Early onset myasthenia gravis in a Malawian woman: Challenges in managing myasthenia gravis in a low-resource setting.

Myasthenia gravis is a rare autoimmune disease, which presents with ocular or generalised symptoms. Few publications describe its prevalence in African populations. We describe a young woman who was diagnosed with myasthenia gravis in a Malawian public hospital and outline the challenges encountered in managing this condition in a low-resource setting.

Evaluation of a surgical training programme for clinical officers in Malawi.

BACKGROUND: Shortages of specialist surgeons in African countries mean that the needs of rural populations go unmet. Task-shifting from surgical specialists to other cadres of clinicians occurs in some countries, but without widespread acceptance. Clinical Officer Surgical Training in Africa (COST-Africa) developed and implemented BSc surgical training for clinical officers in Malawi. METHODS: Trainees participated in the COST-Africa BSc training programme between 2013 and 2016. This prospective study done in 16 hospitals compared crude numbers of selected numbers of major surgical procedures between intervention and control sites before and after the intervention. Volume and outcomes of surgery were compared within intervention hospitals between the COST-Africa trainees and other surgically active cadres. RESULTS: Seventeen trainees participated in the COST-Africa BSc training. The volume of surgical procedures undertaken at intervention hospitals almost doubled between 2013 and 2015 (+74 per cent), and there was a slight reduction in the number of procedures done in the control hospitals (-4 per cent) (P = 0·059). In the intervention hospitals, general surgery procedures were more often undertaken by COST-Africa trainees (61·2 per cent) than other clinical officers (31·3 per cent) and medical doctors (7·4 per cent). There was no significant difference in postoperative wound infection rates for hernia procedures at intervention hospitals between trainees and medical doctors (P = 0·065). CONCLUSION: The COST-Africa study demonstrated that in-service training of practising clinical officers can improve the surgical productivity of district-level hospitals.

'I think we are going to leave these cases'. Obstacles to surgery in rural Malawi: a qualitative study of provider perspectives.

OBJECTIVES: Surgical services at district level in Malawi are poor, yet the majority of the population resides in rural areas. This study aimed to explore the perceived obstacles to surgery from the perspective of the cadre directly responsible for surgical service delivery at district hospitals. METHODS: Qualitative interviews were conducted with 16 clinical officers (COs) receiving surgical training in eight public district hospitals and their 12 trainers. Thematic analysis of data was conducted using a top-down coding method. RESULTS: Despite readiness of the COs to conduct operations, other staff essential for surgery were sometimes unavailable to support them. Respondents attributed this to lack of skills, weak motivation or poor work ethic of their colleagues. Lack of commitment to do surgery, passiveness, lack of initiative in problem-solving and 'laziness' of surgical team members were among the reasons provided by study participants, accounting for unnecessary cancellations of elective surgery and inappropriate referrals of emergency cases. Other factors included infrastructure breakdowns and stock-outs of surgical supplies. There were instances where COs, and their supervisors, showed initiative in finding solutions to problems resulting from poor district hospital management practices. CONCLUSIONS: This study demonstrates how the motivation of surgical team members is a key factor in deciding whether or not to perform operations; and that shortages of supplies or infrastructure need not be an absolute obstacle to service delivery. Scale-up of surgical services at district level requires investments to improve surgical and anaesthetic skills, to strengthen human resources and facility management, and to ensure the availability of reliable infrastructure and essential supplies.

Transverse colon volvulus in children: A case series and a review of the literature.

Volvulus of the transverse colon is very rare in children. Three cases that occurred in a short time span are described and the scarce literature is reviewed. All patients presented with colonic obstruction and in all three the condition was diagnosed at laparotomy, as CT-scanning is not an available option in either of the two institutions. The transverse colon had not become gangrenous but was resected after detorsion for redundancy. The postoperative course was complicated and relaparotomy had to be performed for reobstruction in all cases. Only if the 'reverse' bean sign is recognized on the preoperative plain abdominal radiograph this rare diagnosis can be suspected.

Relationship between Plasmodium falciparum malaria prevalence, genetic diversity and endemic Burkitt lymphoma in Malawi.

