Linkage to Care Intervention to Improve Post-Hospital Outcomes Among Children with Sickle Cell Disease in Tanzania: A Pilot Study.

We conducted a pilot study to determine the effectiveness of a linkage to care intervention with social workers to improve 12-month post-hospital mortality for children in Tanzania with sickle cell disease. Comparison was done with a historical cohort. Mortality was 6.7% in the interventional cohort compared with 19.2% (adjusted Hazard Ratio, 0.26; 95% CI, 0.08-0.83).

Renal abnormalities and its associated factors among school-aged children living in Schistosoma mansoni endemic communities in Northwestern Tanzania.

BACKGROUND: In sub-Saharan Africa, renal abnormalities are a major public health concern, especially in children living in Schistosoma haematobium endemic areas. However, there is a dearth of data on renal abnormalities among children living in Schistosoma mansoni endemic areas. The objective of the study was to assess the prevalence of renal abnormalities among school children in a Schistosoma mansoni endemic community in Northwestern Tanzania. METHODS: A cross-sectional study was conducted between January and March 2017 among school children aged 6-13 years, attending three primary schools located along the shoreline of Lake Victoria. A single urine sample was collected from each child and screened for S. mansoni using circulating cathodic antigen and for S. haematobium eggs using a urine filtration technique. A urine dipstick was used to screen for urine protein levels, creatinine levels, microalbuminuria, and red blood cells. Venous blood was obtained for estimation of creatinine level and for malaria diagnosis. The primary outcomes were the prevalence of renal abnormalities, defined by the presence of low estimated glomerular filtration rate (eGFR), proteinuria or microalbuminuria, and hematuria in urine. RESULTS: Of 507 children included in the final analysis, 49.9% (253/507) were male with a mean age of 8.51 ± 1.3 years. Overall, 64.0% (326/507) of the children were infected with S. mansoni, and 1.6% (8/507) of the children were infected with S. haematobium. A total of 71 (14%) of the children had proteinuria, 37 (7.3%) had hematuria, and 8 (1.6%) had a low estimated glomerular filtration rate (eGFR). Overall prevalence of renal abnormalities was 22.9%. Renal abnormalities (proteinuria) were associated with S. mansoni infection (OR = 4.9, 95% CI 2.1-11.2, p < 0.001) and having red blood cells in urine (OR = 5.3, 95% CI 2.5-11.2, p < 0.001). CONCLUSION: Twenty-two percent of school children who participated in this study had renal abnormalities associated with S. mansoni infection. Given the high prevalence of S. mansoni, longitudinal epidemiological surveillance is warranted to measure the burden of renal abnormalities and assess the impact of the praziquantel treatment on these abnormalities.

Assessing the Impact of Patient-Facing Mobile Health Technology on Patient Outcomes: Retrospective Observational Cohort Study.

