Clinical presentation and improvised management of neonatal pneumothorax in the setting of a low-resource country: Conakry, Guinea.

Two neonates were presented at the Neonatology Department of the Institute of Child Nutrition and Health in Conakry, Guinea, with tension pneumothoraces as confirmed by chest X-ray. They were initially managed with needle thoracentesis but required continuous thoracic drainage. Due to scarce resources in the public health sector, no prepacked and dedicated pleural drainage systems were available as is the case in many developing countries. Therefore, we fabricated an improvised underwater seal drain out of a plastic infusion bottle and a Heimlich valve out of a vicryl fingerstall. Both devices have shown to be effective. Pneumothorax is a common and potentially life-threatening disease in neonates that often requires prompt treatment. This case series demonstrates how tension pneumothorax in two newborns was successfully managed by improvising different chest drainage systems. The depicted techniques shall serve as an instruction manual to healthcare professionals working in low-resource settings and facing similar challenges.

"Assessing Today for a Better Tomorrow": An observational cohort study about quality of care, mortality and morbidity among newborn infants admitted to neonatal intensive care in Guinea.

BACKGROUND: Neonatal mortality in Guinea accounts for about 30% of all fatalities in children younger than five years. Countrywide, specialized neonatal intensive care is provided in one single clinic with markedly limited resources. To implement targeted measures, prospective data on patient characteristics and factors of neonatal death are needed. OBJECTIVE: To determine the rates of morbidity and mortality, to describe clinical characteristics of admitted newborns requiring intensive care, to assess the quality of disease management, and to identify factors contributing to neonatal mortality. METHODS: Prospective observational cohort study of newborns admitted to the hospital between mid-February and mid-March 2019 after birth in other institutions. Data were collected on maternal/prenatal history, delivery, and in-hospital care via convenience sampling. Associations of patient characteristics with in-hospital death were assessed using cause-specific Cox proportional-hazards models. RESULTS: Half of the 168 admitted newborns underwent postnatal cardiopulmonary resuscitation. Reasons for admission included respiratory distress (49.4%), poor postnatal adaptation (45.8%), prematurity (46.2%), and infections (37.1%). 101 newborns (61.2%) arrived in serious/critical general condition; 90 children (53.9%) showed clinical signs of neurological damage. Quality of care was poor: Only 59.4% of the 64 newborns admitted with hypothermia were externally heated; likewise, 57.1% of 45 jaundiced infants did not receive phototherapy. Death occurred in 56 children (33.3%) due to birth asphyxia (42.9%), prematurity (33.9%), and sepsis (12.5%). Newborns in serious/critical general condition at admission had about a fivefold higher hazard to die than those admitted in good condition (HR 5.21 95%-CI 2.42-11.25, p = <0.0001). Hypothermia at admission was also associated with a higher hazard of death (HR 2.00, 95%-CI 1.10-3.65, p = 0.023). CONCLUSION: Neonatal mortality was strikingly high. Birth asphyxia, prematurity, and infection accounted for 89.3% of death, aggravated by poor quality of in-hospital care. Children with serious general condition at admission had poor chances of survival. The whole concept of perinatal care in Guinea requires reconsideration.

Nutritional status of people living with HIV/AIDS in the Ratoma community medical center (Republic of Guinea).

The nutritional status of people living with HIV/AIDS (PLHIV) has a direct impact on their health. The aim of this study was to describe the nutritional status of PLWHA receiving care at the Ratoma community medical center in Conakry, Guinea. This quantitative cross-sectional study assessed the nutritional status of 184 people PLHIV receiving antiretroviral treatment. The sample comprised 184 PLHIV selected by a systematic random sampling from the complete list of PLHIV. The data were collected by individual questionnaires. Two groups were defined according to their nutritional status, based on their body mass index (BMI): a malnourished group (BMI < 18,5kg/m2) and a well-nourished group (BMI ≥ 18,5kg/m2). CD4 lymphocyte counts were also collected. Of the 184 PLVIH, 19.6% were malnourished (95%CI: 14.1-25.0). The mean BMI was 22.2 ± 4.3 kg/m2). The mean CD4 count differed significantly between the groups (328.7±237.7 CD4 cells/mm3 for the malnourished group and 432.9±256.9 for the well-nourished, p <0.017). Malnutrition was significantly associated with a low CD4 count. To improve survival and quality of life among PLHIV, this high frequency of malnutrition calls for sustained attention to the prevention and early detection and treatment of malnutrition in the early stages of HIV.

