Psychiatric assessment of patients with “20th-century disease” (“total allergy syndrome”)

“Twentieth-century disease”, or “total allergy syndrome”, is a condition attributed to hypersensitivity to the environment that may sometimes be seen as so serious that the patient is incapable of living in the modern world. Although the popular media frequently carry stories about it, there is little scientific literature. It is diagnosed by clinical ecologists, who maintain, among other theories, that susceptible individuals experience an overload in assaults by artificial materials in the environment. The patients usually have multiple ill defined symptoms for which no organic cause can be found, but they vigorously resist psychiatric referral, as they attribute their symptoms to allergy. A group of 18 patients who were purportedly suffering from 20th-century disease were referred to a university psychiatric consultation liaison service. They virtually all had a long history of visits to physicians, and their symptoms were characteristic of several well known psychiatric disorders. The case histories and management of three of them are presented. Although this group of patients may have been atypical in that they had more severe psychologic symptoms, the experience indicates that a psychiatric diagnosis ought to be considered. The symptoms of 20th-century disease have much in common with other conditions known to physicians for centuries.     

Onset,Early Stages,and Prognosis of Rheumatoid Arthritis: A Clinical Study of 100 Patients with 11-year Follow-up

One hundred patients with “definite” or “classical” rheumatoid arthritis were followed in a hospital clinic from within one year of the onset of the arthritis. The average interval between onset and first attendance was 3·7 months. Onset was commoner in the winter, transient prodromal symptoms being noted in 23, with possible precipitating factors in 14. The serum rheumatoid factor test was positive at some time in 88.The patients were reassessed between eight and 14 years later. Seventeen died during this period, five possibly as a result of the disease or its treatment.The remaining patients had improved as a whole in terms of the blood sedimentation rate, haemoglobin, titre of the rheumatoid factor test, and status of the disease, but there was an overall deterioration in functional capacity. Both the rheumatoid factor titre and the functional capacity at an earlier review could be directly correlated with the outcome, but other factors were not found to influence the ultimate prognosis.     

Epidemiological features of some acute inflammatory morbid conditions in the neonatal period

In this study, medical records of 231 Prague, Czechoslovakia and 234 Moscow, USSR newborn infants treated for various forms of acute inflammatory diseases acquired in the neonatal period, i.e. during both hospital stay and home nursing period, were reviewed with the aim of assessing the epidemiological characteristics of these morbid conditions. As shown by the analysis of available epidemiological data, most of these inflammatory disease, both in Prague and Moscow, occurred shortly after birth, with a peak at 7 postnatal day, which pointed to hospital stay as a decisive factor in the onset of neonatal inflammation. In Prague, the overall number of inflammation cases diagnosed within the first decade of postnatal days was about three time the number recorded during the second decade; the respective figures for Moscow infants were in both decades identical. Assessed by clinical forms of inflammation, both groups of newborn infants showed concordance for conjunctivitis only (28%), frequencies of other clinical forms varied. In Moscow, the most common form of inflammation, predominant over all other clinical forms especially in the second decade, was pyoderma (29%), followed by conjunctivitis (28%), phlegmon (13%) and gastroenteritis (13%). Cases of gastroenteritis, acquired mostly during the home nursing period, were hospital-unrelated and predominated in the last decade of neonatal life. In the Prague group of infants, cases of catarrhal omphalitis were predominant, accounting for 37% of all diseases; this was due to a local outbreak of epidemic at the time of observation. The frequency of pyoderma, phlegmon and gastroenteritis was here lower than that among the Moscow infants, situation with the inflammation cases classed as "other diseases" was opposite.(ABSTRACT TRUNCATED AT 250 WORDS)     

Fabry Disease – Underestimated in the Differential Diagnosis of Multiple Sclerosis?