BACKGROUND: Despite the growth of and media hype about mobile health (mHealth), there is a paucity of literature supporting the effectiveness of widespread implementation of mHealth technologies. OBJECTIVE: This study aimed to assess whether an innovative mHealth technology system with several overlapping purposes can impact (1) clinical outcomes (ie, readmission rates, revisit rates, and length of stay) and (2) patient-centered care outcomes (ie, patient engagement, patient experience, and patient satisfaction). METHODS: We compared all patients (2059 patients) of participating orthopedic surgeons using mHealth technology with all patients of nonparticipating orthopedic surgeons (2554 patients). The analyses included Wilcoxon rank-sum tests, Kruskal-Wallis tests for continuous variables, and chi-square tests for categorical variables. Logistic regression models were performed on categorical outcomes and a gamma-distributed model for continuous variables. All models were adjusted for patient demographics and comorbidities. RESULTS: The inpatient readmission rates for the nonparticipating group when compared with the participating group were higher and demonstrated higher odds ratios (ORs) for 30-day inpatient readmissions (nonparticipating group 106/2636, 4.02% and participating group 54/2048, 2.64%; OR 1.48, 95% CI 1.03 to 2.13; P=.04), 60-day inpatient readmissions (nonparticipating group 194/2636, 7.36% and participating group 85/2048, 4.15%; OR 1.79, 95% CI 1.32 to 2.39; P<.001), and 90-day inpatient readmissions (nonparticipating group 261/2636, 9.90% and participating group 115/2048, 5.62%; OR 1.81, 95% CI 1.40 to 2.34; P<.001). The length of stay for the nonparticipating cohort was longer at 1.90 days, whereas the length of stay for the participating cohort was 1.50 days (mean 1.87, SD 2 vs mean 1.50, SD 1.37; P<.001). Patients treated by participating surgeons received and read text messages using mHealth 83% of the time and read emails 84% of the time. Patients responded to 60% of the text messages and 53% of the email surveys. Patients were least responsive to digital monitoring questions when the hospital asked them to do something, and they were most engaged with emails that did not require action, including informational content. A total of 96% (558/580) of patients indicated high satisfaction with using mHealth technology to support their care. Only 0.40% (75/2059) patients opted-out of the mHealth technology program after enrollment. CONCLUSIONS: A novel, multicomponent, pathway-driven, patient-facing mHealth technology can positively impact patient outcomes and patient-reported experiences. These technologies can empower patients to play a more active and meaningful role in improving their outcomes. There is a deep need, however, for a better understanding of the interactions between patients, technology, and health care providers. Future research is needed to (1) help identify, address, and improve technology usability and effectiveness; (2) understand patient and provider attributes that support adoption, uptake, and sustainability; and (3) understand the factors that contribute to barriers of technology adoption and how best to overcome them.

A clinical trial of enteral Levetiracetam for acute seizures in pediatric cerebral malaria.

BACKGROUND: Acute seizures are common in pediatric cerebral malaria (CM), but usual care with phenobarbital risks respiratory suppression. We undertook studies of enteral levetiracetam (eLVT) to evaluate pharmacokinetics (PK), safety and efficacy including an open-label, randomized controlled trial (RCT) comparing eLVT to phenobarbital. METHODS: Children 24-83 months old with CM were enrolled in an eLVT dose-finding study starting with standard dose (40 mg/kg load, then 30 mg/kg Q12 hours) titrated upward until seizure freedom was attained in 75% of subjects. The RCT that followed randomized children to eLVT vs. phenobarbital for acute seizures and compared the groups on minutes with seizures based upon continuous electroencephalogram. Due to safety concerns, midway through the study children allocated to phenobarbital received the drug only if they continued to have seizures (either clinically or electrographically) after benzodiazepine treatment. Secondary outcomes were treatment failure requiring cross over, coma duration and neurologic sequelae at discharge. PK and safety assessments were also undertaken. RESULTS: Among 30 comatose CM children, eLVT was rapidly absorbed and well-tolerated. eLVT clearance was lower in patients with higher admission serum creatinine (SCr), but overall PK parameters were similar to prior pediatric PK studies. Within 4 h of the first dose, 90% reached therapeutic levels (> 20 µg/mL) and all were above 6 µg/mL. 7/7 children achieved seizure freedom on the initial eLVT dose. Comparing 23 eLVT to 21 phenobarbital patients among whom 15/21 received phenobarbital, no differences were seen for minutes with seizure, seizure freedom, coma duration, neurologic sequelae or death, but eLVT was safer (p = 0.019). Phenobarbital was discontinued in 3/15 due to respiratory side effects. CONCLUSION: Enteral LVT offers an affordable option for seizure control in pediatric CM and is safer than phenobarbital. TRIAL REGISTRATION: NCT01660672 . NCT01982812 .

Very severe anemia and one year mortality outcome after hospitalization in Tanzanian children: A prospective cohort study.

