Rare treatable limb girdle muscle disease.

We report two cases of Limb Girdle pattern of muscle weakness caused by hyperparathyroidism due to parathyroid adenoma. It can be easily missed as early symptoms are non specific but once diagnosed it is easily treatable and complete recovery occurs over a period of time.

Two cases of a rare treatable limb girdle muscle disease.

We report two cases of limb girdle pattern of muscle weakness caused by hyperparathyroidism due to parathyroid adenoma. It can be easily missed as early symptoms are non specific but once diagnosed it is easily treatable and complete recovery occurs over a period of time.

Antifilarial drugs, in the doses employed in mass drug administrations by the Global Programme to Eliminate Lymphatic Filariasis, reverse lymphatic pathology in children with Brugia malayi infection.

Lymphatic filariasis is increasingly viewed as the result of an infection that is often acquired in childhood. The lymphatic pathology that occurs in the disease is generally believed to be irreversible. In a recent study in India, Doppler ultrasonography and lymphoscintigraphy were used to explore subclinical pathology in 100 children from an area endemic for Brugia malayi infection. All the children investigated showed some evidence of current or previous filarial infection. Some were microfilaraemic but asymptomatic, some were amicrofilaraemic but had filarial disease or a past history of microfilaraemia and/or filarial disease, and the rest, though amicrofilaraemic, asymptomatic and without any history of microfilaraemia or filarial disease, were seropositive for antifilarial IgG(4) antibodies. All the children were treated every 6 months, with a single combined dose of diethylcarbamazine (6 mg/kg) and albendazole (400 mg), and followed up for 24 months. By the end of this period all but one of the children were amicrofilaraemic and the 'filarial dance sign' could not be detected in any of the 14 children who had initially been found positive for this sign. Although lymphoscintigraphy revealed lymph-node and lymph-vessel damage in 82% of the children at enrolment, in about 67% of the children this pathology was markedly reduced by the 24-month follow-up. These results indicate that the drug regimens used in the mass drug administrations run by the Global Programme to Eliminate Lymphatic Filariasis are capable of reversing subclinical lymphatic damage and can provide benefits other than interruption of transmission in endemic areas. The implications of these findings are presented and discussed.

Relevance of anti-BmR1 IgG4 antibodies in children from an area endemic for Brugia malayi infection in Kerala, India.

Brugian filariasis prevalent mostly in South-East Asian countries including India contributes to a small but significant proportion of the socioeconomic burden due to lymphatic filariasis. Along with bancroftian filariasis, brugian filariasis has been targeted for elimination globally. The lack of a reliable daytime diagnostic test has been seen as an important barrier to the successful implementation and monitoring of elimination programmes in brugia endemic areas. We evaluated an anti-BmRI-IgG4 antibody test namely, 'Brugia Rapid' in a large study meant to understand the clinical and pathological manifestations of brugian filariasis in children. We found the test superior to traditional night blood screening for microfilaraemia. Although an antibody detection test, we found it to be a reliable indicator of brugian infection. Among the 100 children studied extensively, 94% of the microfilaraemics, 86% of those showing filarial dance sign indicating presence of, live adult worms and 78% having abnormal lymphatics on lymphoscintigraphy were IgG4 positive. Coupled with its advantages like ease of use any time of the day, high sensitivity and specificity, this test may be the ideal tool to assist programme managers in their efforts to eliminate lymphatic filariasis where brugian infections are found.

Clinical and pathological aspects of filarial lymphedema and its management.