Objective

Fabry disease is a rare X-linked inherited lysosomal storage disorder affecting multiple organ systems. It includes central nervous system involvement via micro- and macroangiopathic cerebral changes. Due to its clinical symptoms and frequent MRI lesions, Fabry disease is commonly misdiagnosed as multiple sclerosis. We present an overview of cases from Fabry centres in Germany initially misdiagnosed with multiple sclerosis and report the clinical, MR-tomographical, and laboratory findings.

Methods

Eleven Fabry patients (one male, ten females) initially diagnosed with multiple sclerosis were identified from 187 patient records (5.9%) and analyzed for presenting symptoms, results of the initial diagnostic workup, and the clinical course of the disease.

Results

Four patients were identified as having a “possible” history of MS, and 7 patients as “definite” cases of multiple sclerosis (revised McDonald criteria). On average, Fabry disease was diagnosed 8.2 years (±9.8 years) after the MS diagnosis, and 12.8 years after onset of first symptoms (±10.3 years). All patients revealed white matter lesions on MRI. The lesion pattern and results of cerebrospinal fluid examination were inconsistent and non-specific. White matter lesion volumes ranged from 8.9 mL to 34.8 mL (mean 17.8 mL±11.4 mL). There was no association between extra-neurological manifestations or enzyme activity and lesion load.

Conclusion

There are several anamnestic and clinical hints indicating when Fabry disease should be considered a relevant differential diagnosis of multiple sclerosis, e.g. female patients with asymmetric, confluent white matter lesions on MRI, normal spinal MR imaging, ectatic vertebrobasilar arteries, proteinuria, or lack of intrathecally derived immunoglobulin synthesis.     

Familial Fibrocystic Pulmonary Dysplasia: A Detailed Family Study

Six cases of familial fibrocystic pulmonary dysplasia are described involving five siblings and their father. The clinical findings and radiological features were similar in all six patients although there was some variation in the period of survival following the onset of the disease. In three the diagnosis was confirmed pathologically; the two brothers, who did not have lung biopsies, had disturbances in respiratory function which are considered typical of the impaired diffusion produced by interstitial fibrosis. One hundred and five members of the family were surveyed for evidence of this disease, but no further cases were discovered. Four of the patients had some elevation of their gamma globulin. Immunoelectrophoretic analysis, which was performed on three of the patients, the two healthy siblings, and 16 of their offspring, showed elevated immunoglobulin patterns. This evidence suggests the possibility of an inherited aberration in the immune response in this family.     

Interferon γ in acute and subacute encephalitis

Intrathecal synthesis of interferon γ was shown in 14 out of 16 samples of cerebrospinal fluid collected in the first days of disease in adults, children, and newborn infants with herpes encephalitis. This synthesis was concomitant with that of interferon α and was switched off when the specific antibodies in the central nervous system increased. No endogenous interferon γ was detected in 11 serum samples or 13 samples of cerebrospinal fluid collected early in the course of the disease from patients with measles encephalitis and rubella encephalitis, or in serum and cerebrospinal fluid samples from seven patients with subacute sclerosing panencephalitis. In serum collected after the 10th day after the onset of neurological symptoms interferon γ was present at low concentrations in only three out of 11 serum specimens from patients with measles encephalitis or rubella encephalitis.Interferon γ was present in patients with acute herpes encephalitis and there was active virus replication, but it was not present in postinfectious encephalitis. Possibly the local production of specific antibodies masks the viral antigens and switches off the induction of interferons.     