gamma-heregulin is the product of a chromosomal translocation fusing the DOC4 and HGL/NRG1 genes in the MDA-MB-175 breast cancer cell line.

gamma-heregulin is a recently described novel isoform of the heregulin/neuregulin class of EGF-like ligands that bind to and activate receptors of the ErbB family. Deregulated signaling through the heregulin-ErbB pathway is thought to be implicated in the development of a subset of human breast cancers. gamma-heregulin has been found to be expressed in the culture supernatant of MDA-MB-175, a breast carcinoma cell line. gamma-heregulin is characterized by the presence of a large N-terminal peptide extension that is not found in other heregulin isoforms. Here we report that this unique N-terminal extension of gamma-heregulin is identical to the N-terminus of DOC4, a product of a recently identified CHOP-dependent stress-induced gene. Human DOC4 and the heregulin-encoding genes map to different chromosomes and the MDA-MB-175 cell line contains a chromosomal translocation that leads to the fusion of DOC4 and HGL, on chromosomes 11 and 8, respectively. Thus, gamma-heregulin is a product of a mutant fusion gene and not a bona fide normal isoform. We speculate that the mutation may be selected for by virtue of its ability to activate ErbB signaling through the production of an autocrine ligand.

New perspectives in localizing enlarged parathyroids by technetium-thallium subtraction scan.

Double-tracer scanning (Tc-99m as pertechnetate and Tl-201 as chloride) with an Anger camera and computerized image subtraction was performed in 61 patients whose clinical and biochemical findings had suggested primary hyperparathyroidism. This study showed intra- or extrathyroidal focal uptake of thallium in 37 cases. Among these, 24 patients underwent surgery, and 18 parathyroid adenomas, five carcinomas, and one hyperplastic gland were found exactly in the sites predicted by scintigraphy. Among 24 patients with negative scans, only two underwent surgery; a hyperplastic parathyroid gland (diam less than 0.5 cm) was found in both. The success rate was 92% in the cases in which operation was performed. We believe that the new method may be useful in the preoperative detection of parathyroid enlargements.

Translocation and coamplification of loci from chromosome arms 8p and 11q in the MDA-MB-175 mammary carcinoma cell line.

Rearrangement and coamplification of the 8p12 and 11q13 chromosomal regions occurs in a significant proportion of breast cancers. It usually involves a complex hybrid structure in which the FGFR1 and CCND1 genes are amplified. We report here a different type of 8p12-11q13 rearrangement in the MDA-MB-175 mammary carcinoma cell line. This amplification contains the NRG1/HGL (from 8p12-21) and DOC4 (from 11q13) genes, encoding respectively a ligand for ERBB receptors and a stress-induced protein which is a mammalian ortholog of Drosophila Tenm/Odz. It has been shown previously (Wang et al, Oncogene 18: 5718-5721, 1999) that these two genes are rearranged and fused by a translocation event. This type of event was not found in 30 tumors tested that showed coamplification of the 8p12 and 11q13 regions.

Semen analysis following allogeneic bone marrow transplantation. Additional data for evidence-based counselling.

