Arthur S. Tucker

A 5 YEAR CLINICAL EVALUATION OF A THERAPEUTIC PROGRAM FOR PATIENTS WITH CYSTIC FIBROSIS.

A clinical evaluation of a comprehensive prophylactic therapeutic program for patients with cystic fibrosis is presented. Ninety-six consecutive patients were followed for 18 to 60 months (average 37 months) and evaluated with the use of a modification of the Shwachman scoring system. Eighty-two per cent of these patients showed improvement, 11% remained the same, 4% showed progression beyond their initial status, and only 3% died. None of the deaths occurred before 5 years of age. Evidence is presented supporting the desirability of early diagnosis and the early institution of an intensive prophylactic and therapeutic regimen.

Course of cystic fibrosis in 95 patients

The course of 95 patients with cystic fibrosis is presented. Survivors have a mean follow-up period of over 14 years (minimum: 13 years). Of 45 patients diagnosed prior to extensive irreversible pulmonary involvement, only one has died and none is disabled. In contrast, of the other 50 patients diagnosed after substantial irreversible pulmonary disease was present, 26 have died. Mortality and morbidity has been greater in females. Possible factors contributing to the improving prognosis include early diagnosis, aggressive management with comprehensive care, easy access to specialized care, and improved antimicrobial therapy.

Cystic Fibrosis Diagnosed after Age 13: Twenty-five Teenage and Adult Patients Including Three Asymptomatic Men

Cystic fibrosis was diagnosed after age 13 in 25 patients. All had an elevated sweat chloride and either a sibling with cystic fibrosis or typical pulmonary infection or digestive symptoms caused by exocrine pancreatic deficiency. Fourteen had long-standing pulmonary or digestive symptoms. In contrast, four of eight patients whose symptoms began after age 13 presented with biliary cirrhosis. Three male patients were asymptomatic at diagnosis. Opacification of all paranasal sinuses was found in all patients examined radiologically. At diagnosis, pulmonary-function testing showed obstructive changes in 19 patients and sputum cultures showed Pseudomonas aeruginosa in 15 patients. Delayed menarche in five of seven female patients and infertility in the asymptomatic male patient (two of whom were found to have aspermia) could have led to earlier diagnosis. Teenagers and young adults with long-standing pulmonary or digestive symptoms, unexplained cirrhosis, aspermia, or a sibling with cystic fibrosis should be sweat-tested by pilocarpine iontophoresis.

Nonobstructive hydronephrosis and hydropureter associated with peritonitis.

Abstract Three patients with peritonitis (ruptured appendix in two and primary in one) were found to have associated hydronephroses and hydroureters. The hydronephroses and hydroureters were of the nonobstructive type, were not accompanied by urinary-tract infection, and disappeared spontaneously within four to 20 months after onset. It is possible that, as in paralytic ileus, the kidney pelvis and ureter participate in impaired peristalsis associated with peritonitis and thus produce reversible, nonobstructive hydronephrosis and hydroureter.

Course of cystic fibrosis in black patients

Seventeen black patients showed typical sweat gland, gastrointestinal, and pulmonary manifestations of cystic fibrosis. There was an unusually high incidence of meconium ileus equivalent (35%). Three infant siblings of patients in this study had died of cystic fibrosis prior to referral of a family member to this center. One patient died at age four years after emotional problems interfered with the treatment program; status asthmaticus played a major role in his terminal respiratory failure. The remainder of the patients have had substantially less morbidity from pulmonary disease than a comparable, much larger group of white patients. Only one patient, age 28, who is married and employed full-time has severe pulmonary involvement as assessed by pulmonary function studies and chest roentgenogram. The 16 surviving patients have a mean age of 13 1/2 years after a mean follow-up period of 10 years. If black patients with cystic fibrosis survive infancy, they then, as a group, may have a relatively good prognosis.

Pulmonary, Pleural, and Thoracic Changes Complicating Chemotherapy

Chronic respiratory failure slowly developed in two pediatric patients following long-term cyclophosphamide therapy for lymphocytic malignancy. One patient survived 12 years after acute lymphocytic leukemia was diagnosed but died of respiratory failure at age 16 still in initial remission; the other, a 12-year survivor of Hodgkin disease, has progressive deterioration of pulmonary function. Each patient received cyclophosphamide (less than 70 g) during the initial years of the disease. Autopsy of the first patient and lung biopsy in the second revealed severe pulmonary fibrosis. There was loss of compliance and a dramatic change in the shape of the thorax which produced a markedly reduced anteroposterior diameter in both patients, and recurrent pneumothoraces in one.

Renal duplication and dysplasia

Abstract Seventeen patients with duplication and coexisting ureteroceles have been studied. In sixteen instances renal dysplasia was also present. This entity has been shown to lead to disability and continued sepsis and therefore dictates direct extirpative surgery.

Congenital hypoplasia of the abdominal muscles and associated genitourinary tract abnormalities.

Congenital hypoplasia of the abdominal musculature is a rare but important affliction of children, first described in the German literature in 1839 by Frohlich (2). The earliest reports in the English literature are those by Henderson in 1890 (5), Parker in 1895 (12), Guthrie in 1896 (4), and Osler in 1901 (11). Since that time over 100 cases have been recorded in the literature (1, 3, 6#x2013;10, 13, 14). The appearance of the abdomen in these children is characteristic (Fig. 3). The abdominal wall is protuberant, flabby, and thin, with intestinal peristalsis often visible. There may be complete or partial absence of the abdominal muscles. Those of the anterior abdomen are most commonly involved, but the posterior muscles such as the quadratus lumborum may be affected. The flanks are bulging and the skin over the abdomen is wrinkled. While the disease is usually found in males and bilateral cryptorchidism is almost invariably present, several instances have been reported in females. The often overlooked ...

The kidney in childhood leukemia.

Involvement of the kidney at some stage of childhood leukemia is a frequent if not invariable occurrence, varying from microscopic infiltration to gross enlargement with subsequent aberration of function (5). Clinically the involvement may not be recognized until enlargement and palpable nodularity take place in the kidney. Merrill and Jackson (7) reported 10 cases of impaired renal function prior to death in an autopsy series of 18 leukemic patients. All of these were adults. Gilbert, Rice, and Lechaux (3) studied renal function in 35 children with leukemia and found no laboratory evidence of impairment. Pierce (9), on the other hand, made note of signs of impending uremia caused by leukemic infiltrates and found that enlargement of the kidneys from this condition was an ominous sign, rendering control by antileukemic drugs more difficult of achievement. Gowdey and Neuhauser (4) were the first to report (1948) a roentgenographic appearance of the kidneys which seemed to be characteristic for leukemia. Th...

How serious are gastrointestinal perforations in infancy

The presence of free air in a body cavity of an unoperated infant is considered to be evidence of perforation and is generally looked upon as having an exceedingly grave prognosis. In our experience, neither presumption is necessarily correct. Thelander (1) in 1939 collected from the literature 85 cases of “spontaneous” perforations of the gastrointestinal tract in infants under one year of age (excluding perforations associated with obstruction and intrauterine rupture of the appendix). There evidently was a great disparity in respect to the extent and nature of what various authors considered to be “perforations.” In most instances there was no record of pneumoperitoneum, especially since many of the cases were reported before the era of the roentgen ray. Even so, the mortality was exceedingly high, only one survival being reported among the 85 infants (Selinger, 2). Many recent reports have been only slightly less dire. Among 75 cases of ruptured stomach in newborn infants reported from the literature ...