Lymphatic filariasis, transmitted by mosquitoes is the commonest cause of lymphedema in endemic countries. Among 120 million infected people in 83 countries, up to 16 million have lymphedema. Microfilariae ingested by mosquitoes grow into infective larvae. These larvae entering humans after infected mosquito bites grow in the lymphatics to adult worms that cause damage to lymphatics resulting in dilatation of lymph vessels. This earliest pathology is demonstrated in adults as well as in children, by ultrasonography, lymphoscintigraphy and histopathology studies. Once established, this damage was thought to be irreversible. This lymphatic damage predisposes to bacterial infection that causes recurrent acute attacks of dermato-lymphangio-adenitis in the affected limbs. Bacteria, mainly streptococci gain entry into the lymphatics through 'entry lesions' in skin, like interdigital fungal infections, injuries, eczema or similar causes that disrupt integrity of skin. Attacks of dermato-lymphangio-adenitis aggravates lymphatic damage causing lymphedema, which gets worse with repeated acute attacks. Elephantiasis is a late manifestation of lymphatic filariasis, which apart from limbs may involve genitalia or breasts. Lymphedema management includes use of antifilarial drugs in early stages, treatment and prevention of acute attacks through 'limb-hygiene', antibiotics and antifungals where indicated, and physical measures to reduce the swelling. In selected cases surgery is helpful.

Lymphoscintigraphic evidence of lymph vessel dilation in the limbs of children with Brugia malayi infection.

Lymphatic filariasis (LF) is targeted for global elimination by the year 2020. It was earlier believed that LF is mostly a disease of adults. Recent studies indicate that in endemic countries filarial infection starts mostly in childhood even though the disease manifestations occur much later in life. The initial damage to the lymph vessels where the adult worms are lodged is dilation, thought to be irreversible even with treatment. Most of these studies relate to bancroftian filariasis. Studies that address this early pathology in brugian filariasis in humans are scarce. We report here for the first time, the lymphatic abnormalities seen on lymphoscintigraphy (LSG) in children with Brugia malayi filariasis. LSG was performed in 100 children aged between 3-15 years, who were enrolled in the study either because they were microfilaremic; had present or past filarial disease or were positive for antifilarial IgG4 antibodies. Inguinal and axillary lymph nodes were imaged in most children. Dilated lymph vessels were visualized in 80 children and this pathology was evenly distributed in all the three study groups. Lymph vessels dilation was seen even in three year old children. The implications of these findings for management of LF and control programmes are discussed.

A prospective clinical review of "multi model" approach for treating ear keloids.

This is a prospective clinical study of 46 ear keloids in 31 patients (with a mean follow-up of 18 months) treated from January 2006 to December 2006 at The Queen Elizabeth Public Hospital, Barbados, West Indies by a single surgeon. The mean age is 21.9 years (range 3-66 years). Seven out of 46 lesions were recurrent lesions following previous surgery. All the lesions were excised surgically (extralesional). Ten out of 31 patients were given postoperative, Intralesional Triamcinolone starting from the 1(st) post operative visit on three visits at monthly intervals. Fourteen patients were given postoperative superficial X-ray therapy of 12 Gy in three equal fractions on three consecutive days starting from the 3(rd) postoperative day. Seven recurrent keloids of this study were given a combination of both superficial X-ray therapy and intralesional triamcinolone. All patients were followed at monthly intervals for three visits from the time of surgery and every three months until the end of the 1(st) year and then every six months thereafter. Five of 46 postoperative surgical wounds showed evidence of recurrence during the 1(st) year but could be suppressed with Intralesional triamcinolone. This study confirms that surgical excision of keloids supplemented with radiotherapy and/Intralesional triamcinolone is a reliable method with few complications. In addition, the study concludes that the key in preventing recurrence is regular clinical follow-up to encounter early recurring lesion (clinical evidence of raised scars or palpable nodules if deep seated) which is 100% susceptible to Intralesional triamcinolone for 2-3 times at monthly intervals.

Routine preoperative abdominal computed tomography in colon cancer: a utility study.

BACKGROUND: There is no proven utility for preoperative scanning of intraperitoneal colonic cancer with computed tomography (CT). A prospective study was conducted where CT was preoperatively incorporated into the management algorithm to ascertain its role in patient treatment. METHODS: Thoraco-abdominal CT was performed in both the elective and emergency settings in an unselected group of 73 patients referred to a coloproctology unit established over the last 30 months at the Queen Elizabeth Hospital, Barbados. CT and operative findings were compared to determine the influence that the preoperative CT had on surgical decision making. RESULTS: Unexpected additional pathology was found in 6.8% of patients. Sensitivity for the detection of visceral metastatic disease was 84.6%. Preoperative CT was deemed to be of clinical value in 24.7% of cases and to definitively alter surgical management in 13.7% of patients. These alterations in management included several types of multivisceral resections, the need for ureteric stenting or reimplantation, the utilization of stomas alone and the avoidance of resection in known metastatic disease. CONCLUSIONS: Preoperative CT of intraperitoneal colonic cancer adds important technical information to the conduct of the surgery and alters therapy in a selected patient group.