Membranoproliferative Glomerulonephritis and Persistent Hypocomplementaemia

The clinical, laboratory, and histological findings of 50 patients with membranoproliferative glomerulonephritis are described. Three-quarters of the patients, who were mostly older children and young adults, presented clinically with a mixture of “nephritic” and “nephrotic” symptoms; the remaining quarter had no symptoms and were diagnosed after the discovery of proteinuria and microscopic haematuria.Though this clinical picture may occur in other forms of glomerulonephritis, the patients described here were unified as a group by their glomerular morphological appearance—namely, a combination of mesangial proliferation and capillary wall thickening, mainly due to subendothelial accumulations of mesangial matrix.In 68% serum C3 (β10-globulin) levels were reduced initially, while a further 16% subsequently showed a fall to abnormally low levels. All patients had substantial proteinuria, usually of moderately impaired selectivity, and all but one had haematuria in addition. Children frequently presented with an illness resembling acute nephritis, whereas adults usually had a nephrotic syndrome from the start.In 31 patients, followed for periods of one to eight and a half years, serial measurements of glomerular filtration rate were made. Sixteen have experienced no deterioration of renal function, though their proteinuria continues unchanged. Fifteen have shown progressive deterioration; six of them are still well, six are on regular dialysis treatment, and three have died. Treatment with corticosteroids, azathioprine, or cyclophosphamide, alone or in combination, did not seem to influence the course of the disease, and another two patients died from complications of steroid therapy. The disease usually runs a chronic course and appears to be progressive.     

Comparison of Birth Weight/Gestation Distribution in Cases of Stillbirth and Neonatal Death According to Lesions Found at Necropsy

The birth weight/gestation distribution of a large series of cases of perinatal death has been analysed according to the lesion (or lesions) present at necropsy. Among the lesions associated with low gestation babies dying with hyaline membranes have a much higher mean birth weight for gestation than either the babies with intraventricular haemorrhage or “no cause found.” Among infants dying of intrapartum asphyxia or cerebral birth trauma it was found that those who were stillborn with trauma had, at term, a significantly higher mean birth weight than the control livebirths, and that, at all gestations, the stillbirths with trauma were, on average, heavier than the babies dying neonatally with this lesion. Cases of intrapartum asphyxia were smaller than the stillbirths with trauma, but those dying during the second stage of labour were larger than those dying during the first stage.Cases of intrauterine pneumonia—that is, stillbirths and first-day deaths—were also shown to be larger for length of gestation than cases of extrauterine pneumonia—that is, deaths between the second and the 28th day. The cases with haemorrhagic pneumonia, however, were, at least at term, smaller than the cases of extrauterine pneumonia, and cases of massive pulmonary haemorrhage showed evidence of growth retardation at all gestations.     

Neonatal Necrotizing Enterocolitis: Clinical and Radiological Features

Necrotizing enterocolitis is an uncommon but dangerous disease in premature infants. Ten cases, seen over a three-year period at the Stanford University Medical Center, represented an incidence of 0.4 percent. The patients, six of whom died, derived from a general population, in contrast to the large series of patients reported in the literature in which the incidence was from 0.9 percent to 3.7 percent.^3-6The initial symptoms—rapid respiration, periodic breathing, lethargy and irritability—were identical to those which occurred in numerous infants who had respiratory disease. Subsequent symptoms (abdominal distension, in 100 percent; vomiting, 80 percent; apneic spells, 70 percent; jaundice, 70 percent; guaic-positive stools, 60 percent) were those of nonspecific acute abdominal disease.The radiologist first made the diagnosis in 90 percent of cases. Interstitial air in the wall of the gut and the retroperitoneum, and portal vein gas were the most diagnostic radiographic features. Barium contrast studies were not helpful, and in one case led to the erroneous diagnosis of small bowel volvulus.Plain abdominal radiographs must be taken of all premature infants with symptoms of nonspecific acute abdominal disease. If the radiographs are negative, but symptoms continue, they should be repeated at frequent intervals, for early diagnosis is critical to institution of proper therapy.     

Hypoglycemia Associated with Symptoms in the Newborn Period

Hypoglycemia in the neonatal period is a well-recognized phenomenon, but many authors have commented upon the infrequent association of symptoms attributable to it. Six infants were seen who appeared normal at birth but who, between 24 and 72, hours of age, developed apnea, irritability, lethargy, muscular twitchings and convulsions. Blood sugar concentrations of 10 mg./100 ml. or less were found in each case. The mothers of four of the babies had toxemia of pregnancy. Three babies were premature. The hypoglycemia was self-limiting in all cases, and four of the babies recovered completely without sequelae. The other two showed evidence of permanent brain damage, but it is not known whether this was the cause of their symptoms or the result of the hypoglycemia. It is concluded that hypoglycemia may cause neurological symptoms in the newborn period and that treatment by glucose administration is necessary. Whether symptomless hypoglycemia requires treatment remains an open question.     

