Iron stores in pregnant women with sickle cell disease: a systematic review.

BACKGROUND: Gradual improvements in the management of sickle cell disease (SCD), have led to an increase in the number of women with SCD who reach the age of procreation. However, evidence on the iron status of pregnant women with sickle cell disease (PWSCD) remains inconclusive. We conducted the first systematic review on the prevalence, determinants and maternal/foetal outcomes of iron deficiency anaemia among PWSCD. METHODS: We searched MEDLINE, EMBASE, Global Health, Africa Index Medicus, the Cochrane library databases and reference lists of retrieved publications for studies describing the iron status of PWSCD. The literature search was done over a period of 1 month, with no language or date restrictions applied. Data were extracted on a Microsoft excel sheet. Two authors assessed all included studies for methodological quality and risk of bias. RESULTS: A total of 710 reports were identified for title and article screening. Five retained studies were conducted before or during the 90s and included 67 participants. After quality assessment, the observational studies were designated to have a "fair" quality assessment while the randomised control trial had an "unclear" quality assessment. The prevalence of iron deficiency anaemia among PWSCD varied by study design and diagnostic method. The overall prevalence ranged from 6.67-83.33%. None of the studies provided evidence on factors associated with iron deficiency anaemia and the randomized trial reported no difference in outcomes between PWSCD who had iron supplementation and those who did not. CONCLUSION: Evidence on factors associated with iron deficiency anaemia among PWSCD and maternal/foetal outcomes in PWSCD who have iron deficiency anaemia is poor. The studies included in this review suggests that iron deficiency anaemia may be highly prevalent in PWSCD but due to the very small sample sizes and varied study designs, this evidence is inconclusive. The review shows that there is a need for more studies with robust designs and adequate sample sizes to assess the disease burden of iron deficiency anaemia in PWSCD.

Exploring the role of obesity and overweight in predicting postoperative outcome of abdominal surgery in a sub-Saharan African setting: a prospective cohort study.

OBJECTIVE: Current literature on the role of excess weight in predicting surgical outcome is controversial. In sub-Saharan Africa, there is extreme paucity of data regarding this issue in spite of the increasing rates of obesity and overweight in the region. This prospective cohort study, carried out over a period of 4 months at Limbe Regional Hospital in the Southwest region of Cameroon, assessed 30-day postoperative outcome of abdominal surgery among consecutive adults with body mass index (BMI) ≥ 25 kg/m2. Adverse postoperative events were reported as per Clavien-Dindo classification. RESULTS: A total of 103 patients were enrolled. Of these, 68.9% were female. The mean age was 38.2 ± 13.7 years. Sixty-four (62.1%) of the patients were overweight and the mean BMI was 29.2 ±4.3 kg/m2. The physical status scores of the patients were either I or II. Appendectomy, myomectomy and hernia repair were the most performed procedures. The overall complication rate was 13/103 (12.6%), with 61.5% being Clavien-Dindo grades II or higher. From the lowest to the highest BMI category, there was a significant increase in the proportion of patients with complications; 25-29.9 kg/m2: 6.25%, 30-34.9 kg/m2: 18.75%, 35-39.9 kg/m2: 25.0%, and ≥ 40 kg/m2: 66.70%; p = 0.0086.

Iron supplementation in pregnant sicklers: an opinion.