BACKGROUND: Africa has the highest rates of child mortality. Little is known about outcomes after hospitalization for children with very severe anemia. OBJECTIVE: To determine one year mortality and predictors of mortality in Tanzanian children hospitalized with very severe anemia. METHODS: We conducted a prospective cohort study enrolling children 2-12 years hospitalized from August 2014 to November 2014 at two public hospitals in northwestern Tanzania. Children were screened for anemia and followed until 12 months after discharge. The primary outcome measured was mortality. Predictors of mortality were determined using Cox regression analysis. RESULTS: Of the 505 children, 90 (17.8%) had very severe anemia and 415 (82.1%) did not. Mortality was higher for children with very severe anemia compared to children without over a one year period from admission, 27/90 (30.0%) vs. 59/415 (14.2%) respectively (Hazard Ratio (HR) 2.42, 95% Cl 1.53-3.83). In-hospital mortality was 11/90 (12.2%) and post-hospital mortality was 16/79 (20.2%) for children with very severe anemia. The strongest predictors of mortality were age (HR 1.01, 95% Cl 1.00-1.03) and decreased urine output (HR 4.30, 95% Cl 1.04-17.7). CONCLUSIONS: Children up to 12 years of age with very severe anemia have nearly a 30% chance of mortality following admission over a one year period, with over 50% of mortality occurring after discharge. Post-hospital interventions are urgently needed to reduce mortality in children with very severe anemia, and should include older children.

Post-hospital mortality in children aged 2-12 years in Tanzania: A prospective cohort study.

BACKGROUND: Sub-Saharan Africa has the highest rates of child mortality worldwide. Little is known about post-hospital outcomes after an index hospitalization for older children. We determined 12-month post-hospital mortality rate and identified factors associated with higher mortality. METHODS: In this prospective cohort study, we enrolled children 2-12 years of age admitted to the pediatric wards of two public hospitals in northwestern Tanzania. Participants or proxies were contacted at 3, 6 and 12 months post-hospitalization. The primary outcome measured was mortality. Factors associated with mortality were determined using Cox regression analysis. RESULTS: A total of 506 participants were enrolled. In-hospital mortality rate was 7.7% (39/506). Of the 467 participants discharged, the post-hospital mortality rate was 10.1% (47/467). Sickle cell disease (Hazard Ratio (HR) 3.32, 95% CI 1.44-7.68), severe malnutrition (HR 3.19, 95% CI 1.18-8.57), neurologic diseases (HR 3.51, 95% CI 1.35-9.11), heart disease (HR 7.11, 95% CI, 2.89-17.51), cancer (HR 11.79, 95% CI 4.95-28.03), and septic shock (HR 4.64, 95% CI 1.42-15.08) had higher association with mortality compared to other diagnoses. The risk factors significantly associated with mortality included older age (HR 1.01, 95% CI 1.00-1.08), lower hemoglobin level (HR 0.83, 95% CI 0.76-0.90), lower Glasgow Coma Scale (HR 0.66, 95% CI 0.59-0.74), history of decreased urine output (HR 2.87, 95% CI 1.49-5.53), higher respiratory rate (HR 1.02, 95% CI 1.00-1.03), estimated glomerular filtration rate less than 60 ml/min/1.73m2 (binary) (HR 1.84, 95% CI 1.10-3.10), and lower oxygen saturation (HR 0.96, 95% CI 0.92-0.99). CONCLUSIONS: Post-hospital mortality is disturbingly high among children 2-12 years of age in Tanzania. Post-hospital interventions are urgently needed especially for older children with chronic illnesses.

Nerve abscess in Hansen's disease as part of immune reconstitution inflammatory syndrome: a case report.

Immune reconstitution inflammatory syndrome is an inflammatory reaction in HIV-infected patients after initiation of antiretroviral therapy resulting from restored immunity to specific infectious or non-infectious antigens. A 36-year-old male patient on highly active antiretroviral therapy of six months duration, presented with reddish, tender lesions over medial aspect of arm and a single, anaesthetic patch. Tender fluctuant swellings were seen on the medial aspect of left forearm. A few of them had ruptured spontaneously discharging pus. A skin biopsy from the anaesthetic patch showed caseating epitheloid granulomas. A diagnosis of Hansen's disease borderline tuberculoid in type 1 reversal reaction, with formation of nerve abscess due to Immune Reconstitution Inflammatory Syndrome was made. The patient was started on multibacillary multidrug therapy as per WHO guidelines and highly active antiretroviral therapy was continued.

Use of an Android application "clinometer" for measurement of head down tilt given during subarachnoid block.