Knowledge of the impact of different conditioning regimens used in bone marrow transplantation on spermatogenesis is important in pre-BMT counselling for three reasons: (1) Most young patients who have not had children are concerned with their subsequent fertility; (2) For a number of diseases there are competing therapeutic options that may affect spermatogenesis more or less seriously; (3) Since spontaneous recovery of spermatogenesis is rare, it would be necessary to offer cryopreservation as soon as possible after diagnosis and prior to any treatment. This retrospective study evaluates 99 semen samples obtained in 64 patients who underwent BMT between 1982 and 1996. Recovery of spermatogenesis was observed in 90% of patients conditioned with cyclophosphamide (CY), in 50% of patients with CY plus busulphan (BU) or thiotepa and in 17% of patients with CY plus total body irradiation (TBI) or thoracoabdominal irradiation (TAI). Sperm quality following CY was within the normal range (WHO) in the majority of patients, whereas it was consistently severely impaired in patients who received irradiation or two alkylating agents. Following CY, spermatogenesis recovery was observed in 60% of patients tested 1 year post transplant and it was accomplished within the third year in 80% of cases. Following CY + TBI/TAI recovery of spermatogenesis never occurred before the 4th year post transplant and was demonstrated as late as 9 years in one patient who was azoospermic 1 year earlier. No statistical correlation between age and recovery of spermatogenesis could be demonstrated. The overall high incidence of azoospermia (70.3%) supports the indication for semen cryopreservation in young patients undergoing BMT. These results have implications for semen sample timing before and after BMT and underline a need to collect further data through prospective multi-center studies.

Increased serum beta 2-microglobulin concentrations in hyperthyroid states.

Serum beta 2-microglobulin concentrations were determined in 21 untreated hyperthyroid patients (12 with Graves' disease, and nine with toxic nodular adenoma) and in 20 healthy controls. All subjects had normal serum creatinine concentrations and urine analysis. Both total and free thyroid hormones were significantly higher in the hyperthyroid groups than in controls. Beta 2-microglobulin concentrations were significantly increased in both groups of hyperthyroid patients compared with controls. No difference was found in the thyroid hormone and beta 2-microglobulin concentrations between both sets of patients. The beta 2-microglobulin and thyroid hormone concentrations were not correlated. These data show that hyperthyroidism is another cause of increased beta 2-microglobulin production along with viral infections, immunologically mediated diseases, and malignant neoplasms. The increased serum beta 2-microglobulin concentration in thyroid hyperfunction is probably related to metabolic rate, even if autoimmunity might contribute to its overproduction.

The endogenous secretion rate of human calcitonin in normal conditions.

This paper presents a study on secretion rate for calcitonin in normal healthy subjects, evaluated by an indirect method. A specific radioimmunoassay for monomeric calcitonin was used and the obtained results are lower than those reported by Kanis in 1982. In normal healthy subjects the secretion rate for calcitonin is 1.4 MRC U/day, i.e. ten times less than the value calculated by Kanis. These results will be able to clarify the role of calcitonin and its effects in normal conditions and in several osteoarticular and metabolic bone diseases.

[Beta-2-microglobulin in hyperthyroidism]. / La beta 2-microglobulina nell'ipertiroidismo.

The aims of the study were to clarify the cause of increased serum beta 2-microglobulin as a marker of thyroid hyperfunction. Serum beta 2-microglobulin was measured in 31 untreated hyperthyroid patients, all of them with normal renal function. Twenty-one subjects were affected by diffuse toxic goiter and 10 by toxic adenoma. Serum free thyroid hormones, TSH, anti-thyroglobulin and anti-microsomal antigen autoantibodies were determined, too. Thyroid hormone and creatinine levels did not differed between both sets of patients. beta 2-microglobulin was higher than normal in 90% of cases with diffuse toxic goiter and in 70% of those with toxic adenoma (p less than 0.05), but mean beta 2-microglobulin concentrations didn't differ between the two groups. No difference was found in beta 2-microglobulin levels in diffuse toxic goiter group according to the presence or absence of autoantibodies. beta 2-Microglobulin and thyroid hormones were not correlated in either diffuse toxic goiter and toxic adenoma groups. These data confirm the high prevalence of elevated beta 2-microglobulin concentrations in hyperthyroidism. As renal function was normal, this rise is due to beta 2-microglobulin overproduction. This increased production is a hormone mediated effect, even if lymphocyte activation may contribute in diffuse toxic goiter. beta 2-microglobulin is not correlated with thyroid hormone concentrations so that at present it isn't a useful marker of hyperthyroidism severity for practical purposes.