Preliminary findings from a cross-sectional study on lymphatic filariasis in children, in an area of India endemic for Brugia malayi infection.

As the more obvious clinical manifestations of the disease are very uncommon in children, lymphatic filariasis has been considered to be primarily a disease of adults. In many recent reports, however, there is evidence indicating not only that filarial infection is commonly acquired in childhood but also that many infected children already have irreversible damage to their lymphatics. The preliminary results of a cross-sectional study on the patterns of Brugia-attributable pathology in 7934 children (aged 3-15 years) who live in an area of India with endemic B. malayi infection confirm these trends. The children were screened for microfilaraemia, evidence of filarial disease, and the presence of antifilarial IgG(4) antibodies. One hundred children who were microfilaraemic but asymptomatic (32), with filarial disease or an history of such disease or microfilaraemia (29) or amicrofilaraemic and asymptomatic but seropositive for antifilarial IgG(4) (39) were investigated further. They were given detailed clinical examinations, their levels of microfilaraemia were evaluated (by counting microfilariae filtered out of blood samples), their lymphatics were explored by Doppler sonography, and their limbs were checked by lymphoscintigraphy. The 'filarial dance sign', which indicates the presence of live adult worms, was detected by sonography in 14 children (apparently the first time this sign has been observed in brugian filariasis). Lymphoscintigraphy revealed dilated lymphatic channels in the limbs of 80 of the children. At the end of the study, each of the 100 hospitalized children was treated with a single combined dose of diethylcarbamazine and albendazole; the aim is to follow-up the treated children every 6 months for 3 years. Even these preliminary results have important implications for filariasis-control programmes and emphasise the need for disability-alleviation efforts among children as well as adults.

Doppler ultrasonography reveals adult-worm nests in the lymph vessels of children with brugian filariasis.

Although ultrasonography has allowed 'nests' of live adult worms and dilated lymphatics to be detected in the early stages of infection with Wuchereria bancrofti, previous attempts to locate such adult-worm nests in brugian filariasis have been unsuccessful. In this study, the successful location of live adult Brugia malayi parasites, in the lymphatics of the axilla, thigh, epitrochlear region and/or popliteal fossa of children aged 3-15 years, is described for the first time. The 'filarial dance sign' (FDS), which indicates the presence of live adult worms, was observed in six children with microfilaraemia and in eight children who, though amicrofilaraemic, either had experienced an episode of lymphoedema (one) or were only positive for antifilarial IgG4 antibodies (seven). In bancroftian infection, the adult-worm nests have mostly been seen in asymptomatic but microfilaraemic subjects. The suspected worm nests, 18 in the 14 children, were all confirmed using colour-power and pulse-wave Doppler examinations. The worm nests were distinctly smaller and the wriggling movements were less rapid and less conspicuous than those seen in bancroftian filariasis. The importance of these findings in the management and control of lymphatic filariasis is discussed.

Carcinoma of the jejunum with multideposit peritoneal seeding, resection and intraperitoneal chemotherapy.

Jejunal adenocarcinoma is rare, often presenting late with widespread intraperitoneal disease. Intraperitoneal chemotherapy (IPC) has been shown in non-randomized studies to improve the survival of patients presenting with intraperitoneal metastases from carcinoma of the colon, appendix and stomach and in primary peritoneal malignancies including mesothelioma and pseudomyxoma peritonei, providing that adequate operative cytoreduction can be performed. A case is presented of obstructive jejunal adenocarcinoma in which 19 intraperitoneal deposits were excised. The patient was treated successfully with immediate postoperative IPC followed by systemic chemotherapy. This condition is reviewed along with the rationale for IPC.