CONTEXT: Head down tilt is given to patients after sub arachnoid block for adjustment of height of block. However, the amount of tilt given is subjective and cannot be documented. AIMS: We used an android application named "clinometer" to measure exact degree of tilt given by anesthesiologists as their routine practice. SETTINGS AND DESIGN: This observational study, at a medical college hospital, was done in 130 patients given sub arachnoid block for lower abdominal surgeries. MATERIALS AND METHODS: We observed and documented vital data of patients and measured tilt given by application "clinometer." RESULTS: We observed that the application was easy to use and measured tilt each time. The result obtained can be documented, digitally saved and transferred. In 130 patients studied, we observed incidence of degree of tilt as follows: 6-8° tilt in 38 patients (29.23%), 8-10 in 36 patients (27.69%), 10-12 in 30 patients (23.08%), 12-14 in 12 patients (9.23%) and 14-16° tilt in 14 patients (10.77%). Use of application was received with enthusiasm by practicing anesthesiologists. Various possible uses of this application are discussed.

Surgical management of vascular anomalies in children at a tertiary care hospital in a resource-limited setting: a Tanzanian experience with 134 patients.

BACKGROUND: Vascular anomalies pose major diagnostic and therapeutic challenges among pediatricians and pediatric surgeons practicing in resource limited countries. There is paucity of published data regarding this subject in Tanzania and Bugando Medical Centre in particular. This study describes our experiences on the challenges and outcome of surgical management of childhood vascular anomalies in our environment. METHODS: Between January 2009 and December 2013, a prospective study on the surgical management of vascular anomalies was undertaken at Bugando Medical Centre. RESULTS: A total of 134 patients (M; F = 1:2.5) were studied. The median age at presentation was 6 years. Of the 134 patients, 101 (75.4%) were diagnosed as having vascular tumors and 33 (24.6%) had vascular malformations. The head and the neck were the most frequent anatomical site recorded as having a tumor (56.7% of patients). Out of 134 patients, 129 (96.3%) underwent surgical treatment. Failure to respond to non-operative treatment (86.8%), huge disfiguring/obstructing mass (4.7%), infection (3.1%), ulceration (3.1%) and hemorrhage (2.3%) were indications for surgical intervention. Tumor excision and primary wound closure was the most common type of surgical procedure performed in 80.6% of patients. Surgical site infection was the most frequent complications accounting for 33.8% of cases. Mortality rate was 1.5%. Tumor excision and primary wound closure gave better outcome compared with other surgical options (p < 0.001). Outcome of injection sclerotherapy in 3 (3.7%) children, serial ligation of feeder vessels employed in 2 (1.6%), and conservative treatment in 5 (3.7%), were poor and required conversion to surgical excision. Despite low mortality rate recorded in this study, but ugly scar, 14 (20.6%) and limb deformity, 6 (8.8%) were problems. The overall result of surgical treatment at the end of follow up period was excellent in 108 (87.1%) patients. CONCLUSION: Surgical excision and primary wound closure gave good outcome which could be employed in complicated and vascular anomalies which failed to respond to other treatment in regions with limited resources.

Head and neck cancers: a clinico-pathological profile and management challenges in a resource-limited setting.

