and high grade subtypes were more common. The majority of cases (72.3%) were nodal lymphomas with cervical lymph node being the commonest site (48.2%). Only classical Hodgkin lymphoma (HL) (20.5%) was seen of which the mixed cellularity subtype was the most common. Epstein Barr virus (EBV) encoded ribonucleic acid was detected in 7 cases (8.4%) including 4 cases of HL, 2 cases of Burkitt lymphoma and the only case of plasmablastic lymphoma. About five cases were reclassified as non-lymphoid malignant lesions. CONCLUSION Immunohistochemistry is vital to the proper classification of lymphomas even in a resource poor environment. Although nine subtypes of lymphomas were identified, diffuse large B-cell lymphomas formed the largest single group. Epstein-Barr virus probably plays an important role in lymphomatogenesis in this environment. A larger multicentre study is required to prove this.

Stage 2 hypertension in a child with a rapidly enlarging kidney: answer

1. Burkitt’s lymphoma (BL) is the most likely aetiology, especially in an endemic region. BL is a nonmetastatic, highly proliferative, multicentric tumour. It is the fastest growing tumour in humans, with a doubling rate of 24 h [2]. 2. These will include nephroblastoma, acute unilateral obstructive uropathy and renal vein thrombosis. 3. Confirmation is either by fine-needle aspirate of the tumour for cytology or renal biopsy for histopathology. Both procedures confirmed BL on admission day 4 (Fig. 1a). There was also glomerular evidence of proliferative glomerulonephritis with Bowman’s capsule ruptures and tuft adhesions (Fig. 1b). Renal biopsy findings at 4 weeks are shown in Fig. 1c. 4. It is possible that the extensive renal infiltration by Burkitt’s tumour with glomerulotubular compression and destruction (Figs. 1a–c), glomerulonephritis and enhanced renin-angiotensin-aldosterone-system (RAAS) activity played dominant roles in the causation of hypertension (HTN) in this patient. The proteinuria could have been due to the combination of glomerulonephritis and systemic and intraglomerular HTN brought about by increased RAAS activity. HTN may cause high glomerular filtration pressure in both kidneys with resultant proteinuria. Hypernatraemia, hypokalaemia, avid tubular sodium reabsorption and kaliuresis suggest enhanced RAAS activity in the patient. 5. BL is highly responsive to cytotoxic drugs. Tumour remission was induced with low-dose cyclophosphamide infusion, 125 mg/m/48 h for four doses [3]. He also received antitumour lysis syndrome regimen, including allopurinol [3]. Significant reduction in the renal mass occurred within 72 h of cyclophosphamide infusion. Tumour remission was subsequently maintained with cycles of combination chemotherapy that comprised cyclophosphamide 1,000 mg/m/dose, methotrexate 75 mg/m/dose and vincristine 1.5 mg/m/dose as stat intravenous doses. Intrathecal (IT) methotrexate 12.5 mg/m on chemotherapy days 1 and 8, and IT cytosine arabinoside 50 mg/m on day 4 were also given. The cycle was repeated fortnightly after ensuring normal blood counts. Overall, he received only three of six prescribed cycles because he was immediately lost to follow-up after hospital discharge. His hypertension was appropriately treated with parenteral hydralazine, frusemide, oral spironolactone and lacidipine. BP normalised (100/60 mmHg) by day 10 of induction chemotherapy. The antihypertensives were subsequently discontinued without adverse events. 6. Given the severity of the glomerular lesion that included Bowman’s capsule ruptures with interstitial communications (Fig. 1b) and tuft synechiae as shown in the histopathology of the second renal biopsy (Fig. 1c), progression to chronic renal failure is a potential complication. Pediatr Nephrol (2008) 23:729–731 DOI 10.1007/s00467-007-0646-4

Histopathological pattern of primary bone tumours and tumour-like lesions in Ile-Ife, Nigeria

Introduction Bone tumours are relatively rare in comparison with neoplasms in other parts of the body. Previous studies have noted higher frequencies of these tumours in young adults with potentially devastating consequences. Methods This study aimed to demonstrate the histopathological pattern of primary bone tumours and tumour-like lesions in Ile-Ife, Nigeria with emphasis on relative frequencies and distribution according to age, sex and anatomical location. A 13 year (1991-2003) retrospective study was carried out on 100 cases of primary bone tumours and tumour-like lesions. Records were retrieved from the surgical registers of the Histopathology Department, Qbafemi Awolowo University Teaching Hospitals Complex, Ile-Ife. Original histopathological slides were retrieved for examination and when they were unavailable, the paraffin blocks were searched out and new slides were made. Results A total of 100 cases met the inclusion criteria for this study accounting for 1.55% of the 6,464 cases of all neoplasms seen within this period. Of the 100 cases, 50 were malignant (50%), 28 were benign (28%) and 22 had tumour-like lesions (22%). The male to female ratio was 1.3:1 and the most common benign and malignant tumours were osteochondroma and osteosarcoma respectively. The femur was the most frequently involved bone in primary malignant lesions (24%) followed by the pelvis and the maxilla (14% each). Conclusion The age, sex and morphological distribution of benign and malignant bone tumours is similar to earlier reports in other African and international journals. Tumour-like lesions occur more frequently in females than in males. The femur is the most favourable site for malignant primary bone tumours and the jaw bones for tumour-like lesions.

Biopsy-proven renal disease in Ile-Ife, Nigeria: A histopathologic review

Although various patterns of renal diseases have been reported from different renal biopsy registries worldwide, data from Nigeria remain scanty. A 10-year retrospective review of renal biopsies was conducted in our tertiary health care facility. All cases were reclassified based on their light microscopic features after the application of standard histochemical stains. A total of 165 cases were reviewed with a male:female ratio of 1.8:1 and a mean age of 15.4 ± 12.0 years. About 69.7% of the cases were below the age of 16 years, while only 2.4% were older than 50 years. The most common indications for biopsy were nephrotic syndrome (72.1%) and acute renal failure of unknown etiology (11.5%). Overall, glomerulonephritis (80%) was the most common histologic category and occurred only in individuals younger than 50 years old. Minimal change disease (22.9%) and membranoproliferative glomerulonephritis (21.9%) were the most common varieties in children, while membranous glomerulonephritis (30.6%) and focal segmental glomerulosclerosis (27.8%) were the commonest among the adult population. The initial histologic diagnosis was revised in 18 cases while a diagnosis was arrived at in seven cases initially adjudged as inadequate for assessment. This study showed that renal biopsy was predominantly performed in children and adolescents. Although glomerulonephritis was the predominant disease, the predominant histologic patterns varied with the patient age. Despite the scarcity of advanced diagnostic tools in resource-poor environments, routine use of histochemical stains is helpful in the evaluation of renal biopsies.