Morbidity associated with iron deficiency anaemia in pregnancy is increased in the presence of sickle cell anaemia. Iron supplementation in pregnant sicklers in a bid to resolve iron deficiency anaemia is recommended only after laboratory confirmation of iron deficiency. However, the greatest burden of sickle cell disease is seen in low and middle income countries where equipment for measuring body iron indices are unavailable.Sickle cell anaemia is an inherited disorder of haemoglobin synthesis characterized by life-long severe haemolytic anaemia. People with sickle cell anaemia are at increased risk of iron overload from haemolysis and recurrent multiple transfusions. Iron overload a complication of sickle cell disease, which is more often in thalassemias, is typically given undue fear in sickle cell anaemia especially in patients with no recent transfusion history. About a third of the haemolysis in sickle cell anaemia is intravascular, and the resulting excess iron is lost in urine. This may lead to a negative iron balance and iron deficiency. There is little evidence of iron overload in pregnant sicklers, and iron deficiency may be more common than suspected. Even when iron overload does occur in a condition called siderosis, the deposited iron is irreversible and thus cannot be reused by the body in case of susceptibility to iron deficiency. More so, in pregnancy there is an increase in the body's iron requirement by about 1000-1200 mg which is usually not met by dietary intake. Iron supplements could be given to pregnant sicklers, caution should however be taken in patients with history of recurrent transfusion.Anaemia is a common and feared complication in pregnancy. The co-existence of iron deficiency anaemia and sickle cell anaemia worsens prognosis of pregnancy. Iron overload a possible complication of sickle cell anaemia is related to multiple transfusions. The urinary losses from intravascular haemolysis and increased dietary requirement in pregnancy predispose even pregnant sicklers to iron deficiency anaemia. Iron supplements should thus conveniently be given to pregnant sicklers with no history of recurrent transfusions.

Audit of HIV counselling and testing services among primary healthcare facilities in Cameroon: a protocol for a multicentre national cross-sectional study.

INTRODUCTION: HIV testing is an invaluable entry point to prevention, care and treatment services for people living with HIV and AIDS. Poor adherence to recommended protocols and guidelines reduces the performance of rapid diagnostic tests, leading to misdiagnosis and poor estimation of HIV seroprevalence. This study seeks to evaluate the adherence of primary healthcare facilities in Cameroon to recommended HIV counselling and testing (HCT) procedures and the impact this may have on the reliability of HIV test results. METHODS AND ANALYSIS: This will be an analytical cross-sectional study involving primary healthcare facilities from all the 10 regions of Cameroon, selected by a multistaged random sampling of primary care facilities in each region. The study will last for 9 months. A structured questionnaire will be used to collect general information concerning the health facility, laboratory and other departments involved in the HCT process. The investigators will directly observe at least 10 HIV testing processes in each facility and fill out the checklist accordingly. ETHICS AND DISSEMINATION: Clearance has been obtained from the National Ethical Committee to carry out the study. Informed consent will be sought from the patients to observe the HIV testing process. The final study will be published in a peer-reviewed journal and the findings presented to health policy-makers and the general public.

Evolutionary aspects of non-metastatic breast cancer after primary treatment in a sub-Saharan African setting: a 16-year retrospective review at the Douala general hospital, Cameroon.

BACKGROUND: Breast cancer has a high case fatality rate in sub-Saharan Africa, and this is chiefly because of late detection and inadequate treatment resources. Progressive renovations in diagnostic and management modalities of non-metastatic breast cancer (NMBC) have been noted in the region but there is paucity of data describing the clinical progress of patients with NMBC. This study sought to determine the rates of local relapse, distant metastasis and sequelae and the time span from initial treatment to the occurrence of these adverse events among patients with NMBC. METHODS: This was a retrospective review of medical records of patients with histologically confirmed NMBC at the department of radiation therapy and oncology of the Douala General Hospital in Cameroon from the January 1997 to December 2012 period. Clinicopathological and treatment characteristics as well as occurrences of adverse events were studied. RESULTS: A total of 260 cases were reviewed of which 224/260 (86.2%) had invasive ductal carcinoma. Surgery was performed on 258/260 cases (99.2%) with 187/258 (72.5%) being modified radical mastectomies. Various treatment combinations were used in up to 228/260 patients (87.5%) while surgery alone was the treatment in the remaining 32 cases (12.5%). Metastasis occurred in 142/260 cases (54.6%) of which 68/142 (26.2%) were local relapses and 74/142 (28.5%) were distant metastases. Among the cases of distant metastasis, 9.2% were bone, 8.5% lungs, 6.9% nodal, and 5.4% brain. Metastasis to multiple organs was noted in 4.7% of these cases. The median periods of occurrence of local relapse and distant metastases were 13 and 12 months respectively. Sequelae occurred in 26/260 cases (10%) and were noted after an average of 30 months. The main sequelae were lymphoedema (6.5%) and lung fibrosis (1.5%). At the end of the period under review, 118/260 patients (45.4%) were alive and disease-free with a median follow up time of 24 months. CONCLUSIONS: Adverse events were frequent among patients who received primary treatment for NMBC. Available cancer therapeutic modalities ought to be supplemented with efficient strategies of follow-up and monitoring so as to optimize the care provided to these patients and improve on their survival.