BACKGROUND: Head and neck cancer (HNC) is one of the most common cancers worldwide and its incidence is reported to be increasing in resource-limited countries. There is a paucity of published data regarding head and neck cancers in Tanzania, and Bugando Medical Centre in particular. This study describes the clinicopathological profile of HNC in our local setting and highlights the challenges in the management of this disease. METHODS: This was a retrospective study of histopathologically confirmed cases of head and neck cancers treated at Bugando Medical Center between January 2009 and December 2013. RESULTS: A total of 346 patients (M:F = 2.1:1) were studied representing 9.5 % of all malignancies. The median age of patients was 42 years. Cigarette smoking (76.6 %) and heavy alcohol consumption (69.9 %) were the most frequently identified risk factors for head and neck cancer. The majority of patients (95.9 %) presented late with advanced stages. Twenty-five (7.2 %) patients were HIV positive with a median CD4+ count of 244 cells/µl. The oral cavity (37.3 %) was the most frequent anatomical site affected. The most common histopathological type was carcinomas (59.6 %) of which 75.7 % were squamous cell carcinoma. A total of 196 (56.6 %) patients underwent surgical procedures for HNC. Radiotherapy and chemotherapy was reported in 9.5 and 16.8 % of patients, respectively. Only 2 (0.6 %) patients received chemo-radiation therapy. The mortality rate was 24.4 %. The overall 5-year survival rate (5-YSR) was 20.6 %. The predictors of overall 5-YSR were age of patient at diagnosis, stage of disease, extent of lymph node involvement, HIV seropositivity and CD4+ count <200 cells/µl (P < 0.001). Local recurrence was reported in 22 (23.4 %) patients and this was significantly associated with positive resection margins, stage of the tumor and presence of metastasis at diagnosis and non-adherence to adjuvant therapy (P < 0.001). CONCLUSION: Head and neck cancers are not uncommon at Bugando Medical Centre and show a trend towards a relative young age at diagnosis and the majority of patients present late with advanced stage cancer. Therefore, public enlightenment, early diagnosis, and effective cost-effective treatment and follow-up are urgently needed to improve outcomes of these patients in our environment.

Concurrent Presentation of Erythrodermic Lichen Planus and Squamous Cell Carcinoma: Coincidence or Malignant Transformation?

Lichen planus is a common papulosquamous disorder affecting about 1-2% of the population, neoplastic transformation of cutaneous lichen planus lesions occurs very rarely. A 40 year old female patient presented with a 1 year history of developing multiple, itchy, pigmented lesions over both lower legs which gradually spread to involve the whole body. A few tense bullae were seen on the extremities. An erythematous fleshy lesion was seen on the upper aspect of the left buttock. Skin biopsy from a plaque on the right forearm showed features suggestive of lichen planus. Skin biopsy of a bullae showed a sub epidermal bulla filled with a mixed inflammatory infiltrate. Direct immunofluorescence revealed no immunoreactants along the basement membrane zone. A diagnosis of erythrodermic lichen planus with bullous lichen planus was made. Biopsy of fleshy lesion of left buttock revealed a moderately differentiated squamous cell carcinoma. Erythrodermic lichen planus with bullous lesions and secondary squamous cell carcinoma; these occurences in a single patient is extremely rare and has not been previously reported to the best of our knowledge.

Infantile hypertrophic pyloric stenosis at a tertiary care hospital in Tanzania: a surgical experience with 102 patients over a 5-year period.

BACKGROUND: Infantile hypertrophic pyloric stenosis (IHPS) is the most common cause of gastric outlet obstruction in infants. There is paucity of published data regarding this condition in our setting. This study describes the clinical presentation, mode of treatment and outcome of treatment of this disease and identifies factors responsible for poor outcome of these patients. METHODS: This was a descriptive retrospective study of infants with HPS admitted to Bugando Medical Centre and subsequently underwent surgery between February 2009 and January 2014. RESULTS: A total of 102 patients (M:F = 4.7:1) were studied. The median age at presentation was 5 weeks. The median duration of illness was 4 weeks. Fifty-four (52.9 %) patients occur in first-born children. Associated anomalies were reported in 7 (6.9 %) patients. Non-bilious vomiting was the most frequent symptom and it was described in all (100 %) patients. A palpable mass was found in 23.5 % of infants. The diagnosis of IHPS was made clinically in 86 (84.3 %) and by ultrasound in 16 (15.7 %) patients. The treatment was Ramstedt's pyloromyotomy in all cases. There were 6 (5.9 %) intra-operative mucosal perforations which were repaired successively. Postoperative complication was 11.8 %. The median length of hospital stay was 12 days and it was significantly associated with prolonged pre-operative hospitalization (p = 0.001). The mortality rate was 4.9 %. Age below 2 weeks, late presentation (≥14 days), severe dehydration on admission, hypokalaemia on admission and surgical site infection were the main predictors of mortality (p < 0.001). CONCLUSION: This study has shown that IHPS is a common condition in our setting. Age <2 weeks, delayed presentation, prolonged preoperative hospital stay, surgical site infection and high proportion of dehydration and electrolyte disturbance were the main predictors of poor outcome. A high index of suspicion is needed in infants with non-bilious vomiting to avoid delay in diagnosis.