High Utility of Contact Investigation for Latent and Active Tuberculosis Case Detection among the Contacts: A Retrospective Cohort Study in Tbilisi,Georgia, 2010–2011

Setting

The study was conducted at the National Center for Tuberculosis and Lung Diseases (NCTBLD) in Tbilisi, Georgia.

Objective

To assess the utility of contact investigation for tuberculosis (TB) case detection. We also assessed the prevalence and risk factors for active TB disease and latent TB infection (LTBI) among contacts of active pulmonary TB cases.

Design

A retrospective cohort study was conducted among the contacts of active pulmonary TB cases registered in 2010–2011 at the NCTBLD in Tbilisi, Georgia. Contacts of active TB patients were investigated according to an “invitation model”: they were referred to the NCTBLD by the index case; were queried about clinical symptoms suggestive of active TB disease; tuberculin skin testing and chest radiographs were performed. Demographic, laboratory, and clinical data of TB patients and their contacts were abstracted from existing records up to February 2013.

Results

869 contacts of 396 index cases were enrolled in the study; a median of 2 contacts were referred per index case. Among the 869 contacts, 47 (5.4%) were found to have or developed active TB disease: 30 (63.8%) were diagnosed with TB during the baseline period (co-prevalent cases) and 17 (36.2%) developed active TB disease during the follow-up period (mean follow up of 21 months) (incident TB cases). The incidence rate of active TB disease among contacts was 1126.0 per 100 000 person years (95% CI 655.7–1802.0 per 100,000 person-years). Among the 402 contacts who had a tuberculin skin test (TST) performed, 52.7% (95% CI 47.7–57.7%) had LTBI.

Conclusions

A high prevalence of LTBI and active TB disease was found among the contacts of TB cases in Tbilisi, Georgia. Our findings demonstrated that an “invitation” model of contact investigation was an effective method of case detection. Therefore, contact investigation should be scaled up in Georgia.     

Cholelithiasis and Cholecystitis in Childhood

Six cases of cholecystitis and cholelithiasis confirmed by x-ray examination and surgical operation were observed in a ten-year period. Due to the wide variability in signs and symptoms in children, cholecystitis and cholelithiasis can be diagnosed only with a high degree of clinical suspicion and roentgenological examination. Gallbladder disease is uncommon in childhood but should be considered in children with vague abdominal pains or bouts of unexplained jaundice. If a normal appendix is found at laparotomy in the “acute abdomen,” the surgeon would be wise to palpate other specific organs within the abdomen, including the liver and gallbladder.The treatment of choice is cholecystectomy. The prognosis for recovery is excellent if there is no complicating systemic disease.     

Unusually High Incidence of Paediatric Coeliac Disease in Sweden during the Period 1973 – 2013

Objective

The prevalence of coeliac disease in Sweden during the “epidemic period” (1984−1996) was one of the highest in the world. The aim of this study was to assess the coeliac disease incidence in our region over the 41-year period, and how diagnostic activity and diagnostic accuracy were affected by the introduction of antibody testing. We also looked into how patients with mild enteropathy were evaluated.

Methods

In the county of Östergötland in Sweden, 2790 paediatric patients were investigated for suspected coeliac disease between 1973 and 2013. Notes were scrutinised for data on sex, age, histopathological reports and final diagnosis. For comparative purposes this period was divided into three sub-periods (1973−1983, 1984−1996 and 1997−2013) named pre-epidemic, epidemic and post-epidemic.