Spina bifida cystica and severe congenital bilateral talipes equinovarus in one twin of a monoamniotic pair: a case report.

BACKGROUND: Spina bifida and congenital talipes equinovarus (CTEV) are common congenital malformations which may occur together and increase morbidity. Monozygous twins are particularly at risk of these malformations and discordance in one type of malformation is typical. The occurrence of both spina bifida and CTEV in one twin of a monozygotic pair is rare. CASE PRESENTATION: A 22 year-old Cameroonian primigravida at 36 weeks of a twin gestation was received in our district hospital at the expulsive phase of labour on a background of sub-optimal antenatal care. A caesarean section indicated for cephalo-pelvic disproportion was performed and life monoamniotic male twins were extracted. The first twin was normal. The second twin had spina bifida cystica and severe bilateral CTEV. Routine postnatal care was ensured and at day 2 of life, the affected twin was evacuated to a tertiary hospital for proper management. He was later on reported dead from complications of hydrocephalus. CONCLUSIONS: Spina bifida cystica with severe bilateral CTEV in one twin of a monoamniotic pair illustrates the complexity in the interplay of causal factors of these malformations even among monozygotic twins who are assumed to share similar genetic and environmental features. The occurrence and poor outcome of the malformations was probably potentiated by poor antenatal care. With postnatal diagnoses, a better outcome was difficult to secure even with prompt referral. Early prenatal diagnoses and appropriate counseling of parents are cardinal.

Delayed diagnosis of transanal prolapse of an ileo-colic intussusception in a 10-month-old infant in rural Cameroon: a case report.

BACKGROUND: Transanal protrusion of intussusception is a complication of intussusception which involves the exteriorization of the apex of the intussusceptum through the anus. However, it is rarely reported and its confusion with rectal prolapse often leads to a diagnostic delay. CASE PRESENTATION: A 10-month-old female with no significant past history from a rural area in the Extreme North region of Cameroon  was referred from a local health centre to our emergency deparment for an irreducible mass. It was reported that the child had spent 5 days at home on over-the-counter medication, then 3 days at a health centre where she was being treated for a respiratory tract infection and a rectal prolapse. On arrival at our hospital, she was conscious and moderately dehydrated. Cardiopulmonary examination revealed generalized coarse crackles over both lung fields. Her abdomen was tender, with a left upper quadrant mass, absent bowel sounds and a dark anal mass. In view of these, diagnoses of bronchopneumonia, intestinal obstruction and a probable rectal prolapse were made. An exploratory laparotomy was carried out after resuscitation with per-operative findings of a prolapsed ileo- colic intussusception and a necrosed intussusceptum. The necrosed portion was resected and an end-to-end ileo-transverse anastomosis was carried out. The immediate post- operative period was uneventful, but the patient died 3 days after the surgery, from an overwhelming sepsis. CONCLUSIONS: Transanal protrusion of intussusception requires timely surgical intervention to prevent mortality. The similarity in presentation to rectal prolapse coupled with inadequate knowledge on the condition by primary healthcare personnel causes a delay in the diagnosis and an increased mortality. A high index of suspicion is essential for an early diagnosis and an improved referral system for timely and definitive treatment.

Diagnostic and therapeutic challenges of an ambiguous cystic kidney disease in a resource limited setting: a case report.