Endemic Burkitt lymphoma (eBL) has been linked to Plasmodium falciparum (Pf) malaria infection, but the contribution of infection with multiple Pf genotypes is uncertain. We studied 303 eBL (cases) and 274 non eBL-related cancers (controls) in Malawi using a sensitive and specific molecular-barcode array of 24 independently segregating Pf single nucleotide polymorphisms. Cases had a higher Pf malaria prevalence than controls (64.7% versus 45.3%; odds ratio [OR] 2.1, 95% confidence interval (CI): 1.5 to 3.1). Cases and controls were similar in terms of Pf density (4.9 versus 4.5 log copies, p = 0.28) and having ≥3 non-clonal calls (OR 2.7, 95% CI: 0.7-9.9, P = 0.14). However, cases were more likely to have a higher Pf genetic diversity score (153.9 versus 133.1, p = 0.036), which measures a combination of clonal and non-clonal calls, than controls. Further work is needed to evaluate the possible role of Pf genetic diversity in the pathogenesis of endemic BL.

Ward round--A rare tumor of the kidney resulting in hypertension, renal failure and a cerebrovascular accident in a young female.

A 17-year-old girl was admitted to our hospital with severe refractory hypertension evolving over approximately 4 years. Despite not having the resources to identify plasma-renin levels and using standard imaging techniques, a juxtaglomerular cell tumor was suspected and was histologically confirmed after surgical excision. This is a potentially lethal condition if left untreated and surgical excision is curative. The benign nature of the tumor is emphasized and its chemical, radiological and microscopic appearance discussed according to the literature. To the best of our knowledge, this is the first reported case of a patient surviving a cerebrovascular accident associated with a juxtaglomerular cell tumor.

Challenges and outcome of Wilms' tumour management in a resource-constrained setting.

BACKGROUND: To review the results of Wilms' tumour patients in a tertiary referral hospital in a developing country and to find ways of improving long-term survival. PATIENTS AND METHODS: Between January 1998 and May 2004, 40 patients with Wilms' tumour (WT) were admitted to Queen Elizabeth Central Hospital. Their files were reviewed and general physical condition on admission, pre-operative investigations, management and outcome were noted. RESULTS: The mean age of presentation was 4.2 years with an equal distribution between the sexes. The mean BMI was 15 kg/m2 and more than 80% of the patients were either mildly (PCV <33%) or severely anaemic (PCV <24%). All patients presented with abdominal distension. Half of them had additional complaints including abdominal pain, haematuria, dyspnoea, oedema and or weight loss. Thirty-nine out of the forty patients received pre-operative chemotherapy. Of the 36 patients that underwent surgery, 32 underwent total nephrectomy, one a partial nephrectomy, and in three the tumour was irresectable. There were no intra-operative tumour ruptures. Only 15% of the patients completed their post-operative course of chemotherapy. The 1-year survival lies between 25% and 53%. Fifteen of the 36 patients operated were known to have a recurrence. CONCLUSION: The patients presented in an advanced stage of the disease. Survival rates are disappointing and recurrence rates are high. Some improvement in outcome may be expected with the implementation of more aggressive treatment protocols but early diagnosis, completion of treatment regimens are needed. Pro-active follow-up is essential to measure outcome.

Improved method for assessing iron stores in the bone marrow.

BACKGROUND: Bone marrow iron microscopy has been the "gold standard" method of assessing iron deficiency. However, the commonly used method of grading marrow iron remains highly subjective. AIM: To improve the bone marrow grading method by developing a detailed protocol that assesses iron in fragments, in macrophages around fragments and in erythroblasts. METHODS: A descriptive study of marrow aspirates of 303 children (aged 6-60 months) with severe anaemia and 22 controls (children undergoing elective surgery) was conducted at hospitals in southern Malawi (2002-04). RESULTS: Using an intensive marrow iron grading method, 22% and 39% of cases and controls had deficient iron stores, and 40% and 46% had functional iron deficiency, respectively. Further evaluation of the iron status classification by the intensive method showed that functional iron deficiency was associated with significantly increased C-reactive protein concentrations (126.7 (85.6) mg/l), and iron stores deficiency with significantly increased soluble transferrin receptor concentrations (21.7 (12.5) mug/ml). CONCLUSIONS: Iron assessment can be greatly improved by a more intense marrow examination. This provides a useful iron status classification which is of particular importance in areas where there is a high rate of inflammatory conditions.