Role of C-reactive protein as a marker of disease severity and cardiovascular risk in patients with psoriasis.

BACKGROUND: There is a paucity of studies addressing the elevation of C-reactive protein (CRP) among psoriatic patients and the role of this marker in assessment of disease severity and association with cardiovascular diseases (CVDs). OBJECTIVE: To assess the difference in CRP levels between psoriatic patients and healthy population and to determine their role in disease severity. Also to compare CRP levels in psoriatic patients with and without the metabolic syndrome. MATERIALS AND METHODS: A total of hundred patients with chronic plaque psoriasis and an equal number of age- and gender-matched healthy controls were enrolled in the study over a period of one year. Serum CRP levels of both cases and controls were estimated. Metabolic syndrome was identified among psoriasis patients using National Cholesterol Education Program's Adult Panel III (ATP III) guidelines. Clinical activity of psoriasis was evaluated using Psoriasis Area and Severity Index Score. RESULTS: Patients with psoriasis reported significantly higher levels of CRP than healthy controls (P value 0.001). Patients with severe disease had significantly higher levels of CRP (P value < 0.003). Elevated level of CRP was observed among psoriatic patients with the metabolic syndrome than patients without the metabolic syndrome and the difference was statistically significant (P value = 0.001). CONCLUSION: CRP may be considered as a useful marker of psoriasis severity that could be used to monitor psoriasis and its treatment. Elevated levels of CRP may be an independent risk factor for CVD in patients with psoriasis.

The influence of HIV and schistosomiasis on renal function: a cross-sectional study among children at a hospital in Tanzania.

BACKGROUND: Schistosomiasis and HIV are both associated with kidney disease. Prevalence and factors associated with abnormal renal function among HIV-infected children in Africa compared to uninfected controls have not been well described in a schistosomiasis endemic area. METHODOLOGY/PRINCIPAL FINDINGS: This cross-sectional study was conducted at the Sekou Toure Regional Hospital HIV clinic in Mwanza, Tanzania. A total of 122 HIV-infected children and 122 HIV-uninfected siblings were consecutively enrolled. Fresh urine was obtained for measurement of albuminuria and Schistosoma circulating cathodic antigen. Blood was collected for measurement of serum creatinine. Estimated glomerular filtration rate (eGFR) was calculated using the modified Schwartz equation. Renal dysfunction was defined operationally as eGFR<60 mL/min/1.73 m2 and/or albuminuria>20 mg/L in a single sample. Among 122 HIV-infected children, 61/122 (50.0%) met our criteria for renal dysfunction: 54/122 (44.3%) had albuminuria>20 mg/L and 9/122 (7.4%) had eGFR<60. Among 122 HIV-uninfected children, 51/122 (41.8%) met our criteria for renal dysfunction: 48/122 (39.3%) had albuminuria>20 mg/L and 6/122 (4.9%) had eGFR<60. Schistosomiasis was the only factor significantly associated with renal dysfunction by multivariable logistic regression (OR = 2.51, 95% CI 1.46-4.31, p = 0.001). CONCLUSIONS/SIGNIFICANCE: A high prevalence of renal dysfunction exists among both HIV-infected Tanzanian children and their HIV-uninfected siblings. Schistosomiasis was strongly associated with renal dysfunction.

Kidney disease among children in sub-Saharan Africa: systematic review.