BACKGROUND: Unilateral renal cystic disease is a rare condition that shares morphological similarities with multicystic dysplastic kidney, the former often distinguished from the latter on some clinical and histopathological grounds. However serious diagnostic and therapeutic dilemmas set in when there is a considerable overlap in the distinguishing features between these entities. CASE PRESENTATION: A 19-year-old African female presented with a chronic severe debilitating right lower quadrant abdominal pain refractory to analgesics. Biochemical investigations and imaging studies revealed a non-functional polycystic right kidney and no identifiable pelvicalyceal system or ureter but with preserved renal function. The marked overlap in clinical presentation between unilateral renal cystic disease and multicystic dysplastic kidney in this patient necessitated further investigation to pose an appropriate diagnosis. A right nephrectomy was performed and histopathological analysis of the resected kidney done, the results of which were more consistent with unilateral renal cystic disease. The post-operative course was favorable. CONCLUSION: Unilateral renal cystic disease with an ipsilateral non-functional kidney and an atretic pelvicalyceal system is a very rare condition that needs to be distinguished from multicystic dysplastic kidney in order to guide management and set prognosis. A suspicion of either of these diseases therefore warrants a thorough clinical evaluation and the appropriate combination of biochemical and imaging investigations.

Occurrence of Emery-Dreifuss muscular dystrophy in a rural setting of Cameroon: a case report and review of the literature.

BACKGROUND: Emery-Dreifuss muscular dystrophy is a rare genetic muscular disease, presenting mainly with contractures, weakness and cardiac conduction abnormalities. Its clinical and laboratory similarities to other muscular dystrophies, and rarity poses diagnostic challenges, requiring a high index of suspicion in resource limited settings. CASE PRESENTATION: An 8 year old sub-Saharan male presented with rigidity and deformity of both elbows and ankles, and weakness of the upper limbs and lower limbs for duration of 4 months. This progressed to inability to stand and walk. There was no mental impairment. Physical examination was remarkable for contractures of the elbows and ankles, and wasting of muscles of the limbs and trunk, with a scapulohumeroperoneal pattern, and tachycardia. After laboratory investigations, a diagnosis of Emery-Dreifuss muscular dystrophy was suspected. Physiotherapy was started, wheel chair was prescribed, and referral to a specialist center was done for appropriate management. CONCLUSIONS: Emery-Dreifuss muscular dystrophy is a rare disabling muscular disease which poses a diagnostic challenge. High index of suspicion is paramount for its early diagnoses to prevent orthopedic and cardiac complications. Prompt diagnosis and management is essential to improve on the prognosis of this disease.

Concurrent hyperthyroidism and papillary thyroid cancer: a fortuitous and ambiguous case report from a resource-poor setting.

BACKGROUND: Concurrent thyroid cancer (TC) and hyperthyroidism (HT) is rare though increasingly being reported. HT due to TC is much rarer and more challenging especially in Africa where TC and HT have significant case fatality rates. CASE PRESENTATION: We present a 37-year-old Cameroonian female who had been on irregular regimens of propranolol and digoxin as treatment for worsening palpitations for 12 months. She came to our district hospital for her propranolol medication refill. We fortuitously identified features of HT and found a left uninodular goiter with no cervical lymphadenopathy. She was referred for thyroid assessment which suggested primary HT and an enlarged heterogeneous left lobe with a well-defined homogenous solid mass. We restarted her on propranolol and referred her for a course of methimazole. At the referral hospital, she also underwent a left thyroid lobectomy. The resected lobe was sent for histopathology which revealed a neoplastic nodule with features suggestive of a papillary thyroid cancer (PTC) causing HT. The patient's clinical progress postoperatively was good and there was regression of hyperthyroid symptoms. CONCLUSIONS: The historical, clinical, and laboratory findings were suggestive of HT due to PTC. A high index of suspicion, prompt referral and counter-referral lead to a positive outcome of such a rare case in a resource poor setting. We advocate for systematic and careful evaluation of all thyroid nodules.