Spectrum and presentation of pediatric malignancies in the HIV era: experience from Blantyre, Malawi, 1998-2003.

BACKGROUND: Data on childhood cancers in Africa are sparse, particularly since the spread of HIV. We aimed to document the frequency of pediatric cancers presenting to a large central hospital in Malawi, detailing the presenting features, initial investigations, and HIV status of these children. PROCEDURE: A retrospective audit of the spectrum and clinical presentation of cancers among children (<16 years) seen at Queen Elizabeth's Central Hospital (QECH), between 1998 and 2003. RESULTS: Seven hundred seven children with cancer were seen, the number of cases per year increased over the time period; 50% (351) had Burkitt lymphoma, 13% (89) had retinoblastoma, and 9% (61) had Kaposi sarcoma, with a variety of other tumors comprising the remainder. Kaposi sarcoma markedly increased in frequency over time. Histological verification of diagnosis was available for 49% (348). The proportion of children with cancer who were tested for HIV increased over time, but varied by cancer type. Amongst those tested, the seroprevalence was 93% (52/56) for children with Kaposi sarcoma, 4% (11/289) for those with Burkitt lymphoma, 31% (8/26) for those with other non-Hodgkin lymphomas, 7% (1/15) for those with Hodgkin disease, and 5% (5/103) for those with other cancers. CONCLUSIONS: The number of cases seen per year has increased over the study period for almost all cancers, but in particular for Kaposi sarcoma. Burkitt lymphoma remains the commonest pediatric tumor in Malawi. In the case of Burkitt lymphoma, non-Hodgkin lymphoma, and Kaposi sarcoma there is a significant difference in the presentation of HIV-seropositive and -seronegative children.

Epidemiology of paediatric trauma admissions at Queen Elizabeth Central Hospital, Blantyre.

We conducted an audit of paediatric trauma admissions to QECH, Blantyre, in September 2003. There were 107 trauma cases representing 8.8% of all paediatric admissions and mean age was 6 years. The commonest cause of trauma was falls (42.9%) followed by burns (31.8%) and road traffic accidents (14.9%). Of the road traffic accidents, only one case was a passenger, the rest were pedestrians hit by moving vehicles. Fracture of limbs was the commonest injury sustained (44.9%) and burns the second commonest injury (31.8%). Most (52.6%) children were brought into hospital within 24 hours of injury while 26.3% came in between 24 hours and 48 hours and 21.1% after 48 hours or more. Death occurred in 7.5% of cases. The mean number of days in hospital was 8.9 days.

Management of hydrocephalus using the Chabbra shunt.

Over a two-year period, 223 patients with hydrocephalus were admitted to the wards of Queen Elizabeth Central Hospital, Blantyre. Of these 223 patients, 111 were male and 112 were female. All children of less than 18 months underwent ultrasonography to confirm the diagnosis of hydrocephalus, and 22 older patients had a CT scan. The commonest causes of hydrocephalus were meningitis and congenital hydrocephalus. 201 had ventricular aspiration to assess suitability for shunting and in 157 patients, the cerebrospinal fluid (CSF) was clear and these patients were considered suitable for insertion of a ventriculo-peritoneal (VP) shunt. The outcome was satisfactory in 73% of the patients with 10 patients lost to follow-up. There were 12 (7.6%) patients with shunt infections, 6 (3.8%) patients had peritoneal shunt blockage and 2 (1.3%) had over-drainage. There were 2 cases of shunt prolapsing through the anus. Twenty patients died (12.7%): one had inflammation along the shunt tract, one had valve malfunction and one had the valve exposed on the scalp.

One-year audit of admissions to the Intensive Care Unit of the Queen Elizabeth Central Hospital, Blantyre.

The intensive care unit at Queen Elizabeth Central Hospital (QECH) has 4 beds and offers level 2 care. A retrospective audit of all admissions to the unit during 2002 was carried out. There were a total of 339 admissions giving a bed occupancy rate of 82 %. Surgical patients made up 81 % of admissions. 45% of all admissions were ventilated. Overall mortality was 38%. Ventilated patients had a mortality of 71% compared with 10% for non-ventilated. Data are also presented for mortality within the surgical and paediatric surgical admissions.

Indications for HIV testing in paediatric surgical patients.