Results

Coeliac disease diagnosis was received by 1,030 patients. The peak incidence rate, 301 cases/100,000 in 1994 for the age group 0−1.9 years is the highest figure ever reported. The other age groups, 2−4.9, 5−14.9, and 15−17.9 years, also had high incidence rates. After the 1984−1996 “epidemic period” the incidence decreased for the youngest group but continued to increase for the other groups. The cumulative incidence at 18 years-of-age for children born during the epidemic reached 14 cases/1000 births, the highest figure hitherto reported. Diagnostic activity differed significantly between the three sub-periods (p<0.001) increasing gradually from 1984 and reaching a peak value of 0.87 in 2012. Cases of mild enteropathy were more frequently regarded as non-coeliac disease cases, decreasing significantly in the “post-epidemic” period (p<0.001).

Conclusions

The incidence rate and cumulative incidence of coeliac disease were possibly the highest ever reported. Changes in diagnostic activity and accuracy could not be attributed to the introduction of new antibody tests, possibly because of other changes e.g. variations in the symptoms at presentation and improved knowledge of the disease among parents and health professionals.     

The “Incubation Period” of Subacute Bacterial Endocarditis

In an attempt to gain information about the “incubation period” of subacute bacterial endocarditis, the literature was searched for case reports stating a specific interval between an event likely to cause bacteremia and the onset of symptoms. In 76 cases of streptococcal endocarditis for which this information was given, the median “incubation period” was one week. Symptoms began within two weeks in 64 of these cases (84%). Although there may be a bias toward reporting short incubation periods, it is concluded that the incubation period of subacute bacterial endocarditis is often shorter than is generally realized, and that procedures carried out more than two weeks before onset of symptoms are less likely to be causally related. In postcardiotomy cases, where timing of the bacteremia causing endocarditis is less easy to define, 27% of 122 cases of staphylococcal endocarditis developed within two weeks of surgery. This information is relevant to the planning and evaluation of prophylactic chemotherapy against bacterial endocarditis.     

Neglect of medical evidence of torture in Guantánamo Bay: a case series

Background

In the wake of the September 11, 2001 attacks on the US, the government authorized the use of “enhanced interrogation” techniques that were previously recognized as torture. While the complicity of US health professionals in the design and implementation of US torture practices has been documented, little is known about the role of health providers, assigned to the US Department of Defense (DoD) at the US Naval Station Guantánamo Bay, Cuba (GTMO), who should have been in a position to observe and document physical and psychological evidence of torture and ill treatment.

Methods and Findings

We reviewed GTMO medical records and relevant case files (client affidavits, attorney–client notes and summaries, and legal affidavits of medical experts) of nine individuals for evidence of torture and ill treatment and documentation by medical personnel. In each of the nine cases, GTMO detainees alleged abusive interrogation methods that are consistent with torture as defined by the UN Convention Against Torture as well as the more restrictive US definition of torture that was operational at the time. The medical affidavits in each of the nine cases indicate that the specific allegations of torture and ill treatment are highly consistent with physical and psychological evidence documented in the medical records and evaluations by non-governmental medical experts. However, the medical personnel who treated the detainees at GTMO failed to inquire and/or document causes of the physical injuries and psychological symptoms they observed. Psychological symptoms were commonly attributed to “personality disorders” and “routine stressors of confinement.” Temporary psychotic symptoms and hallucinations did not prompt consideration of abusive treatment. Psychological assessments conducted by non-governmental medical experts revealed diagnostic criteria for current major depression and/or PTSD in all nine cases.

Conclusion

The findings in these nine cases from GTMO indicate that medical doctors and mental health personnel assigned to the DoD neglected and/or concealed medical evidence of intentional harm. Please see later in the article for the Editors'' Summary     

Epidemic Listeriosis in the Newborn

Within a three-month period 13 cases of listeriosis in the newborn were seen at the National Women''s Hospital, Auckland. Eleven presented in the first 24 hours of life, the most common feature being respiratory difficulty in low birth weight infants. Meconium-stained liquor was noted in nine cases. The constant finding in all cases was an aspiration pneumonia which appeared to be of intrauterine origin from an infected amniotic cavity. There was also evidence of septicaemia in nine cases, and two infants survived meningitis which developed at 1 week. Maternal symptoms were mild and variable, and in only one case were they proved to be due to listerial infection. The mothers came from different suburbs of Auckland and no common source of infection was found.     