The global burden of kidney disease is increasing, and several etiologies first begin in childhood. Risk factors for pediatric kidney disease are common in Africa, but data regarding its prevalence are lacking. We completed a systematic review of community-based studies describing the prevalence of proteinuria, hematuria, abnormal imaging, or kidney dysfunction among children in sub-Saharan Africa (SSA). Medline and Embase were searched. Five hundred twenty-three references were reviewed. Thirty-two references from nine countries in SSA were included in the qualitative synthesis. The degree of kidney damage and abnormal imaging varied widely: proteinuria 32.5% (2.2-56.0%), hematuria 31.1% (0.6-67.0%), hydronephrosis 11.3% (0.0-38.0%), hydroureter 7.5% (0.0-26.4%), and major kidney abnormalities 0.1% (0.0-0.8%). Serum creatinine was reported in four studies with insufficient detail to identify the prevalence renal dysfunction. A majority of the studies were performed in Schistosoma haematobium endemic areas. A lower prevalence of kidney disease was observed in the few studies from nonendemic areas. Published data on pediatric kidney disease in SSA are highly variable and dependent on S. haematobium prevalence. More community-based studies are needed to describe the burden of pediatric kidney disease, particularly in regions where S. haematobium infection is nonendemic.

Hirschsprung's disease in children: a five year experience at a university teaching hospital in northwestern Tanzania.

BACKGROUND: Hirschsprung's disease (HD) is the commonest cause of functional intestinal obstruction in children and poses challenges to pediatricians and pediatric surgeons practicing in resource-limited countries. This study describes the clinical characteristics and outcome of management of this disease in our setting and highlights challenges associated with the care of these patients and proffer solutions for improved outcome. METHODS: This was a descriptive prospective study of children aged ≤ 10 years who were histologically diagnosed and treated for HD at our centre between July 2008 and June 2013. RESULTS: A total of 110 patients (M: F ratio= 3.6:1) with a median age of 24 months were studied. Six (5.5%) patients were in the neonatal period. Sixty-four (58.2%) patients had complete intestinal obstruction whereas 42 (38.2%) and 4 (3.6%) patients had chronic intestinal obstruction and intestinal perforation respectively. No patient had enterocolitis. Constipation (94.5%) was the most common complaints. 109 (99.1%) patients had colostomy prior to the definitive pull-through. The median duration of colostomy before definitive pull-through was 4 months. The majority of patients (67.3%) had short segment of aganglionosis localized to the recto-sigmoid region. The definitive pull-through was performed in 94 (85.5%) patients (Swenson's pull-through 76 (80.9%), Duhamel's pull-through (12.8%) and Soave's pull-through 4 (4.3%) patients). Postoperative complication rate was 47.3%. The median length of hospital stay was 26 days. Patients who developed complications stayed longer in the hospital and this was statistically significant (p <0.001). Mortality rate was 21.8%. The age < 4 weeks, delayed presentation and surgical site infection were the main predictors of mortality (p < 0.001). During the follow-up period, the results of Swenson's and Duhamel's pull through procedures were generally good in 87.8% and 42.9% of patients respectively. The result of Soave's procedures was generally poor in this study. CONCLUSION: HD remains the commonest cause of functional intestinal obstruction in children and contributes significantly to high morbidity and mortality in our setting. The majority of patients present late when the disease becomes complicated. Early diagnosis and timely definitive pull through procedure are essential in order to decrease the morbidity and mortality associated with this disease.

Childhood intussusceptions at a tertiary care hospital in northwestern Tanzania: a diagnostic and therapeutic challenge in resource-limited setting.

BACKGROUND: Intussusception remains a common cause of bowel obstruction in children and results in significant morbidity and mortality if not promptly treated. There is a paucity of prospective studies regarding childhood intussusception in Tanzania and particularly the study area. This study describes the pattern, clinical presentations and management outcomes of childhood intussusception in our setting and highlights the challenging problems in the management of this disease. METHODS: This was a prospective descriptive study of patients aged < 10 years operated for intussusception at Bugando Medical Centre. Ethical approval to conduct the study was obtained from relevant authorities. Data was analyzed using SPSS version 17.0. RESULTS: A total of 56 patients were studied. The male to female ratio was 3.3: 1. The median age was 6 months. Three-quarter of patients were < 1 year. Etiology was mainly idiopathic in 91.1% of cases. The classic triad of bloody stool, vomiting and abdominal distention/abdominal pain was found in 24 (42.5%) patients. The diagnosis of intussusception was mainly clinically in 71.4% of cases. All patients were treated surgically. Ileo-colic was the most frequent type of intussusception (67.9%). Twenty-six (46.4%) patients required bowel resection. The rate of bowel resection was significantly associated with late presentation > 24 hour (p = 0.001). Complication rate was 32.1% and surgical site infection (37.5%) was the most frequent complication. The median length of hospital stay was 7 days. Patients who had bowel resection and those who developed complications stayed longer in the hospital and this was statistically significant (p < 0.001). Mortality rate was 14.3%. Age < 1 year, delayed presentation, associated peritonitis, bowel resection and surgical site infection were the main predictors of mortality (p < 0.001). The follow up of patients was generally poor CONCLUSION: Intussusception in our setting is characterized by late presentation, lack of specialized facilities and trained personnel for nonsurgical reduction. Therefore, surgery remains the main stay of treatment in our centre. A high index of suspicion and proper evaluation of patients is essential for an early diagnosis and timely definitive treatment, in order to decrease the morbidity and mortality associated with this disease.