HIV testing is done in paediatric surgical patients at Queen Elizabeth Central Hospital, when HIV infection is suspected to be the underlying cause of the clinical presentation or contributing to morbidity. We conducted a retrospective, descriptive analysis of children with surgical conditions under the age of 15 who underwent HIV tests between January 2001 to April 2004. A total of 279 children were tested - 50.2% were boys and 49.8% were girls. Overall HIV prevalence was 39.8%. The most common indications for testing and those with highest HIV prevalence were sepsis (31% of the diagnoses and HIV prevalence of 38%), head and neck swellings (22% and HIV prevalence of 39%) and urogenital problems (17% of cases and HIV prevalence of 51%).

The prevalence of HIV infection among burn patients in a burns unit in Malawi and its influence on outcome.

In a 1 year study, 342 patients admitted to the Burns Unit at Queen Elizabeth Central Hospital (QECH), Blantyre, Malawi underwent a voluntary HIV test. Forty (11.7%) tested HIV positive: of those aged above 15 years, 31% (34 out of 112) were HIV positive, whilst 3% (6 of 231) aged under 15 were HIV positive of whom the majority were in those aged under 5 years (5 of 125, 4%). Patients who were HIV positive had an increased risk of death (P=0.04) which was mainly due to sepsis, but those HIV patients, who did not develop infection or recovered from an episode of sepsis, had similar hospital stay, need for skin grafting and graft take as nonHIV patients. There was no difference in pathogens cultured from wound swabs taken from HIV positive and negative patients. HIV positive patients had significantly lower CD4 counts as compared to HIV negative patients (mean 383mm3 (S.D. 320) and 937mm3 (S.D. 497), respectively). However, low CD4 counts were also found in the HIV negative patients (mean 901, range 131-1964) and 24% had CD4 <500/mm3. Both HIV status and the total body surface area (TBSA) burned were independent predictors of CD4 count. TBSA was an independent risk factor for death (odds ratio 1.3; 95% CI 1.1, 1.4). In patients with TBSA burns of over 30%, mortality approached 100% irrespective of HIV status, but in patients with burns of 11-20% TBSA and who were HIV positive have a mortality of 25% compared to 12% in HIV negative patients; for 21-30% TBSA burns mortality was 100% compared to 50% for HIV positive and HIV negative patients, respectively.

Tracking familial transmission of Kaposi's sarcoma-associated herpesvirus using restriction fragment length polymorphism analysis of latent nuclear antigen.

Intra-familial transmission of Kaposi's sarcoma associated herpesvirus (KSHV) is likely to occur in geographical regions where KSHV infection is highly endemic. Transmission has been studied previously indirectly using serological techniques, however direct documentation of specific transmission routes has yet to be reported. The internal repeat domain (IRD) of the KSHV opening reading frame (ORF) 73 was shown previously to exhibit restriction-fragment length polymorphism (RFLP). Analysis of such polymorphism was undertaken using nested ORF 73 IRD PCR products derived from the blood and mouth rinse samples of individuals in Malawian family groups. The resulting RFLP patterns were unique to an individual and could be compared between family members. In three of eight families studied, identical RFLP patterns were recovered from family members; in the remaining five families, dissimilar RFLP patterns were revealed. Results from RFLP analysis were compared to sequencing data recovered from family members for the first variable region of the hypervariable KSHV ORF K1. Patterns of intra- and extra-familial transmission inferred from ORF K1 sequencing data were corroborated mainly using ORF 73 IRD RFLP analysis.

Gastroschisis minor.

BACKGROUND: Since 1994 the author has seen a number of cases of what appear to be a minor variant of gastroschisis. METHODS: The case files of all neonates with gastroschisis (GS) presenting to the neonatal ward of the Queen Elizabeth Central Hospital (QECH), a referral hospital in Blantyre, Malawi, from January 1995 to December 1998 were reviewed and the presentation and survival noted. RESULTS: Twenty-one neonates were seen; only 2 of 15 with "normal" GS survived. The other patients had gastroschisis minor (GSM) and all left the hospital alive. CONCLUSION: In the context of the constraints and difficulties encountered in the treatment of gastroschisis in a developing country, this subgroup of patients with a more minor form of gastroschisis shows a distinct survival advantage.