[Acute hyperparathyroidism associated with follicular carcinoma in the thyroid: possible role of juvenile cervical irradiation. Description of a case]. / Iperparatiroidismo acuto associato a carcinoma follicolare della tiroide: possibile ruolo dell'irradiazione cervicale giovanile. Descrizione di un caso clinico.

Acute onset of primary hyperparathyroidism is uncommon; neuropsychiatric signs are prominent clinical features in acute hypercalcemia and they can subside after normalization of serum calcium. Radiation therapy is a well-known risk factor for non medullary thyroid cancer, but it induces also parathyroid tumors. Data from the literature show that patients previously treated with neck radiation have an increased risk of primary hyperparathyroidism. Furthermore concomitant thyroid cancer is more frequent in radiation-induced hyperparathyroidism than in sporadic primary hyperparathyroidism. The case of a 63-year-old female patient who at the age of 14 had been irradiated to the neck for goiter and at the age of 50 had been repeatedly hospitalized for psychosis is presented. She was admitted to the hospital for suspected recurrence of psychosis, but clinical findings and urgent biochemical data showed on the contrary that she had a severe hypercalcemic crisis. Serum parathormone concentrations, neck echography and 99mTc-Sestamibi scintigraphy suggested hyperfunction of the right lower parathyroid gland; therefore the patient was operated on. Pathological examination disclosed a parathyroid adenoma but also two foci of follicular cancer in the right thyroid lobe with a metastasis to a lymph node were observed. Neuropsychiatric signs disappeared after normalization of calcemia and 6 months after operation the patient is free from psychiatric symptoms, despite she had stopped neurolectic drugs. It is underlined that patients who had received neck irradiation must be carefully observed because they are at increased risk of primary hyperparathyroidism and concurrent thyroid cancer.

[Bone metabolism in primary hypothyroidism in the adult]. / Studio del metabolismo osseo nell'ipotiroidismo primitivo dell'adulto.

The aim of the study was to investigate bone metabolism in adult onset idiopathic myxedema. We studied 13 untreated patients (11 women and 2 men, age ranging 24-64 years) and, as a control group 15 healthy subjects (13 women and 2 men, age ranging 24-64 years). The hypothyroid group had significantly lower urinary excretion of hydroxyproline (4.40 +/- 0.63 vs. 8.60 +/- 1.3 mg/g, p less than 0.05) and serum concentration of osteocalcin (4.45 +/- 0.41 vs. 7.76 +/- 0.55 ng/ml, p less than 0.001). This low urinary excretion of hydroxyproline points to reduced osteoclastic bone resorption, while the low serum level of osteocalcin supports the view that osteoblastic bone formation is sluggish, too. Therefore bone metabolism in adult myxedema is characterized by a general reduction of remodelling. The stimulating actions of thyroid hormones on both bone resorption and new synthesis are further supported by the positive correlations between free hormone fractions and either urinary hydroxyproline and serum osteocalcin. Serum calcium, phosphorus, urinary calcium and phosphorus, and serum calcitrophic hormones (vitamin D, calcitonin, parathyroid hormone, measured as intact molecule) did not differed between the two groups; this finding might be related to the low fraction of active bone in hypothyroidism. The slow bone turnover seems to have few clinical consequences, but replacement therapy might produce accelerated osteoporosis, perhaps as a result of bone hypersensitivity to thyroid hormones.

[Serum levels of the tartrate-resistant isoenzyme, acid phosphatase, for the evaluation of bone remodeling in hyperthyroidism]. / Il dosaggio sierico dell'isoenzima tartrato resistente della fosfatasi acida nella valutazione del rimodellamento osseo nell'ipertiroidismo.