Carcinoma of the jejunum with multideposit peritoneal seeding, resection and intraperitoneal chemotherapy / Carcinoma del yeyuno con siembra peritoneal – depósito múltiple resección y quimioterapia intraperitoneal

Jejunal adenocarcinoma is rare, often presenting late with widespread intraperitoneal disease. Intraperitoneal chemotherapy (IPC) has been shown in non-randomized studies to improve the survival of patients presenting with intraperitoneal metastases from carcinoma of the colon, appendix and stomach and in primary peritoneal malignancies including mesothelioma and pseudomyxoma peritonei, providing that adequate operative cytoreduction can be performed. A case is presented of obstructive jejunal adenocarcinoma in which 19 intraperitoneal deposits were excised. The patient was treated successfully with immediate postoperative IPC followed by systemic chemotherapy. This condition is reviewed along with the rationale for IPC.

El adenocarcinoma del yeyuno es raro, presentándose a menudo de forma tardía con enfermedad intraperitoneal extensa. Estudios no randomizados han demostrado que la quimioterapia intraperitoneal (QIP) mejora la supervivencia de pacientes que presentan metástasis intraperitoneal del carcinoma de colon, apéndice y estómago, así como en malignidades peritoneales primarias, incluyendo el mesotelioma y el pseudomixoma peritoneal, siempre que se realice una adecuada citoreducción quirúrgica. Se presenta un caso de adenocarcinoma yeyunal obstructivo en el que se extirparon 19 depósitos del intraperitoneal, tratándose inmediatamente al paciente exitosamente con quimioterapia intraperitoneal postoperatoria, seguida de quimioterapia sistémica. Se examina esta condición junto con las razones para practicar la QIP.

The utilization of intraluminal formalin for intractable rectal haemorrhage in advanced rectal cancer: a case report.

A patient with a metastatic fixed low rectal carcinoma presented severe rectal bleeding requiring massive transfusion over with a 36-hour period. Bleeding was controlled with the instillation of a 4% intrarectal formalin solution permitting the completion of a course of neoadjuvant chemoradiotherapy. This technique has been reserved for intractable transfusion-dependent radiation proctitis and is presented as a primary alternative in severe rectal bleeding from inoperable rectal cancer.

Multicentre laboratory evaluation of Brugia Rapid dipstick test for detection of brugian filariasis.

A multicentre evaluation of the Brugia Rapid dipstick test was performed using 1263 serum samples in four international laboratories, i.e. T.D. Medical College (TDMC, India), National Institutes of Health (NIH, USA), Swiss Tropical Institute (STI, Switzerland) and Leiden University Medical Centre (LUMC, Netherlands). In comparison with microscopy, the dipstick demonstrated sensitivities of 97.2% (70 of 72) at TDMC, 91.6% (175 of 191) at LUMC and 100% (six of six) at STI. Sera of chronic patients showed a positivity rate of 11.3% (19 of 168) and 61.2% (71of 116) at TDMC and LUMC, respectively. All 266 sera of non-endemic normals from STI, NIH and LUMC tested negative with the dipstick. At LUMC, sera of 'endemic normals' (amicrofilaraemics with no clinical disease) from an area with approximately 35% microfilaria positivity showed 60.8% positive results (31 of 51), thus demonstrating the likelihood of many cryptic infections occurring in this population. Specificities of the test with Onchocerca volvulus sera were 98.8% (80 of 81) and 100% (10 of 10) at the NIH and STI, respectively; while specificity with Loa loa sera at the NIH was 84.6% (44 of 52). At the STI, the dipstick test also demonstrated 100% specificity when tested with 75 sera from various protozoan and helminthic infections.

A qualitative study on the feasibility and benefits of foot hygiene measures practiced by patients with brugian filariasis.