The impact of lesion in-painting and registration methods on voxel-based morphometry in detecting regional cerebral gray matter atrophy in multiple sclerosis.

BACKGROUND AND PURPOSE: VBM has been widely used to study GM atrophy in MS. MS lesions lead to segmentation and registration errors that may affect the reliability of VBM results. Improved segmentation and registration have been demonstrated by WM LI before segmentation. DARTEL appears to improve registration versus the USM. Our aim was to compare the performance of VBM-DARTEL versus VBM-USM and the effect of LI in the regional analysis of GM atrophy in MS. MATERIALS AND METHODS: 3T T1 MR imaging scans were acquired from 26 patients with RRMS and 28 age-matched NC. LI replaced WM lesions with normal-appearing WM intensities before image segmentation. VBM analysis was performed in SPM8 by using DARTEL and USM with and without LI, allowing the comparison of 4 VBM methods (DARTEL + LI, DARTEL - LI, USM + LI, and USM - LI). Accuracy of VBM was assessed by using NMI, CC, and a simulation analysis. RESULTS: Overall, DARTEL + LI yielded the most accurate GM maps among the 4 methods (highest NMI and CC, P < .001). DARTEL + LI showed significant GM loss in the bilateral thalami and caudate nuclei in patients with RRMS versus NC. The other 3 methods overestimated the number of regions of GM loss in RRMS versus NC. LI improved the accuracy of both VBM methods. Simulated data suggested the accuracy of the results provided from patient MR imaging analysis. CONCLUSIONS: We introduce a pipeline that shows promise in limiting segmentation and registration errors in VBM analysis in MS.

A 3T MR imaging investigation of the topography of whole spinal cord atrophy in multiple sclerosis.

BACKGROUND AND PURPOSE: Spinal cord atrophy is a common feature of MS. However, it is unknown which cord levels are most susceptible to atrophy. We performed whole cord imaging to identify the levels most susceptible to atrophy in patients with MS versus controls and also tested for differences among MS clinical phenotypes. MATERIALS AND METHODS: Thirty-five patients with MS (2 with CIS, 27 with RRMS, 2 with SPMS, and 4 with PPMS phenotypes) and 27 healthy controls underwent whole cord 3T MR imaging. The spinal cord contour was segmented and assigned to bins representing each C1 to T12 vertebral level. Volumes were normalized, and group comparisons were age-adjusted. RESULTS: There was a trend toward decreased spinal cord volume at the upper cervical levels in PPMS/SPMS versus controls. A trend toward increased spinal cord volume throughout the cervical and thoracic cord in RRMS/CIS versus controls reached statistical significance at the T10 vertebral level. A statistically significant decrease was found in spinal cord volume at the upper cervical levels in PPMS/SPMS versus RRMS/CIS. CONCLUSIONS: Opposing pathologic factors impact spinal cord volume measures in MS. Patients with PPMS demonstrated a trend toward upper cervical cord atrophy. However patients with RRMS showed a trend toward increased volume at the cervical and thoracic levels, which most likely reflects inflammation or edema-related cord expansion. With the disease causing both expansion and contraction of the cord, the specificity of spinal cord volume measures for neuroprotective therapeutic effect may be limited.