Clinical Presentation of Acute Abdomen: Study of 600 Patients

This paper presents the clinical features of 600 patients suffering from abdominal pain of acute onset and admitted to either the General Infirmary or St. James''s Hospital, Leeds. The survey was initially retrospective, but later put on a prospective basis. Roughly two-thirds of these 600 patients presented a “typical” picture of the disease with which they presented, while the remaining third presented one or more atypical features. Since other prospective studies have indicated that the diagnostic accuracy of a group of clinicians in respect of the acute abdomen is roughly 65% it is tentatively suggested (a) that clinical diagnosis contains a large element of “pattern-matching,” and (b) that such a policy can be expected to be ineffective in roughly one-third of all cases of acute abdominal pain.     

Barriers and Enablers of Kangaroo Mother Care Practice: A Systematic Review

Kangaroo mother care (KMC) is an evidence-based approach to reducing mortality and morbidity in preterm infants. Although KMC is a key intervention package in newborn health initiatives, there is limited systematic information available on the barriers to KMC practice that mothers and other stakeholders face while practicing KMC. This systematic review sought to identify the most frequently reported barriers to KMC practice for mothers, fathers, and health practitioners, as well as the most frequently reported enablers to practice for mothers. We searched nine electronic databases and relevant reference lists for publications reporting barriers or enablers to KMC practice. We identified 1,264 unique publications, of which 103 were included based on pre-specified criteria. Publications were scanned for all barriers / enablers. Each publication was also categorized based on its approach to identification of barriers / enablers, and more weight was assigned to publications which had systematically sought to understand factors influencing KMC practice. Four of the top five ranked barriers to KMC practice for mothers were resource-related: “Issues with the facility environment / resources,” “negative impressions of staff attitudes or interactions with staff,” “lack of help with KMC practice or other obligations,” and “low awareness of KMC / infant health.” Considering only publications from low- and middle-income countries, “pain / fatigue” was ranked higher than when considering all publications. Top enablers to practice were included “mother-infant attachment” and “support from family, friends, and other mentors.” Our findings suggest that mother can understand and enjoy KMC, and it has benefits for mothers, infants, and families. However, continuous KMC may be physically and emotionally difficult, and often requires support from family members, health practitioners, or other mothers. These findings can serve as a starting point for researchers and program implementers looking to improve KMC programs.     

Immunoglobulin Levels in Newborn Infants with Hepatosplenomegaly

Serum IgM and IgA levels were measured in 97 newborn infants with and 141 without hepatosplenomegaly. All were considered normal at birth and had no apparent disease on clinical examination. Thirty per cent. of the infants with hepatosplenomegaly were found to have IgM levels of 20 mg/100 ml or greater, a level established as abnormal when compared with the control group. The number of infants with raised IgM level and the geometric mean level were significantly different (P < 0·001) from those in the control group. No difference, however, was found in serum IgA levels between the two groups of infants. The results suggest that intrauterine infections may sometimes be the cause of hepatosplenomegaly in newborn infants without apparent disease.     

PARATHYROID TUMORS—Intermittent Function,a Pitfall in Diagnosis

Seventy cases of functioning parathyroid tumors encountered at Columbia-Presbyterian Medical Center were reviewed. The clinical and chemical findings in parathyroid tumors were variable and were suggestive of intermittent function. The indirect diagnostic tests available today usually paralleled the basic serum calcium and phosphorus determinations. Urolithiasis was the most common complication observed, but the symptoms of it varied from simple colic and single calculus problem to extensive calculus disease. There were two instances of hyperparathyroid crisis or “hypercalcemic poisoning.”