Disability alleviation is an important component of 'Global Programme for Elimination of Lymphatic Filariasis'. In Brugia malayi infection the disability is largely due to acute attacks of adenolymphangitis (ADL), which frequently prevent patients from attending their normal activities, causing much suffering and economic loss. The foot care programme has been shown to reduce the frequency and severity of these episodes. In the present study we used semi-structured interviews to evaluate the impact of the foot care in 127 patients with brugian filariasis. They were previously trained in this procedure and were advised to practice it regularly, unsupervised. All except one could recollect the various components of foot hygiene and were practicing it regularly. They were aware of the factors causing ADL attacks and were able to avoid them. Majority (95.2%) expressed their happiness with the relief provided by foot care, which prevented or reduced the ADL episodes. The motivation was such that they transmitted this knowledge to others suffering in the community and even physically helped them to carry out foot care. This study fully endorses the advocacy of foot care programme as an easy to carry out, effective, sustainable and economically feasible procedure to prevent acute ADL attacks.

A qualitative study of the perceptions, practices and socio-psychological suffering related to chronic brugian filariasis in Kerala, southern India.

Lymphatic filariasis is a major health problem in many parts of the tropical world. Although the disease itself is rarely fatal, the disability caused by the swollen extremities, the acute attacks of adenolymphangitis and the consequent sufferings of those afflicted are considerable. The economic burden imposed by lymphatic filariasis is not fully quantified and information on the social and psychological problems caused by the disease is scanty. Semi-structured interviews were therefore used, in southern India, to assess the perceptions, practices and socio-psychological problems of 127 patients with brugian filariasis. The patients were aware of the causative factors and the precautions to be taken to prevent progression of the disease. However, depression and loss of job opportunities were common in the study population. Patients also complained that the disease eroded their standing in the community and diminished their prospects of marriage. Awareness of these factors will be of help in planning suitable disability-management packages, including the rehabilitation of those who find it difficult to carry on with their existing jobs because of the severity of their disease.

The pharmacokinetics, safety and tolerability of the co-administration of diethylcarbamazine and albendazole.

The pharmacokinetics, safety and tolerability of single, oral doses of diethylcarbamazine (DEC) and albendazole, given alone or in combination, were investigated in a double-blind, randomized and placebo-controlled trial involving 42 amicrofilaraemic subjects living in an area of India where lymphatic filariasis is endemic. The subjects (34 males and eight females, aged 18-52 years and weighing 46-66.5 kg) were randomly allocated to one of the three drug groups. Fourteen were given just DEC (6 mg/kg), another 14 were given just albendazole (400 mg) and the remaining 14 were given both DEC (6 mg/kg) and albendazole (400 mg). Blood samples for pharmacokinetic study were collected at specified intervals before and after drug administration. Plasma concentrations of DEC and albendazole/albendazole sulphoxide were estimated using gas chromatography and HPLC, respectively. The safety and tolerability of the treatments were evaluated through clinical and laboratory assessments. Both the DEC and albendazole were well tolerated when given alone or in combination, no adverse events being observed. In all three treatment groups, the drugs were rapidly absorbed from the gastro-intestinal tract although there was marked inter-individual#10; variation. The pharmacokinetics of DEC, albendazole and albendazole sulphoxide were similar, whether each drug was given alone or in combination. These results indicate that there is no adverse pharmacokinetic or pharmacodynamic reason why DEC and albendazole should not be co-administered to control lymphatic filariasis.

Efficacy and sustainability of a footcare programme in preventing acute attacks of adenolymphangitis in Brugian filariasis.

Lymphatic filariasis is associated with considerable disability related to the intensity and frequency of acute adenolymphangitis (ADL) attacks. The global programme for elimination of lymphatic filariasis emphasizes the need to combine transmission control with alleviation of disability. Footcare aimed at the prevention of secondary bacterial infections is the mainstay of disability alleviation programmes. We evaluated the efficacy and sustainability of an unsupervised, personal footcare programme by examining and interviewing 127 patients who had previously participated in a trial that assessed the efficacy of diethylcarbamazine, penicillin and footcare in the prevention of ADL. During the trial period these patients had been educated in footcare and were supervised. During the unsupervised period, which lasted 1 year or longer, 47 patients developed no ADL, and ADL occurred less frequently in 72.5%. Most patients were practising footcare as originally advised, unsupervised and without cost, which proves that such a programme is sustainable and effective.