Tartrate-resistant acid phosphatase (TRAP) is one of the acid phosphatase isoenzymes. It is secreted by osteoclasts so it has been proposed as a marker of bone resorption. Bone turnover is high in hyperthyroidism due to an increase in both bone resorption and formation. The aim of the study was to measure serum TRAP as well as other markers of bone metabolism in 20 fertile age females affected by Graves-disease; 11 patients were also studied after euthyroid state was attained by means of a 6 month course of methimazole treatment. TRAP was measured with the colorimetric method using p-nitrophenylphosphate as substrate. Free thyroid hormones, TSH, serum calcium (corrected for albumin concentration), phosphate, osteocalcin, alkaline phosphatase, parathormone intact molecule, and urinary excretions of calcium, phosphate and hydroxyproline were measured, too. Twenty-eight healthy fertile women made up the control group. Untreated patients had a significant increase of TRAP, osteocalcin, serum calcium, alkaline phosphatase and urinary excretion of calcium and hydroxyproline. A significant fall in all these parameters but alkaline phosphatase was disclosed comparing patients before and after treatment, nevertheless only urinary calcium became not significantly different from the controls. TRAP showed a significant correlation with free T3 levels but not with hydroxyproline excretions. This survey on fertile age women with Graves' disease shows a significant increase in serum concentration of TRAP, which decreases, but doesn't get normalization, when euthyroidism is attained by a six month course of methimazole therapy.(ABSTRACT TRUNCATED AT 250 WORDS)

[Hypogonadism in idiopathic hemochromatosis]. / L'ipogonadismo nell'emocromatosi idiopatica.

We studied the prevalence and the pathogenesis of hypogonadism in 16 male patients affected by idiopathic haemochromatosis. Thirteen patients were untreated, 14 had liver cirrhosis; alcohol intake was actually less than 80 g/die. LH and FSH were measured in the basal state and after iv. bolus of 100 micrograms of synthetic gonadotropin-releasing hormone. Plasma concentrations of testosterone, LH-FSH were determined, respectively, by RIA and LIA. Ten patients complained of loss of libido and potency (Group A): this group, as compared to controls, had significant reductions of testosterone, basal gonadotropins and pituitary responses. Nine of these patients disclosed testicular hypotrophy and low blood testosterone: 8 showed hypogonadotropic hypogonadism with low testosterone and LH-FSH responses, often accompanied by reduced basal concentrations of gonadotropins; one patient had a primitive testicular failure with low testosterone but a high response of LH to the GnRH. The other 6 patients had normal sexual activity (Group B): their testicular volumes and testosterone concentrations were normal, but 2 patients disclosed both LH and FSH hyperresponsiveness to the GnRH, which suggests an early primitive testicular failure. Our data emphasize the high prevalence of hypogonadism in male haemochromatosis subjects and disclose that sexual activity, testicular volume and laboratory results are tightly correlated. Stimulation with GnRH proved that hypothalamic-pituitary dysfunction is by far the most frequent cause of testicular failure in idiopathic haemochromatosis.

Apparently nonfunctioning metastases of parathyroid carcinoma.

A 41-year-old woman was operated on for severe hyperparathyroid syndrome. At surgery a parathyroid tumor with the histopathologic pattern of carcinoma was found. After surgery serum calcium settled within normal limits (10.5 mg/dl, N.V. 8.5-10.8), whereas parathormone and calcitonin reached progressively high levels, respectively 400 ng/dl (N.V. up to 250) and 500 pg/ml (N.V. up to 100 ng/ml). Serum ultrafiltration analysis for parathormone and calcitonin showed many peaks of immunoreactivity with high molecular weight of both hormones. One year after surgery, metastases developed in the lymph nodes of the neck and the mediastinal, pleural and pancreatic regions. After death for tumor wasting, immunohistochemical study of the tumoral tissue with the peroxidase-antiperoxidase technique showed a relatively high density of calcitonin-containing cells. The findings in this case suggest that: several cells in this parathyroid cancer could secrete both parathormone and calcitonin; the hormonal secretion was impaired as suggested by the high molecular weight of both hormones found at gel-filtration analysis; the macromolecular profile of parathormone could explain the apparent function of the parathyroid cancer.

Hypercalcemia in malignancies: treatment with dichloromethylene diphosphonate (Cl2MDP).

Eleven hypercalcemic patients affected by various kinds of cancer were treated with dichloromethylene diphosphonate (Cl2MDP), 400 mg diluted in 500 ml of saline solution, i.v., during 3 h. Drug administration normalized plasma calcium levels in all treated patients, within 72 h for patients with lung cancer; within a longer period for other kinds of cancer. Moreover, Cl2MDP also reduced calciuria in treated patients. The use of the drug was not followed by side effects and was also well tolerated in patients with kidney insufficiency.

[Cardiotoxicity induced by 5-fluorouracil. A case report]. / Cardiotossicità da 5-fluorouracile: descrizione di un caso clinico.

A case of ischemic cardiopathy which was observed after 5-fluorouracil administration for a carcinoma of the small intestine is described.

Serum bone GLA-protein in hypercalcemia of primary hyperparathyroidism and malignancy.

Serum bone GLA-protein, a modern and sensitive marker of bone turnover, was measured in 15 patients with primary hyperparathyroidism, 18 patients with hypercalcemia of malignancy, 41 patients with bone metastasis without hypercalcemia, and 29 healthy subjects. Serum bone GLA-protein was increased in primary hyperparathyroidism (17.6 +/- 3.9 ng/ml) and normal in hypercalcemia of malignancy (5.2 +/- 2.8 ng/ml; p less than 0.001 vs hyperparathyroidism) and in normocalcemic patients with bone metastases. In primary hyperparathyroidism parathyroid hormone correlated positively with urinary calcium excretion (p less than 0.05) and with urinary hydroxyproline excretion (p less than 0.001). The sensitivity of serum bone GLA-protein measurements in differentiating between primary hyperparathyroidism and hypercalcemia of malignancy was 91% and the specificity 84%. Thus this marker appears to be a useful tool for the differential diagnosis of hypercalcemias.

Serum immunoreactive calcitonin in lung cancer.

Serum immunoreactive calcitonin (iCT) assay was performed in 92 patients suffering from different kinds of cancer of the lung and in 42 healthy control subjects. Gel filtration of serum of patients suffering from microcytoma was carried out on Sephadex G75 to study the forms of circulating iCT. The obtained results (pg/ml M +/- SE) were: 1) normal subjects, 73 +/- 3; 2) epidermoidal cancer, 105 +/- 19; 3) adenocarcinoma, 116 +/- 47; 4) anaplastic carcinoma with large cells, 156 +/- 74; 5) microcytoma , 354 +/- 74; 6) chronic obstructive bronchitis, 38 +/- 6. Gel filtration of serum of patients with microcytoma demonstrated the same behavior as in normal subjects. We can conclude that iCT increases significantly in microcytoma with extensive disease (84% of cases): in this condition, the iCT assay can be useful as a marker in follow-up of disease.

Endocrine effects of tamoxifen in postmenopausal breast cancer patients.

The effects of tamoxifen on plasma concentration of gonadotropins, prolactin (PRL), estrone (E1), estradiol-17 beta (E2), and sex hormone-binding globulin (SHBG) were studied in 40 postmenopausal breast cancer patients. In addition, the changes induced by the drug on endometrium and vaginal epithelium were investigated. After 6-8 weeks of tamoxifen treatment, a significant decrease in FSH, LH and PRL basal levels was observed, whereas the concentrations of E1 and E2 were not significantly affected. A significant increase in SHBG levels was induced by prolonged treatment with the drug. In addition, tamoxifen caused a partial estrogenization of vaginal smears, and a weak stimulatory effect on endometrium was also apparent. These findings indicate that tamoxifen produced agonistic effects on some targets and antagonistic effects on the others.